ISOLATED DEFECT IN MOTIVATED BEHAVIOR FOL LOWING BILATERAL STRIATO-CAPSULAR INFARCT CAUSED BY ADULT-ONSET MOYAMOYA DISEASE

A 49 par-old woman with no medical history suddenly presented bilatera l striatocapsular infarct causing frontal-like behavioural disturbance s associating inertia with loss of drive, interest and affect, and pre servation of intellectual function (''athymhormic syndrome'' or ''loss of psychic self-activation''). Ischaemic lesions mainly affected righ t globus pallidus a,ld left lentiform nucleus with no involvement of t he caput of the nuclei caudati. Such changes were close to anoxic lesi ons known to cause tile same symptomatology. Infarction was attributed to a Moyamoya disease on angiographic data. Bilateral involvement of basal ganglia, arterial borderzones or both, may explain tile frequent occurrence of neuropsychological disturbances in Moyamoya disease.