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Irene Litvan, MD; Jane S. Paulsen, PhD; Michael S. Mega, MD; Jeffrey L. Cummings, MD

Arch Neurol. 1998;55:1313-1319.

Background  The role of the basal ganglia in neuropsychiatric behaviors is not well known. Anatomical, neurophysiological, and neurochemical evidence supports the notion of parallel direct and indirect basal ganglia thalamocortical motor systems, the differential involvement of which accounts for the hypokinesia or hyperkinesia observed in basal ganglia disorders.

Objectives  To evaluate the neuropsychiatric manifestations of patients with a hyperkinetic movement disorder, such as Huntington disease (HD), vs a hypokinetic disease, such as progressive supranuclear palsy (PSP). To verify if patients with HD show a greater frequency of hyperactive behaviors (eg, agitation, irritation, euphoria, or anxiety), while those with PSP exhibit hypoactive behaviors (eg, apathy).

Patients and Methods  The Neuropsychiatric Inventory, a tool with established validity and reliability, was administered to 29 patients 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 duration, and overall degree of dementia.

Results  There was no difference between the groups in the total Neuropsychiatric Inventory scores. However, there was a double dissociation in behaviors: patients with HD exhibited significantly more agitation (45%), irritability (38%), and anxiety (34%), whereas patients with PSP exhibited more apathy (82%) (P<.01). Euphoria was present only in patients with HD.

Conclusions  We found that patients with HD manifested predominantly hyperactive behaviors, while those with PSP manifested hypoactive behaviors. Based on our findings and the anatomical lesions known to occur in these disorders, we suggest that the hyperactive behaviors in HD are secondary to an excitatory subcortical output through the medial and orbitofrontal cortical circuits, while in PSP the hypoactive behaviors are secondary to hypostimulation.

From the Medical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, and the Neuropharmacology Unit, Defense & Veteran Head Injury Program, Henry M. Jackson Foundation, Bethesda, Md (Dr Litvan); the Departments of Psychiatry and Neurology, University of Iowa, Iowa City (Dr Paulson); and the Departments of Neurology (Drs Mega and Cummings) and Psychiatry and Biobehavioral Science (Dr Cummings), University of California at Los Angeles School of Medicine, Los Angeles.

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