Cerebral blood flow deficits in hereditary essential myoclonus.
Hereditary essential myoclonus is a disease in which segmental myoclonus is the sole clinical abnormality and whose cause is unknown. It is characterized by an early onset, a benign course, an autosomal dominant pattern of inheritance, the absence of any other neurologic dysfunction, and normal results of auxiliary tests. Cerebral blood flow studies of a father and son with this disease showed a cortical blood flow reduction contralateral to the myoclonus symptoms. We postulate the cause to be a focal unilateral subcortical cerebral lesion, either in the basal ganglia or in the brain stem, with subsequent cortical deafferentation.
Delecluse
F
F
Department of Neurology, Hopital Erasme, Brussels, Belgium.
Waldemar
G
G
Vestermark
S
S
Paulson
O B
OB
D000328:Adult; D001921:Brain / Q000000981:diagnostic imaging; D002560:Cerebrovascular Circulation; D002648:Child; D006801:Humans; D008297:Male; D009207:Myoclonus / Q000235:genetics / Q000503:physiopathology*; D015899:Tomography, Emission-Computed, Single-Photon