neuro- NM disorders Flashcards
what is bells palsy? S&S?
paralysis of muscles supplied by facial nerve (CN VII), usually unilateral, caused by inflammation w/in facial canal or stylomastoid foramen. (nerve too big or whole too small)
-sudden onset, ipsilateral dry eye (inadequate lid closure), hyperacusis (hearing sensitive), earache, loss of taste, numbness
what is the pathophys of bells palsy? who gets it? what percent resolve spontaneously?
idiopathic, possibly linked to HSV-1 (ramsey-hunt syndrome)
- men more than women
- possibly family linked- passing down of small foramen? (no genetic basis )
- 85% resolve spontaneously in a year
studies for bells palsy?
none necessary - no labs are determinant
treatment for bell’s palsy
- lubricating eye drops
-oral prednisone (w/ anti-virals to cover HSV) - 6-10 days
-surgery if bad
-
what is guillien-barre syndrome? what causes it?
autoimmune disorder where body attacks nervous system: acute inflammation, demyelination by antiganglioside antiBs, ascending paralysis
-often preceded by URI/diarrhea viral illness 1-3wks before (campylobacter)
S&S of GB?
- dysesthesias (feelings in absence of stimuli)
- parestethias (numbness/tingle)
- areflexia
- dec. positional and vibratory sensation
- back and leg pain
- ascending weakness
- resp. muscle paralysis = death
- labile (easily changed) BP, arrythmias
2 labs/tests for GB?
- CSF- elevated protein w/ normal WBC
- nerve conduction studies (most sensitive)- conduction block is common
txt for GB? 2 effective therapies?
no cure
-hospitalize w/ concern for resp. function failure - intubate
–> support body until it is cleared from it
2 effective therapies speed recovery and reduce severity: plasma exchange & IVIG
what is myasthenia gravis? what is is assoc w/?
- MG antiBs blockage of NM transmission at nicotinic receptors
- ocular, masticatory, facial muscles - easily fatigued, inc weakness at end of day
- onset: anything that can activate immune system
- assosciated w/ other autoimmune diseases, thymoma (thymus tumor)
how does MG present?
- any age, females >males
- ptosis, diplopia, ptosis, chewing/swallow issues, resp. difficulty, limb weakness
- weakness in PE (not in nerve root/dermatome distribution)
- activity = weakness
2 ways to distinguish bells palsy from MG?
- bells- focal neuro deficits like affected pupillary response, MG does not - only has extraocular muscles effected (ptosis)
- bells- areflexive, MG-no reflex change
what is the tensilon challenge?
Dx of MG: give pt tensilon (which temporarily blocks axn of acetylcholinesterase)
-response in 5min, improvement of muscles = MG!
2 lab tests for MG?
- serum anti-AChR
2. Xray or CT of chest may show thymoma
5 possible txts for MG?
- anticholinesterase drugs (pyridostigmine or neostigmine)
- thymectomy (w/ thymoma) in pts <60yo (decreases immune hyperfunction)
3.immunosuppressants - plasmapheresis/IVIG
- pt education: heat and exercise may make symptoms worse
(mostly supportive care, usually well-managed, fatalities from resp complications)
what is multiple sclerosis?
chronic, unpredictable, neuro-immuno disease of CNS
- likely autoimmune cause
- inflamm, destruction of myelin, glial scarring
- eventual irreversible axonal loss (in many cases)
what causes MS?
likely. .. autoimmune, genetic
- trigger to cause break in BBB (CNS is usually safe from immune system), immune cells can then infiltrate and attacks central myelin
- -> maybe infection or trauma cause…
who gets MS?
young, white, females
-common farther away from equator (migration from high risk areas before age 15 = less risk)
how does MS present?
- weak, numb, tired, tingle
- imbalance, spastic, tremors
- speech and swallowing impairments
- diplopia: 90% w/ optis dysfunction
- sphincter dysfunction
- depression, cognitive decline
- symptoms often fleeting
how many general kinds of MS are there?
4
imaging for MS?
MRI - Dx based on lesions and clinical picture
CAN NOT Dx w/ CT, neg. CT DOES NOT rule out MS
-sed rate: very nonspecific, very sensitive
PE for MS shows…5 things
nystagmus, visual field deficits (diplopia), dysarthria (speech disorder from muscle weakness), sensorimotor deficits, cerebellar signs
Txt goals for MS- 5 major points
- preservation of function between flares, and exercise (keep out of wheelchair for as long as possible!)
- treat symptoms w/ drugs (spasticity, incontinence, fatigue, pain, depression)
- PT, OT, cooling vest (overheat = flare)
- high dose steroids for acute flares (# of times needed coincides w/ severity of disease)
- plasmapharesis for relapsing forms
disease modifying drugs for MS…what do they do and when do you take them? downsides? which one was found to improve walking ability?
decrease plaque formation and improve life and longevity
- these are used earlier, rather than just managing symptoms
- but they have ADRs and drug rxns
- fampridine
ALS/Lou Gehrig Disease : what is it and what are the 3 kinds?
degeneration disease that affects upper and lower motor neurons
- amytrophic lateral sclerosis: sporadic and most common form of disease (bulbar palsy, muscular atrophy, primary lateral sclerosis)
- familial ALS
- ALS-parkinson dementia: complex of Guam (specific type- enviro component)
who gets ALS?
men, after age 40
S&S of ALS?
focal wasting of muscle groups, fasciculations
-spares cognitive (watching their bodies fail), ocularmotor, sensory and autonomic functions
what is amyotrophic?
type of ALS, most common, muscles degenerate due to lack of trophic support from nerves
what is lateral sclerosis?
component of amyotrophic ALS, lateral horn of spinal cord becomes fibrotic as motor neurons die
what is sporadic ALS?
degeneration of upper and lower motor neurons , toxic levels of glutamate in serum and CSF (increase cell lysis = inc glutamate = inc cell lysis…)
what does a muscle biopsy for ALS show?
shrunken angulated muscle fibers
txt for ALS?
no cure, just supportive
what is cerebral palsy?
motor dysfunction, non-progressive
-a result of CNS insult that occurs prenatally, perinatally or in first 3 years of life
up to 15% of infants with low birth weight will have…
cerebral palsy
what are the major risk factors for cerebral palsy?
hypoxia, seizures during prenatal period, and other birth complications
S&S of cerebral palsy?
- spastic (40%), contractures
- mental retardation and aphonia w. quadriplegia and mixed forms
- normal intelligence and tremors w. hemiplegia or paraplegia
- scissors gait, to walking
what is athetotic CP?
CP w/ writhing/chorea movements, speech issues, muscular hypertrophy
2 main types of CP?
ataxic: normal intelligence, clumsy, difficulty w/ fine movements
mixed: spastic/chorea, mental retardation
early warning signs with CP
toe-walking, fisting, delay in motor milestones, seizures, hand dominance before age 2 (dominant hand not effected in hemiplegia)
what allows you to rule out CP in your Ddx?
progression
CP txt?
drugs for symptoms- spastic, sleep disturbances, irritability, monitor for UTI (sphincter control), botox to release spasm, surgery- tendon release
babinski test
for UMN lesion - increased tendon reflexes and extensor plantar response
tetany
painful muscle spasm, from faulty Ca+ metabolism
clonus
muscle spasm w/ regular contractions
fasciculation
involuntary muscle twitch (localized and temporary)
flaccid paralysis
LMN lesion- interruption between muscles and spinal cord
