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Neuro Recap > Epilepsy/sleep > Flashcards

Epilepsy/sleep Flashcards

1
Q

Temporal lobe epilepsy

A

automatisms, altered consciousness, deja vu, complex partials, olfactory hallucinations

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2
Q

Fencers posture

A

associated with frontal lobe epilepsy; external rotation and abduction of the contralateral arm from the shoulder with head turning toward the same side of the arm posture

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3
Q

medication worsens generalized/myoclonic epilepsy

A

gabapentin

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4
Q

Gabapentin MOA

A

L type voltage gated Ca channel. less bioavailable than pregabalin

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5
Q

Complex Febrile seizures

A

> 15 mins, focal features, recurrence <24hr, post-ictal signs, more likely d/t underlying condition. prophylaxis could be indicated such as short term (antipyretics/diazapam) and long term (phenobarb/depakote) . Age range >5 years.

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6
Q

Simple Febrile seizures

A

<15 mins, generalized sz, lack of focality, no persistent deficits, no family hx of szs. Antipyretics only. Age range of 6 months- 5 years

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7
Q

GEFS + genotype?

A

SCN1A which encodes pore forming alpha subunit of the Na channel.

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8
Q

Complex Febrile seizures EEG findings?

A

generalized spike-wave or polyspikes

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9
Q

Rasmussens syndrome (chronic focal encephalitis)

A

intractable focal seizures (epilepsia partialis continua), hemiparesis cognitive dysfunction followed by unilateral encephalitis –> hemispheric cortical atrophy
Antibodies against AMPA receptors (glutamate). Intractable to Rx. Perivascular cuff of lymphocytes, glial nodules in brain tissue.
Rx hemispherectomy

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10
Q

Landua-Kefflner syndrome

A

gradual or sudden loss of the ability to understand and use spoken language. Develop mutism. Auditory agnosia. LKS may also be called infantile acquired aphasia. Usually occurs at night.

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11
Q

Developmental cortical malformations

A

pachygyria, schizencephaly, lissencephaly, porencephaly- in utero ischemic insult

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12
Q

Progressive myoclonic epilepsies (PMEs)

A

lysosomal storage or mitochondrial disorders. Progressive cognitive decline, myoclonus (epileptic/non-epileptic) and seizure, possible mvt disorder

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13
Q

Progressive myoclonic epilepsies (PMEs) examples?

A

Lafora body disease, Unverricht-lundborg syndrome, neuronal ceroid lipofuscinosis, myoclonic epilepsy with ragged red fibers (MRRF) and sialidosis.

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14
Q

PMEs treatment?

A

AVOID DEPAKOTE with mitochondrial variant! may cause fulminant hepatic failure. However, first line is depakote!

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15
Q

Myoclonic epilepsy AED ?

A

Avoid Lamotrigine, gabapentin, carbamazepine, vigabatrin. Safe to use Topomax, depakote

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16
Q

Depakote increases levels of ?

A

warfarin, lamictal

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17
Q

Normal variants?

A

14 and 6 positive spikes, 6 hz spike and wave

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18
Q

Abscence seizure exacerbated by?

A

Gabaergeic drugs

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19
Q

Juvenile myoclonic epilepsy (JME)

A

idiopathic generalized epilepsy. Age range 8-24 yrs. Large-amplitude and bilateral simultaneous jerks. Commonly seen on awakening, clumsy. No LOC.

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20
Q

JME eeg finding/AED choice?

A

EEG reveals generalized 4-6 hz polyspike and wave interictally and trains of spikes ictally
First line is Depakote. Avoid carbamezapine/phenytoin

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21
Q

benign rolandic epilepsy with centrotemporal spikes

A

EEG bilateral centrotemporal spikes. Age 2-13 yrs, resolves during teenage yrs.

Focal motor,sensory,autonomic predominantly face,mouth,throat or extremities usually occurring in sleep

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22
Q

Rolandic first line AED?

A

Carbamezepine

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23
Q

Infantile spasms

A

first year of life. sudden tonic extension or flexion of limbs occurring in clusters shortly after awakening
hypsarrhythmia- high amplitude slows on a background of irregular multifocal spikes.

24
Q

Wests syndrome

A

triad of infantile spasms, hypsarrhthmia, and psychomotor arrest or regression 2/2 intero/postnatal insults, cerebral dysgenesis.
Rx ACTH

25
Q

Phenytoin pharmokinetics

A

zero order kinetics (non-linear) from 10-20ug/ml

Target total level-current total phenytoin level) x (Kg x volume of distribution)

26
Q

Carbemezapine

A

oxidized to 10,11-carbamazepine epoxide metabolite, however trileptal does not!

27
Q

Infantile spasm associations?

A

hypoxic-ischemic injuries, brain malformations or structural abnormalities, chromosomal abnormalities

28
Q

Aicardis syndrome

A

X linked. Presence of infantile spasms, choriorioretinal lacunae, corpus callosum agenesis. Predominant in girls

29
Q

Doose syndrome (myoclonic-astatic epilepsy)

A

Generalized seizures w/myoclonic or astatic components. Loses postural tone and falls. EEG interictal bilateral synchronus irregular 2-3 Hz spike/wave complexes along with parietal rhythmic theta activity

Rx Valproic acid

30
Q

Dravets syndrome (severe myoclonic epilepsy of infancy)

A

Febrile sz in first year of life. Frequent seizures and various seizure types (absence, myoclonic, GTC).

31
Q

Otaharas syndrome (infantile epileptic encephalopathy)

A

<1 yr. Epileptic tonic spasms throughout the day. EEG shows burst suppression pattern. Intractable.

32
Q

Benign myoclonic epilepsy of infancy (BMEI)

A

M>W. Brief myoclonic seizures easily treatable. Myoclonias are brief and isolated, do not cluster like infantile seizures. EEG generalized spikes and waves or polyspikes/waves. Respond well to depakote, prognosis is good.

33
Q

Benign neonatal seizures “fifth day fits”

A

Normal babies develop partial clonic seizures associated with apneic spells around day 5. EEG “theta pointu alternant” pattern. No Rx indicated

34
Q

Early onset childhood occipital epilepsy (panayiotopoulous syndrome)

A

Peak 4 yr. Tonic eye deviation/vomiting, visual aura. GTC during sleep. EEG shows high voltage occipital spkes in 1-3 Hz bursts, which appears with eyes closed. Rx not indicated

35
Q

Lennox-Gastuat Syndrome (LGS)

A

Triad of seizure types (atypical absence, tonic, atonic, myoclonic), EEG w/diffuse slow 2 Hz spike-wave complexes and cognitive developmental impairment d/t uncontrolled szs.
Rx Klonipin and depakote

36
Q

AD nocturnal frontal lobe epilepsy

A

Begin in childhood, persist into adult life. Bizarre episodeic behaviors in context of hypermotor seizures, such as thrashin or jerking. Occur during non-REM sleep, wake up with motor manifestations. EEG is normal
Rx carbamazepine or trileptal

37
Q

Electrical status epilepticus during slow wave sleeps

A

Peak 5 yr. Psychomotor impairment and multiple seizure types that occur during sleep. EEG showing slow spike-wave complexes occuring more often during sleep.

38
Q

Extension of arm seizure focus?

A

Contralateral SMA

39
Q

Unilateral dystonic hand/arm posture sz focus?

A

contralateral temporal lobe

40
Q

Gelastic seizures

A

uncontrollable episodes of laughter in clusters. Originate in hypothalamus 2/2 hamartomas.

41
Q

Complex FS

A

known risk factor for subsequent development of epilepsy, wheras family hx of FS does not increase risk.

42
Q

Unverricht-Lundborg (baltic myoclonic epilepsy)

A

Myoclonic epilepsy and progressive neurological deteriotion. Autosomal recessive gene EPM1 on 21q22 encodes for cystatin B–> initiation of apoptosis.
Presents w/stimulus induced myoclonus which develop into various seizure types. EEG shows spike waves and polyspikes.

43
Q

Myoclonic epilepsy with ragged red fibers (MERRF)

A

mitochondrial disorder. Presents with migraines, short stature,ataxia,cognitive impairment, deafness,epilepsy, elevated lactate.

44
Q

Sialidosis type I

A

PME. Type I caused by defieciency of alpha neuramindase presents in adolescents/adults with action myoclonus/ataxia, GTC, vision loss. Fundoscopic exam shows cherry red spot.

45
Q

Sialidosis type II

A

PME. Type II caused by deficiency of N-acetyl neuraminidase/B galactosialidase. Myoclonus with coarse facial features, corneal clouding, hepatomegaly, skeletal dysplasia and learning disabilities. Autosomal recessive.

46
Q

Lafora body disease

A

Autosomal recessive. Associated gene EPM2A on chromosome 6 q, encoding laforin. Presents with myoclonus, atypical absences, atonic, occipital seizures with transient blindness, ataxia, dyarthria, EEG generalized bursts in occipital lobe. Lafora bodies are periodic- acid schiff positive intracellular inclusion bodies on skin bx.

47
Q

AED in elderly

A

Lower gastric acidity making weakly basic drugs less easily absorbed and weakly acidic drugs more easily absorbed. Volume of distribution with hydrophilic/lipophilic drugs smaller. Hepatic metabolism decreases.

48
Q

Stages of sleep

A

NREM (N1)- 5% of total sleep time. Slow rolling eye movements, attenuation of PDR. Vertex waves/POSTS
NREM (N2)- 45% of sleep. Presence of sleep spindles/K complexes
NREM (N3)-slow wave sleep, 20%. Alpha activity in occipital leads. REM. Atonic EMG on leg.
REM

49
Q

Central sleep apnea syndrome

A

Cheyne stokes. Periods of absent airflow along with cessation of vent effort during sleep, alternating peirods of crescendo-decrescendo resp flow. Not airway obstruction! Comorbid heart failure is underlying issue

50
Q

Sleep terrors

A

non-REM parasomnia. N3. Confused, no recall of waking up vs nightmares

51
Q

Sleep walking

A

non-REM parasomnias.

52
Q

Non-REM parasomnias

A

confusional arousals, sleep walking and sleep terrors. Arising from slow wave sleep or N3

53
Q

REM parasomnias

A

arousal disorders, sleep-wake transition disorders

54
Q

REM sleep behavioral disorder (RBD)

A

highly associated with alpha synucleinopathies such as parkinsons, MSA, dementia w/lewy bodies

55
Q

Kleine-levin syndrome

A

recurrent hypersomnia. Early adolescence. Sleep about 18 hours/day

56
Q

Narcolepsy with cataplexy

A

excessive sleepiness, cataplexy, sleep paralysis, and hypnopompic/hypnagogic hallucinations. Suffer sleep attacks. Loss of hypocretin neurons in the the lateral hypothalamus
Rx SSRIs/TCAs decrease REM sleep

57
Q

Delayed/advanced sleep phase syndrome

A

circadian rhythm sleep disorder.

Neuro Recap (6 decks)

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