Med III lowy exam 1 - neuro

5 common neurodegenerative disorders

alzheimer’s, ALS, dementia with lewy bodies, huntington dz, parkinson’s

what neuro disorders are described as a movement disorder

ALS, essential tremors, huntington’s, parkinson dz, restless leg syndrome, TD, tourette’s

3 demyelinating disorders in pt with sx of muscle weakness

ALS, GBS, MS

3 neuromuscular disorders

CP, MS, MG

signs of a neurodegenerative disease of the upper motor neuron (UMN)

hyper reflexivity, inc tone, muscle spasm, pos babinski sign

what is an example of a neurodegenerative condition that is caused by a LMN lesion

muscle atrophy

neurodegenerative condition where there is both UML and LMN lesions. this dz is

ALS

what part of the neuron is GBS affecting

only LMN

MS affects what part of the motor neuron

UMN

this dz is a neurodegenerative movement disorder that affects both the upper and lower motor neurons of the CNS. muscle weakness occurs bc of progressive loss of motor neurons. there is preserved cognitive and sensory function. the phrenic nerve can get involved, leading to resp paralysis and a need for vent.

ALS

how to tx ALS

sx relief, maintain QoL. riluzole (a glutamate antagonist) to slow progression of dz. many pts have inc glutamate in blood and CSF

what is the greatest indicator of ALS

muscle weakness that spreads and worsens

ascending paralysis syndrome

GBS

what dz is attributed to an autoimmune reaction that developed in response to a previous infxn, vaccination, or allergic rxn

GBS

this dz presents with symmetrical muscle weakness, demyelination of the PNS nerves, ascending weakness/paralysis, hyporeflexia, and papilledema of the optic nerve. caused by an overproduction of CSF leading to an inc in protein. there is the potential to have mass-effect sx.

GBS

GBS or acute inflammatory demyelinating polyradiculoneuropathy develops in a previously healthy person in response to which of the following

allergic rxn, GI infxn, URI infxn, vaccination. *** muscle weakness/paralysis is a PRESENTATION, NOT a cause

MS is what type of dz

demyelinating dz of the CNS

GBS is what type of dz

demyelinating dz of the PNS

pt is concerned they might have a genetic disorder. father had early onset dementia and passed at 45. father had twitching and dance like movements of his arms. what dz is assoc with dementia, choreatheroid movements of the face and extremities, and early death?

Huntington dz

what relieves the sx of an essential tremor

alcohol

what sx are assoc with restless leg syn

pt wakes up at night with shaking leg

what sx are assoc with parkinson dz

shuffling gait, mask-like facies, cogwheel rigidity

what sx are assoc with ALS

fasciculations and progressive weakness

pt is concerned they might have a genetic disorder. father had early onset dementia and passed at 45. father had twitching and dance like movements of his arms. a mutation of what chromosome is likely to be responsible for the suspected dx

chromosome 4

pt is concerned they might have a genetic disorder. father had early onset dementia and passed at 45. father had twitching and dance like movements of his arms. what transmission patterns is most likely to be responsible for the suspected dx

AD

pt is worried about being dx with CNS demyelinating dz MS. what are RF for developing MS in this pt?

age onset 20-50 yrs, living in temperate climate, being female, fhx of MS

what is likely to be noted in a pt with MS

difficulty speaking, muscle weakness, essential/intention tremor, urinary incontinence (from muscle weakness), visual disturbances (from demyelinating optic nerves)

54 yr old lady complains of difficulty chewing and swallowing food, double vision. sx absent in the am but worsen as day progresses. what is the pathophys contributing to the development of her sx

Ab against ACh receptors

pathophys for MS

CNS plaque formation, demyelination of the CNS

pathophys for parkinsons dz

dec dopaminergic input into basal ganglia, neuronal loss in substantia nigra

autoimmune polyneuropathy, PNS damage

demyelination of PNS

what conditions presenting with neurologic sx is likely to also have dementia

alzheimer’s dz, Creutzfeldt-Jacob dz, Huntington’s dz, parkinson’s dz

etiology of alzheimer’s dz

neurodegenerative

how do alzheimer’s pts present

dementia of short term memory, confusion, personality changes, impaired judgement

ALS etiology

neurodegenerative, movement disorder, CNS, UMN and LMN

ALS presentation

UMN = fasciculations, twitching, spasticity.

LMN = muscle atrophy, weakness, hyporeflexia

CP etiology

congenital

CP presentation

spasticity, dyskinetic, ataxia

GBS etiology

autoimmune rxn, PNS demyelination, LMN lesion

this pt has rapid onset numbness, tingling, pain followed by weakness, change in sensations, muscle weakness, hypo/areflexia. tingling & dec reflexes in feet/legs, ascends to upper body. ascending begins in feet/hands, ascends to upper body/arms. can lead to paralysis in 3 phases = acute, plateau, recovery. once there is resp muscle weakness you need mechanical ventilation

GBS

Huntington dz etiology

neurodegenerative, AD (cr 4)

huntington dz presentation

dementia, dance like movements, early death

MS etiology

CNS demyelination, UMN

MS presentation

relapsing neuro deficits, diplopia, optic neuritis, weakness, paresthesia

MG etiology

neuromuscular d/o, Ab made to ACh receptors at neuromuscular junction

MG presentation

diplopia, ptosis, dysphagia, weakness worse when active and better at rest.

Parkinson’s dz etiology

neurodegenerative movement disorder, neuronal loss in substantia nigra

Parkinson’s dz presentation

resting tremor, cogwheel rigidity, akinesia/bradykinesia, postural instability, shuffling gait, masked facies

restless leg syn etiology

dopamine dysfxn or iron def anemia

restless leg syn presentation

urge to move your leg at night. can be involuntary or a feeling forcing you to move it

tourette’s syn etiology

AD

tourette’s presentation

involuntary motor and phonic tics

how to tx alzheimer’s

no tx, progressive deterioration

how to tx ALS

sx relief, better QoL, riluzole (glutamate antagonist)

dementia with lewy bodies presents how

gradual parkinson-like presentation. memory loss. PE shows visual hallucinations

how to tx dementia with Lewy bodies

rivastigmine for sx relief to improve cognition and dec hallucinations

how to tx essential tremors

BBs

how to test for GBS

nerve conduction studies, CSF LP: nl cel count, elevated proteins

how to tx GBS

IV immunoglobulins, plasmapheresis

how to test for huntington dz

genetic testing: trinucleotide CAG; CT/MRI: cerebral atrophy

how to tx huntington dz

supportive care

MS charcot’s triad

scanning speech, intranuclear ophthalmoplegia, nystagmus

Lhermitte’s sign MS

neck flexion = lightening shock radiating from spine down the leg

tests for MS

MRI test of choice (see demyelinating plaques), CSF (inc immunoglobulins, inc IgG, oligoclonal bands). sensory evoked potential (dec conduction velocity)

how to tx MS

IF-B1b, hi dose CCS during an acute attack

how to test for MG

ice pack over eyelids improves sx. edrophonium/tensilon test = temporary improvement in strength

how to tx MG

anticholinesterase drugs (pyridostigmine). ppx thymectomy <60, plasmapheresis/IVIG = temporary relief

myerson’s sign of parkinson’s dz

tap on the nose and the pt blinks

how to tx parkinson’s dz

levodopa + carbidopa. dopamine agonist (bromocriptine, pergolide), anticholinergics, amantadine, selegeline

what sx is characteristic of parkinsons

resting tremor

how to tx tourette d/o

dopamine antagonists (fluphenazine, pimozide)

what is the difference between dementia and delirium

dementia is progressive and delirium is short term

general decline in cognitive ability. continuum of impairments in memory, thinking, language, behavior, motor control

dementia/cognitive impairment

altered level of consciousness, acute confusional state due to psych/organic process. short course with fluctuations. reduced ability to focus, perceptual disturbances.

delirium

what diseases cause dementia

alzheimers, vascular dementia, Lewy body disease, huntington dz, parkinson dz, korsakoff dementia (excessive alc use —> b12 def)

what diseases cause delirium

encephalopathic disorders, wernicke’s encephalopathy (excessive alc use = b1 def), chronic alcoholism, eating disorders, chemotherapy

progressive neurodegenerative movement disorder that affects males> females. loss of dopaminergic neurons from substantia nigra in basal ganglia.

parkinson dz

m/c type of dementia

alzheimer’s dz

what is alzheimer’s dz

progressive deterioration of cognitive fxn. loss of ability to perform daily activities/functions at work/social situations

what are defining traits about alzheimer’s disease

inc homocysteine, APOE4 gene

what would MRI/CT show in a pt with alzheimer’s

temporal/parietal atrophy

definitive dx of alzheimer’s is made

on autopsy

what is vascular dementia

strokes in the cerebral cortex

neoplasms/tumors can cause

dementia/cognitive impairment

when trauma/injury causes dementia you must order

CT

what dz cause dementia

alzheimer’s, vascular dementia, neoplasms/tumors, trauma/injury, parkinson’s, huntington’s, normal P hydrocephalus, pick’s dz, Lewy body dz, infxns like AIDS encephalopathy, neurosyphilis, CJD

what does a LP show in a pt with normal P hydrocephalus

normal CSF pressure

what are the 3 Ws of normal P hydrocephalus

wacky (dementia), wet (urinary incontinence), wobbly (gait instability)

what is pick’s dz

pick bodies form in the frontal lobe.

what does MRI/CT show in a pt with Pick’s dz

frontotemporal atrophy

what is CJD caused by and how do these pts present

prion proteins, abrupt presentation

what are the DEMENTIAS (acronym)

degenerative disorders, endo d/o, metabolic d/o (uremic/hepatic encephalopathy), exogenous d/o, neoplasms, trauma, infx, affective d/o (depression), stroke (vascular dementia)

normal P hydrocephalus on CT/MRI shows

ventricular enlargement

what are causes of delirium

drugs, infxn, metabolic abnl, brain d/o, systemic d/o (cardiac/liver/renal failure), burns, hyper/hypothermia, trauma

what is an irreversible dementia

alzheimer’s

what are reversible dementias

depression (pseudodementia), hypothyroidism (memory loss, depression, cold intolerance, autoimmune, radioactive iodine, lithium), pernicious anemia (IF/B12 def = impaired proprioception and vibratory sensation), nl pressure hydrocephalus (dementia, abnl gait, urinary incontinence), meds = oral bismuth (for h pylori), amiodarone.

is delirium reversible or irreversible

reversible

what is an encephalopathic disorder

brain dz that causes brain dysfxn and AMS

how do pts with encephalopathic disorders present

cognitive, personality, muscular changes. confusion, disoriented, memory loss, personality changes, agitation, neuropsych sx, poor coordination, twitching

what causes encephalopathic d/o

infxn, tumor, bleeding, toxins