Nervous System Diseases: JME

Introduction

JME (Juvenile Myoclonic Epilepsy) is not an uncommon type of epilepsy and it belongs to the category of idiopathic generalized epilepsy syndromes.

The following are some of the features of idiopathic generalized epilepsy syndromes;

• Patients do not have mental retardation
• Seizures originate from both cerebral hemispheres simultaneously
• Seizures are not secondary to structural pathologies
• Genetic linkage is high in this category of epilepsy syndromes

Who gets Juvenile Myoclonic Epilepsy?

As the name implies the seizures usually start manifesting around the juvenile or adolescent age. Probably there are no significant gender differences or only a minor one. This disease affects all races and ethnicities.

What cause it?

It is a hereditary disease with possibility of AD (autosomal dominant) inheritance. Several genetic mutations have been identified.

What is the clinical picture?

Patients may experience 3 different kinds of seizures and all are idiopathic generalized types. They are myclonic, generalized tonic clonic (GTC) and absence (petit mal) type seizures.

Diagnosis

Diagnosis is primarily done on clinical basis. EEG helps significantly too. It may show generalized burst of spikes and polyspike activity at > 3 Hz/second (fast spike & slow wave complexes). The presence of polspikes indicate underlying tendency to have myoclonic seizures.

CT or MRI is most often not required and they are expected to be normal.

Treatment

The following anti seizure medicines are usually tried;

• Sodium valproate
• Lamotrigine
• Topiramate
• Levetiracetam
• Zonisamide etc

Patients respond very well to seizure medications generally but on discontinuation of AEDs the seizures may recur, so long term AED treatment is common for this condition.

A neurologist is frequently involved in the care of Juvenile Myoclonic Epilepsy patents.

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