Juvenile Myoclonic Epilepsy (JME)

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Janz syndrome is another name of juvenile myoclonic epilepsy (JME) and it is one of the most common syndromes of idiopathic generalized epilepsy. In 1867, Herpin was first person who described JME. Generalized tonic-clonic or absence seizures may or may not occur but myoclonic seizures occur compulsory. JME generally occurs in early morning after waking. Children between ages of 12-18 are mostly affected by JME. It gives sudden jerks which involve the arms, legs and shoulders but there is no loss of consciousness. Chances for having JME is slightly grater in female. It is hereditary syndrome and most of patients have family history with epilepsy. Intellectual ability of patient is same as that of normal person. Psychiatric disorders also occur in many patients with JME.

Gene Associated with Juvenile Myoclonic Epilepsy

Many times JME is genetic disorder. Parents with JME have 15% chances of having JME to their child. Gene who is responsible for JME is located on chromosomes 6, 8, or 15. EFHC1 gene is responsible for JME.

Causes for Juvenile Myoclonic Epilepsy

• Sometime seizure may caused by flickering light such as light of TV, video game, blinking light at dance. Seizure occurred because of this reason is called as photosensitive seizures.
  
• Because of calculations or decision-making myoclonic seizure may occur.
  
• There are 15% chances for having JME later if child have childhood absence epilepsy.
  
• People having family members with generalized epilepsy also have chances for JME.

Symptoms for Juvenile Myoclonic Epilepsy (JME)

1. Symptoms generally start in adolescence.
   
2. Twitches or jerks occur without loss of consciousness in early morning.
   
3. Patient or object from hand may fall rarely.
   

Diagnosis for Juvenile Myoclonic Epilepsy

1. Many times depending on patients history diagnosis is made.
   
2. The EEG carried out in sleep and after some time of awakening gives best result. The EEG shows a pattern of irregular spike and wave or polyspike and wave for child having JME disorder.
   
3. Sometimes CT and MRI scans of the brain are also done which most of the time shows normal result and most of the time even not required.

How is Juvenile Myoclonic Epilepsy Treated?

Treatment may reduce seizures but syndrome is for life long.

• Antiepileptic drug sodium valproate (Depakote) is mostly used as it controls all three types of seizure that occurred in JME.
  
• Topiramate, acetazolamide, phenobarbital, lamotrigine or clonazepam are also used as secondary drugs in treatment of JME.
  
• Avoid carbamazepine, ethosuximide and phenytoin because it shows opposite effect and worse your seizure.