Ramen and Redbulls: Neuropath

An 82-year-old man is found unresponsive by his neighbor. The patient lives alone in his suburban home. When the neighbor went to check on him, the patient was on the floor and not answering any questions. His medical history is unknown. On arrival of emergency medical services, the patient is obtunded and responds only to painful stimuli. Supportive measures are begun, including endotracheal intubation, but the patient dies en route to the hospital. Autopsy reveals extensive atherosclerotic disease involving the coronary and internal carotid arteries. Histopathologic examination of the brain in the right middle cerebral artery territory shows neurons with intensely eosinophilic cytoplasm and nuclear fragmentation. Based on these findings, this patient's cerebral injury most likely occurred approximately how long ago? A. Less than an hour B. 12-24 hours C. 1-2 weeks D. 2 months E. 2 years

12-24 hours when it comes to hypoxic changes 1-24 hours: pyknosis, microvacuolization, eosinophilic cells 2 days- 2 weeks: macrophages, necrosis, vascular proliferation, gliosis more than 2 weeks: glial scar, removal of necrotic tissue

A 42-year-old man has a 2-year history of increasing progressive muscle weakness in both arms and legs and dysarthria and difficulty swallowing. He is now wheelchair-bound. Physical examination shows 3/5 motor strength in all extremities. He has no muscle pain on palpation, no deformities or loss of joint mobility, and no tremor. There is no sensory loss. Family history is significant for a similar condition in first-degree relatives. A biopsy specimen of the quadriceps muscle shows atrophy of the myofibers but no inflammation. Molecular testing reveals a mutation affecting the gene encoding superoxide dismutase. Which of the following is the most likely diagnosis? Amyotrophic lateral sclerosis Becker muscular dystrophy Guillain-Barré syndrome Myasthenia gravis Lambert-Eaton syndrome

ALS

A 40-year-old man is brought to the emergency department after sustaining multiple lacerations during a bar fight. The patient's wife says that he has been showing increasing aggression and has been involved in a lot of arguments and fights over the past 2 years. He has no significant past medical or psychiatric history and currently takes no medications. He cannot provide any relevant family history since he was adopted as an infant. Vital signs are within normal limits. On physical examination, the patient looks apathetic and grimaces repeatedly. Suddenly, his arms start to swing by his side in an uncontrolled manner. Which area of the brain is most likely affected by this patient's disease process? A. A. Caudate nucleus B. B. Cerebellum C. C. Cerebral cortex D. D. Medulla oblongata E. E. Substantia nigra

ATROPHY of caudate agression, grimacing, chorea--> huntingtons

A 25-year-old man presents with a 5-month history of headaches. During the last two weeks, he has had 4 generalized seizures. On physical examination, he has no papilledema or movement disorder. CT scan of the head shows a 2-cm mass in the right frontal lobe. A stereotactic biopsy specimen of this lesion shows only gliosis and evidence of recent and remote hemorrhage. The mass is removed, and histologic examination shows a conglomerate of various-sized tortuous vessels surrounded by gliosis. Which of the following is the most likely diagnosis? Ruptured saccular aneurysm Arteriovenous malformation Multiple sclerosis plaque Organizing abscess Prior head trauma

AVM histology is showing a "bag of worms" appearance--> indicative of AVM

An 86-year-old man has become progressively unable to live independently for the past 10 years and now requires assistance with activities of daily living. On physical examination, he has no motor or sensory deficits. He cannot give the current date or state where he is. Six months later, he develops pneumonia and dies of respiratory failure. At autopsy, histologic examination of the brain shows numerous neocortical neuritic plaques and neurofibrillary tangles. Which of the following mechanisms is most likely responsible for his disease? Aggregation of Aβ peptide Conformational change in the prion protein (PrP) Dopamine deficiency Expansion of polyglutamine repeats Mutations in the gene that encodes tau

Aggregation of Aβ peptide sensory and motor intact, old age, loss of orientation neuritic plaque and neurofibrillary triangles--> Alzeimers

A 30-year-old woman comes to the physician because of increasing weakness in both legs for 7 days. She also reports a tingling sensation in her legs that is exacerbated by taking a hot shower. She is sexually active with one male partner and does not use condoms. Her temperature is 37.8°C (100.1°F). Physical examination shows decreased muscle strength and clonus in both lower extremities. Patellar reflex is 4+ bilaterally and plantar reflex shows an extensor response on both sides. Abdominal reflex is absent. Which of the following findings is most likely to further support the diagnosis in this patient? A. Positive antibody response to cardiolipin-cholesterol-lecithin antigen in the serum B. Oligodendrocytes that stain positive for polyomavirus proteins on brain biopsy C. Presence of immunoglobulin bands on cerebrospinal fluid immunofixation D. Identification of gram-positive cocci in groups on blood culture E. Detection of high-affinity antibodies to dsDNA in the serum

C. Presence of immunoglobulin bands on cerebrospinal fluid immunofixation weakness, tingling sensation, decreased muscle strength and some reflexes, WORSE WITH HOT TEMPERATURE--> multiple sclerosis Diagnose: High protein in CSF, oligoclonal bands in CSF

A 58-year-old homeless man is brought to the emergency department after being found unresponsive on a sidewalk. The patient has been hospitalized on several occasions for alcohol and illicit drug intoxication. He also has a history of hypertension but is not adherent with medical therapy. On arrival, the patient is obtunded, febrile, and hypotensive. Physical examination reveals lung crackles and a new blowing diastolic murmur consistent with aortic regurgitation. Appropriate treatment is started, but the patient dies several days later. Autopsy examination shows symmetric, bilateral wedge-shaped strips of necrosis over the cerebral convexity, parallel and a few centimeters lateral to the interhemispheric cerebral fissure. Which of the following is the most likely cause of this patient's cerebral findings? A. Amyloid deposition in cerebral arteries B. Cerebral embolization of heart valve vegetations C. Generalized reduction of cerebral perfusion D. Hypertension-induced microaneurysm formation E. Localized weakness and rupture of arterial walls

C. Generalized reduction of cerebral perfusion Wedge shaped necrosis--> Watershed infarct Most common cause of watershed infarct: systemic hypotension from reduced perfusion

A 59-year-old woman comes to the physician because of progressively worsening coordination and involuntary movements in her left hand for the past 6 months. Her husband also reports that she has been withdrawn and apathetic during this period. She is oriented to time, place, and person. Examination shows a bimanual, rhythmic, low-frequency tremor that is more prominent in the left hand. There is normal range of motion in the arms and legs; active movements are very slow. Muscle strength is normal, and there is increased resistance to passive flexion and extension in the limbs. She walks with a shuffling gait and takes small steps. Which of the following is the most likely underlying cause of this patient's symptoms? A. Accumulation of neurotoxic metabolites secondary to hepatocyte damage B. Copper accumulation due to mutations in hepatocyte copper-transporting ATPase C. Neuronal degeneration due to α-synuclein protein misfolding D. Cerebellar ischemia due to chronic hypertension E. Proliferation of beta-adrenergic receptors from excessive circulating T4

C. Neuronal degeneration due to α-synuclein protein misfolding TREMOR, SLOW ACTIVE MOVEMENTS, SHUFFLING GAIT, SMALL STEPS--> PARKINSONS parkinsons associated with abnormal synuclein

A 75-year-old man is brought to the emergency department due to problems with vision and right-sided hemisensory loss that started an hour ago. While in the emergency department, his symptoms gradually worsen and he develops a headache. Head CT reveals multiple, small lobar hemorrhages of varying ages in the occipital and parietal areas with a medium-size acute bleed in the left parietooccipital lobe. Two years ago, the patient developed sudden right arm weakness; neuroimaging at that time demonstrated a small left frontal lobe hemorrhage. He has no head trauma and does not use anticoagulants. This patient most likely suffers from which of the following? A. Arteriovenous malformation B. Cardiac embolism C. Carotid artery atherosclerosis D. Cerebral amyloid angiopathy E. Charcot-Bouchard aneurysm F. Hypertensive encephalopathy G. Hypoxic encephalopathy H. Saccular aneurysm

D. Cerebral amyloid angiopathy This elderly patient likely has recurrent lobar hemorrhage due to cerebral amyloid angiopathy. Amyloid angiopathy is a consequence of β-amyloid deposition in the walls of small- to medium-sized cerebral arteries, resulting in vessel wall weakening and predisposition to rupture. The disease is not associated with systemic amyloidoses; rather, the amyloidogenic proteins are usually the same as those seen in Alzheimer disease. Amyloid angiopathy is the most common cause of spontaneous lobar hemorrhage, particularly in adults age >60. Hemorrhage tends to be recurrent and most often involves the occipital and parietal lobes. Occipital lobe hemorrhage is typically associated with homonymous hemianopsia; parietal hemorrhages can cause contralateral hemisensory loss. Frontal lobe hemorrhage is less common but may result in contralateral hemiparesis.

A 74-year-old woman comes to the office due to progressive forgetfulness over the past 2 years. Her family states that the patient asks the same questions repeatedly over the course of a day and often forgets what she is doing in the middle of chores. The patient has also been more irritable but has had no movement or gait abnormalities, feelings of depression, hallucinations, or delusions. Medical conditions include hypertension and renal artery stenosis. Vital signs are within normal limits. Mini-Mental State Examination score is 21 out of 30 and she recalls 1 out of 3 objects after 5 minutes. There are no focal neurologic deficits. Which of the following is most likely involved in the pathogenesis of this patient's condition? A. Alpha-synuclein protein aggregation B. CD4+ T lymphocytes activated by myelin basic protein C. Inflammatory vasculitis affecting the arterioles D. Microtubule-associated protein polymerization E. Perivascular infiltration of virally infected macrophages

D. Microtubule-associated protein polymerizatio Patient has alzeimers.

A 54-year-old hospitalized man develops new-onset slurring of speech and extremity weakness. Medical history is significant for alcohol abuse. Physical examination reveals confusion, dysarthria, dysphagia, and quadriparesis. MRI of the brain shows symmetric areas of demyelination in the pons. Which of the following most likely predisposed this patient to his current condition? A. Atheroma formation in the vertebrobasilar arteries B. Hematogenous dissemination of microbial agents C. Nutritional deficiency of water-soluble vitamin D. Overly rapid correction of electrolyte abnormalities E. Paraneoplastic production of anti-neuronal antibodies

D. Overly rapid correction of electrolyte abnormalities

A 64-year-old woman is evaluated for progressive gait disturbance over the last 2 years. The patient feels her feet are "stuck on the floor" and that she is unsteady while walking. She also recently began experiencing urinary urgency and has wet herself several times due to difficulty getting to the bathroom on time. The patient stopped working 6 months ago because of difficulty concentrating, and she has also lost interest in her hobbies and social activities. She has had no tremor, visual changes, headaches, nausea, or vomiting. The patient has a remote history of closed traumatic brain injury. Vital signs are within normal limits. On physical examination, lower extremity tone and deep tendon reflexes are increased. Sensation to light touch, pain, and vibration are normal. The patient's gait is slow with small steps and a wide base. Which of the following is the most likely diagnosis? A. Alzheimer disease B. Frontotemporal dementia C. Idiopathic Parkinson disease D. Major depressive disorder E. Normal pressure hydrocephalus F. Obstructive hydrocephalus G. Vitamin B12 deficiency

E. Normal pressure hydrocephalus Wet, wobbly, wacky.

62-year-old woman comes to the office due to tingling and numbness in her feet over the last several months. The patient has also noticed a small ulcer on her right foot that "doesn't seem to be healing." Medical history is significant for long-term diabetes, hypertension, and gout. Physical examination shows decreased pain sensation over both feet and bilateral absence of ankle reflexes. This patient's neurological symptoms are most likely associated with which of the following features? A. Endoneurial arteriole hyalinization B. Endoneurial inflammatory infiltration C. Mutation of a myelin protein gene D. Nerve compression within an anatomic compartment E. Poor signal transmission at the neuromuscular junction

Endoneurial arteriole hyalization - long term diabetes--> accumulation of sorbitol and glycosylation end products--> hyalinization and narrowing of endoneurial arterioles

A 34-year-old woman comes to the office due to diplopia, clumsiness of the left hand, and numbness of the right foot. Six months ago, the patient had numbness of the right hand, which resolved without treatment. Ten years ago, she was hurt by an explosion at work that resulted in a metallic foreign body in her left eye. She has no other medical problems and takes no medications. The patient has a 10-pack-year smoking history. Both parents are healthy. Vital signs are normal. BMI is 27 kg/m2. Neurologic examination shows slowing of left eye adduction and left arm power of 4/5 with mild spasticity. Sensation to pinprick is decreased over the right foot. Cerebrospinal fluid analysis shows mononuclear cell pleocytosis and oligoclonal banding. Which of the following pathologic findings is most likely to be present in this patient's brain? A. Astrocyte shrinkage B. Eosinophilic inclusions in neurons C. Extracellular fibrillar protein accumulation D. Inflammatory vasculitis E. Local hemorrhage F. Oligodendrocyte depletion

F. oligodendrocyte depletion CSF shows oligoclonal bands--> diagnostic of MS Oligodendrocytes mylenate CNS

A 43-year-old man with a history of HIV infection who is noncompliant with highly active antiretroviral therapy comes to the office due to progressive memory loss and cognitive decline over the last several years. The patient has problems with attention, concentration, and speech. In the past few months, he has become more apathetic and increasingly lethargic. More recently, he has had difficulty with balance and has fallen on multiple occasions. The patient has had no headache, tremor, change in vision, fever, nausea/vomiting, or urinary incontinence. His brother has a history of severe intellectual disability and epilepsy. Mini-Mental State Examination score is 18/30, and the patient appears emotionally blunted. He also has postural instability and difficulty performing fine motor tasks. Noncontrast head CT scan reveals diffuse cortical atrophy with some enlargement of the ventricles, as shown in the exhibits. This patient's condition can be best described as which of the following? A. Communicating hydrocephalus B. Hydrocephalus ex-vacuo C. Lissencephaly D. Noncommunicating hydrocephalus E. Pseudotumor cerebri F. Transtentorial (uncal) herniation

Hydrocephalus Ex vacuo Patient has compensatory rise in CSF due to atrophy of brain.

A 45-year-old woman comes to the emergency department due to sudden-onset, severe headache. On arrival, the patient is alert and oriented and has no focal neurological deficits. CT scan of the head reveals subarachnoid hemorrhage, and she is admitted to the critical care unit. Over the next several hours, the patient gradually becomes somnolent and less responsive. She opens her eyes to painful stimuli only and does not follow commands. Repeat imaging of the brain shows no new hemorrhage, but there is enlargement of the entire ventricular system compared to the previous CT scan. Which of the following is the most likely cause of this patient's neurologic deterioration in the hospital? A. Blockage of the cerebral aqueduct by blood products B. Cerebral vasospasm due to subarachnoid blood C. Edema from a disruption in the blood-brain barrier D. Excessive production of cerebrospinal fluid E. Impaired absorption of cerebrospinal fluid

Impaired absorption of cerebrospinal fluid patient is experiencing communicating hydrocephalus - mechanism: impaired CSF absorption other complications: vasospasm (occurs 4-14 days after, not deterioration) rebleed

A 26-year-old man is brought to the emergency department after a high-speed motorcycle collision. The patient was thrown several feet after his motorcycle collided with a car. On arrival, he is profoundly comatose. A CT scan of the head performed within an hour of the event shows no abnormalities. The patient dies several hours later, and autopsy examination is performed. Brain histopathology shows widespread axonal swelling, predominantly at the gray-white junction. Immunohistochemical staining of these axons reveals accumulation of alpha-synuclein and amyloid precursor proteins. Which of the following is the most likely cause of these observed findings? A. Diffuse cerebral hypoperfusion B. Disruption of the bridging cortical veins C. Glutamate-mediated neuronal injury D. Interruption of the white matter tracts E. Secondary microvascular spasm

Interruption of white matter tracts Motor collision, comatose state and eventual death. CT comes back clear. Axonal swelling

A 59-year-old woman had sudden loss of consciousness 4 months ago. On physical examination, she now has left hemiplegia. CT imaging shows a large, cystic space in the right parietal region. MR angiography shows occlusion of a peripheral cerebral artery branch at the gray-white junction near the lesion. Which of the following underlying disease processes is this woman most likely to have? AIDS with a low CD4+ T-lymphocyte count Chronic alcoholism Chronic renal failure with hypertension Colonic adenocarcinoma with migratory thrombophlebitis Ischemic heart disease with left ventricular mural thrombus

Ischemic heart disease with left ventricular mural thrombus ischemia of the brain--> occlusion most likely due to an embolus

A 34-year-old woman comes to the office due to a 2-day history of reduced sensation in her legs. Lately, the patient has also had significant fatigue, especially with exercise. Six months ago, she had an episode of visual blurring that resolved spontaneously after a few days. Medical history is significant for psoriasis, for which she uses a topical glucocorticoid ointment. The patient does not use tobacco or illicit drugs. Vital signs are normal. Examination indicates decreased pain and light touch perception in both legs below the level of the umbilicus. Mild bilateral spasticity is noted. Which of the following pathologic brain lesions is most specific for this patient's disease process? A. Microglial nodules B. Neuritic plaques and neurofibrillary tangles C. Periventrular plaques D. Spongiform changes E. Viral inclusions

Periventricular plaques

A 66-year-old man undergoes a coronary artery bypass grafting. Upon regaining consciousness, he reports that he cannot see from either eye and cannot move his arms. Physical examination shows bilaterally equal, reactive pupils. A fundoscopy shows no abnormalities. An MRI of the brain shows wedge-shaped cortical infarcts in both occipital lobes. Which of the following is the most likely cause of this patient's current symptoms? A. Amyloid angiopathy B. Ruptured saccular aneurysm C. Lipohyalinosis D. Cardiac embolism E. Atherothrombosis F. Systemic hypotension

Systemic hypotension Cardiac surgery, cortical infarcts, inability to see--> points to a water-shed infarct. Most common cause is systemic hypotension

A 68-year-old woman with a 7-year history of progressive dementia dies of bronchopneumonia. At autopsy, there is cerebral atrophy in a predominantly frontal and parietal lobe distribution. Microscopic examination of the brain shows numerous neuritic plaques in the hippocampus, amygdala, and neocortex. Neurofibrillary tangles in the hippocampus contain tau protein. Which of the following mechanisms is the most important factor in the development of her disease? Expansion of glutamine encoding CAG repeats Inheritance of HLA-DR3/DR4 alleles Increased tandem repeats in the FMR1 gene Mutation of a prion protein gene Self-aggregation of Aβ peptide

Self-aggregation of Aβ peptide dementia, cerebral atrophy, neuritic plaques, neurofibrillary tangles in hippocampus, tau protein--> alzeimers

A 55-year-old woman is brought to the office by her husband for evaluation of strange behavior. She has been spending large amounts of money to buy expensive clothes and jewelry. The patient has also offered sex to unknown men in the neighborhood and has become increasingly indifferent toward her family, about whom she used to care deeply. She usually drinks 1 or 2 glasses of wine on weekends but lately has been drinking 2 or 3 glasses almost every day. When asked about these changes during the interview, the patient states that she has not noticed any change in her behavior. Vital signs are normal. The general physical and neurologic examinations are normal. This patient's condition is most likely due to abnormal accumulation of which of the following? A. Ataxin B. Beta-amyloid C. Polyglutamine D. Prions E. Ubiquitinated TDP-43

Ubiquitinated TDP-43 weird behavior, loss of impulse control. signs of frontal temporal degeneration

A 65-year-old woman is brought to the clinic by her husband due to worsening memory problems. She has a medical history significant for long-standing hypertension, type 2 diabetes mellitus, and depression. Physical examination shows cognitive impairment but no focal neurologic deficits. Two years later, the patient dies suddenly from a massive myocardial infarction. Congo red staining of brain samples obtained from the hippocampus and cerebral arterioles reveals patchy red deposits that turn yellow-green under polarized light. This patient most likely suffered from which of the following conditions? A. Alzheimer disease B. Huntington disease C. Parkinson disease D. Pick disease E. Pseudodementia F. Vascular dementia G. Vitamin B12 deficiency

alzeimers explaining the presence of amyloid beta

A 55-year-old man has a 2-month history of chronic headaches. He presents at the Emergency Department following a generalized tonic-clonic seizure. Physical examination reveals left-sided weakness. An MRI of his brain shows a large, irregular, 6-cm mass in the right cerebral hemisphere that extends across the corpus callosum. Histologic examination of a stereotaxic biopsy of the mass reveals pleomorphic cells positive for glial fibrillary acidic protein (GFAP). Molecular analysis shows IDH1 mutations but no 1p/19q deletions. Which of the following neoplasms is he most likely to have? Diffuse large B-cell lymphoma Anaplastic astrocytoma Hemangioblastoma Medulloblastoma Pilocytic astrocytoma

anaplastic astrocytoma mass extends across corpus callosum (butterfly), GFAP--> astrocytoma unlikely pilocytic astrocytoma due to age, and shape/symmetry of tumor

A 54-year-old man is brought to the office by his daughter. She says that her father has been acting strangely over the past 2 years. He makes inappropriate sexual jokes, has little regard for social rules, is often irritable, and is borderline aggressive at times. The patient denies that his personality is any different from normal. When speaking with the patient, the physician notices that he has minimal verbal output and repeats "it is what it is" when asked questions about his strange behavior. On physical examination, he appears unkempt but otherwise has no significant findings. This patient most likely has a condition that predominantly involves which of the following structures? A. Anterior temporal and prefrontal cortices B. Caudate nucleus and putamen C. Locus ceruleus and substantia nigra D. Mamillary bodies and medial thalamus E. Medial temporal and parietal cortices F. Parietal and occipital cortices

anterior temporal and prefrontal cortices strange behavior

A 28-year-old man presents for a physical examination at a new job. His past medical history is significant for (1) paresthesia of his left arm and difficulty walking 3 years ago which subsequently resolved; (2) blurred vision in his left eye for the past year; and (3) difficulty writing with his right hand during the last 6 months. On physical examination, there is decreased visual acuity on the left, no papilledema, and no retinal lesions, decreased motor strength and decreased sensation in the right hand and forearm. MRI of the brain shows focal areas of increased signal intensity in periventricular white matter and in the left optic nerve. Which of the following is the most likely mechanism of this disease? Inherited disorder of myelin synthesis Autoimmune reaction against myelin Viral meningoencephalitis Misfolding of PrPc Schwann cell dysfunction

autoimmune reaction against myelin paresthesia, visual problems, decreases motor strength and sensation--> signs of MS In MS, Th1 and TH17 mediated autoimmune damage against myelin

A 23-year-old, previously healthy man was involved in a motor vehicle collision. He lost consciousness at the scene but recovered by the time emergency medical services arrived. The patient was alert and awake in the ambulance but lost consciousness again soon after arriving at the emergency department. His breath smells of alcohol. Blood pressure is 130/90 mm Hg, pulse is 68/min, and respirations are 12/min. A bruise is noted over the left temple area. The left pupil is mildly dilated. Brain imaging would most likely reveal blood accumulating between which of the following tissues? A. Aponeurosis and outer periosteum B. Arachnoid mater and pia mater C. Bone and dura mater D. Dura mater and arachnoid mater E. Pia mater and brain surface

bone and dura matter loss of consiousness followed by a lucid interval and back to loss of consciousness after a head trauma points to an epidural hematoma.

A 56-year-old man is brought to the emergency department due to progressive weakness and lethargy. The patient has also had a nonproductive cough for the past several weeks. He has no prior medical conditions and takes no medications. He has smoked a pack of cigarettes daily for 30 years. Physical examination shows no abnormalities. Chest x-ray reveals a lung mass. Further evaluation reveals an electrolyte abnormality induced by ectopic antidiuretic hormone production from a lung mass. This patient is at greatest risk of which of the following conditions if his electrolyte abnormality is corrected too quickly? A. Cardiac arrhythmias B. Cerebral demyelination C. Cerebral edema D. Diffuse myonecrosis E. Intravascular hemolysis

cerebral demyelination (central pontine demyelination) can occur due to quick correction of hyponatremia

A 68-year-old woman with a history of atrial fibrillation suddenly loses consciousness and collapses. When she becomes arousable, she is unable to move her left arm and has difficulty speaking. On physical examination, her temperature is 37° C, pulse is 81 bpm, respirations are 18 breaths/min, and blood pressure is 135/85 mm Hg. Despite optimal medical therapy, she does not regain consciousness and the family elects to withdraw care. What most likely led to the state of this patient? Arteriovenous malformation (AVM) Embolic arterial occlusion Metastatic carcinoma Organizing subdural hematoma Cerebral amyloid angiopathy

embolic arterial occlusion Patient had fib-->risk of embolus formation

A 60-year-old man had increasing irritability over 3 years. He wandered about his neighborhood, complaining to the neighbors about everything. He had no memory loss and was able to find his way home. More recently, he developed aphasia. On physical examination, there were no motor or sensory deficits and no gait disturbances or tremor. MRI of the brain showed bilateral marked temporal and frontal lobe gyral atrophy. He died of pneumonia 1 year later. At autopsy, the frontal cortex microscopically shows extensive neuronal loss, and some remaining neurons show intracytoplasmic inclusions that stain immunohistochemically for tau protein. Which of the following is the most likely diagnosis? Alzheimer disease Huntington disease Frontotemporal lobe degeneration Parkinson disease Prion disease

frontotemporal lobe degeneration odd behavior, aphasia, no motor or sensory loss, tau inclusions--> FTDG

A 63-year-old man collapses at home after experiencing severe retrosternal chest pain. The emergency medical team finds him with no pulse, and cardiac monitoring shows ventricular fibrillation. Cardiopulmonary resuscitation is performed, and the patient regains spontaneous circulation after several minutes. On arrival at the hospital, he has stable vital signs but does not follow commands or respond to strong stimuli. Which of the following areas is most likely to be affected first by ischemic damage in this patient? A. Caudate nucleus B. Hippocampus C. Hypothalamus D. Substantia nigra E. Thalamic nuclei

hippocampus patient is experiencing global ischemia secondary to myocardial infection. most notable areas affected: hippocampus, neocortex, purkinje fibers of cerebellum, watershed areas

A 45-year-old man comes to the clinic due to a 2-month history of progressive left arm clumsiness and weakness. The patient initially attributed his symptoms to a left rotator cuff injury he sustained several years ago; however, he has recently developed problems with his gait. The patient's symptoms have interfered with daily activities such as bathing and dressing. He has had no recent injury, headaches, or bowel/bladder symptoms. On physical examination, there is reduced muscle strength in the left upper extremity. Further evaluation with brain MRI reveals a lesion involving the corticospinal tract. Which of the following additional signs is most likely to been seen in this patient? A. Hyperactive deep tendon reflexes B. Muscle atrophy C. Muscle fasciculation D. Muscle hypotonia E. Resting tremor F. Sensory loss in the affected area

hyperactive tendon reflexes key point: distinguish between upper and motor neuron lesion. give away: brain MRI. upper motor neurons are found in the brain, low motor neurons are found in the spinal cord. if there was some type of trauma involving his spinal cord, and the doctors detected a lesion within a lumbar or thoracic MRI, this would point to LMN lesion.

A 5-year-old Caucasian boy is brought to the physician's office with recent-onset gait instability and gait ataxia. MRI of the brain shows a midline posterior fossa mass. Biopsy of the mass reveals sheets of primitive cells and many mitotic figures. This patient most likely suffers from which of the following? A. Pilocytic astrocytoma B. Glioblastoma C. Oligodendroglioma D. Ependymoma E. Medulloblastoma F. Primary CNS lymphoma G. Meningioma H. Craniopharyngioma I. Schwannoma J. Pituitary adenoma

medulloblastoma child with gait and ataxia points towards a cerebellar tumor of the posterior fossa (consistent with a medulloblastoma) microscope used to determine between medulloblastoma and astrocytoma medulloblastoma: primitive sheet cells, homer wright rosettes, small blue cells astroctyoma: rosenthal eosinophilic rods, hair like projections

A 45-year-old woman has a 5-month history of right-sided unilateral headaches. Physical examination is unremarkable. CT scan of the brain reveals a parasagittal dural mass that compresses the underlying brain. A representative gross appearance of the lesion is shown in the figure. The mass is surgically removed and microscopic examination shows elongated cells with pale, oblong nuclei and pink cytoplasm with occasional psammoma bodies. Cytogenetic analysis shows 22q- with loss of NF2 gene. Which of the following is the most likely diagnosis? Astrocytoma Ependymoma Meningioma Metastasis Tuberculoma

meningioma dural mass, psamomma bodies, NF2 association

An autopsy is requested for a 75-year-old man who died in the ICU after 3 days in a coma following a cerebrovascular accident (stroke) involving the right middle cerebral artery (MCA), confirmed by CT scan. Clinical history is significant for multiple transient attacks of CNS ischemia during the previous 2 years. Microscopic examination of sections in the ischemic area reveals a population of cells with intensely eosinophilic cytoplasm. Which of the following cell types do these eosinophilic cells most likely represent? Neurons Astrocytes Ependymal cells Microglia Oligodendroglia

neurons

A 35-year-old woman comes to the office due to trouble seeing out of her right eye. The patient started having blurry vision and pain with eye movement 2 days ago. She has no headache, vertigo, slurred speech, numbness, or weakness. Two years ago, the patient had an episode of double vision that spontaneously resolved after a few weeks. On physical examination, she has an afferent pupillary defect with markedly reduced visual acuity on the right. Funduscopy reveals blurring of the optic disk margins in the right eye. MRI of the brain reveals multiple, ovoid white matter lesions with perpendicular orientation to the lateral ventricles. This patient's condition is most likely caused by an inappropriate immune response directed against which of the following cells? A. Astrocytes B. Ependymal cells C. Microglial cells D. Oligodendrocytes E. Schwann cells

oligodendrocytes symptoms of MS + white matter lesions perpendicular to lateral ventricles

A 10-year-old boy has had persistent headaches for the past 3 months. He is afebrile and displays an ataxic gait. CT scan of the head shows a 4-cm cystic mass in the right cerebellar hemisphere. The CSF protein concentration is elevated but the glucose level is normal. Neurosurgery is performed, and the mass is removed and sectioned. On gross examination, the mass is a cyst filled with gelatinous material. The cyst has a thin wall and a 1-cm mural nodule. Microscopically, the mass is composed of cells that stain positive for glial fibrillary acidic protein (GFAP) and have long, hair like processes. BRAF mutations are present, but no IDH mutation is identified. Which of the following is the most likely diagnosis? Pilocytic astrocytoma Ependymoma Hemangioblastoma Medulloblastoma Meningioma

pilocytic astrocytoma age, cerebellum location, GFAP, hair like processes

A 17-year-old right-handed boy is brought to the emergency department due to brief loss of consciousness after colliding with another player during a football game. The patient reports "fogginess" but has had no headache, vomiting, focal weakness or numbness, or seizures. He has no other medical conditions and takes no medications. Vital signs are within normal limits. On physical examination, the patient demonstrates retrograde amnesia about the game but no focal neurologic deficits. CT angiography of the head reveals no skull fractures or intracranial bleeding, but a small lesion in the left temporoparietal region composed of abnormal vascular channels is noted and is shown in the exhibit. Without treatment of this abnormality, which of the following will most likely occur in this patient? A. Brain abscess B. Flaccid paralysis C. Malignant transformation D. Spontaneous hemorrhage E. Spontaneous resolution

spontaneous hemorhage abnormal vascular channels in left temporal region--> bag of worms appearance AVM: connection of arteries to veins without a capillary network. Risk of hemorrhaging, a type of nonhypertensive-hemorrhage that can occur in the brain

A 45-year-old previously healthy man has developed headaches over the past month. There are no remarkable findings on physical examination. A cerebral MR angiogram shows a 7-mm saccular aneurysm at the trifurcation of the right middle cerebral artery. Which of the following is the most likely complication from this lesion? Cerebellar tonsillar herniation Hydrocephalus Epidural hematoma Subarachnoid hemorrhage Subdural hematoma

subarachnoid hemorrhage most common cause of subarachnoid hemorrhage is a berry aneurysm

A 55-year-old man has had increasing difficulty with initiation of voluntary movements and increasing inability to perform activities of daily living for 1 year. On physical examination, he has tremors at rest and rigidity of the limbs. The right side of the figure shows the gross appearance of the midbrain of this patient; on the left is a section through normal midbrain (picture shows dipigmentation of substantia nigra) Pathologic effects of this disease are most likely related to which of the following inclusions in the nucleus? Huntingtin Frataxin α-Synuclein Tau Aβ amyloid

synuclein rigidity, tremors, dipigmentation of SN--> Parkinsons Parkinsons is associated with synuclein inclusions

Question 16 A 55-year-old man suddenly loses consciousness while driving at slow speed and comes to a stop without a collision. Paramedics arrive but are unable to arouse him. On physical examination, there is bilateral papilledema. He has no spontaneous movements. The figure shows the gross appearance of the brain at autopsy (picture shows brain with a large area of blood. Which of the following underlying conditions is most likely to have caused this lesion? Vitamin B1 deficiency Saccular aneurysms Multiple sclerosis (MS) Systemic hypertension Thromboembolism

systemic hypertension No trauma, papilledema is most likely due to increased intracranial pressure--> indicating a possible hemorrhage--> non, traumatic cause is usually hypertension

An 80-year-old nursing home resident is admitted to the hospital due to the recent onset of fluctuating levels of consciousness with headache and confusion. On physical examination, she is arousable, but disoriented and irritable. Vital signs include temperature of 36.9° C and blood pressure of 130/85 mm Hg. There is papilledema of the right eye. CT scan of the head shows a collection of blood in the brain that crosses the suture lines. Which of the following vascular lesions most likely produced these findings? Bleeding from an arteriovenous malformation (AVM) Laceration of the middle meningeal artery Rupture of a saccular aneurysm Tearing of the cerebral bridging veins Thrombosis of the middle cerebral artery

tearing of cerebral bridging veins crosses suture lines--> subdural bleed--> bridging veins

A 49-year-old woman has a 2-day history of a severe headache. On physical examination, she is afebrile and normotensive. Funduscopic examination shows papilledema in the right eye. One day later, she has right pupillary dilation and impaired ocular movement. She then becomes obtunded. Which of the following lesions best explains these findings? Chronic subdural hematoma Temporal lobe herniation Noncommunicating hydrocephalus Hydrocephalus ex vacuo Ruptured middle cerebral berry aneurysm

temporal lobe (transtentorial) herniation

A 35-year-old man is brought to the emergency room after suffering a catastrophic fall while skiing during a training session. He was found unconscious by the aeromedical emergency services team, who established an airway and cervical spine control measures. Upon arrival at the emergency department, ventilatory support is started, followed by an evaluation of the patient by the neurosurgical team. His blood pressure is 210/125 mm Hg, the heart rate is 55/min, and the respiratory rate is 15/min with a Cheyne-Stokes breathing pattern. Neurological examination shows a Glasgow Coma Score of 4/15, with dilated, fixed pupils, and a decerebrate posture. An MRI of the brain shows diffuse axonal injury with severe cerebral edema, multiple frontal and occipital lobe contusions, and multiple flame-shaped hemorrhages in the brainstem. Despite the medical team's efforts, the patient dies 24 hours later. What would be the most likely type of herniation found at this patient's autopsy? A. A. Uncal herniation B. B. Subfalcine herniation C. C. transtentorial herniation D. D. Downward cerebellar herniation E. E. Upward cerebellar herniation

transtentorial herniation key word: flame shaped (duret) hemorrhages

A 46-year-old woman comes to the emergency department due to sudden-onset, severe occipital headache and nausea. The patient has a history of hypertension and cigarette smoking. Physical examination shows mild disorientation but no focal weakness, sensory loss, or cranial nerve abnormalities. Noncontrast CT scan of the head reveals diffuse hyperdensity in the subarachnoid space. CT angiography reveals a ruptured anterior communicating artery aneurysm. The patient undergoes endovascular coiling of the aneurysm. Four days later, there is sudden deterioration in her level of consciousness and new, right-sided hemiparesis is observed. Repeat CT scan shows no significant changes. Which of the following is the most likely cause of this patient's neurological deterioration? A. Arterial thrombosis B. Embolism C. Hydrocephalus D. Rebleeding E. Vascular inflammation F. Vasospasm

vasospam Vasospasm is most common complication arising from subarachnoid hemorrhage occurring 3-14 days after onset

A 27-year-old woman had an episode of weakness 3 months ago, which she attributed to job stress and fatigue. The neurologic examination shows mild residual weakness, with 4/5 motor strength in the right lower extremity. Laboratory examination of the CSF from a lumbar puncture shows increased IgG levels with prominent oligoclonal bands. MRI of the brain shows small, scattered, 0.5-cm areas consistent with demyelination, most of which are located in periventricular white matter. Which of the following complications is she most likely to develop? Non-Hodgkin lymphoma Progressive dementia Visual impairment Seizure disorder Tremor at rest

visual impairment weakness, fatigue, decreased motor strength, oligoclonal bands in CSF, demyelination--> MS It is common for MS to cause visual problems