Cerebral blood flow deficits in hereditary essential myoclonus
F. Delecluse, G. Waldemar, S. Vestermark and O. B. Paulson
Department of Neurology, Hopital Erasme, Brussels, Belgium.
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.
