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Medicine

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Neurology

Question
6 out of 18
 

All of the following are true about Juvenile Myoclonic epilepsy, except: (PGI Nov 2012)



A Focal seizures
B Generalized seizures

C Myoclonus
D Responses to sodium valproate

E Spike and waves in EEG

Ans. A

Focal seizures

Epilepsy Syndromes

Epilepsy syndromes are disorders in which epilepsy is a predominant feature and there is sufficient evidence to suggest a common underlying mechanism.

Three important epilepsy syndromes are – (Ref. Hari-18th ed., pg- 3253)

1). Juvenile myoclonic epilepsy (JME) also known as JANZ syndrome. It is an epilepsy syndrome.

It is characterized by:

a. Appearance in early adolescence

b. Bilateral myoclonic jerks that may be single or repetitive

c. Most frequent in morning after awakening

d. Can be provoked by sleep deprivation

e. Consciousness is usually preserved unless myoclonus is specially severe

f. Association with generalized tonic-clonic seizures and absence seizure is seen

g. Benign condition although complete remission is uncommon

h. Seizure responds well to appropriate anticonvulsant medication

i. Family history of epilepsy is common

j. Treatment is with valproate

2). Lennox – Gastaut syndrome –Occur in children and consist of triad of

a. Multiple seizure type (GTCS, atonic, atypical absence)

b. EEG <3HZ spike & wave discharge

c. Impaired cognitive function.

Causes – Developmental ab normal. Perinatal hypoxia/ ischemia, trauma, infection. Response to therapy is poor.

3). Mesial Temporal Lobe Epilepsy – H/O febrile seizure. Family h/o positive. Complex partial seizure

Poor response to therapy. Characteristic hippocampal sclerosis. Surgery is curative.

Causes of epilepsy

a. Neonates (<1month) – Perinatal hypoxia/ ischemia, Intracranial hmg. CNS infection.

b. Infants / children (<12) – Febrile seizures, metabolic. CNS infection, Idiopathic

c. Adolescents (12 – 18) – Trauma, infection

d. Young adults (<35) – Trauma, alcohol

e. Older (>35) – Cerebrovascular disease (Most common cause in elderly), Brain tumor, Degenerative CNS disorders.

f. Epileptogenic factors – Head trauma (50% risk in case of penetrating injury)

Table: Features that distinguish generalized tonic – clonic seizure from syncope

Features

Seizure

Syncope

Immediate precipitating factors

Usually none

Emotional stress, Valsalva, orthostatic hypotension, cardiac etiologies

Posture at onset

Variable

Erect

Transition to unconsciousness

Often immediate

Gradual over seconds

Duration of unconsciousness

Minutes

Seconds

Disorientation and sleepiness after event

Many minutes to hours

Transient

Return of consciousness

After many minutes or hrs

Almost immediately (AIPG 07)

Biting of tongue

Sometimes

No

Incontinence

Sometimes

No

Headache

Sometimes

No

Neurology Flashcard List

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