Cutaneous sympathetic function and cardiovascular function in patients with progressive supranuclear palsy and Parkinson's disease
Introduction
Progressive supranuclear palsy (PSP) is a neurodegenerative disorder clinically characterized by supranuclear ophthalmoplegia, akinetic-rigid syndrome with dominant axial rigidity, postural instability, pseudobulbar palsy [1], [2], and higher cortical dysfunction [3], [4]. Because the clinical features of PSP sometimes resemble those of Parkinson's disease (PD), it is occasionally difficult to differentiate between the two [5], [6], [7]. Signs and symptoms of autonomic involvement have been seen frequently in PD [8]. However, controversy exists as to whether or not PSP patients present prominent dysautonomia. Some investigators have reported abnormalities of the autonomic nervous system, including orthostatic hypotension [9], [10], impaired heart rate response to orthostatic stress [10], and a diminished pressor response to isometric exercise [9], [10], while other studies did not show prominent autonomic dysfunction [11], [12], [13]. On the other hand, some procedures, such as deep inspiration and exercise, provoke increased sweat output and reduced cutaneous blood flow in the palm. Those responses, respectively, termed the sympathetic sweat response (SSwR) [14], [15] and skin vasomotor reflex (SVR) [16], are useful indexes for the evaluation of cutaneous sympathetic function [14], [15]. However, there have been few reports on cutaneous sympathetic function in PSP. We therefore evaluated sudomotor functions, cutaneous vasomotor functions, and cardiovascular functions in PSP, PD patients and healthy control subjects, and compared the differences in autonomic involvement between these two patient groups.
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Subjects
We studied 12 PSP patients (mean age 70.4±6.3 years, nine males and three females), 13 PD patients (mean age 61.5±7.0 years, seven males and six females), and nine normal controls (mean age 61.1±11.8 years, four males and five females) (Table 1).
All PSP patients satisfied the clinical and research criteria for PSP [17]. The mean duration of the disease was 3.7±2.8 (range 1–10) years. The mean Hoehn and Yahr score, a measure of the severity of parkinsonism [18], was 3.5±0.8 (range 3–5). Six
Results
The mean age was significantly higher in the PSP group than in the PD group (Table 1, p<0.05). There was no significant age difference between the PSP group and the controls. There were no significant differences in disease duration or severity between the PSP and PD groups. The mean score of MMSE was significantly lower in the PSP group (21.9±5.4) than in the PD group (28.8±1.4) (Table 1, p<0.0005). One PSP patient was unable to take the WCST because of the severity of his dementia. The PSP
Discussion
In our study, SSwRs were more severely diminished in the PSP patients than in the PD patients. The major function of sweating is to regulate body temperature (thermoregulatory sweating). A previous study revealed that the degree of impairment of thermoregulatory sweating did not differ significantly between PSP and PD patients [24]. However, sweating on the palm or sole, which is elicited by emotional stimuli, mental stress, or physiological stimuli, is independent of ambient temperature
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