Idiopathic epilepsy and cortical tremor of adult onset Volume 16, issue 1, Janvier-Février-Mars 2004

Clinique neurologique, 1, place de l‘Hôpital, 67091 Strasbourg Cedex

Several Japanese and European families have been reported with adult‐onset tremulous finger movements, myoclonus and infrequent generalized seizures, with a nonprogressive course. More recently, partial seizures and mental retardation were also observed in the pedigree of such families. The electroencephalogram studies showed bilateral posterior or diffuse spike‐and‐waves complexes and sometimes a photoparoxysmal response ; the cortical reflex myoclonus was defined according to giant somatosensory evoked potential, enhancement of C‐reflex and jerk‐locked premyoclonus spikes. Based on evidence of autosomal dominant inheritance, genetic analysis showed linkage to chromosome 8q23.3‐24.1 or 2p11.1‐q12.2 in some families. In this article, we reviewed the main clinical, neurophysiological and genetic data related to so‐called Familial Adult Myoclonic Epileptic syndromes.