山中 義崇

Objective: It is difficult to differentiate multiple system atrophy (MSA) from Parkinson's disease (PD) at least in the early stage. Urodynamic study (UDS) is useful in differentiating MSA from PD. We aimed to clarify which UDS parameter was useful in differentiating MSA from PD.Methods: We retrospectively reviewed 273 cases and performed UDS and external anal sphincter electromyography (EAS-EMG) in patients with MSA (n = 182) and PD (n = 91). We analyzed the utility of UDS parameters, including postvoid residuals (PVR), detrusor overactivity (DO), degree of bladder contraction, and mean duration of motor unit potentials (MUPs) in EAS-EMG, for differentiating MSA from PD.Results: PVR > 150 ml during free-flow study strongly indicated MSA rather than PD (OR 8.723, 95% CI 2.612-29.130, p < 0.001). 'Weak detrusor' also suggested MSA, but it was not a statistically significant indicator (OR 10.598, 95% CI 0.359-312.473, p = 0.172). DO and neurogenic changes in EAS-EMG (mean duration of MUPs > 10 ms). did not significantly contribute to the differentiation of MSA from PD.Conclusions: PVR > 150 ml during free-flow study might be more useful than other UDS parameters in clinically differentiating MSA from PD.

Introduction: We aimed to examine temporal changes in health-related quality of life (HRQOL) and its relationship with motor and cognitive functions in patients with Parkinson's disease (PD) after subthalamic nucleus deep brain stimulation (STN-DBS). Methods: In total, 31 patients with PD were enrolled in this study (mean age: 66.7 +/- 0.9 years; mean disease duration: 11.6 +/- 3.7 years). Participants completed the Unified Parkinson's Disease Rating Scale and the Parkinson's Disease Questionnaire-39. Cognitive function was assessed using the Mini Mental State Examination, the Frontal Assessment Battery, and the Montreal Cognitive Assessment. Postoperative evaluation was performed at three months, one year, three years, and five years after surgery; temporal changes in the correlation between HRQOL and motor and cognitive functions were evaluated at all follow-up periods. Results: All patients completed postoperative clinical evaluations after three months, after one year. Of the 31 participants, twelve completed postoperative clinical evaluations after three years and seven after five years. Motor functions showed significant improvement over the five-year follow-up period. The mobility subdomain of the HRQOL worsened whereas the total score did not change significantly over years. Cognitive functions were not significantly impaired during follow-up periods. HRQOL was basically not significantly correlated with motor and cognitive functions during the follow-up period. Conclusions: The mobility subdomain of the HRQOL worsened after surgery, and the improvement in motor functions was basically not correlated with HRQOL after STN-DBS in patients with PD. Cognitive functions were not significantly impaired during follow-up periods. (C) 2017 Published by Elsevier B.V.

Objective To determine the ability of sphincter electromyography (EMG) and post-void residual urine volume (PVR) during a free-flow study and a pressure-flow study (PFS) for distinguishing multiple system atrophy (MSA) from Parkinson's disease (PD). Methods We retrospectively reviewed 241 case records; both urodynamic study and sphincter EMG were performed in patients with MSA (n = 147) and PD (n = 94). Results There was a statistically significant difference (p &lt; 0.01) in the mean PVR during the free-flow study (113.1 +/- 7.5 mL in MSA and 40.4 +/- 3.8 mL in PD), mean PVR during PFS (230.1 +/- 12.6 mL in MSA and 71.7 +/- 6.6 mL in PD), and mean duration of MUP for sphincter EMG (9.3 +/- 0.1 ms in MSA and 7.7 +/- 0.1 ms in PD). The area under the curve used for differentiating MSA from PD was 0.79 and 0.73 for PVR during PFS and the free-flow study, respectively. There was a mean duration of 0.69 ms for the sphincter EMG. Conclusions The present results suggested that PVR was more appropriate than sphincter EMG for differentiating MSA from PD.

Background: MSA is clinically classified into two phenotypes: parkinsonism predominant (MSA-P) and cerebellar ataxia predominant (MSA-C). However, little is known about the differences in urinary dysfunctions between MSA-C and MSA-P. We investigated the differences in urinary and cardiovascular dysfunctions between MSA-C and MSA-P.Methods: We retrospectively reviewed the medical records of patients with MSA diagnosed as having probable or possible MSA according to Gilman's second consensus criteria from January 2007 to September 2013 in our hospital. Data regarding the initial symptoms, onset of urinary symptoms, and results of urodynamic (including anal sphincter electromyography) and head-up tilt tests were collected.Results: A total of 100 patients with MSA, including 59 patients with MSA-C and 41 with MSA-P, were reviewed. Initial symptoms were autonomic (n = 12) and cerebellar (n = 47) in the MSA-C phenotype and were autonomic (n = 14) and parkinsonian (n = 27) in the MSA-P phenotypes. Urodynamic study indicated that bladder contraction was more severely impaired in patients with MSA-P than in those with MSA-C. In the head-up tilt test, the decrease in diastolic blood pressure was significantly larger in the MSA-P phenotype than that in the MSA-C phenotype. Acontractile bladder during the pressure flow study increased likelihood that the phenotype is MSA-P (odds ratio: 6.67; 95% confidence interval: 1.004-44.284; P = 0.050).Conclusions: Urinary dysfunctions were more severe in MSA-P compared with MSA-C. Detailed urodynamic study was helpful for detecting subtle differences between MSA-C and MSA-P.

BackgroundAutonomic urinary dysfunction affects patients with progressive supranuclear palsy (PSP); however, the severity and prevalence of urinary dysfunctions in these patients compared with those observed in patients with Parkinson's disease (PD) and multiple system atrophy (MSA) are unknown.ObjectiveWe compared urinary dysfunction characteristics in patients with PSP, PD, and MSA.Patients and MethodsForty-seven patients who satisfied the probable or possible criteria of the National Institute for Neurological Diseases and Stroke and Society for PSP were assessed using the urinary symptoms questionnaire and the urodynamic study at Chiba and Toho Universities (n = 26 and 21, respectively). The results were compared with those of patients with PD and MSA (n = 218 and 193, respectively).ResultsThe mean disease duration of PSP and the mean age were 2.97 +/- 0.26 and 71.4 +/- 0.88 years, respectively. The mini-mental state examination and frontal assessment battery scores were 22.6 +/- 0.70 and 10.7 +/- 0.49, respectively. Urinary storage and voiding symptoms were observed in 57% and 56% of patients with PSP, respectively. Detrusor overactivity in the urodynamic study was detected in 81% of patients with PSP, which was slightly more than that found in patients with PD (69%) and MSA (67%); however, this was not statistically significant. Postvoid residual volume in patients with PSP was significantly more than that in patients with PD (P < 0.01), but was equivalent to that in patients with MSA.ConclusionsThe present study demonstrated that patients with PSP experienced various urinary dysfunctions. Urinary storage dysfunction in patients with PSP was not different from that in patients with PD or MSA, whereas urinary voiding dysfunction in patients with PSP was milder than that in patients with MSA and more severe than that in patients with PD. These features should be taken into account for the differentiation of PSP from PD and MSA.

Purpose Cutaneous sympathetic pathophysiology in complex regional pain syndrome type 1 (CRPS-1) is not yet completely understood. To evaluate cutaneous sympathetic dysfunction in CRPS-1, we evaluated sympathetic sweat response (SSwR) and skin vasomotor reflex (SkVR) in CRPS-1 patients. Methods We studied 10 CRPS-1 patients (age 41 +/- A 13 years; 5 females and 5 males; disease duration 20 +/- A 22 months) and 10 healthy subjects (age 44 +/- A 13 years; 3 females and 7 males). SkVRs and SSwRs to several sympathetic activating procedures were recorded on the palms of the CRPS-1 patients (affected side) and controls (right side). Results There were no significant differences in the baselines of sweat output and skin blood flow between the CRPS-1 and control groups. SSwR and SkVR amplitudes were significantly lower in the CRPS-1 group than in the control group. There was no significant correlation between disease duration and SSwR or SkVR amplitudes among the patients. Conclusions The reduced SSwRs and SkVRs in the affected limb of our CRPS-1 patients may reflect underlying damage to the sympathetic postganglionic fibres.

Aims Although subthalamic nucleus deep brain stimulation (STN-DBS) is effective in patients with advanced Parkinson's disease (PD), its physiological mechanisms remain unclear. Because STN-DBS is effective in patients with PD whose motor symptoms are dramatically alleviated by L-3,4-dihydroxyphenylalanine (L-DOPA) treatment, the higher preoperative catecholamine levels might be related to the better clinical outcome after surgery. We aimed to examine the correlation between the preoperative catecholamine levels and postoperative clinical outcome after subthalamic nucleus deep brain stimulation. The effectiveness of STN-DBS in the patient who responded well to dopaminergic medication suggest the causal link between the dopaminergic system and STN-DBS. We also examined how catecholamine levels were modulated after subthalamic stimulation. Methods In total 25 patients with PD were enrolled (Mean age 66.2 +/- 6.7 years, mean disease duration 11.6 +/- 3.7 years). Mean levodopa equivalent doses were 1032 +/- 34.6 mg before surgery. Cerebrospinal fluid and plasma catecholamine levels were measured an hour after oral administration of antiparkinsonian drugs before surgery. The mean Unified Parkinson's Disease Rating Scale scores (UPDRS) and the Parkinson's disease Questionnaire-39 (PDQ-39) were obtained before and after surgery. Of the 25 patients, postoperative cerebrospinal fluid and plasma were collected an hour after oral administration of antiparkinsonian drugs during on stimulation at follow up in 11 patients. Results Mean levodopa equivalent doses significantly decreased after surgery with improvement in motor functions and quality of life. The preoperative catecholamine levels had basically negative correlations with postoperative motor scores and quality of life, suggesting that higher preoperative catecholamine levels were related to better outcome after STN-DBS. The preoperative plasma levels of L-DOPA had significantly negative correlations with postoperative UPDRS-III score in off phase three months after STN-DBS. The preoperative cerebrospinal fluid (CSF) 3,4-dihydroxyphenylacetic acid (DOPAC) and 5-hydroxytryptamine (5-HT) levels had significantly negative correlations with postoperative UPDRS-III score in off phase one year after STN-DBS and the preoperative CSF homovanilic acid (HVA) levels had significant negative correlations with postoperative UPDRS-III score in on phase three months after STN-DBS. In PDQ-39 SI (summary index), preoperative plasma dopamine (DA) level had significantly negative correlations with postoperative PDQ-39 SI one year after STN-DBS suggesting that higher preoperative plasma DA level resulted in better quality of life (QOL) one year after STN-DBS. The stepwise multiple linear regression study revealed that higher preoperative plasma HVA levels had negative influence on the postoperative motor symptoms (i.e., increase in the score of UPDRS), whereas higher preoperative CSF L-DOPA levels had positive influence on the postoperative motor symptoms and QOL (decrease in the score of UPDRS and PDQ-39 SI) The catecholamine levels were not significantly reduced postoperatively in 11 patients despite the significant reduction in levodopa equivalent doses. Unexpectedly, CSF HVA levels significantly increased from 0.00089 +/- 0.0003 ng/mu l to 0.002 +/- 0.0008 ng/mu l after STN-DBS. Conclusion The preoperative catecholamine levels might affect the postoperative motor symptoms and quality of life. The catecholamine levels were not significantly reduced postoperatively despite the significant reduction in levodopa equivalent doses.

Introduction. Patients with Parkinson's disease (PD) showed reduced myocardial 123I-MIBG uptake, which may affect autonomic regulation. We investigated correlation between MIBC accumulation and cardiovascular autonomic function in PD. Methods. We performed myocardial MIBG scintigraphy, heart rate variability (HRV) analysis, and the head-up tilt test (HUT) in 50 PD patients (66.4±7.8 years duration 5.5±5.9 years). Autonomic function tests were also performed in 50 healthy controls (66.5±8.9 years). As HRV parameters, a high-frequency power (HF, 0.15-0.4 Hz), a low-frequency power (LF, 0.04-0.15 Hz), and LF/HF ratio were used. Results. Our PD patients had a significant reduction in LF and HF compared with the controls (P=0.005 and P=0.01). In HUT, systolic and diastolic blood pressure falls in the PD group were significantly greater than those in the controls (P=0.02 and P=0.02). The washout rate of MIBG was negatively correlated with blood pressure changes during HUT. Conclusion. Our PD patients showed reduced HRV, blood pressure dysregulation, and reduced MIBG accumulation, which was correlated with blood pressure dysregulation. Orthostatic hypotension in PD may be mainly caused by sympathetic postganglionic degeneration.

Introduction. Patients with Parkinson's disease (PD) showed reduced myocardial I-123-MIBG uptake, which may affect autonomic regulation. We investigated correlation between MIBC accumulation and cardiovascular autonomic function in PD. Methods. We performed myocardial MIBG scintigraphy, heart rate variability (HRV) analysis, and the head-up tilt test (HUT) in 50 PD patients (66.4 +/- 7.8 years; duration 5.5 +/- 5.9 years). Autonomic function tests were also performed in 50 healthy controls (66.5 +/- 8.9 years). As HRV parameters, a high-frequency power (HF, 0.15-0.4Hz), a low-frequency power (LF, 0.04-0.15Hz), and LF/HF ratio were used. Results. Our PD patients had a significant reduction in LF and HF compared with the controls (P = 0.005 and P = 0.01). In HUT, systolic and diastolic blood pressure falls in the PD group were significantly greater than those in the controls (P = 0.02 and P = 0.02). The washout rate of MIBG was negatively correlated with blood pressure changes during HUT. Conclusion. Our PD patients showed reduced HRV, blood pressure dysregulation, and reduced MIBG accumulation, which was correlated with blood pressure dysregulation. Orthostatic hypotension in PD may be mainly caused by sympathetic postganglionic degeneration.

Background: According to Braak staging of Parkinson's disease (PD), detection of autonomic dysfunction would help with early diagnosis of PD. Objective: To determine whether the autonomic nervous system is involved in the early stage of PD, we evaluated cardiovascular and sudomotor function in early untreated PD patients. Methods: Orthostatic blood pressure regulation, heart rate variability, skin vasomotor function, and palmar sympathetic sweat responses were examined in 50 early untreated PD patients and 20 healthy control subjects. Results: The mean decrease in systolic blood pressure during head-up tilt in PD patients was mildly but significantly larger than in controls (p = 0.0001). There were no differences between the 2 groups in heart rate variability, with analysis of low frequency (LF; mediated by baroreflex feedback), and high frequency (HF; mainly reflecting parasympathetic vagal) modulation. However, LF/HF, an index of sympatho-parasympathetic balance, was lower in the PD group than in controls (p = 0.02). Amplitudes of palmar sweat responses to deep inspiration (p = 0.004), mental arithmetic (p = 0.01), and exercise (p = 0.01) in PD patients were lower than in controls, with negative correlations with motor severity. Amplitudes of palmar skin vasomotor reflexes in PD patients did not differ from controls. Conclusions: Our study indicates impairment of sympathetic cardiovascular and sudomotor function with orthostatic dysregulation of blood pressure control, reduced LF/HF and reduction in palm sweat responses even in early untreated PD patients.

Aim: A previous study on a small number of patients showed that low skin temperature of the hands, the so called "cold hands sign", may be useful for distinguishing multiple system atrophy (MSA) from Parkinson's disease (PD). We have further investigated skin temperature of the hand in a larger number of patients. Methods: Skin temperature on the palm was measured in 50 MSA (11 MSA-P and 39 MSA-C patients) and 50 PD patients, and 25 normal healthy subjects. Results: Palm skin temperature was significantly lower in MSA patients (32.0 +/- 2.7 degrees C) than in controls (34.1 +/- 0.9 degrees C, p = 0.0002), but was not different compared with the PD group (32.9 +/- 1.8 degrees C, p = 0.06). Temperatures of &lt;28 degrees C were observed in 3 MSA patients (6%) and none of the PD patients and controls. There was no significant difference in palm skin temperature between patients with and without orthostatic hypotension for each patient group, or between MSA-P and MSA-C patients. Conclusion: The cold hand (&lt;28 degrees C) is a useful marker for distinguishing MSA from PD, but it is not common in MSA patients, and its sensitivity may be low for differentiating between MSA and PD. (C) 2013 Elsevier Ltd. All rights reserved.

Postprandial hypotension (PPH) is a major clinical problem in patients with autonomic failure such as that observed in multiple system atrophy (MSA). The pathophysiology of PPH remains unclear, although autonomic dysfunction and gastrointestinal vasoactive peptides have been suspected to participate in its pathogenesis. We measured blood pressure and plasma levels of glucose, insulin, noradrenaline, neurotensin, glucagon-like peptide (GLP)-1 and GLP-2 before and after meal ingestion in 24 patients with MSA to reveal the roles of the autonomic nervous system and gastrointestinal vasoactive peptides in PPH. We performed a second meal-ingestion test by administering acarbose to evaluate the effects of acarbose (an alpha-glucosidase inhibitor) on PPH and vasoactive peptides in 14 patients with MSA and PPH. We also evaluated blood pressure responses to the head-up tilt test and heart rate variability in all the patients. Severities of PPH and orthostatic hypotension were significantly correlated. Patients with PPH had significantly worse orthostatic hypotension and lower heart rate variability than those without PPH. Postprandial GLP-1 secretion was higher in patients with PPH than in those without PPH. No significant differences were observed in the postprandial increases in plasma levels of glucose, insulin, noradrenaline, neurotensin or GLP-2. Acarbose significantly attenuated postprandial hypotension and tended to decrease GLP-2 secretion. Our results indicate that autonomic failure is involved in the pathogenesis of PPH and confirm that acarbose has a preventive effect against PPH in patients with MSA. Decreased postprandial secretion of GLP-2, which increases intestinal blood pooling, may attenuate PPH in patients with MSA.

Although the clinical symptoms of Machado-Joseph disease (MJD) vary widely, those involving the autonomic nervous system, such as cutaneous sympathetic dysfunction, have rarely been investigated. In addition, there are no reports on cutaneous vasomotor function in patients with MJD. To determine the effects of MJD on cutaneous sympathetic function, we evaluated cutaneous vasomotor and sudomotor responses in the palms of 15 patients (mean age, 49 +/- 15 years; seven men and eight women) who were genetically diagnosed with MJD as well as in the palms of 15 age-matched, healthy controls (mean age, 48 +/- 16 years; nine men and six women). Sweat response was absent in 10 (67 %) patients with MJD, and the mean amplitude of sweat response was significantly lower (p &lt; 0.0001) in patients with MJD than in healthy controls following mental stress (mental arithmetic) and physiological stimuli. Although vasoconstrictive response was absent in three patients with MJD (20 %), there were no significant differences in the mean amplitude of vasoconstrictive response between patients with MJD and healthy controls. These results indicate that patients with MJD have reduced cutaneous sympathetic response, including severely impaired sudomotor functions and mildly affected vasomotor functions.

Aim: We systematically performed autonomic testing on patients with polyneuropathy, organomegaly, endocrinopathy, M-protein and skin changes syndrome (POEMS) to determine whether autonomic function is preserved in such patients. Methods: We studied 17 POEMS patients, 17 diabetic neuropathy (DN) patients and 17 age-matched normal subjects. Blood pressure responses to the head-up tilt test and heart rate variability were used to evaluate cardiovascular autonomic function. Sweat responses and cutaneous vasoconstriction to several stimuli were recorded via the finger tips to estimate cutaneous sympathetic function. In addition, motor nerve conduction studies were performed. Results: Although the results of the autonomic testing were normal in POEMS patients, motor disability was severe, and motor nerve conduction studies provided evidence of extensive axonal loss. The DN patients showed significantly impaired autonomic responses despite mild motor dysfunction. Conclusions: Autonomic function was normal in POEMS patients, indicating the preservation of autonomic fibers and selective involvement of large fibers. (C) 2012 Elsevier B.V. All rights reserved.

It is established that deep brain stimulation of the subthalamic nucleus improves motor function in advanced Parkinson's disease, but its effects on autonomic function remain to be elucidated. The present study was undertaken to investigate the effects of subthalamic deep brain stimulation on gastric emptying. A total of 16 patients with Parkinson's disease who underwent bilateral subthalamic deep brain stimulation were enrolled. Gastric emptying was expressed as the peak time of (CO2)-C-13 excretion (T-max) in the C-13-acetate breath test and was assessed in patients with and without administration of 100-150 mg levodopa/decarboxylase inhibitor before surgery, and with and without subthalamic deep brain stimulation at 3 months post-surgery. The pattern of (CO2)-C-13 excretion curve was analysed. To evaluate potential factors related to the effect of subthalamic deep brain stimulation on gastric emptying, we also examined the association between gastric emptying, clinical characteristics, the equivalent dose of levodopa and serum ghrelin levels. The peak time of (CO2)-C-13 excretion (T-max) values for gastric emptying in patients without and with levodopa/decarboxylase inhibitor treatment were 45.6 +/- 22.7 min and 42.5 +/- 13.6 min, respectively (P = not significant), thus demonstrating levodopa resistance. The peak time of (CO2)-C-13 excretion (T-max) values without and with subthalamic deep brain stimulation after surgery were 44.0 +/- 17.5 min and 30.0 +/- 12.5 min (P &lt; 0.001), respectively, which showed that subthalamic deep brain stimulation was effective. Simultaneously, the pattern of the (CO2)-C-13 excretion curve was also significantly improved relative to surgery with no stimulation (P = 0.002), although the difference with and without levodopa/decarboxylase inhibitor was not significant. The difference in peak time of (CO2)-C-13 excretion (T-max) values without levodopa/decarboxylase inhibitor before surgery and without levodopa/decarboxylase inhibitor and subthalamic deep brain stimulation after surgery was not significant, although motor dysfunction improved and the levodopa equivalent dose decreased after surgery. There was little association between changes in ghrelin levels (delta ghrelin) and changes in T-max values (delta T-max) in the subthalamic deep brain stimulation trial after surgery (r = -0.20), and no association between changes in other characteristics and delta T-max post-surgery in the subthalamic deep brain stimulation trial. These results showed that levodopa/decarboxylase inhibitor did not influence gastric emptying and that subthalamic deep brain stimulation can improve the dysfunction in patients with Parkinson's disease possibly by altering the neural system that controls gastrointestinal function after subthalamic deep brain stimulation. This is the first report to show the effectiveness of subthalamic deep brain stimulation on gastrointestinal dysfunction as a non-motor symptom in Parkinson's disease.

Objective: Sweating on the palms of the hands and soles of the feet, so-called emotional sweating, is considered to be mediated by the limbic system, including the amygdala and anterior cingulate cortex. To reveal involvement of the limbic system in emotional sweating, we evaluated emotional sweating on the palms in patients with limbic encephalitis. Methods: Sweat and skin vasoconstriction responses to arousal stimuli were recorded on the palms of 7 patients with limbic encephalitis caused by viral infection (n = 3) or immune-mediated encephalitis (n = 4). All patients had amnesia, and magnetic resonance imaging revealed mesial temporal lobe lesions, including those on the amygdala, in 6 of these patients. Results: Sweat responses were absent or markedly reduced in patients with limbic encephalitis compared to normal controls following deep inspiration (p&lt;0.05), mental arithmetic (p&lt;0.01), exercise (p&lt;0.05), and tactile stimulation (p&lt;0.01). Skin vasoconstriction responses in these patients were also impaired, but the extent of such impairment was mild compared to that of the sweating reductions. Conclusion: Sweating on the palm was significantly impaired in patients with mesial temporal lesions. Sweating on the palm could be a useful index of limbic function. (C) 2011 Elsevier B.V. All rights reserved.

Objective: To assess the acute cardiovascular response to aquatic exercise in patients with osteoarthritis. Design: Blood pressure (BP) and heart rate (HR) were measured in 13 female patients with osteoarthritis (63.3 +/- 8.4 yrs) during aquatic walking for 40 mins. A double product (DP) value was calculated by multiplying systolic BP by HR to evaluate the workload of the heart. Results: BP and DP increased transiently with a decrease in HR after water immersion. Aquatic walking induced increases in BP, DP, and HR. Furthermore, BP and DP increased sharply with an increase in HR on leaving the water. The mean maximum increases in systolic BP and DP during each process were 23.5 +/- 18.2 mm Hg and 2931.1 +/- 2758.5 mm Hg/min when entering the water, 36.5 +/- 16.5 mm Hg and 4557.2 +/- 3435.1 mm Hg/min during aquatic walking, and 38.5 +/- 18.9 mm Hg and 5132.3 +/- 3228.8 mm Hg/min on leaving the water. Conclusions: Water immersion, aquatic walking, and the process of leaving the water induced marked increases in BP in patients with osteoarthritis.

Objective: Tachycardia is a clinical feature of Machado-Joseph disease (MJD), and it may be attributable to cardiac autonomic dysfunction. We investigated heart rate variability in MJD patients to reveal heart rate dysregulation in MJD. Methods: Power spectral analysis of successive R-R intervals (300 beats) was performed in 10 genetically diagnosed MJD patients and 10 healthy control subjects to obtain frequency-domain measures, including high- (HF: 0.15-0.4 Hz) and low frequency power (LF; 0.04-0.15 Hz) and the ratio of LF to HF (LF/HF) of heart rate variability. In addition, the coefficient of R-R intervals (CV(R-R)) was calculated as a time-domain measure. Results: HF in the MJD patients (26.2 +/- 25.3 ms(2)) was lower than that in the controls (82.1 +/- 82.6 ms(2), p = 0.004). LF was also lower in the MJD patients (70.6 +/- 69.4 ms(2)) than in the controls (106.9 +/- 81.3 ms(2); p = 0.029). There was no significant difference in LF/HF or CV(R-R) between the groups. Conclusion: HF, which is modulated by parasympathetic vagal activity, was low in our MJD patients. The reduced parasympathetic activity may be a cause of tachycardia in MJD patients. (C) 2009 Elsevier B.V. All rights reserved.

Objective: To elucidate the differences among dementia with Lewy bodies (DLB), Parkinson disease with dementia (PDD), and Parkinson disease without dementia (PD), with respect to the involvement of the autonomic nervous system, we clinically investigated the cutaneous and cardiovascular autonomic functions in patients with Lewy body disease. Methods: We studied 36 patients with Lewy body disorders, including 12 patients with DLB (age, 75.4 +/- 5.9 years), 12 patients with PDD (71.0 +/- 6.8 years), and 12 patients with PD (70.9 +/- 4.2 years), and 12 healthy control subjects (69.9 +/- 5.3 years). Sympathetic sweat response (SSwR) and skin vasomotor reflex (SkVR) on the palm were recorded to estimate the cutaneous sympathetic function, and the head-up tilt test was performed and coefficient of variation of R-R intervals (CV R-R) was studied to estimate the cardiovascular function. Results: The patients with DLB, patients with PDD, and patients with PD showed severely reduced SSwR amplitudes, significantly lower than that in the controls. The mean SkVR amplitudes in the patients with DLB and patients with PDD were significantly lower than that in the controls, but not in the patients with PD. The mean decreases in the systolic blood pressure during the head-up tilt test in the patients with DLB and patients with PDD were less than that in the controls. The mean CV R-R value was significantly lower in the patients with DLB. Conclusion: Sudomotor function on the palm may be severely affected in Lewy body disorders, while skin vasomotor function and the cardiovascular system may be more severely affected in dementia with Lewy bodies and Parkinson disease with dementia than in Parkinson disease. Neurology (R) 2009; 73: 59-65

Objective: Certain stimuli evoke increased sweat secretion (sympathetic sweat response: SSwR) and reduced skin blood flow (skin vasomotor reflex; SkVR) in the palm/sole. We evaluated SSwR and SkVR in patients with multiple system atrophy (MSA) and pure autonomic failure (PAF). Methods: SSwR and SkVR on the palm in response to deep inspiration and mental arithmetic were recorded in 11 MSA patients, 11 PAF patients, and 11 healthy controls. In addition, the head-up tilt test was performed. and the coefficient of variation of R-R. intervals (CV(R-R)) was obtained. Results: SSwR amplitudes were significantly lower in the MSA and PAF patients than the controls. SkVR amplitudes in the PAF patients were significantly lower than the controls, but preserved in the MSA patients. In head-up tilt tests, all MSA and PAF patients showed orthostatic hypotension, with similar severity. CV(R-R) was low in the MSA and PAF patients, but a significant difference was found only between the PAIF and control groups. Conclusion: In the MSA patients, SkVR was preserved, but SSwR was diminished. In the PAF patients, both SkVR and SSwR were attenuated. The combination of SkVR and SSwR tests may differentiate MSA and PAF. (C) 2008 Elsevier Ltd. Ail rights reserved.

Aims: Some physical or arousal stimuli induce a rise in sweat secretion (sympathetic sweat response or SSwR) and a reduction in skin blood flow (skin vasomotor reflex or SkVR) to the palm. We recorded SSwRs and SkVRs in diabetic patients and assessed the usefulness of these parameters for evaluating autonomic dysfunction in diabetes. Methods: We studied 42 diabetic patients (58+/-12 years) and 42 normal control subjects (59+/-11 years). Focal sweat secretion and skin blood flow were measured on the palm by a sudorometer and a Doppler flowmeter, respectively. SSwRs and SkVRs to deep inspiration, mental arithmetic, and isotonic exercise were recorded. SSwR amplitude was measured from baseline to peak, and SkVR amplitude (reduction rate) was calculated as: (blood flow reduction/basal blood flow) x 100%. We also conducted head-up tilt tests and R-R interval variation tests (coefficient of variation of R-R intervals or CVR-R). Results: The SSwR or SkVR amplitudes in the diabetic group were significantly lower than those in the control group for any stimulus. CVR-R in the diabetic group was significantly less than that in the control group. The diabetic group showed a significantly greater reduction in systolic blood pressure during head-up tilt compared with the control group. in the diabetic group, there were significant correlations in SSwR or SkVR amplitudes versus blood pressure falls during the head-up tilt test, and CVR-R values. Conclusion: We believe that SSwR and SkVR are useful indexes for the evaluation of autonomic involvement in diabetic patients. (C) 2008 Elsevier Inc. All rights reserved.

Objective: To investigate cutaneous sympathetic functions in carpal tunnel syndrome (CTS) using sympathetic sweat responses (SSwRs) and skin vasomotor reflexes (SVmRs). Methods: In 29 hands (20 patients) with idiopathic CTS, SSwRs were recorded with a sudorometer from the thenar eminence, and SVmRs were used to measure cutaneous blood flow using a Doppler flowmeter placed on the index finger tip. Normal data were obtained from 15 volunteers of similar age. Results: SSwRs or SVmRs were abnormal in 23 (80%) hands; SSwRs were absent in 38%, whereas SVmRs were abnormally decreased in 59%. Autonomic symptoms were present in 18 (62%) hands; finger edema (38%) and dry hand (35%) were frequent symptoms. Autonomic symptoms, and abnormal SSwRs and SVmRs did not correlate with results of nerve conduction studies. Conclusions: Skin sudomotor or vasomotor sympathetic function is frequently impaired in CTS. Susceptibility to compression ischemia may be different in sympathetic unmyelinated and large myelinated fibers. (c) 2008 Elsevier B.V. All rights reserved.

Local heating of nonglabrous skin increases skin blood flow (SkBF) in two phases. The initial peak (P1) is mediated by a sensory-axon reflex and the plateau phase (P2) by local production of substances such as nitric oxide. We evaluated the SkBF response to local heating in 15 multiple system atrophy (MSA) patients with autonomic failure and 12 age-matched healthy controls. The mean ratio of SkBF at P1 to that at baseline (SkBF(P1)/SkBF(base) ratio) in MSA was significantly lower than that in controls (P &lt; 0.01). The mean ratio of SkBF at P2 seemed to be slightly reduced in the MSA patients, compared with controls, although there was no significant difference. The P1 phase is thought to be mediated by a sensory-axon reflex modulated by sympathetic nerve activity. These findings are indicative of the skin sympathetic vasomotor dysfunction in MSA. (c) 2007 Movement Disorder Society

Background: Local heating of non-glabrous skin increases skin blood flow (SkBF) in two phases: the initial peak (P I) is mediated by sensory axon reflex, and the plateau phase (P2) is thought to be mediated by local production of substances including nitric oxide. We evaluated PI and P2 responses in subacute myclo-optico-neuropathy (SMON). Methods: SkBF response to local heating from 32 degrees C (5 min of baseline) to 42 degrees C (at least for 30 min) of the dorsal surface of the hand skin were measured in 7 SMON patients (67.6 +/- 10. 0 years) and 7 normal control volunteers (65.0 +/- 7.4 years) participated. Results: Mean values of SkBF at PI (SkBFP1) and SkBF during P2 (SkBFP2) were significantly lower in SMON patients than in controls (p &lt; 0.05, p &lt; 0.05). Mean SkBFPI/SkBF at baseline (SkBFbase) and Skl3FP2/SkBFbase ratios were significantly lower in SMON patients than in controls (p &lt; 0.01 and p &lt; 0.05, respectively). Conclusions: The SkBF response to local heating was diminished in SMON patients. This may reflect the involvement of the spinal cord, peripheral sensory nerves, and sympathetic post-ganglionic nerves in SMON. (c) 2007 Elsevier B.V. All rights reserved.

In the human palm/sole, mental or physical stimuli induce an increase in sweat secretion and a decrease in skin blood flow (SkBF). However, the central pathways A these responses remain unclear. We measured sweat secretion and SkBF in the cat footpad by electrically stimulating the raphe. Stimulation of the rostra] raphe magnus/pallidus elicited a reduction in SkBF without affecting sweat secretion. Stimulation of the mid to caudal raphe magnus/pallidus elicited an increase in both sweat secretion and SkBF The raphe magnus/pallidus may play a crucial role in skin vasomotor and sudomotor responses, in the cat footpad. (c) 2007 Elsevier Ireland Ltd. All rights reserved.

In a 66-year-old man with autonomic failure, pyrido-stigmine (180 mg/day orally) improved both postural hypotension and underactive detrusor bladder dysfunction. Acetylcholinesterase inhibition may be useful in the management of orthostatic hypotension and bladder dysfunction in autonomic failure patients.

Objective: Dementia of Lewy body (DLB) type is the second commonest degenerative cause of dementia and autonomic dysfunction has been recognised in DLB. Lower urinary tract (LUT) function in DLB has not been fully delineated. We investigated LUT function in DLB by evaluating clinical and urodynamic data. Methods: We examined 11 patients (eight men, three women; age range 65-81; disease duration 2-14 years) with probable DLB. Urodynamic studies consisted of: measurement of postvoid residual in all patients, uroflowmetry in five, and electrorriyograpKy (EMG) cystometry in seven. Results: All patients had symptoms of LUT: urinary incontinence (urgency type/functional type due to dementia and immobility/both urgency and stress type in 7/2/1 patients, respectively); night-time frequency; Urgency; and daytime frequency and voiding difficulty Seven had postvoid residuals, and three had residual urine volume >100 ml. Decreased urinary flow was seen in all five and detrusor pveraetivity in 5/7 patients who underwent flowmetry and EMG cystometry, respectively. Low compliance detrusor (storage phase; n=2; with bethanechol supersensitivity), an underactive detrusor (n=4), an acontracfile defrusor (n=1), and detrusor-sphincfer dyssynergia (voiding phase) (n=1) were also seen; 2/3 patients who underwent motor unit potential analysis had neurogenic changes. Conclusion: LUT dysfunction is a common feature in DLB, not only due to dementia and immobility but also to central and peripheral types of somato-autonomic dysfunction.