Neuromuscular Function I

Myasthenia gravis is characterized by muscle weakness caused by antibody-mediated destruction of: Acetylcholine receptors Skeletal muscle fibers Thymus gland cells Periorbital muscles

Acetylcholine receptors Rationalization: MG is an autoimmune disease caused by the loss of functioning acetylcholine (Ach) receptors in the neuromuscular junction. The end result of this process is inefficient neuromuscular transmission, which is manifested as muscle weakness and easy fatigability. Ach is needed to stimulate muscle.

Characteristics of autonomic dysreflexia include which of the following? Pick all that apply. Spinal Cord Injury below Lumbar 5 Hypotension Exaggerated sympathetic response Headache Dangerously high blood pressure

Exaggerated sympathetic response Headache Dangerously high blood pressure Rationalization: For autonomic dysreflexia... Spinal cord injury is in the upper spinal area, above t6. It causes hypertension.

In patients diagnosed with Guillain Barre Syndrome you will see the manifestation of their symptoms starting in the: Lower extremities Face Trunk Speech

Lower extremities Rationalization: Paresthesias and numbness are usually the first symptoms. The weakness can vary from mild weakness in the lower limbs, causing difficulty walking, to nearly complete paralysis of all extremities and respiratory muscles. Weakness begins in the lower extremities and then spreads to the upper body.

since acetylcholine receptor activates muscle contraction....

MG doesn't have that activation - receptor site is destroyed. muscle with no depolarization will not contract

MG: MS:

MG: levator muscle MS: optic nerve

what is a demyelinating, autoimmune, inflammatory destruction of the CNS myelin?

MS

Paralysis of the legs/lower extremities A) Quadriplegia B) Paraplegia

Paraplegia Rationalization: Quadriplegia is paralysis of all 4 limbs.

how do you treat MG?

TENSILON patient comes in with droopy eyelid or fatigue. tensilon causes an immediate acetylcholine uptake, but it only lasts for a few seconds/minutes. immediately the patient will have an immediate improvement in symptoms. its an acetylcholine boost so the symptoms resolve. if the patient doesn't have MG, they will have no affect and will not improve

appendicitis - a sharp pain to the belly is a type of pain telling your body something is wrong. this type of pain is PURPOSEFUL

acute pain

what are examples of a partial spinal cord injury?

central cord syndrome anterior cord syndrome brown-seguard syndrome (normally in spinal shock or injury, it will knock out that entire section. this is not the case for partial)

tremor at rest ridigitiy/hypertonia: excess muscle contraction. try to move arm - very rigid bradykinesia/akinesia: slowness of movement postural disturbance: flexed, forward leaning dysphagia: difficulty swallowing speech slurred demetia depression constipation and urinary retention no true paralysis: still have contraction but its rigid bilateral symptoms, but starts in 1 side late cognitive decline

clinical manifestations of parkinson's

relaxation and contraction alternating

clonus

the skin

cutaneous pain

polyneuropathy is damage to many peripheral nerves what is an example of this?

gullain-barre

both limbs on one side

hemi-

one sided weakness

hemiparesis

where are lower motor neurons?

send axons out of the spinal cord

what do action potentials innervate?

skeletal muscles

a nurse moves a patient's bed higher than 30 degrees. this will affect a patient who has:

spinal cord injury

explain ascending motor paralysis

usually have had a viral infection 2-3 before and the immune response goes out of whack and attacks the myelin sheath. it will attack the longest ones first which are at the lower extremities. it goes from the lower extremities and then moves upwards.

stroke or paralysis

-plegia

1. Match the characteristics, manifestations, etc. with the following neurologic alterations: 1. Myasthenia gravis 2. Peripheral mononeuropathies 3. Peripheral polyneuropathies 4. Parkinson disease 5. Amyotrophic lateral sclerosis 6. Multiple sclerosis a. Caused by conditions that affect a single nerve b. Demyelinating disease of the CNS c. Selectively affects motor function d. Autoimmune disorder caused by antibody-mediated destruction of acetylcholine receptors in the neuromuscular junction e. Carpal tunnel syndrome f. Guillain-Barré syndrome g. Tremor, rigidity, bradykinesia h. Tensilon test is done to assist in diagnosis i. Occurs more often in males in middle to late adulthood j. Symptoms depend on site of interrupted neural conduction, but often include optic neuritis, diplopia, numbness, tingling, fatigue k. Death of LMNs → denervation → musculature shrinkage and fiber atrophy l. Occurs most often in females in young adulthood m. Late in the disease, autonomic symptoms may include: orthostatic hypotension, sweating, constipation, impotence n. Muscle weakness, most commonly affected eye and periorbital muscles, and fatigability with sustained effort o. Degenerative disorder of basal ganglia resulting in decreased concentrations of dopamine p. Involves demyelination or axonal degeneration of multiple peripheral nerves

1d, 1h, 1n 2a, 2e 3f, 3p 4g, 4m, 4o 5c, 5i, 5k 6b, 6j, 6l

weakness in any or all muscles of the body paralysis occur with progressive muscle atrophy normal intellectual and sensory functions

ALS

what disorder involves the lower and upper motor neurons with an onset at any time from the fourth decade?

ALS

what disease has the shortest life expectancy after onset of symptoms?

ALS 2-5 year life expectancy

Which of the following neurotransmitters acts directly on the synapses? Acetylcholine Epinephrine Dopamine Beta cells

Acetylcholine Rationalization: Acetylcholine is found within the CNS, PNS, and ANS. It can act as either an excitatory or inhibitory neurotransmitter, depending on what neurons secrete it. For example, within the PNS, it acts as an excitatory neurotransmitter at neuromuscular junctions (synapses). Dopaminergic neurons are mainly located in the substantia nigra of the midbrain's basal ganglia region.

The cardinal symptoms of Parkinson disease include: Paresthesia Lack of sweating Bradykinesia Hypotonia

Bradykinesia Rationalization: Bradykineisa is a state of slowed movements inhibiting independent functioning. Early disease patients may first see the effect in distal muscles of the arms and legs. Patients may have difficulty initiating gait or rising from a chair. It manifests into the upper extremities including decreased arm swing, handwriting, and decreased fine motor function. Other symptoms of Parkinson's includes: tremor, rigidity, postural instability (very quick few steps forward and steps backward rapidly and uncontrollably), and nomotor symptoms (constipation, drooling, sexual dysfunction, sleep disturbances, cognitive changes - dementia, anxiety)

Which of the following disorders of neuromuscular function typically has the most rapid onset? Duchenne muscular dystrophy (DMD) Guillain Barre syndrome Parkinson disease Myasthenia gravis

Guillain Barre syndrome Rationalization: GBS leads to progressive limb weakness over the course of several days up to 4 weeks. Symptoms manifest quickly.

**optic nerve involvement: visual field plaques form on brain - MRI ocular motor nerve cerebellum system crisis and remission paresthesias, fatigue

MS clinical manifestations

Restoration of the integrity of myelin sheaths would likely result in a slowing or stopping of the progression of: Duchenne muscular dystrophy (DMD) Multiple sclerosis (MS) Amyotrophic lateral sclerosis (ALS) Paralysis caused by Clostridium botulinum

Multiple sclerosis (MS)

Review normal anatomy and physiology of nervous system including embryonic development and normal changes that occur with the aging process. Identify and describe physiological processes that are BSN Essentials to "normal" functioning of the nervous system and their relationship: Central nervous system Peripheral nervous system Ventricular system Blood supply Metabolic needs Functions of localized regions Discuss pathophysiological processes and assessment findings for the following acute neurological/cerebral alterations: Increase Intracranial Pressure Head injury Cerebrovascular Accident (CVA) Cerebral Hemorrhage Meningitis Discuss pathophysiological processes and assessment findings for the following chronic neurological/cerebral alterations: Epilepsy Parkinson's disease Multiple Sclerosis/Amyotrophic Lateral Sclerosis Guillian Barre Discuss general treatment/management strategies for acute and chronic neurological/cerebral alterations. Discuss potential complications/sequela (e) of undiagnosed/untreated acute and chronic neurological/cerebral alterations.

Objectives

A patient with a diagnosis of myasthenia gravis has required a mastectomy for the treatment of breast cancer. The surgery has been deemed a success, but the patient has gone into a myasthenic crisis on postoperative day one. Which of the following measures should the care team prioritize in this patient's immediate care? Positioning the patient to minimize hypertonia and muscle rigidity Seizure precautions Respiratory support and protection of the patient's airway Monitoring the patient for painful dyskinesias

Respiratory support and protection of the patient's airway Rationalization: aspiration and respiratory failure are major cause of death in severe MG so it is important to assess respiratory function and gag reflex because the diaphragm, intercostal, and swallowing muscles can be affected.

any patient with an injury of _____ is at risk for autonomic dysreflexia

T6 or above

A patient is devastated to receive a diagnosis of amyotrophic lateral sclerosis (ALS). The symptomatology of the disease is a result of its effects on: Neuromuscular junction The vestibulocerebellar system Upper and lower motor neurons Upper motor neurons

Upper and lower motor neurons Rationalization: upper motor disorder - problem in the brain's area of motor control. lower motor disorder - problem in the region where motor nerves exit the spinal cord. This disease eventually results in respiratory failure. Symptoms include painless muscle weakness and atrophy.

what are the clinical presentations of autonomic dysreflexia?

a pounding headache, blurred vision Exaggerated sympathetic response Dangerously high blood pressure - hypertension most common causes - distended bladder or rectum

what is the shrinking of cells referred to?

atrophy

an exaggerated sympathetic response to stimuli that is below the level of the spinal cord. if someone has a t6 injury or above, they will not feel anything below that

autonomic dysreflexia

in spinal shock reflex function is lost...

below the lesion ex) T3 injury.. below the level of the transection where the chord has been completely damage, there is a loss of reflexes. involuntary reflexes are not working

caused by anticholinesterase drug toxicity. anticholinesterase are used to help MG symptoms. an anticholinesterase drug prevents the breakdown of the enzyme that breaks down acetylcholine. allows more acetycholine to be taken up by healthy acetylcholine receptor sites. too much of this drug --> too much acetylcholine on board --> SLUG

cholinergic crisis

serves NO PURPOSE. lasts more than 6 months after an injury or disease process.

chronic pain

paresis of legs to complete quadriplegia respiratory insufficiency ascending paralysis may progress to respiratory arrest or cardiovascular collapse

clinical manifestations of gullain barre

insidious onset: it happens vaguely and then it can worsen muscle fatigue and progressive weakness **EOM, levator muscles are most affected! (muscle that brings eyelid up) diplopia (double eyelid) ptosis (drooping eyelid) difficulty chewing and swallowing diaphragmatic involvement

clinical manifestations of myasthenia gravis

this type of headache occurs in a cluster, behind the eye, one sided or just in one spot. this is a:

cluster headache

muscles or tendons. sprained ankle, soft tissues, muscle, tendons

deep somatic pain

a patient has a spinal chord injury. the nerve is not sending signals. the muscle cannot respond to signals because the nerve transmission is impaired.

denervation atrophy

both upper limbs or both lower limbs

di- or para-

a bedbound patient's muscle cells are shrinking when the muscle is not used. this is an example of:

disuse atrophy

what is the treatment for parkinson's?

drugs to decrease akinesia: only lasts for a short amount of time brain implants to activate dopaminergic sites

what factors can lead to people displaying different responses to pain?

emotional factors - am i agitated or anxious physical or chemical factors - do i reposition myself, do i apply ice or heat

what neurological disorder is a demyelinating disorder caused by immunologic reaction directed at the peripheral nerves with an acute onset of ascending motor paralysis?

gullain barre

a muscle contraction that is stiff

hypertonia/rigidity

there is no tone/no muscle contraction think of floppy baby syndrome. amount of tension/resistance to stretch of muscle is low - involves reduced muscle strength.

hypotonia/flaccidity

where are upper motor neurons?

in the brain and spinal cord

this type of headache is seen more in women than men - may be hormone related. can be associated with other symptoms outside of just the headache like photosensitivity. can present with an aura. can involve vomiting, facial droop - signs similar to a stroke. this is a:

migraine headache

one limb

mono-

damage to one peripheral nerve like carpal tunnel syndrome

mononeuropathies

what is the leading cause of spinal cord injury?

motorcycle crashes

a contractile protein called dystrophin is absent and does not allow the muscle to maintain its integrity. the muscle fills with fat instead of this protein causing shrinkage of the muscle. it is a genetic disorder

muscular dystrophy

the thymus tumor/overgrowth of thymus gland is associated with...

myasthenia gravis

what neurological disorder attacks the acetylcholine receptor sites?

myasthenia gravis

symptoms begin in the body and are triggered by stress leading to manifestation of symptoms like respiratory failure

myasthenic crisis

triggered by pain receptors in the nerves. nerve injury

neuropathic

triggered by pain receptors in the tissues. tissue injury

nociceptive

what is the most common cause of dysreflexia and why?

over extended bowel or bladder. they cannot voluntarily urinate or defecate they need assistance emptying their bladder and bowels. if these patients get constipated or we lose tract of the last time they got urinary catheterized. the peripheral nerves sense this and try to send a signal to the brain that they have to poop or pee. the brain doesn't receive this message. the peripheral nerves will react and will stimulate catecholamine surge. BP goes up to uncontrollable levels. patient can have a stroke and burst the vessel in their brain. once we resolve the issue, the patient will go back to normal pretty quickly

the level at which pain is perceived by the person. subjective

pain threshold

the point at which pain is more than what the person can bare. subjective

pain tolerance

loss of movement

paralysis

weakness

paresis

what is a degenerative/progressive, happens over time disorder of the basal ganglia and dopaminergic pathway?

parkison disease

damage to many peripheral nerve, like guillain barre

polyneuropathies

all four limbs

quadri- or tetra-

a gall bladder is inflammed. it is tucked behind the liver and the patient presents right shoulder pain. heart attack patients may have jaw pain. the way nerves are bundled/pathways leads to this sensation. the pain shows up at a different site than where the organ is affected. this is an example of:

referred pain

what is myasthenia crisis?

respiration is compromised. this can be severe and life threatening if the diaphragm is affected (remember this is a muscle) - patient may not be able to breathe

what is important to do when evaluating parkinson's?

rule out secondary causes like stroke at basal ganglia, medications on board with parkinson's like symptoms like acetylcholine meds

flaccid paralysis: the leg is flaccid, hypotonia loss of sensation loss of bladder and rectal control hypotension/bradycardia hypothermia below the level of the lesion - body can't self regulate

spinal shock

what occurs onset at the time of injury? this happens right after spinal chord injury. Time that the spinal chord is swollen and inflamed due to the injury. There is damage to the peripheral nerves below the level of the injury.

spinal shock

this is a hat-band distribution - a more typical headache. this feels like a tight band around the head. this is a:

tension type headache

after spinal shock resolves ______, but the patient will always be at risk for ____

the peripheral nerves are working; always be at risk for autonomic dysreflexia/hyperreflexia

what is the gate control theory?

the way a person perceives pain can be moderated by several factors and is SUBJECTIVE two people can experience pain much differently. pain is subjective to each person. if i change my behavior, i may change my perception of pain

treatment for ALS

there is a theory that glutamate toxicity is involved and damages neurons. anti-glutamate meds may help symptoms

this is pain that is compromised movement of the jaw joint and surrounding muscles. this is:

tmj (temporomandibular joint pain)

T/F dopamine is the inhibitory to muscle contraction. acetylcholine is excitatory. if we have too little dopamine, we have too much acetylcholine. this leads to excess muscle contraction in parkinson's

true

T/F parkinson's is linked to head injury, concussion

true

what happens before gullain barre appears?

viral infection that causes this immune response like the epstein barr or flu

in the organ bed distension of an organ

visceral pain

what happens when swelling of the spinal shock resolves?

you will illicit a response, but the reflex will be abnormal. the peripheral nerves are working - but the patient can't feel sense, or control it. the peripheral nerves can't send messages to the brain because the spinal chord no longer functions. at baseline and where the patient will be the rest of their life.