Epilepsy Syndrome-Childhood

Question 1

Genetic Epilepsy With Febrile Seizures Plus

Answer 1

AD

• genes such as SCN1A (overlap with Dravet)
• infancy or childhood (6 months to 6 years)

Clinical:
1.) range of phenotype; simple febrile to mixed febrile, afebrile seizures that are prolonged, focal

2. ) Severe end of Specturm: Dravet syndrome or myoclonic-astatic epilepsy (doose syndrome)
3. ) normal neurologically
4. ) Hippocampal sclerosis
5. ) at least 2 family members must be affected

***avoid sodium channel blockers such as phenytoin, lamotrigine, carbamazepine, oxcarbamazepin

Question 2

Doose syndrome (Myoclonic-Atonic Epilepsy)

Answer 2

• large amplitude symmetric myoclonic jerks (arms, legs, neck, shoulders, head drop), followed by loss of muscle tone and fall
• could be part of GEFS spectrum

EEG: recurrent paroxysms of generalized spike or polyspike and wave; myoclonic jerks associated with 2-4Hz

Treatment: VPA, benzos, ethosuximide, lamotrigine, Topamax, etc,
-ketogenic diet

Question 3

Benign Epilepsy with Centrotemporal spikes (benign Rolandic epilepsy)

Answer 3

7-8 years
Clinical Features:

1.) shortly after sleep onset or just before awakening

2.) begin with paresthesia on one side of the
tongue or mouth–>followed by dysarthria or gagging-type noises, jerking of the
ipsilateral face, and excessive drooling
-duration brief; may to go secondary generalization

EEG:

1.) high-voltage sharp or blunt
spike discharges involving either centrotemporal region

2.) dipole with anterior positivity and
centrotemporal negativity

3.) no poistictal slowing

Question 4

Panayiotopoulos Syndrome aka early-onset childhood occipital epilepsy

Answer 4

benign childhood occipital epilepsy
-3-6 years of age
-Autnomic: recurrent vomiting with seizure onset during sleep, bowel/bladder incontinence, pallor, pupillary changes, syncope
→ events can be prolonged (upto hours)
→ eye deviation with preserved consciousness
→ secondary generalization may occur

EEG: occipital spikes (may be multifocal)

Question 5

Late-onset Childhood Occipital Epilepsy (Gastaut type)

Answer 5

8-11 years 
Clinical features: 
1.) transient vision loss
2.) visual hallucinations
3.) eye deviation
4.) eye flutters 
5.) ocular pain 

EEG: bilateral occipital spike-and-eave discharges→ precipitated by eye closure and attenuated by eye opening

Question 6

Childhood Absence Epilepsy

Answer 6

4-10 years
Clinical:
1.) Abrupt impairment of conciousness
-a/w behavioural arrest, staring, eye flutter, or automatisms
2.) EEG: Generalized bilateral synchronous 3-4Hz spike and wave discharges

Treatment: Ethosuximide, VPA, clonazepam, lamotrigine

Question 7

Generalized Epilepsy With Eyelid Myoclonia (Jeavons Syndrome)

Answer 7

Clinical:

1. ) Eyelid myoclonia with absences
2. ) brief episodes with upward eye deviation and eye blinking
3. ) triggered by eye closure

EEG: 3 Hz to 6 Hz polyspike-and-wave
complexes;

Treatment: Ethosuximide, VAP, benzos