Origin

The tremor can be demonstrated to be of cortical origin, and represents a manifestation of cortical reflex myoclonus.

Classification

Since 1990, a number of similar conditions have been described from Japan, typically characterized by a benign clinical course with rare seizures, normal cerebellar function, absence of dementia, and associated with tremor.

A number of synonyms exist, likely describing the same entity, although genetic loci are not established:

  1. “Cortical tremor” (reported by Ikeda in 1990)
  2. “Benign adult familial myoclonus epilepsy (BAFME)” (reported by Kuwano in 1996)
  3. “Familial cortical myoclonic tremor” (reported by Terada in 1997)
  4. “Familial cortical tremor with epilepsy” (FCTE) (reported by Okuma in 1997)
  5.  “Familial benign myoclonus epilepsy of adult onset” (reported by Okino in 1997)
  6. “Familial adult myoclonic epilepsy (FAME)” (reported by Plaster in 1999)

In addition, similar conditions have been described in European families, namely ADCME with complex partial and generalized seizures and Familial Cortical Tremor, Epilepsy and Mental Retardation. Ultimately, the condition has been termed Familial Cortical Myoclonic Tremor With Epilepsy.

Clinical Features

Although epilepsy is present, the condition may be distinguished from progressive myoclonic epilepsies by very slow progression, normal cognition and infrequent seizures.

Age of onset:

3rd to 7th decades.

Diagnosis:

Differential Diagnosis

Diagnostic Tests

Treatment