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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
OBJECTIVE
To 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.
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