J Neurol Neurosurg Psychiatry 1998;65:717-721 ( November )

Neuropsychiatric features of corticobasal degeneration

Irene Litvan,^{a b} Jeffrey L Cummings,^{c d} Michael Mega^c

^a Neuropharmacology Unit, Defense and Veteran Head Injury Program, Jackson Foundation and the Medical Neurology Branch, ^b National Institutes of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Maryland, USA, ^c Department of Neurology, ^d Department of Psychiatry and Biobehavioral Science, UCLA School of Medicine, Los Angeles, CA, USA

Correspondence to: Dr Irene Litvan, NINDS, NIH, Federal Building, Room 714, Bethesda, MD 20892-9130, USA. Telephone 001 301 496 1189; fax 001 301 496 2358.

Received 2 February 1998 and in revised form 27 April 1998; Accepted 7 May 1998

OBJECTIVETo characterise the neuropsychiatric symptoms of patients with corticobasal degeneration (CBD).
METHODSThe neuropsychiatric inventory (NPI), a tool with established validity and reliability, was administered to 15 patients with CBD (mean (SEM), age 67.9 (2) years); 34 patients with progressive supranuclear palsy (PSP) (66.6 (1.2) years); and 25 controls (70 (0.8) years), matched for age and education. Both patient groups had similar duration of symptoms and mini mental state examination scores. Semantic fluency and motor impairment were also assessed.
RESULTSPatients with CBD exhibited depression (73%), apathy (40%), irritability (20%), and agitation (20%) but less often had anxiety, disinhibition, delusions, or aberrant motor behaviour (for example, pacing). The depression and irritability of patients with CBD were more frequent and severe than those of patients with PSP. Conversely, patients with PSP exhibited significantly more apathy than patients with CBD. The presence of high depression and irritability and low apathy scale scores correctly differentiated the patients with CBD 88% of the time. The irritability of patients with CBD was significantly associated with disinhibition (r=0.85) and apathy (r=0.72). In CBD, apathy was associated with disinhibition (r=0.67); disinhibition was associated with aberrant motor behaviour (r=0.68) and apathy (r=0.67); and aberrant motor behaviour with delusions (r=1.0). On the other hand, depression was not associated with any other behaviour, suggesting that it has a different pathophysiological mechanism. Symptom duration was associated with total motor scores (r=0.69). However, total motor score was not associated with any behaviour or cognitive scores.
CONCLUSIONSThe findings indicate that frontosubcortical pathways mediating cognition, emotion, and motor function in CBD are not affected in parallel. Patients with CBD and PSP have overlapping neuropsychiatric manifestations, but they express distinctive symptom profiles. Evaluating the behavioural abnormalities of parkinsonian patients may help clarify the role of the basal ganglia in behaviour.

Keywords: corticobasal degeneration; progressive supranuclear palsy; neurobehaviour

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© 1998 by Journal of Neurology, Neurosurgery, and Psychiatry
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