L. Milandre et al., ISOLATED DEFECT IN MOTIVATED BEHAVIOR FOL LOWING BILATERAL STRIATO-CAPSULAR INFARCT CAUSED BY ADULT-ONSET MOYAMOYA DISEASE, Revue neurologique, 151(6-7), 1995, pp. 383-387
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.