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69 Cards in this Set

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what is the etiology of multiple sclerosis (MS)?
1. unknown etiology
2. genetic and environm. predispositions
3. common in pts who lived 1st decade of life in northern latitudes
what are si/sx of MS?
1. relapsing asymmetric limb weakness
2. increased deep tendon reflexes (DTRs)
3. nystagmus
4. tremor
5. scanning speeck
6. paresthesias
7. optic neuritis
8. pos. Babinski sign
how do you dx MS?
1. Hx
2. MRI (periventricular plaques, muliple focal demyelination scattered in brain and spinal cord)
3. lumbar puncture (increased CSF immunoglobulins manifested as multiple oligoclonal bands on electrophoresis)
what does an MRI show in MS?
1. periventricular plaques
2. multiple focal demyelination scattered in brain and spinal cord
3. lesions disseminated in space and time!!
what does a lumbar puncture show in MS?
1. increased CSF immunoglobulins
2. manifested as multiple oligoclonal bands on electrophoresis or increased IgG
what is tx for MS?
1. interferon-B
2. corticosteroids
3. glatiramer (immunomodulator)--copaxone
what is prognosis of MS?
1. variable types of dz
2. long remissions sometimes seen
3. can progressively decline--death in only a few yrs
what is Guillain-Barre Syndrome?
1. acute autoimmune demyelinating dz involving peripheral nerves
2. most often preceded by gastroenteritis (classically Campylobacter jejuni)
what are si/sx of Guillain-Barre Sx?
1. m. weakness and paralysis ascending up from lower limbs
2. decreased reflexes
3. can cause b/l facial nerve palsy
4. most often preceded by gastroenteritis (classically campylobacter jejuni), mcyoplasma or viral infxn, immunization or allergic rxn
what should you think of in a pt with a hx of gastroenteritis (classically campylobacter jejuni), mycoplasma or viral infxn, immunization or allergic rxns?
Guillian-Barre Syndrome!!!
how do you dx Guillain-Barre Sx?
1. Hx of antecedent stimuli (gastroenteritis, campylobacter jejuni, Mycoplasma, viral infxn, immunization, allergic rxn)
2. CSF albumin-cytologic dissociation (CSF protein is increased s/ increase in cells)
what is albumin-cytologic dissociation and what syndrome do you find it in?
1. CSF protein increased s/ increase in other cells seen
2. Guillian-Barre Synd
how do you tx Guillain-Barre Sx?
1. plasmapheresis
2. intravenous immunoglobulin (IVIG)
3. intubation for respiratory failure
4. do NOT use steroids!!
what is px for Guillain-Barre Sx?
1. dz usually stops spontaneously--excellent px for 80-90% of pts
2. spontaneously regresses
3. respiratory failure and death can occur in remainder!!
what causes central pontine myelinolysis?
1. rapid correction of hyponatremia
2. occurs from osmotically induced demyelination d/t overly rapid correction of serum sodium (an increase of more than 1 mEq/L/hr or 25 mEq/L in the 1st day of therapy)
what can result from rapid correction of hyponatremia?
central pontine myelinolysis!!
how do you characterize central pontine myelinolysis?
1. diamond-shaped region of demyelination in basis pontis
2. results from rapid correction of hyponatremia, liver dz
what is tx for central pontine myelinolysis?
1. no tx once condition has begun
2. coma or death is common outcome
3. results from rapid correction of hyponatremia, liver dz
what is seen in pts enclosed in burned areas or during the start of a cold winter (people using their new gas heaters)?
1. carbon monoxide poisoning
2. b/l pallidal necrosis
how does b/l pallidal necrosis occur?
1. carbon monoxide poisoning
2. pts enclosed in burned areas
3. people using new gas heaters
what are si/sx of carbon monoxide poisoning?
1. HA
2. nausea
3. vomiting
4. delirium
5. cherry-red color of lips
what is dx of carbon monoxide poisoning?
elevated carboxyhemoglobin levels
what is tx of carbon monoxide poisoning?
hyperbaric oxygen (1st line) or 100% O2
what is found in thiamine deficiency (B1)?
1. usually secondary to alcoholism
2. Beriberi peripheral neuropathy d/t Wallerian degeneration
what is Wernicke's triad?
1. confusion (confabulation)
2. ophthalmoplegia (lateral rectus m. weakness, conjugate gaze palsies)
3. ataxia
*nystagmus leading to ophthalmoplegia
*periphearl neuropathy may also be present
*seen in thiamine deficiency
how do you tx thiamine def?
1. thiamin 50 mg is given IV immediately (prior to glucose!!)
2. IV glucose given before thiamin may precipitate the sxndrome or worsen the sx--will exacerbate mamillary body damage
what lesions is Wernicke's related to?
lesions of mamillary bodies
when do you give glucose to a thiamine-def pt?
AFTER giving thiamine!!! (run thiamine in IV fluid s/ glucose!!)
describe B12 def
subacute degeneration of posterior columns and lateral corticospinal tract
what are si/sx of B12 def?
1. weakness
2. decreased vibration sense (both worse in legs)
3. paresthesias
4. hyperreflexia
5. ataxia
6. personality change
7. dementia
Note: Neurologic deficits can occur even if no hematologic abnormalities are present!!
what is tx for B12 def?
1. B12 replacement (can use high-dose oral inlieu of injection)
What is Hepatolenticular degeneration?
Wilson's disease!!
1. defect in copper metabolism
2. causing lesions in basal ganglia
what is Wilson's disease?
hepatolenticular degeneration!!
1. defect in copper metabolism
2. causing lesions in basal ganglia
what are si/sx of Wilson's dz?
1. extrapyramidal tremors and rigidity
2. psychosis
3. manic-depression
what is pathognomonic for Wilson's dz?
Kayser-Fleischer ring around the cornea
what is the dx of Wilson's dz?
1. decreased serum ceruloplasmin (plasma copper-carrying protien)
2. excessive deposition of copper in the lever and brain
what is the pathophy of Wilson's dz?
1. AR disorder
2. affects a copper-transporter leading to excessive deposition of copper in the liver and brain
3. excessive absorption of copper from the sm intestine
4. decreased excretion of copper by the liver
5. the plasma copper-carrying protien (serum ceruloplasmin) is low
6. urinary excretion of copper is high
how do you tx Wilson's dz?
1. penicillamine
2. liver transplant if drug fails
what causes hepatic encephalopathy?
1. seen in cirrhosis
2. may result from brain toxicity secondary to excess ammonia and other toxins not degraded by malfunctioning liver
what are si/sx of hepatic encephalopathy?
1. hyperreflexia
2. asterixis (flapping of extended wrists)
3. dementia
4. seizures
5. obtundation/coma
what is tx for hepatic encephalopathy?
1. lactulose (leads to a change in bowel flora so that less ammonia-forming organisma are present, lacutulose also favors the formation of a non-absorb form of ammonia NH4+)
2. neomycin (also controls ammonia-producing intestinal flora)
3. protein restriction to decrease ammonia-related toxins
p. 641 of CMDT
what is Tay-Sachs dz?
1. hexosaminidase A defect
2. causes an increase in ganglioside GM2 (defect=gangliosidosis)
3. AR
where do you see a cherry-red spot on macula?
in Tay-Sachs dx!!
what are si/sx of Tay-Sachs dz?
1. cherry-red spot on macula
2. retardation
3. paralysis
4. blind
how do you dx tay-sachs dz?
1. dx by bx of rectum or by enzymatic assay
2. no Tx
what is definition of Complex seizure?
LOC (loss of consciousness)
what is definition of simple seizure?
NO LOC (no loss of consciousness)
what is generalized seizure?
the entire brain is involved
what is a partial seizure?
focal area of the brain
what is tonic seizure?
prolonged contraction
what is clonic seizure?
twitches
what is an absence seizure?
1. complex (LOC),
2. generalized (entire brain)
what is a grand mal seizure?
1. complex (LOC),
2. generalized (entire brain)
3. tonic (prolonged contractions)-
4. clonic (twitches)
what is tx for partial (focal) sz?
1. phenytoin (1st line)
2. carbamazepine (1st line)
3. valproate
what is tx for grand mal seizure?
1. valproate (1st line)
2. carbamazepine
3. phenytoin
what is tx for absence seizure?
complex, generalized sz:
1. ethosuximide (1st line)
2. valproate
3. clonazepam
what is tx for myoclonic seizure?
1. valproate (1st line)
2. clonazepam
what are si/sx of a seizure?
1. hx of prior head trauma, stroke, other CNS dz increases risk for seizures
2. loss of bowel/bladder control
3. tongue maceration
4. postictal confusion/lethargy
5. focal findings indicate epileptogenic foci
if pt has a hx of seizures, what should you always check?
the blood level of medication
what else should you consider when tx for seizures?
*tx the seizure that recurs
*tx the underlying cause:
1. electrolyte
2. infxn
3. toxic ingestion
4. trauma
5. azotemia
6. storke/bleed
7. delirium tremens
8. hypoglycemia
9. hypoxia
which seizure med causes gingival hyperplasia and hirsutism?
phenytoin!!!!
what side effects does phenytoin cause?
1. gingival hyperplasia
2. hirsutism
what seizure med causes leukopenia/aplastic anemia and is hepatotoxic?
carbamazepine!!!
what SE does carbamazepine cause?
1. leukopenia
2. aplastic anemia
3. hepatotoxic
which seizure med causes neutropenia, thrombocytopenia and is hepatotoxic?
valproate!!
what SE does valproate cause?
1. neutropenia
2. thrombocytopenia
3. hepatotoxic
when can you stop seizure tx?
if no seizures for two years and normal EKG
what is status epilepticus?
1. continuous seizing lasting >5 min
2. this is a medical emergency
what is tx for status epilepticus?
1. benzodiazepines for immediate control
2. follow by phenytoin loading and
3. phenobarbital for refractory cases
4. this is a med emergency!!