I. Litvan et al., NEUROPSYCHIATRIC ASSESSMENT OF PATIENTS WITH HYPERKINETIC AND HYPOKINETIC MOVEMENT-DISORDERS, Archives of neurology, 55(10), 1998, pp. 1313-1319
Background: The role of the basal ganglia in neuropsychiatric behavior
s is not well known. Anatomical, neurophysiological, and neurochemical
evidence supports the notion of parallel direct and indirect basal ga
nglia thalamocortical motor systems, the differential involvement of w
hich accounts for the hypokinesia or hyperkinesia observed in basal ga
nglia disorders. Objectives: To evaluate the neuropsychiatric manifest
ations of patients with a hyperkinetic movement disorder, such as Hunt
ington disease (HD), vs a hypokinetic disease, such as progressive sup
ranuclear palsy (PSP). To verify if patients with HD show a greater fr
equency of hyperactive behaviors leg, agitation, irritation, euphoria,
or anxiety), while those with PSP exhibit hypoactive behaviors leg, a
pathy). Patients and Methods: The Neuropsychiatric Inventory, a tool w
ith established validity and reliability, was administered to 29 patie
nts with HD (mean +/- SD age, 43.8 +/- 2 years) and 34 with PSP (mean
+/- SD age, 66.6 +/- 1.2 years), matched for education, symptom durati
on, and overall degree of dementia. Results: There was no difference b
etween the groups in the total Neuropsychiatric inventory scores. Howe
ver, there was a double dissociation in behaviors: patients with HD ex
hibited significantly more agitation (45%), irritability (38%), and an
xiety (34%), whereas patients with PSP exhibited more apathy (82%) (P<
.01). Euphoria was present only in patients with HD. Conclusions: We f
ound that patients with HD manifested predominantly hyperactive behavi
ors, while those with PSP manifested hypoactive behaviors. Based on ou
r findings and the anatomical lesions known to occur in these disorder
s, we suggest that the hyperactive behaviors in HD are secondary to an
excitatory subcortical output through the medial and orbitofrontal co
rtical circuits, while in PSP the hypoactive behaviors are secondary t
o hypostimulation.