Few studies have explored the neuropsychiatry symptoms of patients with PSP, a disorder characterized neuropathologically by abundant neurofibrillary tangles in several subcortical nuclei (stria-tum, pallidum, subthalamic nucleus), in addition to the substantia nigra (74). Prefrontal and parahippocampal cortices may also be involved. Locus ceruleus and raphe nuclei are relatively preserved, and noradrenaline and serotonin concentrations are usually not affected (28). Clinical symptoms include supranuclear vertical gaze palsy, akineto-rigid predominant parkinsonism, early postural instability, and subcortical dementia. Some studies of the clinical features in patients with PSP have included neuropsychiatry aspects, although most studies have employed only unstructured assessments based on retrospective review of clinical records. In one study of 52 patients, at the time of diagnosis
45% had memory impairment, 15% were depressed, 23% had personality changes, and 17% emotional lability (75). In another study, caregivers of 437 of the 1184 members of the Society for Progressive Supranuclear Palsy (SPSP) (76) completed a structured questionnaire assessing common symptoms and signs, including cognitive and emotional/personality problems. Personality and emotional problems were uncommon 2 yr prior to the diagnosis. At the time of diagnosis, however, changes in personality appeared in 46% and depression in 44%. Other personality problems, such as losing control over emotions, lack of emotions, and excessive anger, were reported in less than 30% of patients at the time of diagnosis. A UK sample of 187 patients was diagnosed according to the National Institute of Neurological Disorders and Stroke (NINDS)-SPSP criteria for probable or possible PSP. Sixty-two patients were drawn from a prevalence study and examined by the investigators, 49 of these with a structured clinical examination. At onset, 15% had neuropsychiatric features, including memory impairment (7%), personality change (4%), and depression/anxiety/apathy (3%). Apathy was noted in 50% of the patients during the disease course (77). Seven patients had responded to an antidepressive drug. In a group of 24 patients with an autopsy-confirmed diagnosis of PSP, frontal lobe symptomatology, including executive dysfunction and personality changes such as apathy and depression, was the presenting symptom in 8%, 55% had such symptoms at the first visit, and 58% at the last visit (78).
A clinical series of 25 PSP patients were assessed for depression using the BDI. The majority of patients (55%) had scores above the cutoff for probable depression: mild (score 11-17, n = 7), moderate (18-23; n = 3) or severe depression (24 or above, n = 3) (79). Depression scores did not correlate with neuropsychological performance. The proportion of subjects with a BDI score of 18 or more in PSP (24%) is identical to the findings in a large PD population (52). A cutoff score of 16/17 has received empirical support for diagnosing clinically relevant depression in PD (14).
Few studies have focused specifically on neuropsychiatric aspects using standardized assessment procedures. The NPI profile was reported in 61 patients with PSP (49), 13 of whom were diagnosed as definite PSP according to the NINDS-PSP (43) (i.e., autopsy confirmed); the remaining met the criteria for probable (n = 36) or possible (n = 12) NINDS-SPSP criteria, which have high diagnostic specificity. The most common NPI items were apathy (positive score in 84%) and disinhibition (56%), whereas hallucinations and delusions were very rare (Table 1). Compared to age- and gender-matched PD patients, the severity of apathy and disinhibition was higher in PSP than PD patients, whereas hallucinations, delusions, and agitation scores were higher in PD than PSP patients. Interestingly, 44% of the PSP patients were taking at least one anti-parkinsonian agent, usually levodopa, at similar doses as the PD group. Thus, hallucinations and delusions do not seem to occur in PSP even when taking dopaminergic agents. The same group of PSP patients was compared with Huntington's disease and Gilles de la Tourette syndrome, both typical hyperkinetic movement disorders. Patients with PSP, a hypoactive movement disorder, had lower scores on NPI items assessing "hyperactive" behaviors (i.e., agitation, irritability, euphoria, and anxiety), but higher scores on apathy, a "hypoactive" behavior (27,80). Thus, in summary, the neuropsychiatric profile of PSP is characterized by early subcortical dementia and frequent occurrence of apathy and disinhibition, whereas hallucinations, delusions, and agitation are rare, irrespective of whether the patients are treated with anti-parkinsonian agents.
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