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162 Cards in this Set

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  • Back
What are the 3 features of alpha activity that are important to notate? 1
1) symmetry (up to 50% variance of 20 microvolts usually the right is higher side)
2) location- usually posterior dominant, anterior dominant can be seen in alpha coma
3) reactivity- attenuates with eye closure; paradoxical alpha is when atteuates with eye opening (just waking without visual fixation or sedation)
What is the usual frequency of alpha waves?
8 to 13 hz
What is the usual frequency of beta waves?
>13 hertz
What is the usual etiology of frontal predominant or diffuse beta?
medications such as barbiturates or benzodiazepines
What is the usual etiology theta frequency?
drowsiness
What is the usual etiology of temporal alpha?
can be normal variant in elderly
What is jamais vu?
a familiar situation which is not recognized
What are three ways to characterize automatisms?
de novo- complex motor activity begins after sz onset
reactive automatisms- activity is a reaction to ext. stimulus
perseverative automatisms- continuation of motor activity initiated before seizure onset
What does the term uncinate fits refer to?
olfactory halllucinations arising from the temporal lobe
Where can bicycling, pedaling movements or sexual gesturing during aseizure localize to?
frontal lobe
What is the most common extratemporal partial epilepsy?
frontal lobe seizures
What are some examples of occipital seizures?
elementary visual hallucinations, bright lights, flashes of light, geometric objectives and versive eye movements
What are the characteristic of benign neonatal seizures?
clonic or myoclonic seizures/apneic events during first few weeks after birth, stop by 6 weeks, normal development; 10-15% later have epilepsy; treatment not necessary (can give 1 month phenobarb)
What is the etiology of benign familial neonatal convulsions?
autosomal dominant inheritance, mutation of KCNQ2 or 3 genes
What is early myoclonic enphalopaty?
focal myoclonus that migrates to different body parts, occurs in early infancy, other seizure types; many etiologies (cryptogenic, HONK, inherited, developmental malformation)
What is the etiology of AD partial epilepsy with auditory features?
Leucine-rich, glioma inactivated 1 gene
What is a caveat about the EEG in myoclonus?
may not have EEG correlate on EEG and be normal initially
What is the prognosis with early myoclonic encephalopathy?
more than 50% die
What is Ohtahara syndorome?
early infantile epileptic encephalopathy with suppression bursts
What is the difference between Ohtahara syndrome and early myoclonic encephalopathy?
Ohtahara syndrome has burst-suppression on EEG, no myolocnic seizures, can treat with vigabatrin, same prognosis
What is the etiology of pyridoxine dependent seizures in infancy?
possibly decreased glutamic acid decarboxylase activity
What is the difference between pyridoxine dependent and deficient seizures?
pyridoxine dependent requires larger doses of B6, life long B6 and occurs early than pyridoxine deficient (caused by breastfeeding malnourished mothers)
What is the difference between benign myclonus of infancy and myoclonic encephalopathy?
developmentally normal, clusters for weeks then less freuqent by 3 months and stops by 2 years
normal eeg
What is the triad in West's syndrome?
infantile spasms
hypsarrhythmia
developmental arrest
What is the triad for Aicardi's syndrome?
infantile spasms
agenesis of the corpus collosum
and retinal malformations
X linked dominant
What is benign myclonic epilepsy of infancy?
EEG with spike & wave or polyspike and wave
excellent dvelopmental outcome with treatment with VPA
1/3 have family history
What is Dravet syndrome?
severe myoclonic epilepsy in infancy, begins within 1 year afterbirth, no previous brain abnormality except diffuse atropy, partial seizures later, 1/4 have family hx of sz, dev delay, EEG general, focal or multifocal abnL
idiopathic generalized epilepsy
What are the features of benign epilepsy with central temporal spikes?
onset 4-12 resolve by mid-teens
motor, simple sensory, tonic-clonic movements, speech arrests can have secondary generalization
more sz with speech
half of releatives have EEG changes but only 12% would have sz
not necessary to treat
idiopathic focal epilepsy
What is Panayiotopoulos syndrome?
also called early onset benign childhood occipital epilepsy
children 3-6
autonomic sz, status epilepticus, ictal vomiting
good response to anticonvulsants
frequent or nealry continuous rhythmic occipital speaks
What is Gastaut type late onset childhood occipital epilepsy?
children 4-8
visual seizures: hallucinations, blidness, followed by headache, onset with photic stimulation
What is the significance of epilepsy with myoclonic absences?
children age 7
**half have dev delay
no eye twitching unlike other absence seizures
1/2 continue to have seizures as adults**
What is myoclonic-astatic epilepsy of childhood?
onset 2-5 with multiple generalized sz types: tonic-clonic, clonic, atonic, myoclonic (NO TONIC unlike LG)
EEG faster than LG 2-3 HZ spike and wave
What are the characteristics of Lennox Gastaut syndrome?
1. mental retardation (severe, progressive due to subclin sz)
2. slow spike & wave 1.5-2.5 HZ (spikes are slow 150 ms)
3. multiple generalized sz types
onset 2-8 boys >girls
cryptogenic or symptomatic
What is Landau Kelfferner syndrome?
acquired epileptic aphasia, word deafness
onset 3-8
What is the treatment for Lennox gastaut syndrome?
VPA, felbamate (drop attacks), lamotrigine
What is the treatment for Landau Kleffner syndrome?
VPA, Lamotrigine, steroids?
What are the features of childhood absence epilepsy?
onset 6 years old (up to 10)
staring spells (can have automatisms, ictal jerks of eye)
1/3 have GTC
90% outgrow by childhood
caused by thalamic activity of T type calcium channels
What are the differences between juvenile absence epilepsy and childhood absence epilepsy?
age of onset juvenile is 10-16
more likely to have GTC
less likely to outgrow sz
What features of absence epilepsy are considered atypical?
prolonged (more than a few seconds)
assoc with other sz types or MR
spike and wave <2.5 HZ (absence can be 2.5 HZ to 4 HZ)
What is the differential diagnosis for progressive myoclonic epilepsy?
lysosomal
Lafora body disease
Unverricht-Lundborg syndrome (baltic myoclonus)
NCL
Sialidoses
mitochondrial
MERRF
What are the features of GEFS plus?
febrile sz + atypicla feature (sz without fevere, after age 6 or other seizure type)
AD (sodium channel or GABA)
What are the clinical features of Rasmussen's encephalitis?
intractable progressive focal seizures
progressive hemiparesis
cog deterioration
antibodies to GluR3 (glutamate receptor 3)
What is the difference between phenytoin and fosphenytoin?
Fosphenytoin is the intravenous prodrug of phenytoin, converted to phenytoin by erythrocytes (takes 8 to 16 minutes), water soluble solution (less purple glove syndrome), can give more 150 mg of phenytoin equivalents per minute
What are the 4 MOA of phenobarbital?
GABAa
sodium channel blocker
T-type calcium channel blocker
glutamate blocker
What are the 3 MOA of VPA?
sodium channel blocker
gaba a agonist
gaba trasnaminase inhibition
t-type calcium channel blocker
What arethe side effects of Ethosuximide?
drowsiness, dizziness, headaches, GI upset, n/v/d, rash,
monitor lfts and cbc
What are the side effects of felbamate?
potentially fatal hepatotsicity and high rates of aplastic anemia, only in severe refractory cases of Lennox-Gastaut as last resort
How does Felbamate interact with other meds?
inhibits breakdown of pht, phb, vpa, cbz
what is the MOA of tiagabine?
GABA reuptake inhibitor
What are hte SE of Tiagabine?
somnolence, dizziness, cognitive, ataxia, tremor
What is the MOA of Gabapentin?
increases post-synptic GABA
What are hte MOA of Lamotrigine?
inhibits sodium channels and inhibits glutamate release
What are the RFs for Stevens Johnson with LMG?
rapid titration
use of VPA
use in children
What is the MOA of Levetiracetam?
inhibits burst firings of neurons withoutaffecting normal neuronal activity, not exactly known
What are the side effects of LEV?
mild cognitive-drowsiness, dizziness, ataxia
psychiatric-agitation, emotional lability, behavioral
What are the MOA of Topiramate?
sodium channel inhibition
gaba a agonist
NMDA blocker
carbonic anhydrase inhibitor
What are the side effects of TOP?
kidney stones, paresthesias, angle closure glaucoma, weight loss, word findingdifficulty, weight loss
What are the interactions of TOP with OCP?
can decrease effectiveness of OCP
What is the difference between VPA and DVP?
VPA should be given TID
DVP can be given BID
What is autoinduction in CBZ?
after being on CBZ for a few days to weeks can lead to autoinduction and half-life changes from 30 hrs to 10-20 hrs
What is a caveat regarding CBZ interpretation levels?
CBZ epoxide metabolites can accumulate although the levels may look low (autoinduction, use with VPA)
What is the relationship between OXC use and OCPs?
OXC can decrease the effectiveness of OCPs
What should be monitored in LMG?
nothing
What is MOA of BZDZ?
GABAa channels, increase chloride channel opening FREQ
What are the SE of BZDZ?
cardiovascular collapse, somnolence, resp depression, , withdrawal, hepatotoxic, neutropenia/pancytopenia/thrombocytopenia
What is MOA of Ethosux?
T-type calcium channels
Wat are the SE of ethosux?
rash, leukopenia, Stevens-Johnson, pancytopenia, agranulocytosis, aplastic anemia
What should be monitoried with ethosux?
LFTs and CBC
What is the MOA of Tiagabine?
GABA transporter inhibitor
What are the SE of tiagabine?
poor responder rate
GI upset, cognitive, dizziness, ataxia, tremor, requires frequent dosing
What is the MOA of Gabapentin?
increases post-synaptic Gaba
What is the MOA of Lamotrigine?
inhibits sodium channels and glutamate release
How is LMG affected by OCPs?
OCPs can actually change the half life of LMG
What are the 3 MOA of ZNS?
GABAa agonist
inhibits sodium channels
inhibits T-type calcium channels
What is the most feared SE of Vigabatrin?
retina toxicity causing field cus
How does Vigabatrin work?
GABA transaminase
What are the SE of Pregabalin?
drowsiness, ataxia, dizziness, weight gain, euophoria
What is propofol infusion syndrome triad?
severe hypotension
lipidemia
metabolic acidosis
Why does estrogen have a proconvulsant effect?
It downregulates GABAa receptor synthesis
acts as NMDA agonist
What is the localization of an early unforced head turn?
the ipsilateral temporal lobe
What is the abnormality in PARK1 gene?
mutation in alpha synuclein
early onset parkisonism and dystonia
autosomal dominant inheritance
What is the abnormality in PARK2 gene?
early onset parkinsonism and dystonia
autosomal recessive
may be responsible for up to 1/2 cases of PD
most common hereditary cause*
What are the side effects of Sinemet?
nausea (make sure at least 100-150 mg/day of carbidopa)
dyskinesias
hallucinations
orthostatic hypotension
psychosis
What are the side effects of dopamine agonists?
non-ergot derivatives: pramipexole & ropinorole
nausea
less dyskinesias than levodopa
sleep attacks & sedation
peripheral edema
more likely than levodopa to cause hallucinations, psychosis, orthostatic hypotension
What are the side effects of ergot derivative dopamine agonists?
pulmonary, retroperitoneal vavlcular fibrosis?
What are the side effects of COMT inhibitors?
Tolcapone- fatal hepatoxicity
Entocapone
nausea, GI upset
urine discoloration
can predispose to dyskinesias
What are the side effects of anticholinergic agents?
dry mouth, urinary retention, constipation, confusion; can be helpful in tremor (trihexyphenidyl)
What are the side effects of MAO inhibitors?
serotonin syndrome* (low prevalence rates despite concomitant use in many PD patients ADAGIO, DATATOP)
nausea, GI upset, urine, orthostatic hypotension, confusion
What is dopamine dysregulation syndrome?
punding, hypersexuality, occuring mostly with dopamine agonists
What are the SE of amantadine?
can cause livedo reticularis, per Ondo one of worse offenders for confusion/psychosis/hallucinations
What are some clinical features that may suggest MSA rather than PD?
early prominent autonomic features (orthostatic hypotension, urinary incontinence- some even do EMG of ext. urthra or anal sphincter to look for long duration, high amplitude units consistent with denervation of Onuf's nucleus)
What is the histopathologic feature in MSA?
glial cytoplasmic inclusions
What is the histopathologic feature in PSP?
globose neurofibrillary tangles
Is PSP a synucleinopathy or tauopathy?
tauopathy like CBGD
What type of dystonia may be present in MSA?
antecollis
What type of dystonia or axial findings in PSP?
early axial regidity prominent hyperextension on walking
What findings can be seen on imaging for MSA?
putaminal hypointensity
What findings can be found on imaging for PSP?
mickey mouse midbrain
What are the characteristic clinical features of CBD?
alien limb phenomenon
*very asymmetric (dystonia, apraxia, myoclonus, ocular aparaxia, speech apraxia)
ideomotor apraxia
cortical sensory loss
What is the characteristic histopathologic finding of CBGD?
tau positive intraneuronal inclusions
What is the differential diagnosis for symmetric basal ganglia calcification?
Vascular: parinatal anoxia
Infxn: ToRCH, HIV, TB, cysticercosis
Trauma/toxins: perinatal anoxia, lead, metotrexate, radiation
Autoimmune: none
Metabolic: Hypercalcemia, hypoparathyroidism, Amyloid angiopathy, Fahr's disease, Wilson's disease
Idiopathic
Neoplastic
Hereditary: Cockayne syndrome: pigmentary retinal degen, sensorineural hearing loss, dwarfism/short stature, psychomotor retardation, bilateral basal ganglia calcification
What are the characteristics of Fahr's disease?
progressive dementia
parkisonism
schizophrenia/psychosis (if presenting age 20-40)
bilateral basal ganglia calcification
usually sporadic, can be AD chr. 14
Where is the calcification in Fahr's disease?
could be basal ganglia, dentate nucleus and periventricular region
What is the presentation of NBIA type 1 or PKAN?
movement disorders: dystonia, rigidity, gait imbalance,, parkinsonism in adults
progressive dementia
psychosis & personality change
(other: dysarthria, choreoathetosis, retinits pigmentosa, optic n. degen, sz, pyramidal signs)
What is the characteristic imaging finding on MRI for PKAN or NBIA type 1?
eye of the tiger sign, bilateral low density basal ganglia on CT
What are the clinical characteristics of Wilson's disease?
most commonly cranial motor features: (dysarthria, dysphagia, rhisus sardonicus, sialorrhea)
dystonia, parkinsonism, chorea, classic wing beating tremor with change in kinetic
liver disease
neuropsychiatric symptoms
What are the characteristic imaging findings on CT of
Wilson's disease?
hypodensity of putamen bilaterally
What are the characteristic findings of Wilson's disease on MRI?
hyperintensity of dentate nucleus, thalamus and brainstem
What are the toxic exposures that may lead to PD?
mangagnese (miners, industrial workers)
MPTP
herbicides
pesticides
well water
What are the characteristic imaging findings on T1 for manganese toxicity?
bilateral T1 hyperintense signal in pallidum
What are the long-term effects of cyanide toxiciy in patients who survive the acute phase?
parkinsonism
optic atrophy
cerebellar ataxia
myelopathy
peripheral neuropathy
What are the imaging findings in patients with cyanide toxicity?
bilateral T1 hypointensity in pallidum
bilateral T2 hyperintensity in pallidum
What is the differential diagnosis of Parkisonism?
Vascular: vascular parkinsonism, post-anoxic
Infxn:
bacteria: syphilis, trophyrema whipplei
viral: HIV, SSPE, coxsackie virus, polio, western equine, vzv
creutzfeldt jakob disease
Toxins/Trauma: Dementia pugilistica, MPTP, CO, Methanol, Manganese, pesticide, well water, miners, welders, cyanide
Autoimmun: Encephalitis Lethargica or Von Economo's encephalitis
Metabolic: Fahr's disease, hypoparathyroidism, central pontine myelinolysis
Iatrogenic-tardive dyskinesias
Neoplastic paraneoplastic (autoantibodies)
Degenerative: - (uam-Dementia-Parkinsonism; FTD-17 Parkinsonism
What is the primary generator of tremor in ET?
the cerebellum/red nucleus
What is the starting dose & max dose of propranolol for ET?
40 mg/day up to 320 mg/day maximum
What is the effectiveness of propranolol for ET?
60% reduction in tremor
What is the starting/max dose of primidone?
50 mg/day up to 750 mg/day
What is the effectiveness of primidone for ET?
60% reduction in tremor
What are the side effects of propranolol?
AV block, depression, impotence, mask hypoglycemia, worsening asthma or copd
What are the side effects of primidone?
acute rxn: nausea, dizziness
metabolized to barbiturate
cognitive slowing
How can one distinguish PD tremor from essential tremor?
re-emergence of tremor (in PD) and improvement of ET with alcohol
What is orthostatic tremor?
occurs in elderly patients > 60
sensation of falling, instability dizziness
high-frequency 13 to 18 HZ tremor of legs can move to trunks
What is the treatment for orthostatic tremor?
benzodiazepines, primidone, vpa and GBP
What are the features of dystonic tremor?
irregular tremor, focal, kinetic & postural; response to sensory tricks
What is etiology of Holmes tremor?
involve nigrostriatal pathway, midbrain and cerebellar output
What is Guillan Mollaret triangle?
Dentate nucleus via SCP cross red nucleus, CTT ipsi inferior olive to DN via ICP contra
What are the clinical characteristics of Holmes tremor?
low frequency < 5 HZ, high amplitude proximal > distal tremor present at rest & with action, posture, difficult to treat
What is the etiology palatal tremor?
generated from guillan mollaret triangle
What are the features of hereditary geniospasm?
infants/early childhood, chin tremor without any other abnormalities, benign prognosis
What type of tremor is characterized by FXTAS?
premutation with intention tremor in older men with gait instability
What is titubation?
low frequency oscillation involving the axial musculature
What is parakinesia?
chorea that may seem semipurposeful and may blend into purposeful movements
What is the etiology of DRPLA?
chroosome 12 unstable trinucleotide CAG repeat expansion
What are the clinical features of DRPLA?
progressive myoclonic epilepsy
cognitive decline
dystonia, chorea, parkinsonism
usually Japan, Af-Am, North Carolina family
onset in 30s
What are the clinical features of amyotrophic choreoathetosis with acanthocytosis? ?
chorea *orofacial dyskinesias, tics, dystonia, parkinsonism
cognitive decline
neuropsychiatric manifestations
McLeod
muscle weakness, wwasting
seizures
acanthocytes on pbs
onset 20s-30s
What are the clinical features of McLeod's syndrome?
acanthocytosis
chorea, dystonia, parkinsonism, *orofacial dyskinesias (less prominent that other neuroacanthocytosis)
neuropsychiatric manifestations
peripheral neuropathy
myopathy
seizures
What is the inheritance of McLeod's syndrome?
X linked recessive
What are the features of hereditary choreoacanthocytosis?
chorea
acanthocytes on pbs
dystonia, parkinonism
neurpsych manifestations
axonal neuropathy
What is the method of inheritance of heredtiary choreoacanthocytosis?
AR chromosome 9
What are the clinical features of sydenham's chorea?
female:male predominance
months after onset of strep A infxn
motor and vocal tics, chorea can be hemichorea which generalizes, oculogyric crisis, personality changes
What is prognosis of sydenhan's chorea?
self limited resolves after 8-9 months
What is the differential diagnosis of chorea?
Vascular- strokes <1% (ischemic or hemorrhagic)
infarcts related to infxn: TB
moyamoya
vasculitis
Infectious: bacterial mening
aseptic mening
TB mening with infarcts
Toxoplasmosis
post-infxn Sydenham's chorea
Trauma/Toxins: Hemorrhagic? Toluene, Manganese CO
Autoimmune: SLE, Antiphospholipid, Behcet's, HSP
Metabolic: Hyperthyroidism, HONK, pregnancy
Iatrogenic: Tardive, Sinemet, lamotrigine, oral contraceptives, LI, methoadone, Cocaine, Amphetamines
Neoplastic:Paraneoplastic anti-Hu
Degenerative: Huntington's disease, Neuroacanthocytosis, DRPLA, benign familial chorea, senile chorea,
What are the histopathologic findings of NBIA type 1?
excess irone dposits, spheroids, neuronal loss, widespread alpha synuclein
What is eye of the tiger sign on MRI?
high signal anteromedially surrounded by low signal
What are Opalski cells?
rounds cells with eo cytoplasm an small central nucleus assoc with hepatic encephalopathy
What type of astrocytes are associated with hepatic abnormality?
alzheimer type II astrocytes
What is the most common hereidtary dystonia?
ad DYT1 rarly onset dystonia, GAG deletion, tosin A
What is DYT 11 or myoclonus-dystonia?
epsilon-sarcoglycan gene
chromosome 18
first decade onset
sensitive to alcohol, tend to have depression/mood disorder/substance abuse
head, arm, upper body affected
imprinting
What is DYT5 or DRD?
AD chromosome 14
GTP cyclohydrolase 1 sysnthesis of tetrahydrobiopterin
diurnal fluctuations
marked response to levodopa
What is DYT 10?
parosymal kineseogenic dyskinesia
dystonia, chorea lasting a few seconds to 5 minutes
precipitated by sudden movements
treat with na channel blockers
In the context of crime for homicide, the presence of a deadly weapon does what?
Creates an inference of an intent to kill.
What is paroxysmal exertional dyskinesia?
episodes lasting 5 to 30 minutes, induced by exericse, longer than dyt 10 lasting 5 to 30 minutes
different tx: diamox or bzdz
What is DYT3?
X linked dystonia-parkinsonims
40s-50s
poor response to tx
What is Niemann Pick disease type C?
cholesterol metabolism disorderwith dementia, cataplexy, dysarthria, dystonia, dysphagia, vertical supranuclear gaze palsy and sz; preschool age also with hepatosplenomegaly
How can one distinguish between adductor variety and abductor variety of spasmodic dysphonia?
adductor strained
abductor breathy
What are the characteristics clinically of acute dystonic reactions?
abrupt onset of dystonic posturing, oculogyric crisis within 2 to 24 hours after first dose of drug, painful, can treat with benztropine or benadryl but stop drug and should resolve spontaneously
What is the hereditary etiology of hyperekplexia?
AD mutation in glycine receptor chromosome 5q
What is the genetic etiology of sialidosis type I and II?
autosomal recessive chromosome 6
Whatis the etiology of GM2 gangliosidoeses?
beta hexosamindiase
What is the etiology of Lafora's disease?
autosomal recessive, chromosome 6
onset 20s
dementia, myoclonus, seizures
diagnosis by eccrine sweat gland ducts on biopsy
What is the cause of withdrawal emergent syndrome?
abrupt withdrawal of neuroleptics
nonstereotypic choreic hyperkinetic movmeents, parkinsonism
What is painful legs nad moving toes syndrome?
peripherla nerve trauma, peripheral neuropathy, radiculopathy, diopathic
pain precedes movement d/o
mild and can treat with anticonvulsants (gbp), tca, dopaminergic, sympathetic blockades (usually disappointing results)