Progressive myoclonic epilepsy represents a group of epilepsy characterized by tonic clonic seizures at the start with the association of myoclonus secondarily and intellectual deterioration. This epilepsy poses a problem of diagnosis and management in front of a problem of technical platform. The onset of epilepsy is marked most often by generalized tonic clonic or clonic seizures, or absence seizures. Early diagnosis at the onset phase when there are generalized idiopathic tonic clonic seizures can be confused with generalized idiopathic epilepsies. A genetic test and a sweat gland biopsy determine the exact cause of the epilepsies and helps in further diagnosis. The diagnosis of the form of progressive myoclonic epilepsy is based on the conjunction of a comprehensive clinical description, a good knowledge of the genetic, ethnic and geographical background and the evolution of symptoms. Myoclonic syndrome dominates the clinical evaluation, but may remain in the background, behind seizures or signs of cognitive deterioration or sensory symptoms.
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