Epilepsy: Continuum (Pediatrics) Flashcards

1
Q

West Syndrome:

Onset and semiology

A

<2 years old / clusters of neck/truncal flexion with arm abduction often seen in the AM

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2
Q

West Syndrome:

Clinical triad

A

Psychomotor arrest, hypsarrhythmia and spasms

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3
Q

What is hypsarrhythmia

A

Seen in West Syndrom. High amplitude slow waves on a background of irregular multifocal spikes.

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4
Q

West Syndrome:

Treatment

A

ACTH first line. Vigabatrin in tuberous sclerosis.

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5
Q
Dravet Syndrome  (severe myoclonic epilepsy of infancy):
Onset and Semiology
A

< 2 years old. Hemiconvulsions with myoclonic and atypical absence

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6
Q

Dravet Syndrome: EEG specific ?

A

No

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7
Q

Dravet Syndrome: clinical symptoms and tx

A

regression with ataxia. NEVER sodium channel blockers (scn1a mut). VPA/BZD/LTC are common first line.

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8
Q

Benign epilepsy with centrotemporal spikes (BECTS or benign rolandic):
Onset and Semiology

A

age 7 with focal seizures that can be autnomic and focal involving mouth/throat. Tonic clonic as well.

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9
Q

Benign epilepsy with centrotemporal spikes (BECTS or benign rolandic):
EEG prognosis and tx

A

Centrotemporal BIPHASIC spikes/sharps that worsen in sleep. The are usually fine by age 16. Usually no tx but can use carbamazepine.

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10
Q
Lennox Gastaut (LGS: 
Triad
A

multiple seizure types, cognitive impairment, and slow spike and wave (1.5-2.5 Hz)

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11
Q

Syndrome that can precede LGS

A

West syndrome

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12
Q

LGS:

Tx and Prognosis

A

Can try ACTH, ketogenic diet but very pharmacoresistant with poor outcome.

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13
Q

Childhood Absence epilepsy:

Onset and semiology

A

ages 4-8 (age >9 is juvenile absence)

Staring spells but 30% can have GTC

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14
Q

Childhood Absence epilepsy:

EEG

A

3 Hz spike an wave with no post ictal slowing

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15
Q

Bedside test for childhood absence epilepsy

A

Hyperventilation for 3 minutes

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16
Q

Childhood Absence epilepsy:

Tx and Prognosis

A

Ethosuximide

70 % remit with 30% developing GTC which merits an added AED.

17
Q

JME:

Onset and semiology

A
teenage years
Mycolonic jerks (clumsy in AM) with potential for GTC
18
Q

JME:

EEG

A

4-6 Hz Generalized spike/polyspike and wave discharge that are photosensitive

19
Q

JME:

Tx and Prognosis

A

Lamotrigine or VPA indefinitely . Avoid photic stimuli/alcohol/stress and sleep deprivation

20
Q

What is epilepsia partialis continua and what is it associated with ?

A

continuous focal clonic seizures without impairment of consciousness. Associated with Rasmussen’s encephalitis.

21
Q

Gene for Autosomal dominant partial epilepsy with auditory features

A

LGI1

22
Q

Syndrome with language regression and electrical status epileptics in sleep

A

Landau-Kleffner