FOCAL BRAIN-DYSFUNCTION IN A 41-YEAR OLD MAN WITH FAMILIAL ALTERNATING HEMIPLEGIA

The acute pathophysiologic changes during hemiplegic spells and the lo ng-term outcome of alternating hemiplegia remain obscure. In a 41-year -old male with familial alternating hemiplegia we found an increase in right frontal cerebral blood flow 3 h into a 5-h left hemiplegic epis ode. A repeat high-resolution brain SPECT study performed 26 h after t he resolution of the left hemiplegia revealed normalization of the fro ntal blood flow accompanied by hyperperfusion in the right parietal lo be. An interictal SPECT scan several weeks later showed no asymmetries . Head CT and MRI scans were negative. Neuropsychologic assessment and neurologic examination revealed evidence of a diffuse disorder which predominantly involved the right hemisphere. To our knowledge, there a re no previous correlative studies of serial high-resolution brain SPE CT with MRI, or of detailed neuropsychologic assessment, in adult pati ents with such an advanced course of alternating hemiplegia of childho od.