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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 .

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