Psychopathological alterations associated with symmetrical basal ganglia sclerosis have been well characterized. A preponderance of a so-called organic affective syndrome has been reported (König 1989), but schizophrenic syndromes have also been described, in particular in young patients (Cummings et al 1983). Symmetrical basal ganglia sclerosis may be secondary to ischemia, hypoxia, trauma, intoxications, inflammations, or hyporesp. pseudohypoparathyroidism. Among idiopathic forms sporadic as well as familial ones with dominant and recessive inheritance have been observed (Billard et al 1989).