oral clinical neurology

polyneuropathy

"polyneuropathy," "peripheral neuropathy," and "neuropathy" are frequently used interchangeably, but are distinct. Polyneuropathy is a specific term that refers to a generalized, relatively homogeneous process affecting many peripheral nerves, with the distal nerves usually affected most prominently. symmetric and stimulatneous involvement of various nerves. Guillain Barre syndrome: acute inf. polyradiculoneuropathy: presents with, weakness, symmetric that often begins in the legs clinical: sensory: pain, sensory loss motor and reflexes, autonomic --< postural hypotension, coldness of extremities, enlarged nerves

persistent vegetive state, locked in syndrome, brain death

when comatose patients regain wakefulness but not awareness, if it persists for longer than a month it is termed persistent vegetive state. they have intact brainstem and autonomic function however they neither comprehend nor produce language or any motor responces. recovery is very rare after 3 months (non traumatic) and 12 after traumatic. locked in syndrome a functional transection of the brain stem below the portion of the reticular formation responsible for consciousness (level of midpons) . will interrupt descending pathways to produce an akinetic mute state with preserved consciousness diagnosis is by voluntary eye opening, vertical eyemovements Brain death irreversible cessation of all brain function for the diagnosis > unresponsiveness to sensory input, including pain and speech >absent brainstem reflexes: pupillary, corneal and oropharyngeal responses are absent. doll's head and cold-water caloric testing are unsuccessful. respiratory responces are abscent with no effort of ventilation after Pco2 is permitted to rise to 60mmhg for max ventilator stimuli. cause for come must be known, must be able to explain the clinical opicture, and sedative intoxication, hypothermia, neuromuscular blockade and shock must be ruled out.

transient ischemic attack SUPPLIES MOST OF WHATS LEFT OF CER4E

when symptoms and signs of cerebral ischemia resolve completely after briefer periods (1h) --> TIA reccurent TIA,s with identical clinical features are usually caused by thrombosis or emboli within cerebral vasculature if different presentation each time: suggests emboli of cardiac source. 1/3 will have a stroke within 5 years treatment: anticoagulation: heparin, warfarin antiplatelet; asprin, clopidogrel, statins Interventional: carotid endaraectomy; surgical removal of thrombus from common or internal carotid artery.' carotid stenting: for extracranial carotid stenting page 406

entrapment neuropathies

when the nerve is compressed, stretched or angulated by adjacent anatomical structures to such an extent that it causes dysfuntion. and most of the noticed complainets are of sensory symptoms and pain upper limb entrapment Median nerve compression: can occur in the carpal tunnel. common during pregnancy, trauma, arthritis, DM, acromegaly, tenosynovitis (?) pain and paresthesias of median nerve distribution THUMB, INDEX AND MIDDLE FINGER, EVEN HALF OF RING FINGER. MAY EXTEND TO FOREARM AND EVEN SOMETIMES UPPER ARM. as it advances, muscle weakness and atrophy may develop in the thenar muscles examination: decreased cutaneous sensation of median nerve distribution. , wasting of abductor pollicis brevis and opponens TINEL SIGN: PERCUSIION WILL CAUSE PARESTHESIAS PHALEN MANUVER: flexion of wrist for 1 minute worsens symptoms diagnosis may be confirmed by electrophysiologic studies, showing decreased velocity of sensory and motor conduction at the wrist. corticosteroids, surgery, or a nocturnal wrist splint. --> treatment Ulnar neuropathy: dysfunction at the elbow--< paresthesiais, hypesthesia and nocturnal pain in little finger and elbow. often increased by elbow flexion examination, loss of ulnar sensation, weakness of adductor pollicis and deep flexor muscles of the forth and 5th digits reason: may result from external pressure, form entrapment in cubital tunnel, or cubitus valgus deformity (stretch injury) splitting of elbow in extension may sometimes be enough for arresting the progression. surgery may also help Radial neuropathy: mey be compressed in axilla (saturday night palsy) primarily motor motor deficit, causing weakness and even paralysis but recovery usually occurs spontaneously. thoracic outlet syndrome compresses the lower part of the brachial plexus. symptoms include pain, parasthesia and numbness along C8 and T1 distribution -klumpke? lOWER LIMB ENTRAPMENT Fibular (superficial) (peroneal) neuropathy can occur secondary to trauma or pressure about the knee at the head of the fibula. resulting in paralysis of foot and toe extension (plantar flexion), and eversion . impaired sensation of the lower anterior leg and dorsum of foot. The ankle reflex is preserved as is foot inversion Femoral neuropathy:associated with diabetes, vascular diseases,. symptoms: weakness of quadriceps wirh reduced knee reflex. saphenous neuropathy: terminal sensory nerve of the femoral nerve, supplies cutaneous sensation to the medial aspect of the leg, about and below the knee (quadriceps weakness doesn not imply saphenous but femoral) Lateral femoral cutaneous neuropathy: supplies the outer border of the thigh. Angulation or compression by neighbouring structures (especially pregnancy or exaggerated lumbar lordosis) pain and parasthesiss in the lateral thigh and impaired sensation (this disorder is known as meralgia paresthesia) treated with oral analgestics and is usually self limited obturator neuropathy trauma, by pelvic fracture or surgery, may lead to pain radiating from groin with accompanying weakness of adduction of thigh

Lumbar puncture

A diagnostic procedure, in order to collect a sample of cerebrospinal fluid for biochemical, microbiological and cytological analysis L3-L4 between the spinal processes. It must be below where the spinal cord terminates (L1) patient in lateral decubitus position indications: 1. diagnosis of meningitis or other infective, inflammatory disorders, SaH, hepatic encephalopathy, paraneoplastic disorders, meningeal malignacie. suspected abnormalities in intracrainial pressure 2. assessment of response to therapy in meningitis 3.admin of intrathecal medications or radiologic contrast. Contraindications. suspected intracranial mass lesion --> thiscan hasten incipient trantentorial herniation. 2.local infection,like furncle on the skin 3. coagulopathy, deficiencies in clotting factors, thrombocytopenia, risk of hemorrhage 4. suspected spinal cord lesion. only a small quantity can be removed. can produce pressure differences above and below the block analysis of results: appearance normally clear and colourless cloudy or turbid with a white blood count that exceeds 200micr/ pink--> hemoglobin yellow--> (Xanthochromic) bilirubin pressure: normal:180-200mm water. increased pressure may result from, obesity, agitation, increased abdominal pressure related to position (have patient extend legs in that case) pathologic increase: intracranial mass lesions, meningoencephalitis, SaH and pseudotumor cerebri ( occurs when the pressure inside your skull (intracranial pressure) increases for no obvious reason. Symptoms mimic those of a brain tumor, but no tumor is present) microscopic normally: 5 mononuclear leukocyters per microliter, no polymorphonuclear cells or erythrocytes (unless trauma) Blood CSf to distinguish between CNS hemorrhage or traumatic tap. can be established by comparing cell count in first and last tube of CSF obtained. amount will decrease in in nr of RBC if cause is traumatic. after CNS hemorrhage, enzymatic degradation of hemoglobin to bilirubion in situ renders the supernatant yellow.

normal pressure hydrocephalous

A potentially reversible cause of dementia, characterized by a clinical triad: dementia, gait apraxia and incontinence. and: hyperreflexia, spacisity, extensorplantar responses. this disorder may be idiopathic or a secondary condition that interferes with CSF absorption, like meningitis,SaH, disorder can also be called communicating hydrocephalous or nonobstructive hydrocephalous, due to that the ventricle are still intact. and that the main problem is probably impaired CSF absorption from arachnoid granulations. normal pressure hydropehalous usually develops over months, gait disorder usually being the first symptom patient can perform leg movement while lying or sitting but not when legs are bearing weight even though ataxia and weakness. investigative: lumbar puncture will show normal or low opening pressure. MRI large ventricles with normal cortical sulci. Treatment ventriculoatrial shunt, lumboperitonela shunting

stroke definitions and types

A stroke occurs when blood flow is interrupted to part of the brain. Without blood to supply oxygen and nutrients and to remove waste products, brain cells quickly begin to die. Depending on the region of the brain affected, a stroke may cause paralysis, speech impairment, loss of memory and reasoning ability, coma, or death we have ischemic strokes caused by thrombus ( occluding large vessels) or emboli ( when cerebral circulation is occulded by distal passage of thrombus from heart) hemorrhage: intracerebral H. sub arachnoid hemorrhage subdural or epidural hemorrhage Risk factors Increased age Male sex Low birth weight African American ethnicity Family history of stroke Modifiable risk factors Vascular Hypertension (BP >140 mm Hg systolic or 90 mm Hg diastolic) Cigarette smoking Asymptomatic carotid stenosis (>60% diameter) Peripheral artery disease Cardiac Atrial fibrillation (with or without valvular disease) Congestive heart failure Coronary artery disease Endocrine Diabetes mellitus Postmenopausal hormone therapy (estrogen ± progesterone) Oral contraceptive use Metabolic Dyslipidemia High total cholesterol (top 20%) Low HDL cholesterol (<40 mg/dL) Obesity (especially abdominal) Hematologic Sickle cell disease Lifestyle Physical inactivity Diagnosis: has acute onset focal

functional anatomy of brain circulation

Anterior (carotid) circulation: consists of internal carotid and it's branches --> anterior choroidal, anterior cerebral and middle cerebral arteries MA -- gives rise to lentico+ulo striate branches anterior circulation supplies: cerebral cortex, subcortical white matter, basal ganglia and internal capsuče posterior (vertebrobasilar) circulation 2 paired vertebral arteries,--> basilar artery --> post inferior cerebellar anterior inferior cerbellar posterior cerebral arteries --> thalmoperforate and thalmogeniculate (pontine branches) supplies brainstem, cerebellum, thalamus and portions of occipital and temporal lobes

syncope (didn't read book)

Episodic loss of consciousness associated with loss of postural tone. Syncome Is due to reduced blood/o2 supply to both cerebral hemispheres or brainstem ( reticular activating system) by either pancerebral hypo perfusion by: < vasovagal reflexes--> emotional stimuli, pain,blood, fatigue, prolonged motionless standing. vagally mediated decreases in blood pressure and HR prodomal: 30-60sec, tachycardia, dizziness, eyes open, role back. after loss reagin of consciousness is fast. , orthostatic hypotension: hypovolemia or autonomic dysfunction, neurodegenerative (idiopathic orthostatic hypotension , post ganglionic or shy-drager syndrome, preganglionic degeneration of sympathetic neurons < decreased cardiac output: or selective hypoperfusion of the brainstem from vertebrobasilar ischemia. pre syncopal symproms: light headedness, dimming of vision onset of syncopes posture --> orthostatic exertion-->usually due to cardiac outflow obstruction cognitive symptoms; a sensation of fear, olfactory or gustatory hallucinations Recovery from a simpla faint, is characterized by a prompt return to consciousness with full luciditywithin 20-30 sec

acute headaches

Headaches that are new in onset or clearly different from any the patient has experienced previously are commonly a symptom of serious illness and demand prompt evalua- tion. The sudden onset of "the worst headache I've ever had in my life" (classically due to subarachnoid hemor- rhage), diffuse headache with neck stiffness and fever (meningitis), and head pain centered about one eye (acute glaucoma) are striking examples. Acute headaches may also accompany more benign processes such as systemic viral infections or other febrile illnesses. sudden onset of a new headache, may be a symptom of serious intracranial or systemic disease. Subarachnoid hemorrhage, intracranial hemorrhage cerebral ischemia --> mainly thromotic or TIA's meningitis or encephalitis: inflammation of the brain or its meningeal covers: often described as throbbing, bilateral, with occipital, nuchal location other causes of acute: seizures -->following tonic-clonic seziures lumbar puncture --> postural headache sudden elevation in BP: pheochromocytoma or malignant HTN. 250/150 or higher coitus: men>women, erusal or during orgasm ophtalmic disorders: pain about eye

chronic headaches

Headaches that have recurred over years (eg, migraine or tension-type headaches) usually have a benign cause, although each acute attack may be profoundly disabling. When treating these patients, it is important to determine whether the pres- ent headache is similar to those suffered previously or is new—and thus represents a different process. migraine: reccurent headache, usually unilateral and frequently pulsatile. often associated with nausea and vomiting, photophobia, phonophobia and lassitude Prodomal symptoms. mood, cognition may occur in many patients. the attacks may begin with visual or other neurologic symptoms --> aura (classic migraine) followed by the headache phase at onset of aura, a decrease in blood flow is observed in occipital cortex and spreads anteriorly according to the cytoarchitectural rather that vascular bounderies. it resembles a spreading depression in which a slow wave of neuronal and glial depolarization decreases blood flow and inhibits neuronal activity. most common auras are visual: especially, hemaniopic field defects, scotomas (blind spots) and scintillations (flickerings) during headache phase, sterile inflammation in the meninges may activate the trigerminal nerve sensory fibers that project to the nucleus caudalis, senory thalamic nuceli and primary sensory cortex. OR there is a primary disturbance of the central pain pathways so that normal innocuous sensory input is misinterpreted as signaling pain --< this is called allodynia. most often they are common migraines (without aura) Women>men onset may be early in life 90% before the age of 40. common migraines (without aura) headache is most often bilateral and periorbital chronic (transformed migraine) episodic migraine can change its clinical features and become clinical with a nearly every day pain. precipitating factors: food,; tyramine containing cheese,meat, chocolate containing phenylethylamine. monosodium glutamate. emotions, menses, bright lights, treatments: to abort attacks in progress: triptans--> serotonin agonistsor elkot alkoids, both contraindicated in patients with HTN. or prevent future attacks (prophylactic) : tricyclic antidepressants, b-blockers, anticonvulsants and calcium channel blockers( in that order) >5 recurrent episodes of headache attacks lasting 4-72 hs • With at least 2 of: • Unilateral • Pulsating • Moderate to severe • Worsen by physical activity •And at least 1 of: •Nausea or vomiting •Increased light sensitivity •Increased noise sensitivity

prolapsed lumbar intervertebral disk

Lower back pain most commonly affects L5-S1 or L4-L5 disk often follows minor strain or normal activity the protruded disk material may presss on one or more nerve roots and produce redicular pain, a segmental motor or sensory deficit. together with a painful stiff back pain may reproduced by either spinal percussion or or by the sciatic nerve, by raising the extended leg over the flexed hip. --< laseuge sign is positive a L5 reticulopathy causes a weak dorsiflexion of foot and toes. where as S1 root lesion leads to depressed ankle reflex and weakness of plantar flexion treatment: simple analgesics, diazepam and bed rest on a firm bed for 2-3 days

signs of lower motor neuron lesions

Lower motor neurons (LMNs) are motor neurons located in either the anterior grey column, anterior nerve roots (spinal lower motor neurons) or the cranial nerve nuclei of the brainstem and cranial nerves with motor function (cranial nerve lower motor neurons).[1] All voluntary movement relies on spinal lower motor neurons, which innervate skeletal muscle fibers and act as a link between upper motor neurons and muscles.[2][3] Cranial nerve lower motor neurons control movements of the eyes, face and tongue, and contribute to chewing, swallowing and vocalization.[4] Damage to the lower motor neurons can lead to flaccid paralysis. signs: 1 weakness and paralysis 2. wasting and fasciculations (involved muscles) 3. hypotonia loss of tendon reflexes 5. normal abdominal and plantar reflexes localization: anterior horn, reticular (nerve root), plexus or peripheral nerve lesions. only the muscles supplied fully or partially are weakend

Binocular disorders

Papilledema painless, bilateraloptic disk sewlling, associated with intracranial pressure. may be acute, SaH or intracranial. or more prolonged; tumor pseudotumor cerebri, increase CSF proteins by gillian barre syndrome or spinal cord tumor. treatment: find cause chiasmal lesions major lesion (tumours) at level of optic chasm (pituitary origin) other causes MS, berry aneurysms and trauma. BITEMPORAL HEMANIOPIA involvement of oculomotor nerves suggests expansion of tumor laterally into the cavernous sinus. nonopthalmic symptoms if pituitary timor: acromegaly, cushiness syndrome, galactorrhea, amonrrehea or headache Retrochiasmal lesions optic tract and lateral geniculate: usually due to infarction NONCONGURUNOUS HOMONYMOUS HEMAOPIA,the field is not the same in the two eyes.(nasal and temporal) optic radiations: also homonymous hemanopia, usually due to temporal lobe tumor. deficit is usually more dense superiorly than inferiorly --> superior quadrantopia. lesion of optic radiations in parietal lobe are usually associated with contralateral weakness and sensory loss. a gaze preference to in acute phase to side of parietal lesion. INFERIOR QUADRANTOPIA (binocular) is the normal visual field abnormality (or complete homonymous hemaniopia) occipital cortex homonymous hemanopia with macula sparing a infarction of posterior cerebellar. the most common reason. if basilar infarction. then bilateral blindness with macula spaying du to dual supply (pac and mca)

status epilepticus

Seizures that continue for longer than 30 minutes without ceasing spontaneously or they reccur so frequently that that full consciousness is not regained a medical emergency --> can lead to permanent brain damage management early: ensure airway, positioned to prevent aspiration of stomach contents dextrose Iv drug therapy lorazepam or diazepam proceed immediately to fosphenytoin or phenytoin still proceeds --< phenobarbital management of hyperthermia: consequence of increased motor activity and high levels of circulating catecholamines; include hyperthermia

subacute headaches

Subacute headaches are those that persist or recur over weeks to months. Such headaches may also signify serious medical disorders, especially when the pain is progressive or when it occurs in elderly patients. Patients with subacute headache should be asked about recent head trauma (sub- dural hematoma or postconcussive syndrome); malaise, fever, or neck stiffness (subacute meningitis); focal neuro- logic abnormalities or weight loss (primary or metastatic brain tumor); visual changes (giant cell arteritis, idiopathic intracranial hypertension); or medications predisposing to headache (nitrates). Giant cell arteritis intracranial mass idiopathic intracranial hypertension trigerminal or glossopharyngeal or postherpetic (herpes simplx, zoster) neuralgia

jakob Cretszfeldt disease

a rapidly progressive demetia, with variable focal degeneration of cerebral cortex, basal ganglia, cerebellum, brainstem and spinal cored. this disease is caused by a proteinacous infective particle called a prion. cause may be sporadic or genetic or infectious Familial CJD is AD, caused by mutation in PRNP gene, which codes for the prion isoform. sporadic will under go conformational change of the isoform to a an abnormal prion PrPscc which will serve as a template for additional prions. can enable replication without nucleic acids. this causes an aggregation/ accumulation of prions in the brain. clinical findings, besides dementia, extrapyramidal signs (rigidity, bradykinesia, tremor,chorea, athetosis, myoclnus, cerebellar symptoms it will progress, can lead to akinetic mutism, coma, behavioural changes-> euphoria, delusions , hallucinations depression (anything really) myoclonus is a fequent clinical manifestation mad cow--> characterised by earlier onset

seizures

a transient disturbance of cerebral function caused by an abnormal neuronal discharge. Epilepsy a group of disorders characterized by recurrent seizures causing change in behaviour, including a change in motor activity, in autonomic function, in consciousness or in sensation 2 historic features most suggestive towards a seizure (since they usually happen outside of medical observation) 1. Aura, a prodromal symptom associated with the seizures of focal onset 2. postictal confusional state especially tonic clonic seizures the cause/reason behind seizures, impo to distinguish because right therapy. Primary neurologic disorders <benign febrile convulsions: 2-5% of children 6months-5years, the fibrile convulsions last for less than 10-15 min usually and lack foal features. usually a self limited disease and treatment is not needed. (pronlonged seizure can be treated with diazepam) <Idiopathic, 2/3 of new onset seizures in the general population <Head trauma (seizure can be controlled with anticonvulsants) <stroke <mass lesions <meningitis developmental abnormalities Systemic disorders metabolic diseases and drug overdoses ( antidepressants,antipsychotics, cocaine, insulin, lidocaine, isoniazid) or withdrwal syndromes (from ethanol or sedatiove drugs) associated with seizures theses kind of seziures do not require anticonvsulants <hypoglycemia <hyponatremia <hyperosmolar states <hypocalcemia <uremia porphyria: disorder of heme biosynthesis (neuropathy and seizures) <eclampsia: seizures or coma during pregnancy. magnesium sulfate used to treat eclamptic seizures. causes /risks of provoking seizure Massive sleep deprivation Excessive use of stimulants Withdrawal from sedative drugs od alcohol Substance abuse (cocaine, methamphetamine) High fever Hypoglycemia Electrolyte imbalance Hypoxia general seizures: tonic-clonic/grand mal consciousness is lost 1.tonic phase- tonic contraction of limb muscles for 10-30sec, flexion and then extension. contraction of the resp muscles may produce a cry/sound, cyanosis and contraction of masticatory muscles (tounge trauma) clonic phase: alternating muscle contraction and relaxation) , limb jerking. ventilator efforts return. eventually all movemts cease--> flaccid muscles spinteric relaxation may lead to incontinence. recovery: post seizure confusion and headache, muscleache EEG features: abnormal spikes, polyspike discharges, and spike-wave complexes-. absence genetically transmitted seizures , always begin in childhood and rarely persist to adolescence. brief loss of consciousness without postural tone lost. sometimes smaller motor manifestations (eye blinking, head tilt) EEG shows a characteristic 3 per second spike and wave pattern (3Hz) other generalised seizures: tonic: continous muscle contraction clonic: repetitive clonic jerking myotonic sudden brief shock like contraction of a few localised muscles, no loss of consciousness atonic: loss of postural tone, sometimes after myotonic jerks. creates terrible falls parial seizures simple - consciousness preserved unless/until the seizure spreads to other areas of the bain (secondary generalisation) causing tonic-clonic seizure. motor, sensory or autonomic phenomena depending on cortical area affeceted jacksonian march complex temporal lobe or pssychomotor seizures hese seizures usually start in a small area of the temporal lobe or frontal lobe of the brain. They quickly involve other areas of the brain that affect alertness and awareness. So even though the person's eyes are open and they may make movements that seem to have a purpose, mouth smacking clapping hands. Partial or secondarily tonic-clonic seizures: Phenytoin, carbamazepine and lamotrigine: first choices Generalized seizures: valproic acid, primary generalized seizures, phenobarbital (in adults) phenytoin and carbamazepine Absence seizures: valproic acid or ethosuximide myoclonic seizures: valproic acid, clonazepam carbamazepine and valproic acid, total blood count every 6-12 month due to hapotoxicity and aplastic anemia

lumbar disk prolapse cervical disk prolapse (nerve root and plexus lesions)

lumbar: leads to pain in back in radicular distribution (l5-s1) in the leg (accompanied by paresthesianand numbness) and maybe a motor deficit. L5 reticulopathy--> weakness of dorsiflexion of the foot and toes. S1 root involvement--> weakness in plantar flexion straight leg raising is restricted. (lasegue sign) due to reflex spasms of hamstrings Bedrest on firm bed, mobilization. asprin acetaminophen with codine for pain treatment. lumbar herniated disc may cause sciatica: pain along sciatic nerve all along back of leg. cervical disk prolapse A cervical herniated disc is diagnosed when the inner core of a disc in the neck herniates, or leaks out of the disc, and presses on an adjacent nerve root. It usually develops in the 30-to-50-year-old age group. While a cervical herniated disc may originate from some sort of trauma or neck injury, the symptoms commonly start spontaneously. The arm pain from a cervical herniated disc results because the herniated disc material "pinches" or presses on a cervical nerve, causing pain to radiate along the nerve pathway down the arm. Along with the arm pain, numbness and tingling can be present down the arm and into the fingertips. Muscle weakness may also be present. can happen at any age with no proceeding trauma, pain in neck and radicular pain in arm. increased by neck movement.

hemifacial spasm

characterized by repetitive involuntary contractions of some or all muscles supplied by the facial nerve may be provoked by blinking or voluntary activity. the disorder commonly relates to a anomalous blood vessel compressing the intracranial facial nerve. treatment with carbamazepine or phenytoin may help sometimes or botulin toxin injection other disorders with increased activity of motor unit: stiff person syndrome, (CNS) tetanus (by clostridium tetanus) CNS startle syndromes cramps, neuromyotonia, tetany --> hypocalcemia) muscular disorders that cause abnormal increased activity: myotonia and malignant hyperthermia

cardioembolism

check note pad Afib rheumatic fever mural thrombus( MI) endocarditis( bacterial and noninfective) mechanical prostetic heart valves, especially of mitral vlve. chronic admin pf anticoagulant, warfarin less common mitral valve prolapse paradoxical embolus atrial myxoma Atrial fibrillation predisposes to embolic stroke from thrombi that form in the left atrial appendage due to stasis of blood. Treatment is with oral anticoagulants (see later). The anti- arrhythmic drug dronedarone (400 mg orally twice daily) may provide additional reduction in stroke risk. Tachycardia-bradycardia (sick sinus) syndrome is also associated with cardioembolic stroke, Myocardial infarction is followed by stroke, usually car- dioembolic, within 1 month in about 2.5% of patients. Factors associated with increased risk include left ven- tricular dysfunction with low cardiac output, left ventricular thrombus or aneurysm, and atrial fibrillation. Treatment with aspirin, other antiplatelet drugs, warfarin, or combi- nations of these may reduce the risk of stroke after myo- cardial infarction, but is also associated with a risk for bleeding.

what was the drugs for psychotic dementia..

clonazapine, quantrapine issh

cluster headache and tension (chronic headache)

cluster headache: presents with clusters of brief, very severe, unilateral(always, unlike migraines), constant nonthrobbing headaches. 15-3h. they usually occur during the same time of the day, nightly forexample often waking the patient up actually occurs more often in men than women typically begin as aburning sensation over the lateral aspect of the noseor as a pressure behind the eye. ipsilateral conjuunctival injection, acrimation, nasal stuffiness and horners syndrome treatment: acute: 100% inhalation of oxygen or subcutaneous admin of sumatriptan for prompt relief maintenance: calcium channel blockers, triptans, ergot alkoids. VERAPAMIL -->most widely used Tension headache: non throbbing bilateral, occipital head pain. not associated with nausea or vomiting or visual disturbances. So chronic headache that lack migraine features. At least 10 attacks of: A. Duration 30 min - 7 days B. >2 of the following characteristics: 1. Pressing/ tightening (non-pulsating) 2. Mild/Moderate intensity 3. Bilateral 4. Not aggravated by routine activity C. Both of: 1. absence of nausea and vomiting (anorexia may occur) 2. absence of photophobia or phonophobia > 15 days/ month = Chronic Tension Type Headache (CTTH) Muscle contraction precipitated by stress/anxiety • 20-40 years • Female/male ratio 3:1 • Pressure sensation or pain "As head is going to explode" "On fire or stabbing from knives or needles" Daily increasing through the day Forehead to occiput or neck - or vice versa

Confusional states ( definition, examination, diagnosis,, etiology, difference between delirium and dementia state in which the level of consciousness is depressed. but not ythe the level of coma.

definition: A form of delirium. It may occur in any age group or may accompany preexisting brain disease. It may be triggered by a sudden or unexpected change in the person's environment. The confusion may be characterized by failure to perform activities of daily living, memory deficits, disruptive behavior, and inappropriate speech. It is a state in shich the level of consciousness is depressed (not as much as coma ) approach to dig/examination: 1. characterizing the nature of the disorder. confusional state or coma or is it a chronic disoreder like dementia. 2. determine cause, onset, take history (old charts, family) past history (drugs, alcoholic, Wernicke encephalopathy) 3. general physical examination: fever, neck stiffness or hypothermia ect... 4.mental status exam: evaluate wakefulness, arusability, orientation, memory and attention. a pitfall: to mistake Wernicke aphasia for confusion. they will generally not understands spoken or written language but can understand nonverbal gestures. will also often have rightsided neurological abnormalities; such as hemiparesis, hemisensory def, visual field deficit. causes/ etiology Drugs: alcohol intox, withdrawl sedativive drug intox or withrawl; barbiturates, benzodiazepines, propofol opiates: morphine, heroine, oxycodone, merpedine, fentanyl, methandone : pinpoint pupils, respiratory depression, this can also result from pontine hemorrhage but naloxone (opiate antagonist) or the ability to induce horizontal gaze will help figure that out. anticholinergics: used for parkinsonism,motion sickness, GI disturbances sympathomimetics; cocaine,amphetamine, ectasy Endocrine disorders: hypothyroisism; hashimotos thyroiditis hyperthyroidism: graves disease hypoglycemia: may progress very rapidly, from reversible to irreversible. most often due to insulin overdose. clinical signs, tachycardia, sweating, pupillary dilataion with confusional state treatment is iv dextrose 50ml with 50% dextrose immediately without blood results. hyperglycemia: diabetic ketoacidosis or hperosmolar nonketonic hypoglycemia --> encephalopathy or coma. both are due to diabetes symptoms: blurred vision,dry skin, anorexia, POLYURIA, polydipsia, kaussmal breathing (diabetic ketoacidosis) treatment: for ketoacidosis insulin, saline and after correction of acidosis has started potassium. hypo (addisons) and hyperadrenalism(cushi9ngs syndrome) hyponatermia: causes brain swelling due to hypoosmolality of ECF hypercalcemia: myopathic weakness, confusional state can progress to coma hypo calcemia : tetany, chovstek sign ( cn 7) trousseau sign Wernicke encephalopathy: nutritional disorder, usually due to chronic alcoholism, a deficieny of thiamine (B1) signs are neuronal loss, demyelination a triad of opthalmoplegia (nystagmus, abducens nerve pasly + combine horizontal and vertical gaze center palsy, ataxia and confusional state. Uremia Meningitis, encephalitis, sepsis Hepatic encephalopathy The differences between dementia and delirium; Dementia develops over time, with a slow progression of cognitive decline. Delirium occurs abruptly, and symptoms can fluctuate during the day. The hallmark separating delirium from underlying dementia is inattention. The individual simply cannot focus on one idea or task. delirium (decreased cognition ) may be the only sign/symptom for alzheimers while acute confusional state the underlying cause may show other symptom

multiple sclerosis, a demyelinating myelopathy

disorder is characterized by focal and scattered areas of demyelination, with reactive glisos,c causing axonal damage as neuronal degeneration. this will slow down conduction and lead to progressive neurologic symptoms. women more affected, in colder countries clinical: initial: focal weaknes, numbness, paresthesia (tingling) sudden vison loss/blurring (diplopia) later: may be an interval for months-years before further symptoms appear. or the initial reappear. relapse may be triggerd by infection, after childbirth ( reduced during pregnancy) different forms: relapsing-remitting progression does not occur between attacks seconday progressive: gradual progression after relapse (after ca 25y) primary progressive(10%) progressive from onset progressive-relapsing:rare Diagnosis treatment: acute episode, relapse reduction: corticosteroids, predisonde, methylprednisolone, dexamethasone relapse prevention, firstline treatment: interferon B-1a or B-1b glatiramer acetate

vascular dementia

due to either multiple large cortical infarcts or several small infarcts( lacunar sate) affecting subcortical white matter, basal ganglia, thalamus. Vascular dementia is classically described as a disorder of: − sudden onset − stepwise deterioration − vascular factors are risk factors for AD − mixed disease is common − relationship between degree of vascular damage and dementia is not direct − vascular dementia is found in many forms Clincial findings: patients classically have a history of HTN, a stepwise deficit but with a more or less abrupt onset of dementia + focal neurological symptoms common signs: pseudobulbar palsy with dysarthria, dysphagia, focal motor and sensory deficits. gait apraxia, ataxia , memory disturbance less than in AD. Investigate MRI --< shows multiple large infarcts and small by areas of low density. treatmnet: treat the HTN to reduce incidence, anti platelet agents( aspirin, clopidogrel)

56. dystonia and tics

dystonia: excessive, inappropriate contraction of muscles --> lead to abnormal postures, may be generalized or restricted (neck-torticollis) forearm and hand (writers block) oro-mandibular dystonia). Not present during sleep, can hbe enhanced in emotional states, or during voluntary activity causes/ etiology: drugs: levodopa, antipsychotics SRI's wilsons, Huntington , birthtrauma abnormal development cerebral palsy. parkinsons disease progressive suprenuclear palsy idiopathic torsion dystonan, hereditary or sporadic TICS: sudden recurrent, coordinated abnormal movements, can easily be imitated. occur the same again and again. can be suppressed shortly but causes anxiety 4 types 1)transient simple: children, benign no drugs needed last about 1 year 2)chronic simple: 3)persistent simple or multiple tics: motor and vocal, begin in adolescence usually end at end of adolescence 4) gilles de la Tourette syndrome: chronic multiple motor and vocal tics. lifelong, symptoms begin before 18/21 cortico-thalmocortical pathways seem to be involved. dopaminergic excess. Treatment: clonidine: a2 adrenergic agonist or atypical antipsychotics: risperidone clonazepam ( used in seizures, movement disorders, panic disorders) it is a benzodiazepam tranquilliser simple tics usually don't require therapy there is also tardive tics: with levodopa, chronic use of neuroleptics and amphetamine use, after head trauma or in neurodegenerative disoders of BG: ex, huntington differential diagnosis: Wilsons bobble head syndrome.

35 movement disease

extra pyramidial disorders When the regulation or execution of voluntary motor activity is impaired. There is no muscle palsy, sensation and cerebellar functions are preserved signs include: hyperkinetic disorders (abnormal involuntary movements,) tremor, chorea: rapid irregular muscle jerks, hemiballismus: unilateral chorea that is especially violent, because proximal muscles are involved tics, dystonia, myoclonus: Asterix: episodic cessation of muscular activity, causes sudden flexion when wrist extended --> flappy hands considerd a myoclonus but usually associated with postural tremor, mainly seen in metabolic encephalopathy and hypokinetic disorders (lack /poor movement, bradykinesia, hypokinesia, akinesia) cardinal signs for hypo kinetic are: hypokinesia, tremor, rigidity and impairment of postural reflexes. these disorders result form dysfunction of the structures in the basal ganglia. (putamen, globus pallidus, substansia nigra, subthalmic nucleous, caudate and its basic cicurity aetiology of hypo kinetic syndromes Idiopathic Parkinson Disease Other neurodegenerative diseases characterized by the impoverishment of the movements: Atypical parkinsonism (MSA, PSP, CBD) Huntington's disease Wilson's disease Frontotemporal degeneration Lewy Body Disease 3. The secondary parkinsonism (vascular, medicaments, postinfectious, posttraumatic, normotensive hydrocephalus, tumors, toxic) familial or benign essential tremor parkinsonism: cardinal signs: Hypokinesia Tremor at rest and postural Rigidity Impairment of postural reflexes lewy body disease progressive supranuclerar palsy Tremor: 8-12Hz

management of intracerebarl hemorrhage

headache, vomiting, altered consciousness and focal neurologic deficits causes: hypertension

Essential tremor

postural tremor in a otherwise normal patient, often has a familial basis (AD) most common extrapyramidal disorder in the population clinical: typically one or both hands or head and voice, interferes with the ability to perform fine or delicate tasks with hands and if affected speech (laryngeal muscles) (above 6hz) treatment: propranolol (b-blocker) primidone ( but stepwise unlike in epilepsy) benzodiazepines ( diazepam, alprazolam) botulin toxin nimodipine differential diagnosis problem : is that some times essential tremor is associated with positive cogwheel sig or is present at rest. sometimes ET will also evolve to PD

missfolding disorders

protein miss folding Diseases where specific protein undergoes a conformational rearrangement that endows it with a tendency to aggregate and become deposited within tissues or cellular compartments leading to the cell dysfunction and finally its death Taupathies (AD, PSP, CBD) Alfa-sinucleinopathies (PD, MSA, LBD) Poliglytamate disordres (HD, SCA) Prion disorders TDP-43 proteinopathies (FTD, MND) PD is alpha-synucleopathy Histopathologic markers of PD are Lewy bodies and Lewy neurites (a build-up of pathologicaly conformed alpha-synuclein)

foot palsy

same as foot drop: must lift knee higher due to inability to lift foot ( plantar extension) Nerve injury. Most commonly, foot drop is caused by an injury to the peroneal nerve. The peroneal nerve is a branch of the sciatic nerve that wraps from the back of the knee to the front of the shin. Because it sits very close to the surface, it may be damaged easily. An injury to the peroneal nerve may also be associated with pain or numbness along the shin or the top of the foot. Some common ways the peroneal nerve is damaged or compressed include: sports injuries diabetes hip or knee replacement surgery spending long hours sitting cross-legged or squatting childbirth time spent in a leg cast other causes may be muscle disorders or spinal cord injury ext

cerebellar signs

signs: 1. hypotonia 2.depressed or pendular tendon reflexes 3.ataxia: comples movement disorders--> impaired coordination . occurs in the same side as lesion of cerebellar hemisphere midline lesions may not be evident in limbs, but more marked in the trunk and evident when walking dysmetria: when movements aren't adjusted acuuretly for range asynergia: breakdown of a complex movement. intention tremor rebound phenomnen. overshooting of the limb after resistance is withdrawn. 4. gatitb disorder: unsteady imbalance of station 6. nystagmus, jerks in eye movement. usually seen in unilateral lesion of a cerebellar hemisphere. (NOT anterior cerebellar vermis) jerk is slowest and greatest when eye is turned to side of lesion 7. dysarthria: speech takes irregular and explosive quality. not if only midline is involved.

Lacunar stroke

small penertarting arteries located deep in the brain may become occluded. caused by chronic hypertension --> which changes the small vessel walls --> lipohyalinosis. these lacunar infarcts are are often multiple and cfound in approx, 105 0f brain autopsies. most common in the deep nulcei, affecting the putamen, thalamus, caudate nucleus and pons or internal capsu e (mainly posterior part) cliniclal: .Hard to find ( normal on angiography) but impo to, because prognosis for complete/ nearly complete recovery is good. there are 4 classic and distinctive lacunar syndromes: 1. pure motor hemiparesis: affecting face,arm and leg without any disturbed sensation of sensation. this is usually do to contralateral stroke in the contralateral capsule or pons 2. pure sensory stroke: hemisensory loss, associated with a paresthesia, results from stroke in contralateral thalamus. 3.ataxic hemipareis: pure motor hemiparesis with combined ataxia of same side: contralateral pons, internal capsuleor subcortical white matter. 4. dysarthria- clumsy hand syndrome: dysarthria and facial weakness, dysphagia, and mild weakness and clumsiness of hand on same side as face: lesion in contralateral pons, internal capsule

myelopathies

spinal cord lesions can lead to motor, sensory, or spinchter disturbances Myelopathy describes any neurological deficit related to the spinal cord Below the level of the lesion Spasticity is a common, It supports the patient in the upright position or lead to the deformity and increased disability Disturbances of sensation Unilateral involvement of the posterior columns-ipsilateral (dorsal column nuclei) loss of position (proprioception) and vibration sense, and tactile Unilateral involvement of the anterolateral columns-impairs contralateral pain and temperature and touch below the level of the lesion (spinothalmic) Total cord transection Immediate permanent paralysis and loss of sensation below the level of the lesion Acute stage is the 1. stage of spinal shock- loss of reflexes, sensory loss, urinary and fecal retention 2. Spastic paraplegia or quadriplegia - brisk tendon reflexes and extensor plantar responses. Sensation is lost below the level of the lesion. Sphincters regain some reflex function. 3.Spasms of the legs - patient assumes a posture with the legs in flexion or extension multiple sclerosis - demyelinating myleopathy neuromyelitis optica -< treated with corticosteroids acute disseminated encephalomyelitis--> occurs after immunisation after a nonspecific viral infection. headache, fever and confusion (meningeal irritation), somnolescens--> coma even seizures may occur flaccid weakness and sensory disturbance of legs, extensor plantar response, urinary retention, these neurologic deficits mostly recover after a few weeks. --> treatment with IV corticosteroids, iv immunogobins and plasmapherisis is sometimes helpful infective or inflammatory myelopathies spinal epidural abcess syphilis, tbc,aids, herpes vascular myelopathy spinal cord infarction: most often ant spinal artery, supplies anterior (1 ant, 2 post) cervical spondylosis

vertigo

the illusion of movement of the body or environment. Objective vertigo - environment is spinning around the body Subjective vertigo - body is spinning around the external space/environment It may be accompanied by other symptoms like: impulsion oscilopsia (a visual illusion of moving back and forth) nausea vomiting gait ataxia If vertigo is diagnosed than it implies that the lesion is localized in: peripheral or central vestibular pathways important to distinguish it from nonvertiginous dizziness (light headiness, dizzy due to glucose or O2 deprivation to brain-->may lead to syncope. Peripheral vertigo: the vertigo is severe and often intermittent, it always presents with nystagmus( unidirectional) but never vertical. can be accompanied with hearing loss/tinnitus etiology of pv. Benign paroxysmal positional vertigo - BPPV: --> severe episodes, symptoms with any change in head position, most severe is in lateral decubitus position with affected ear down. resolves spontaneously. BPPV is due to canalolithiasis, stimuli of the semicircular canals by free floatingg debris in endolymph Acute peripheral vestibulopathy (neuronitis vestibularis) --> most severe, always present nystagmus. some degree of vestibular dysfunction may always be present. treatment is steroids Meniere disease: disorder of the inner ear that causes episodes of vertigo, and you have fluctuating hearing loss with a progressive, ultimately permanent loss of hearing, ringing in the ear (tinnitus), and sometimes a feeling of fullness or pressure in your ear.pathophys: endolypmhatic hydrous. a chronic disease and therapy is symptomatic Head trauma (labyrinth commotion) Toxic vestibulopathy (alcohol, aminoglycosides, ciprofloxacine, salicylates)biphasic vertigo (which is based on the fact that alcohol is lighter than water) First attack of vertigo occurs when ethanol diffuses into cupula but not yet into endolymph Cerebellopontine angle tumor Are produced due to compression/displacement of the cranial nerves, brainstem and cerebellum: Insidious hearing loss is usually initial symptom Headache Vertigo in 20-30% of cases Fullness in the ear Facial weakness Absent corneal reflex Sensory loss over face Ataxia of the trunk and limbs central vertigo: results from lesions that effect the brainstennvestibular nuclei. causes less distress than peripheral vertigo. it may occur with or withou nystagmus ant it may be vertical and either uni or multidirectional. central V from lesions in brain stem may be accompanied with other brainstem/cerebellar signs ( hyperrefelxia, extensor plantar responses , limb ataxia. etiology of acute cv. Drug intoxications (i.e. ethanol, sedative-hypnotics, anticonvulsants like CBZ, PHT, MPB) Wernicke encephalopathy caused by B1 vitamin deficiency (triad of ataxia, ophthalmoplegia and acute confusion) Ischemia/infarction in brain stem and cerebellum Hemorrhage in brain stem and cerebellum Acute viral and bacterial infection of cerebellum Acute idiopathic immune-mediated demyelization of the cerebellum (ADEM) there is also chronic reasons for cv.Multiple sclerosis Alcoholic cerebellar degeneration Hypothyroidism Paraneoplastic cerebellar degeneration Inherited cerebellar ataxias (dominant and recessive) Wilson disease Posterior fossa tumors (metastases, astrocytomas, meduloblastomas, acoustic neuromas, meningeomas, ependymomas) Posterior fossa malformations (Syndroma Arnold-Chiari)

neurological complications of alcoholism

Alcoholic cerebellar degeneration <superior vermis ( Wernicke enephalopaty) thyamine deficiency Alcoholic korsakoff amnestic syndrome: memory loss/ inability to form new memories degeneration of dorsomedial thalamic nuclei necrotzing alcoholic myopathy toxic vestibulopathies causing vertigo due to the diffusion of alcohol into the cupula and endolymph toxic - neuropathies polyneuropathy,a very common neurologic complication due to alcoholism, a symmetric distal sensorimotorneuropathy. defective perception of vibration and touch and depressed or abscent ankle reflexes Gait ataxia -> truncal instability dysarthria distal sensory deficit nystagmus treatment: abstence from alcohol and thyamine repletion may help these disorders

paraneoplastic neuropathies

Carcinoma (especially small-cell cancer of the lung) and lymphoma may be associated with neuropathies that are thought to be immunologically mediated, based on the detection of autoantibodies to neuronal antigens in several cases.

chronic subdural hematoma

50-70 year old patients after minor head trauma, alcoholism, cerebral atrophy, epilepsy, anticoagulation, ventricular shunts, and long-term hemodialysis. the symptoms may be delayed for months after the small trauma. headache is the initial symptom, confusion and dementia, hemiparesis and vomiting, papilledema with cognitive disorders --> extenstor plantar response the hematoma will be seen on CT ( a cresent shaped extra-axial area of decreased density. their is a chance for bilateral subdural collections should be surgically evacuated unless contraindicated

acute disseminated encephalomyelitis

Acute disseminated encephalomyelitis (ADEM), or acute demyelinating encephalomyelitis, is a rare autoimmune disease marked by a sudden, widespread attack of inflammation in the brain and spinal cord. As well as causing the brain and spinal cord to become inflamed, ADEM also attacks the nerves of the central nervous system and damages their myelin insulation, which, as a result, destroys the white matter. It is often triggered after the patient has received a viral infection or, perhaps exceedingly rarely specific non-routine vaccinations ADEM's symptoms resemble the symptoms of multiple sclerosis (MS), so the disease itself is sorted into the classification of the multiple sclerosis borderline diseases. However, ADEM has several features that distinguish it from MS.[7] Unlike MS, ADEM occurs usually in children and is marked with rapid fever, although adolescents and adults can get the disease too. ADEM consists of a single flare-up whereas MS is marked with several flare-ups over a long period of time. ADEM is also distinguished by a loss of consciousness, coma and death, produces multiple inflammatory lesions in the brain and spinal cord, particularly in the white matter. broad spectrum antibiotics and acyclovir until bacterial or viral encephalitis are excluded. differential dig: infectibe meningitis, encephelitis, other demyelinating dis. High dose IV methylprednisolone

Subarrachnoid hemorrhage

Acute headache: sudden onset of a new headache should be investigated SAH: spontaneous/non traumatic sah is usually due to a ruptured cerebral aneurysm or an AVM. 75% of them due to saccular (berry) aneurysm also fusiform aneurysms, thought to be by atherosclerosi or disscection or the reason for SAH is intracranial AVM rupture of artery, elevates intracranial pressure and disorts pain sensitive structures--> causing headache clinically, its severe and generalized and described as the worst headache the patient has ever had (unlessAVM, may be less) other clinical features: loss of consciousness vomiting neck stiffness (meningeal irritation, may also elevate the temp 39c) BP rises preretinal globuar sunhyaloid hemorrhages, most suggestive towards diagnosis bilateral extensor plantar responses and abducens nerve palsy are frequent non localising sings due to increased intracranial pressure. focal neurologic are otherwise uncommon in SaH due to aneurysm. more common when due to ruptured AVMs. complications: recurrence of hemorrhage, intraparenchymal extension of hemorrhage arterial vasospasm --> can lead to cerebral ischemia seizure, only a chance if cerebral cortex is damaged lab: CT , CSF--> marked elevated opening pressure, is bloody. CSF may become xanthocromic (breakdown of heme) but after 12h. treatment: prevent high blood pressure that may re-rupture aneurysm or avm. đbed rest with head slightly elevated, anti platelets should be avoided!! but analgesic fro headache, absolute bed rest NIMODIPINE reduce theischemic sequel of cerebral vasospasm

,management of acute ischemic stroke

Blood pressure should usually not be lowered acutely, except for patients with acute ischemic stroke in whom it is high enough (>185 mm Hg systolic or >110 mm Hg diastolic pressure) to make an otherwise suitable candi- date ineligible for thrombolytic therapy (see later). When acute antihypertensive therapy is indicated, rec- ommended drugs include intravenous labetalol or nicardipine. 2. Hyperthermia, which may adversely affect outcome, should be corrected, and any infectious cause identified. 3. Hypoxia (oxygen saturation ≤94%) should be treated with supplemental oxygen. 4. Hypoglycemia (blood glucose <60 mg/dL) should be corrected. 5. Anticoagulation with heparin, given by continuous intravenous infusion to achieve an activated partial thromboplastin time (aPTT) 1.5 to 2.5 times control, followed by warfarin, given orally daily to achieve an INR of 2.5±0.5, or another oral anticoagulant (see Table 13-6), is indicated if a cardiac embolic source (eg, atrial fibrillation, mitral stenosis, or mechanical valve replacement) appears to be responsible for TIA or acute ischemic stroke. 6. Antiplatelet therapy with aspirin (325 mg orally once, followed by 81-325 mg orally daily) is recommended for presumed noncardiogenic TIA or acute ischemic stroke, unless the patient is to undergo thrombolysis. 7. Statins should be continued for patients receiving long- term statin treatment. interventional 1. Intravenous thrombolysis—Intravenous administra- tion of recombinant tissue plasminogen activator (rtPA or alteplase) within 4.5 hours of the onset of symptoms reduces disability and mortality from acute ischemic stroke. 2. intraarterial thrombolysis: acute ischemic stroke who are not candidates for intravenous thrombolysis, such as those treated 4.5 to 6 hours after the onset of symptoms or with a recent history of major surgery, and in patients in whom intravenous therapy is unsuccessful. 3. Clot retrieval—Mechanical thrombectomy with stent or coil retrievers (eg, Solitaire FR, Trevo or Merci), alone or in combination with rtPA surgical: stenting endarectomy: surgical removal of thrombus surgeries to prevent herniation: posterior fossa decompression with evacuation of infarcted cerebellar tissue

hereditary neuropathies

Charcot-Marie-Tooth hereditay neuropathies genetically heterogenous group of disorders (AD disorders) there is weakness and wasting of distal muscles in limbs with or without sensory loss. they are devided into demyelinating (CMT-1) or neuronal (CMT-2, sparing sensory neurons) types CMT-1 has its onset in the first decade, slowly progressive the nerves are palpabr thickened in approximately 50% of cases. nerve conduction velocity is reduced these hereditary disorders are usually divided by their genetic findings. CMT-1 results most often from a duplication or mutation in gene for peripheral myelin protein. CMT-2 is generally less severe, no nerve enlargement and velocity of conduction is not much slower Dejerine-sottas disease, onset by 2 years of age. severe sensorimotor neuropathy, that often extends to the proximal muscles and is associated with skeletal abnormalities such as scoliosis . there is sever demyelinationof the nerves AD muations same as CMT-1 HEREDITARY SENSORY AND AUTONOMIC NEUROPATHIES HSAN type 1, dominant with progressive and gradual symmetric loss of distal pain, temp perception withe relative perception of touch AMYLOIDOSIS can occur in hereditay form and caus polyneuropathy pain temp, and autonomic functions are affected . with distal paresthesias , dysesthesias and numbness, posturtal hypotension, impaired thermoregulatory sweating, disturbance of bladder, bowl or sexual function. distal weakness and wasting will eventually progress, and tendon reflexes wont be lost until the late stage. Carpal tunnel syndrome (entrapment neuropathy) may develop due to the amyloid deposits. Porphyria is a group of diseases in which substances called porphyrins build up, affecting the skin or nervous system. The types that affect the nervous system are also known as acute porphyria.[1] Symptoms of acute porphyria include abdominal pain, chest pain, vomiting, confusion, constipation, fever, and seizures.[1][2] These symptoms typically come and go with attacks that last for days to weeks. Attacks may be triggered by alcohol, smoking, stress, or certain medications. If the skin is affected, blisters or itching may occur with sunlight exposure.[2] The disease is usually inherited from a person's parents and is due to a mutation in one of the genes that makes heme.[2] Some types are autosomal dominant and others are autosomal recessive. treatment dextrose to suppress the heme biosynthetic pathway and propanolol to control tachycardia and hypertension polyneuropathy symmetric and usually more marked proximally tha distally

Coma definition, etiology, examination, emergency management

Coma is a sleep-like state where the patient makes no purposeful response to the environment and can not be aroused to wake up. It results from a disturbance in the function in the brainstem reticular activating system above the mid pons or of both hemispheres. --> these are the brain regions that maintain consciousness Emergency management: 1. Ensure airway isn't blocked, adequacy of ventilation (abcence of cyanosis and Resp rate greater than 8/min) measure blood pressure and pulse for status of circulation --> intubate patient, mechanical ventilation, intravenous fluid replacement, antirythmic drugs. 2. draw blood for lab studies, for measurement of glucose, electrolytes, hepatic and renal tests, prothrombin time and patial thromboplastin time, total blood count. 3. IV infusion to administer, dextrose 25g( for possible hypoglycemic) , thiamine and naloxone -> for possible opiate overdose 4.withdraw arterial blood for gas and pH determination (asses ventilation and cause of metabolic coma. 5.tratment of seizures examination: history, especially onset of loss of consciousness 1. sudden: vascular orgin, esp brain stem or SaH 2. rapid progression, from hemispheric signs such as hemiparesis, hemisensory or aphasia --> to coma. suggest intracerebral hemorrhage 3. protracted cource, days to weeks or more, tumor, abcess, chronic subdural hematoma 4. from confusional state or agitated delirium, metabolic dearrangement or infections 8meningitis, encephalitis) look for signs of trauma: CSF out of ear, nose. fracture, raccoon eyes ect. Blood pressure (HTN--> hemorrhage) , temp (hypothermia suggest intoxication,hypoglycemia ect) nuchal rigidity: meningeal irritation optic fundi: reveal papilledema or retinal hemorrhages (chronic HTN or increasd intracranial pressure) subhyaloid hemorrhages suggest SaH. Pupils: normal 3-4mm: metabolic cause pinpoint pupils: 1-1.5mm opioid overdose or focal lesion in pons --> naloxone fixed(unreactive to light) and dilated: greater than 7mm: compression of oculomotor (CN3) anywhere along nerve, midbrain--> to orbit eye movements: tested by stimulating vestibular system by passive head rotataion (doll's-head maneuver) (1) or ice water irrigation (2) (1) eyes should move in the direction opposite to that of the head rotation. (2) cold water caoric tonic deviation fofn the eyes toward the irrigated side. if not suggests: lesion involving posterior fossa (cerebellum or brainstem) or intoxication with sedative drugs motor response to pain dectoriate : thalamic lesion, (flexion of arm) decerberate Causes: for supratentorial lesions -> subdural hematoma, blood between dura ad arachnoid epidural hematoma cerebral contusion (bruising of the brain) intracerebral hemorrhage (chronic HTN) Brain abcess Subtentorial lesions basilar artery thrombosis or emboli pontine hemorrhage cerebellar hemorrhage or infarction posterior fossa subdural and epidural hematomas diffuse encephalopathies meningitis and encephalitis subarachnoid hemorrhage hypoglycemia--> insulin overdose, alcoholism, liver disease, insulin secreting neoplasms global cerebral ischemia drug intoxication diffuse encephalopathies

Episodic loss of consciousness

Consciousness is lost when both cerebral hemispheres or the brainstem reticular activating system is compromised. Episodic dysfunction of these anatomic structures are often transient and recurrent . epilepsy, hypoperfusion : decreased cardiac outflow postural hypotension vasovagal disorder

Dementia ( definition, etiology, approach to diagnosis , mental status exam)

Dementia is a loss of mental ability severe enough to interfere with normal activities of daily living, lasting more than six months, not present since birth, and not associated with a loss or alteration of consciousness. approach to diagnosis: * History-> to establish that the patients level of cognitive functioning has declined, the time course of this decline and maybe (fam hist, unprotected sex, alcohol) help differentiate diagnosis of dementia. general exam mental status exam: helps determine weather it is the level of consciousness impaired or cognitive dysfunction and if so the scope of the cognitive dysfunction (global or restricted) ex, memory, language. the minimental status examination is useful for bedside screening of dementia language --> frontotemporal den memory (more diffuse) --> alzheimers Etiology: most often a mix between Alzheimers, neurodegenerative and vascular dementia ( multi-infarct) other common diseases of progressive are, frontotemporal dementia, lewybody disease and parkinsons. less common and sometimes reversible,; normal pressure hydrocephalus, intracranial mass lesions, vit B def, , hypothyroidism, neurosyphilis neurodegenerative proteinopathies: missfolded proteins --> that form insoulble aggregates. impo pathology for several neurodegenerative diseases. can be either acquired or genetic. only a few inherited and toxic cases underlying cause is known. cell to cell prionic transmission, allowing them to spre4adthrough the nervous system Treatment for psychosis due to dementia: Quetiapine, and clozapine SSRI's for dementia with depression:

DIX-Hallpike and Epley maneuvers

Dix-Hallpike test: is the first step in the epley manuver, Unidirectional horizontal/rotatory jerky nystagmus that is maximal on gaze away from affected ear - Ny is delayed in onset by several seconds after assumption of the precipitating head position - Nystagmus and vertigo resolve within seconds to minutes Epley maneuver: Step 2 Then, without lifting up your head, the doctor will turn your head to look at the same angle to the opposite side, so that the other side of your head is now facing the floor. The doctor will hold you in this position for 30 seconds or until your vertigo stops. Epley maneuver: Step 3 The doctor will help you roll in the same direction you are facing so that you are now lying on your side. (For example, if you are looking to your right, you will roll onto your right side.) The side that causes the worst vertigo should be facing up. The doctor will hold you in this position for another 30 seconds or until your vertigo stops. Epley maneuver: Step 4 The doctor will then help you to sit back up with your legs hanging off the table on the same side that you were facing. This maneuver is done with the assistance of a doctor or physical therapist. A single 10- to 15-minute session usually is all that is needed. When your head is firmly moved into different positions, the crystal debris (canaliths) causing vertigo will move freely and no longer cause symptoms This is used on benign paroxysmal positional vertigo

Anticonvulsant therapy in pregnancy

Djamona say valproate is ok, better than changes or decreasing to much( increasing risk of seizure during pregnancy) there is a risk of still birth, microencepahly --> effect of epilepsy teratogenic effect on anticonvulsants--> cleft palate/lip, cardiac abnormalities Valporate and carbamazepine are associated with increased risk of neural tube defecets phenobarbital, phenytoin :teratogenic risks folate deficiency: anticonvulsants lower folic acid--> neural tube def. folate supplement (1mg/d) withdrawl before pregnancy is an option for a patient who has been seizure free for several years. or at least maintain treatment with 1 single drug that has been shown to best maintain seizures, to avoid clinical toxicity. anticonvulsant plasma levels may decrease during pregnancy due to increade metabolism

12. frontotemporal dementia and lewy body disease

LWB disease is a diffuse Alpha-synucleinopathy that has 3 phenotypes; parkinsons, LB dementia and Primary autonomic failure Lewy body disease: dementia where the cognitive function may fluctuate markedly over a 24h period, unlike alzheimers and will also either shortly after or before dementia begins show symptoms of parkinsonism clinical: parkinsonism (rigor+hypokinesia, with little or no tremor) typical frequent visual hallucinations oscillations of attention and cognitive performance if neuroleptics are given than parkinsonism is deteriorated if given due to parkinsonism dopaminergic drugs can cause strong side effects (hallucinations) Frontotemporal dementia

Herpes zoster ophthahnicus. Ramsey-Hunt syndrome and postherpetic neuralgia

Herpes zoster ophthahnicus. Ramsey-Hunt syndrome and postherpetic neuralgia

horners syndrome

Horner's syndrome is a combination of symptoms that arises when a group of nerves known as the sympathetic trunk is damaged. The signs and symptoms occur on the same side as the lesion of the sympathetic trunk. It is characterized by miosis (a constricted pupil), partial ptosis (a weak, droopy eyelid), apparent anhidrosis (decreased sweating), with or without enophthalmos (inset eyeball). The nerves of the sympathetic trunk arise from the spinal cord in the chest, and from there ascend to the neck and face. The nerves are part of the sympathetic nervous system, a part of the autonomic (or involuntary) nervous system.

Huntington and wilsons

Huntington: hereditary AD offspring 50%, with anticipation for every generation, gradual onset, with progression of chorea (rapid irregular muscle jerks, involuntary) and dementia initial symptoms: intellectual and abnormal movemnets atypical symptoms: progressive rigidity and Akinesia, epilepsy and cerebellar ataxia diagnosis: gentic testing CT with atrophy of cerebral cortex and caudate nucleus fatal after 10-20 years of onset. nothing to treat for the dementia but dopaminergic inhibition for movement disorder --> reserpine (antipsychotic) wilsons: AR genetic disease of coppar metabolism, produces neurologic and hepatic dysfunction patho: decreased binding of copper to the transport protein ceruloplasmin--> deposited in liver, brain corne, kidney --> free radical formation and oxidative damage neurological clinical: resting/postural tremor, choreiform movements, facial grimacing, rigidity,hypokinesia, dysartheria, dysphagia, abnormal flexed postures and ataxia. seizures Psycholoic: dementia treatment chelating agents; promotes extraction of copper from tissues

management of intracerebelar hemorrhage

IC causes 10-15% of strokes, hypertension most common underlying cause pathophysio In normotensive individuals, the lowest mean BP in which autoregulation is still affective is ca 60mmhg any lower and changes in caliber cant compensate --> lightheadiness, confusion, blured vision. below 40-35 somnolence upper limit of autoregulation is 150-200mmhg if cerebral flow increases this can cause hypertensive encephalopathy. chronic hypertension promote structural changes in the vessel walls; fibrinoid necrosis/liphyalinosis, military aneurysms hypertensive hemorrhage causes compression rather than destruction on adjacent brain tissue, resulting in reduced perfusion and metabolism in the perihematomal area. clinical, occurs without warning, usually while awake, Headache 50%, vomiting, BP elevated, the increasing cerebral edema produces worsening clinical presentation. actual bleeding is short and usually not recurrent fatality usually, due to herniation by mass effect 2 most common sets Of HTN haemorrhage : putamen and thalamus; affecting the postural internal capsule --> producing contraateral sensorimotor deficit thalamic hemorrhage : tonic downward deviation (staring at the tip of your nose) due to affect on the center of pward gaze, located in midbrain. homonymous hemanopia may also occur pontine hemorrhage: coma within seconds, usually death within 48h. pinpoint pupils, horizontal eyemovemnets absent cerebellar hemorrhage: inability to stand deviation away from lesion (impaired gaze) treatment: don't want to the decrease BP due to risk of compromising cerebral blood flow, but a short acting vasodilator may help. lower it to approximately 180mmhg or nitroglycerin topically. easy to rub off in needed to corticosteroids: may help with the vasogenic edema , but only temporary benefit. Dignosis: CT or MRI with gradient echo lumbar puncture is not advised if cerebellar hemorrhage is suspected. it might hasten the process of herniation. surgical: cerebellar hemorrhage: decompressive posterior fossa surgery and removal of hematomas lobar hemorrhage, surgical evacuation no surgery for deep hemorrhage or pontine, not without distruction of overlying brain ``Medical Initial management of intracerebral hemorrhage includes airway support with ventilatory assistance if required and treatment of hypertension. Reducing systolic blood pres- sure to ~140 mm Hg over 1 hour does not compromise perfusion of the perihematoma region or arterial water- shed territories of the affected hemisphere, is as safe as more modest reduction to ~180 mm Hg over 6 hours, and may lead to a better outcome. Antihypertensive drugs that may be used in this setting include labetalol and nicardip- ine. Neither corticosteroids nor antiedema agents are effec- tive, and prophylactic administration of anticonvulsants is not recommended. ``Surgical 1. Cerebellar hemorrhage—Neurologic deterioration, brainstem compression, and hydrocephalus are indica- tions for decompressive posterior fossa surgery, which may avert a fatal outcome. Results are best in conscious patients. 2. Lobar hemorrhage—Surgical evacuation can also be useful for lobar hematomas, especially those larger than30 mL in volume and located within approximately 1 cm of the brain surface. Patients with good neurologic function who begin to deteriorate are optimal candi- dates. Prognosis is related to the level of consciousness before surgery. 3. Deep hemorrhage—Surgery is not beneficial for pon- tine or deep cerebral hypertensive hemorrhage.

54 Atypical parkinsonism

Multisystem atrophy (MSA) is an Alpha- synucleinopathy disorder, but it has a mixture of different features, with parkinsonism (MSA-P) due to neuronal loss in putamen, globes pallidus, caudate nucleus. cerebellar dysfunction (MSA-C) + autonommic insufficiency. --> shy-drager syndrome. Progressive supranuclear palsy: a taupathy, a idiopathic degenerative disorder, primarily affecting subcortical gray matter. clinically: gait disturbance with early falls, supranuclear opthalmoplegia ( failure of voluntary vertical gaze, with later failure of horizontal gaze) neck often assumes an extended posture, resitance to flexion. bradykinesia (mimic parkisons but symmetric!! and with less /no tremor) pseudobulbar palsy: facial weakness, dysarthia, dysphagia and its lack of efficiency of levodopa corticobasal degeneration; rare, cortico basal ganglionic dysfunction the typical parkinson symptoms with other cortical focal deficits, like sensory loss, aphasia ext none of there a typical disorders response very well to parkinson medication/ levodopa Corticobasal degeneration: rare, taupathy very simminal to PD with asymmetric parkinsonism, but the cortical defixts may cause sensory loss-neglect, apraxia, myoclonus and dementia. Common signs/symptoms for atypical parkinsonism: Rigidity Akinesia Postural instability Dysarhtria Dysphagia Lack of efficiency of levodopa Vascular (also referred to as "multi-infarct") parkinsonism is a form of "atypical parkinsonism" Or a secondary parkinsonism in which parkinsonian symptoms (slow movements, tremor, difficulty with walking and balance, stiffness and rigidity) are produced by one or more small strokes, rather than by gradual loss of nerve cells as seen in the more typical neurodegenerative Parkinson's disease. By definition, a stroke is the loss of a discrete brain area (lesion) because of blockage of the blood supply to that brain region. secondar parkinson symptoms Normal pressure hydrocephalus (NPH) is a clinical symptom complex caused by the build-up of cerebrospinal fluid. This condition is characterized by abnormal gait, gait apraxia--> unsteadiness when standing, difficulty to initiate walking even though there is n weakness, , urinary incontinence, hyperrefexia, babinski, and (potentially reversible) dementia. Often positive history of brain trauma, ICH or meningoencephalitis CT scan shows expanded internal CSF spaces with a relatively small degree of cortical atrophy Th.: surgical treatment (drainage of cerebrospinal fluid) shunting

myastina gravis

Myasthenia gravis is caused by variable block of neuro- muscular transmission related to an immune-mediated decrease in the number of functioning nicotinic acetylcho- line receptors (Figure 9-11). In approximately 80% of cases, antibodies to the skeletal muscle nicotinic acetyl- choline receptor are present and lead to loss of receptor function. Myasthenia gravis can occur at any age and is sometimes associated with thymic tumor, thyrotoxicosis, rheumatoid arthritis, or disseminated lupus erythematosus. More com- mon in females than males, it is characterized by fluctuating weakness and easy fatigability of voluntary muscles; muscle activity cannot be maintained, and initially powerful move- ments weaken readily. There is a predilection for the external ocular muscles and certain other cranial muscles, including the masticatory, facial, pharyngeal, and laryngeal muscles. Respiratory and limb muscles may also be affected. pyridostigminewill eventually involve ocular muscles. 90% of the cases. diagnosis is confirmed with a positive response to intravenous edrophinum Myasthina gravis can aslo closely mimic INO ( a lesion of the medial longitudinal fasiculus) so it is impo to rule the other one out. (by MG test)

Myopathic disorders (muscular dystriphies,myotonic dystrophies, inflammatory myopathies)

Myotonic disorders abnormality of the muscle fiber membrane (sarcolemma) causes marked delay of affected muscle before it can relax after contraction. --> stiffness examination, see the difficulty in relaxing hand after sustained grip electromyography may reveal characteristic high frequency discharges of potentials , frequency and amplitude. EMG loudspeaker sounds like a dive bomber or chain saw. Myotonic dystrophies 1: Ad CTG repeat above 34 repeats, myotonia causes weakness and wasting of the facial, sternomastoid and distal limbs. cataracts frontal baldness, testicular atrophy, DM and cardiac abnormalities , intelectuall changes and sudden death are also common. myotonic dystrophy type 2: more proximal limbs , fingers neck. less severe course thann DM1 inflammatory myopathies: dermatomyositis and polymyositis: immune mediated inflammatory myopathies characterised by distraction of muscle fibers. dermatomyositus--> microangiopathy, rash over eyelids and on joints. polymyositis--> muscle fibers express MCH class 1 antigens, CD8 cytotoxic t cells invade, causing necrosis. EMC : abundance of short low amplitude polyphasic motor unit potentials (like myopathic processes) biopsy shows muscle necrosis with infiltration of inflammatory cells treatment corticosteroids. CK generally levanted

neuromyelitis optica

Neuromyelitis optica (NMO) and NMO Spectrum Disorder (NMOSD), also known as Devic's disease, is an autoimmune disorder in which immune system cells and antibodies primarily attack the optic nerves and the spinal cord. The damage to the optic nerves produces swelling and inflammation that cause pain and loss of vision; the damage to the spinal cord causes weakness or paralysis in the legs or arms, loss of sensation, and problems with bladder and bowel function. NMO is a relapsing-remitting disease, like MS. During a relapse, new damage to the optic nerves and/or spinal cord can lead to accumulating disability. Unlike MS, there is no progressive phase of this disease. Therefore, preventing attacks is critical to a good long-term outcome. The cause of NMO is thought to be due to a specific attack on the aquaporin-4 (AQP4) water channel located within the optic nerves and spinal cord. Aquaporins (AQPs) are proteins that transport water across cell membranes. More than 70% of NMO and NMOSD patients test positive for an antibody biomarker in the blood called the NMO-IgG or anti-AQP4 antibody treatment: IV methylprednisolone, followed by oral predisone taper. if poor response--> plasmapheresis. with iv immunoglibins. or longterm immunosuppressive therapy: mycophenolate mofetil

Myasthenia gravis

Pathogenesis: caused by block of neuromuscular transmission, related to an immune-mediated decrease in number of functioning of nicotinic acetylocholine receptors. clinical:can occur at any age, and be associated with thymic tumor, thyrotoxicosis, RA, dissiminated lupus erythematous. Females>males fluctuating weakness and faitigness of vonutary muscles predilection for external ocular muscles, masticatory, facial and pharyngeal --> present with : ptosis (drooping of upper eyelid) diplopia, chewing difficulties and swallowing, speech, resp difficulties. symptoms may fluctuate characteristic is that sustainesdactivity of affected muscles leds to temporary increase in weakness. dignosis can be confirmed by the benefit of anti-cholinesterase administration. endrophonium test. in Mg patients there is obvious improvement in the strength of muscle treatment: drugs that impair neuromuscular junction should be avoided: quinidine, procainamide,propranolol, phenytoin, lithium, tetracyclin, aminoglycoside antibiotics approved treatments: anticholinesterase drugs: symptomatic relief pyridostigmine 2)thyomectomy 3)corticosteroids: 4) azathioprine 5)plasmapheresis, 6) IV immunoglobins 7)mycophenolate mofetil: agent that selectively inhibits proliferation of T and b lymphocytes --> immunosuppression

signs of anterior and posterior circulations stroke

The anterior cerebral circulation supplies most of the cere- bral cortex and subcortical white matter, basal ganglia, and internal capsule. It consists of the internal carotid artery and its branches: the anterior choroidal, anterior cerebral, and middle cerebral arteries. The middle cere- bral artery in turn gives rise to deep, penetrating lenticu- lostriate branches (Figure 13-2). The specific territory of each of these vessels is listed in Table 13-3. Anterior circulation strokes are commonly associated with symptoms and signs that indicate hemispheric dys- function (Table 13-4), such as aphasia, apraxia, or agno- sia (described in Chapter 1, Neurologic History & Examination). They also often produce hemiparesis, hemi- sensory disturbances, and visual field defects, but these can occur with posterior circulation strokes as well. The posterior cerebral circulation supplies the brainstem, cerebellum, thalamus, and portions of the occipital and temporal lobes. It consists of the paired vertebral arteries, the basilar artery, and their branches: the poste- rior inferior cerebellar, anterior inferior cerebellar, superior cerebellar, and posterior cerebral arteries (see Figure 13-2). The posterior cerebral artery also gives off thalamoperforate and thalamogeniculate branches. Areas supplied by these arteries are listed in Table 13-3. Posterior circulation strokes produce symptoms and signs of brainstem or cerebellar dysfunction or both (see Table 13-4), including coma, drop attacks (sudden col- lapse without loss of consciousness), vertigo, nausea and vomiting, cranial nerve palsies, ataxia, and crossed sen- sorimotor deficits that affect the face on one side of the body and the limbs on the other. Hemiparesis, hemisen- sory disturbances, and visual field deficits also occur, but are not specific to posterior circulation strokes. anterior cerebral artery: stroke not so common, supplies parasagittal cerebral cortex. motor and sensory cortex --> contralateral leg (motor and sensory impaired) and maybe impaired voluntary control of micturition. (failure to inhibit reflex bladder contractions) MIDDLE CEREBRAL A: supplies most of whats left of cerebral hemisphere and deep subcortical structures superior devision of mca brach: motor and sensory of face,hand arm, expressive language (broca (in dominant hemisphere) inferior division: supplies visual receptive language --< wernickes area (dominant hemisphere) lenticostriate: branches of the proximal portion of MCA supply the basal ganglia and motor fibers related to face,hand.arm,keg as they descend in the genu and posterior limb of internal capsule. Posterior cerebral a: supplies the occipital cerebral cortex, medial temporal lobes, thalamus and rostral midbran. clinical: emboli like to lodge at the apex of the basilar artery, blocking both PCA. if just one PCA is occluded -- homonymous hemaniopia, affecting contralatreal visual fired. macular vision is spared dominant occipital lobe: anomic aphasia, inability to read but spared writing (alexia without agraphaia) visual agnosia

Transient global amnesia

Transient global amnesia is a syndrome of acute memory loss that tends to occur in middle-aged or elderly patients. Transient cerebral ischemia, spreading depression (waves of reduced electrical activity in the cerebral cortex), and physi- cal or emotional stress have all been suggested as the cause. A syndrome of acute memory loss. transient cerebral ischemia, spreading depression (waves of reduced electrical activity) and physical or emotional stress may be causes. It affects short term memory and lasts up to 24h. patient is agitated and perplexed, ask for whereabouts and time but usually hase knowledge of own identity.

monoocular disorders

Transient monocular blindness caused by optic nerve or retinal schema sudden in onset and resolves rapidly. remains max for 1-5min and resolves within 10-20min think it is embolic material in retinal arteries treatment is the same as for TIA, combo of aspirin and if needed surgical removal of plaque. optic neuritis > subacute ( impairment occurs over hours-days) painful, unilateral visual loss with partial resolution > inflammation of optic nerve, most often cause id demyelination > scotoma (blind spot) is common due to decreased visual acuity. > gadolinium enhancement of optic nerve on MRI scans for acute demyelinating optic neuritis. > methylpredisone (IV) oral prednisone, may hasten recovery but not change outcome. This disease may be a symptom/sign of multiple sclerosis nonarteritic anterior ischemic optic neuropathy > (idiopathic infarction ) less reversible visual loss of sudden onset painelss, Giant cell (temporal) arteritis Giant cell arteritis (GCA), or temporal arteritis, is a systemic inflammatory vasculitis of unknown etiology that occurs in older persons and can result in a wide variety of systemic, neurologic, and ophthalmologic complications This is a arthritic infarction of the optic nerve, which will lead to sudden visual loss less reversible but unlike Nonarteritic on --> it is more treatable, with corticosteroids (IV) and oral for protection of what visoíon is left to distinguish it from na on, features are increased esr and c-reactve protein.

acute inflammatory polyneuropathy and chronic demyelinating polyneuropathy

acute gillian barre :Guillain-Barré syndrome is an acute or subacute polyneu- ropathy that can follow minor infective illnesses, inocula- tions, or surgical procedures or may occur without obvious precipitants. Clinical and epidemiologic evidence suggests an association with preceding Campylobacter jejuni infec- tion. is an autoimmune process that is characterized by progressive areflexic weakness and mild sensory changes. Sensory symptoms often precede motor weakness. About 20% of patients end up with respiratory failure. Plasmapheresis appears to reduce the time required for recovery and may decrease the likelihood of residual neu- rologic deficits. It is best instituted early, and it is indicated especially in patients with a severe or rapidly progressive deficit or respiratory compromise. Intravenous immuno- globulin Chronic inflammatory demyelinating polyneuropathy is clinically similar to Guillain-Barré syndrome except that it follows a chronic progressive course, or a course character- ized by relapses, and no improvement is apparent within the 6 months after onset. Its cause is not known. Its clinical features are summarized in Table 10-4. Examination of the CSF reveals findings resembling those in Guillain-Barré syndrome. The electrophysiologic findings indicate a demyelinative neuropathy with super- imposed axonal degeneration. The disorder is often responsive to treatment with cor- ticosteroids (prednisone 60-100 mg/d for 2-4 weeks, then gradually tapered to 5-20 mg every other day), which may be required on a long-term basis. Treatment with intrave- nous immunoglobulin

diabetic opthalmoplegias

an isolated oculomotor, trochlar or abducens nerve lesion may occur in patients with DM. Diabetic oculomotor lesions are characterized with pupillary sparing (pupillary constriction)which is commonly attributed to infarction of the central portion of the nerve with sparing of the more peripherally situated fibers that mediate pupillary constriction In known diabetics, painful ophthalmoplegia with exophthalmos and metabolic acidosis requires urgent attention to determine the possibility of fungal infection in the paranasal sinus, orbit, or cavernous sinus by mucormycosis. The diagnosis is usually made by biopsy of the nasal mucosa. Urgent treatment with amphoteri- cin B and surgical debridement of necrotic tissue is required.

signs of cerebellar lesions

ataxia : irregularities in rate, rhythm, amplitude and force of voluntary movements (complex movement disorders) impaired coordination hypotonia depressed pendular reflexes

Botulism ( prevents release of ach at neuromuscular junction and autonomic synapses)

causes neuromuscular paralysis after ingestion of home canned food that is contaminated with the toxin. clinical: diplopia, ptosis, facial weakness, dysphagia, nasal speech, respiratory difficulties besides motor deficit. pupils are dialated ( in MG pupils are unaffected) dryness in mouth, paralytic ileus, postural hypotension (autonomic)

frontotemporal dementia

characterised by atrophy of the frontal and temporal lobes generally causing behavioural changes ( blunted emotions, lack of insight, altered interpersonal interactions and language aphasia . due to its frontal-temporal distribution , unlike alzheimers which is parietal-temporal and has a more prominent memory loss. but both exhibit Tau inclusions but FTD does not have amyloid. in MRI PET scan: usually asymmetric--> right sides atrophy usually behavioural, left sided atrophy affecting language predominantly. genetic screening for MAPT or GRN in a with a positive family history. drugs like memantine and anti cholinesterase not effective like in AD. maybe depressants esp Serotonin re-uptake inhibitors for behavioural symptoms. Patients with parkinsonian features ( corticobasal degeneration, progressive supra nuclear),; levodopa may alleviate or dopamine agonists.

Herpes zoster ophthahnicus,Ramsey-Hunt syndrome and postherpetic neuralgia

if v1 is affected and a rash appears´r near to eye, can lead to ocular complications, Late sequelae—glaucoma, cataract, chronic or recurrent uveitis, corneal scarring, corneal neovascularization, and hypesthesia—are common and may threaten vision. Postherpetic neuralgia may develop late. post herpatic neuralgia: persistent pain and abnormal sensation , left for weeks months after herpes rash is gone ramsey hunt syndrome --> facial nerve palsy (CN7) in association with hepatic eruption.

Ataxia

incoordination or clumsiness of movements, not due to muscular weakness. It can affect eye movement, speech ( dysarthria) and the limbs and trunk it can be caused by vestibular, cerebellar or sensory ( proprioceptive) Vestibular: Central or peripheral lesions of the vestibular nerve, canal vestibular This is gravity-dependent ataxia, it is apparent only when patient attempts to stand or walk And truncal ataxia/unsteadiness is more severe in central vertigo than in peripheral vertigo (brainstem or cerebellar strokes Sensory ataxiaResults from affection of proprioceptive pathways in: peripheral sensory nerves spinal cord rarely thalamus and parietal lobe Typically, the trunk coordination/unsteadiness is dramatically worsen with deprivation of visual sensory input (i.e. with closed eyes - positive Romberg's sign) "Steppage gait" - patient lifts foot high and than slap it heavily in order to produce sound of touching the ground Gait is wide-based and the tandem gait is affected as well Vertigo and nystagmus are not seen but depressed ankle reflexes CErebellar ataxia: lesions in cerebellum or its afferent or effort connections the cerebellar peduncles, red nuclei, pons or spinal cord. irregularities in the rate, rhythm,amplitude and force of voluntary movements Hypotonia --> rebound movemnet, failure for antagonist muscles to react after resistance of contracted muscle is released incoordination hemispheric lesion: ipsilateral helitaxia, hypotonia, nystagmus and ipsilateral gaze paresis (inability to look voluntarily towrd the affected side)

polymyalgia rheumatica

inflammatory myopathies; more common in women than in men regarded as a variant of giant cell arteritis characterized by muscle pain and stiffness especially by neck and girdle. headache,anorexia, weight loss and low-grade fever. erythrocyte sedimentation is increased serum enymes, electromyographyand muscle biopsy are normal tests for diagnostics treatment: a long period but low dosage of corticosteroids ( predisone 10-15 mg/d for a year to avoid complications, like in giant cell arthritis) Giant cell arthritis causes subacute headaches is a systemic vasculitis that affects medium sized and large arteries. characterized pathologically by granulaomatous inflammation (lymphocytes, neutrophils, giant cells) these inflammatory changes may stimulate platelet adhesion

Nystagmus

involuntarry biphasic eye movements Typical nystagmus is jerky nystagmus with one slow (pathological) and one fast (corrective, coming from the brain) component/phase pendular ny; when both phases are of same velocity--> usually result of visual impairment that begins in infancy most often du to vestibular aetiology we determine Ny by its fast component If there is destructive lesion in one vestibular part of the labyrinth than slow phase of nystagmus is directed towards that damaged labyrinth, the body tends to fall on that same side and the fast component of nystagmus is directed towards other healthy ear/labyrinth jerk nystagmus usually increases in amplitude with gaze in direction of fast phase (healthy?)

signs of upper motor lesions

is a lesion of the neural pathway above the anterior horn cell of the spinal cord or motor nuclei of the cranial nerves.upper: 1. weakness or paralysis 2. spasticity 3. increased tendon reflexes 4. extensor plantar (Babinski sign) response 5. loss of superficial abdominal reflexes 6. little if any muscle atrophy localization 1. parasagittal intracranial --> both legs and maybe later arms 2. focal spot of cerebral cortex: may be restricted to contralateral leg (anterior cerebral a) or contralateral face and arm if middle c. a. 3. internal capsule: closely packed fibers--> severe hemiparesis involving contralateral limbs and face ( supplied by the lenticulostriate arteries, which are branches of the M1 segment of the middle cerebral artery.) 4.brainstem lesion: bilateral motor deficit commonlywith accompanying sensory, cranial nerves and disequilibrium. or more limited lesion: cranial nerve- ipsilateral, and contralateral hemiparesis 5. spinal cord: above C5: ipislateral hemiparesis,sparing cranial nerves and face. C5-T1--> ipsilateral arm and leg to some extent( variable) below T1: ipsilateral leg if there is extensive but unilateralcord lesion, the motor deficit is accompanied by ipsilateral impairment of vibration, position sence and contralateral loss of pain ans temperature appretiation --> Brown -sequard syndrome) 6.compressive and other lesions involving anterior horn cells in addition to fiber tracts transversing the cord. a focal lower motor neuron deficit exits at the level of the lesion and an upper motot neuron deficit below it + any associated sensory disturbances

electromyography

is a technique for evaluating & recording the electrical activity produced by skeletal muscles is performed using an instrument called an electromyograph, to produce a record called an electromyogram A motor unit is defined as one anterior horn cell, its axon, and all the muscle fibers innervated by that motor neuron Activity at rest: a relaxed muscle shows no spontaneous electrical activity. but various abnormal activity can occur in diseased muscle. fibrillation potentials and positive sharp waves (PSW), reflect muscle fiber irritability . typically found in denervated muscle Conditions associated with PSW and fibrillation potential CHRONIC MUSCLE DISORDERS Inflammation myopathies Muscular dydtrophies Inclusion body myositis Rhabdomyolysis Muscle trauma NEUROGENIC DISORDERS Radiculopathy Axonal peripheral neuropathy Entrapment neuropathies Motor neurone disease--> als, primary ls fasciculation potentials, can be encountered in normal tissue but is characteristic for neuropathic disorders, especially in anterior horn. (ALS) myotonic discharges: jigh frequency charges --< myotonic dystrophy Activity during voluntary movement myopathic d. increased incidence of of small short duration polyphasic motor units. neuropathic d. number of units activated at maximal strength is reduced neuromuscular transmission disorders: variation of individual potential units. nerve conduction The electrical response of a muscle is recorded to stimula- tion of its motor nerve at two or more points along its course (Figure 2-4). This permits conduction velocity to be determined in the fastest-conducting motor fibers between the points of stimulation. Nerve conduction studies can confirm the presence and extent of peripheral nerve damage The goal of Edx studies is to determine whether there is a problem along the peripheral nervous system pathway and if so, where the problem is: Motor nerve cell body (anterior horn cell) - ALS Root - cervical or lumbar radiculopathy Axon - toxic neuropathy Myelin - Guillan Barre syndrome NMJ - MG Muscle - muscular dystrophy

Gaze palsies

lesions in the cortex or brainstem above the levelof oculomotor nuclei may impair conjugate movement of eyes- hemisphereic lesions tonic deviation of bothe eyes towards side of lesion and away from side of hemiparesis midbrain lesions lesion of dorsal midbrain, affect the center responsible for voluntary upward gaze. hence upgaze paralysis. but vertical eye reflex (ex from doll's head ) may still be in intact Pontine lesions lesions at level of pontine gaze center causes disorder of the conjugate horizontal gaze. these unlike hemispheric gaze disorders cause deviation towards side of hemiparesis because the corticobulbar pathways that regulate gaze have crossed but the descending motor pathwys have not. also, gaze palsies from brainstem more often involve abducens nerve

internuclear ophtalmoplegia

lesions of medial longitudinal fasiculus (between midpons and oculomotor nerve) Internuclear ophthalmoparesis (INO), also commonly referred to as internuclear ophthalmoplegia, is a specific gaze abnormality characterized by impaired horizontal eye movements with weak adduction of the affected eye, and abduction nystagmus of the contralateral eye. It is one of the most localizing brainstem syndromes, resulting from a lesion in the medial longitudinal fasciculus INO usually implies intrinsic brainstem disease. bilateral involvement most common cause is multipls sclerosis. uniolateral and older patients usuallyn suggest stroke. MG should be ruled out.

Bell palsy mononeuropathy simplex

mononeuropathy simplex : involvement of a single peripheral nerve. usually entails both sensory and motor deficit. facial weakness of lower motor neuron caused by idiopathic facial nerve involvement. --> Bell palsy Bell's palsy is a paralysis or weakness of the muscles on one side of your face. Damage to the facial nerve that controls muscles on one side of the face causes that side of your face to droop . The nerve damage may also affect your sense of taste and how you make tears and saliva. This condition comes on suddenly, often overnight, and usually gets better on its own within a few weeks. cause is unclear, but happens most often in pregnant women, diabetics, after reactivation of herpes simplex, or zoster or varicella zoster in the geniculate ganglion

Alzheimer disease

the most common cause of dementia, 60-70% ca 15% of population over 65.y. a progressive degenerative disorder caused by a an abnormal deposition /clearence of two proteins, B amyloid and tau protein (neurofibrillary tangles- cytoplasmic) pathogenesis Genetics + environmental neuritic (senile) plaques that are extrecellular deposits of B-amyloid proteins, producing cerebral amyloid antipathy. and tau protein (neurofibrillary tangles- cytoplasmic) risk factors: age, female, APOE4 genotype ( fan history) low education, diabetes, hypertension, fatty diet --> smoking may be preventive due to activating nicotinic receptors?? symptoms Amnesia − memory loss is early and invariable − recent memory loss before remote memory • Aphasia − nominal dysphasia early − both expressive and receptive dysphasia in moderate stages − severely disrupted speech in late phases • Apraxia − functional difficulties, initially instrumental, subsequently basic activities of daily living • Agnosia − difficult to assess, but probably more prevalent than often realised • Behavioural and psychiatric symptoms (BPSD) − depression − psychotic features − personality change − activity disturbance treatment: nothing to reverse, memantine, an NMDA glutamate receptor antagonist cholinergic replacement for symptomatic treatment, like acetylcholinesterase inhibitors like rivastigmine (pseudo -irreversible, greater efficacy) , donezepil (reversible) galantine (competitive reversible) antipsychotic for behavioal disturbances clozapine antidepressants: benzodiazapine

Parkinson disease

the most common variety of parkinsonism: tremor, hypokinesia, rigidity and abnormal gait PD is Progressive neurodegenerative disease. the most common one after AzD. The motor symptoms arise due to loss of dopaminergic neurons in the pars compacta substantia nigre and consequent striatal dopaminergic denervation the non motor signs arise due to affection of there are also Nonmotor signs and symptoms arise due to affection of other parts of the central and autonomic nervous system and some organs Prevalence increases with increasing age (above 60) Prevalence and incidence of PD are higher in men 95% sporadic 5 % genetic but usually a mix a build-up of pathologicaly conformed alpha-synuclein (proteins). is a newer hypothesis (protein aggregation) of the cause of PD Histopathologic markers of PD are Lewy bodies and Lewy neurites (a build-up of pathologicaly conformed alpha-synuclein) classical clinical signs of PD: TRAP Tremor at rest Rigidity Akinesia Postural instability Unilateral : rigidity, tremor and akinesia The most frequent premotor symptoms include: (may occur earlier) Depression Constipation REMBD --> sleep disorders Anosmia (can't smell) dysautonomic symptoms (urinary urgency, constipation) and these signs might even begin years before any signs of motor deficit Risk factors Age Gender Family history Physical and emotional traumas Environmental factors: Heavy metals Well water Agriculture and rural life Manufacture of wood into cellulose Industrial steel alloy Exposure to pesticides and MPTP Smoking and drinking coffee are considered as protective against PD Pathogenesis: Cause of disease: Loss of dopaminergic inervation/input from substantia nigra to basal ganglia which is the reason for the majority of motor signs and symptoms or a new theory being similar to a prion disorder, with a conformational protein. alpha-synucleinopathy diagnosis: predominantly a clinical procedure I step: Bradykinesia + Plus one or more of the following: Tremor at rest Rigidity Postural instability step 2 is to exclude other disorders that may resemble parkinsons, like atypical parkinsons, cerebellar disorders, supernuclear palsy?? step 3 additional signs that point towards parkinsosns, unilateral rigidity, progression, responds well to levodopa therapy: symptomatic. drugs for PD achieve their beneficial effects by correcting a deficit of dopaminergic innervation in the CNS levodopa --> stepwise follow degeneration, may later cause dyskinesia , wearing off and on-off phenomena dopamine agonist: pramiprexole, good early treatment COMPT- inhib, enhance bioavailability (entacapone) blocks levodopa turning into methyldopa ( has similar effects as carbidopa) the best bioavailability is when used together. goldenstandard but put of as treatment for as long as possible ( start low and go slow) MAO-B inhibitors: selegline and rasagline, mild on side effects and can postpone the use of levodopa anticholinergics: biperidene ( not for the elderly due to strong side effects) only really helps the tremor and not hypokinesia like L-dopa. propranolol for tremor options in late phase when levodopa can't prevent motor complications? --> transplantation of fetal cells,

malignat hyperthermia

thought to result from abnormal excitation-contraction coupling in skeletal muscle. symptoms are usually by administration of neuromuscular blocking agentsor inhalation anesthetics. clinical features: rigidity, hyperthermia, metabolic acidosis, myoglobinuria. mrotaklity rate is high 70% treatmnent: promt cessation of anesthesia and admin of dantrolene (excitation-contraction uncoupler), reduction of body temp and correction of acidosis. impo to distinghuish from neuroleptic malignat syndrome a life-threatening idiosyncratic reaction to antipsychotic drugs characterized by fever, altered mental status, muscle rigidity, and autonomic dysfunction NMS: rigidity, fever, altered mental status and autonomic dysfunction

restless leg syndrome

unpleasant creeping discomfort sensation in the legsoccurs when patient Is relaxed. at night--> delay onset of sleep U - urge to move R - rest worsens G - getting up improves E - evening The etiology is unknown, but it is considered that symptoms occur due to the lack of dopaminergic inhibition of spinal reflex mechanisms It may be a primary disorder (AD inheritance) Secondary: Anemia PD PNP (eg, renal) hyperthyroidism symptoms may resolve after correction of anemia or may respond to treatment like dopamine agonist, levodopa- carbidopa,(golden standard) pregabalin and gabapentin (dopa agonistsI ., oxycodone (opioid) Periodic Limb Movements of Sleep Can occur together with RLS, or as isolated syndrome While the RLS is a clinical diagnosis, PLMS is diagnosed by polysomnography Clinical features: spasmodic jerks in the hip, knee and ankle during non-REM sleep Usually the patient is not aware of them but this condition can lead to excessive daytime sleepiness due to poor quality of sleep Th.: clonazepam or a dopamine agonist

electroencephalography

useful in evaluating epilepsy often not possible to obtain an EEg during a seizure, but there stzill my be abnormal EEG findings interictally (when patient is not experiencing a clinical attack) the interictal prescence of epileptiform activity: abnormal paroxysmal activity containing spike discharges. but this can alos be found in patients who have never had a seizure and does not mean that the patient has epilepsy or ever needs to take anticonvulsants. EEg may help to classify type of epilepsy and hence help with drug of choice to patient.