Wednesday, August 26, 2009

35 - Juvenile Myoclonic Epilepsy ( Janz syndrome )

Here are some important points about Juvenile Myoclonic Epilepsy :

1. Juvenile Myoclonic Epilepsy is otherwise called Janz syndrome .

2. It usually begins between the ages of 12 and 16 year .

3. It accounts for approximately 5 % of all the epilepsies .

4. Patients note frequent myoclonic jerks on awakening , making hair combing and tooth-brushing difficult .

5. As the myoclonus tends to abate later in the morning, most patients do not seek medical advice at this stage and some deny the episodes .

6. A few years later, early morning generalized tonic-clonic seizures develop in association with the myoclonus.

7. The EEG shows a 4-6/sec irregular spike and wave pattern, which is enhanced by photic stimulation .

8. The neurologic examination is normal.

9. Majority of the patients respond dramatically to valproate, which is required lifelong .

10. Discontinuance of the drug causes a high rate of recurrence of seizures .

MCQs in Pediatrics 2nd edition
MCQs in Pediatrics ; Over 600 Questions with Explanatory Notes

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