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Kiyoshi Shikino1,2, Shoichi Ito2,3, Yoshiyuki Ohira,1 Kazutaka Noda,1 Mayumi Asahina2,3, Masatomi Ikusaka1,21Department of General Medicine, Chiba University Hospital, Chiba, Japan; 2Health Professional Development Center, Chiba University Hospital, Chiba, Japan; 3Office of Medical Education, Chiba University Graduate School of Medicine, Chiba, JapanObjective: To evaluate a short-time simulation training seminar on how to handle difficult patients using professional simulated patients (SPs) such as actors.Participants: Sixty-three second-year residents at Chiba University Hospital between 2015 and 2017 who only attended the seminar once.Intervention: The participants were divided into small groups, each of which was assigned a supervisory doctor as facilitator. Those who were playing the doctor’s role enacted a medical interview with an SP. After the interview, the facilitator, the SP, and the observing residents participated in a debriefing while watching a recorded video of the interview.Outcome measures: Pre- and post-seminar questionnaires using a 7-point Likert scale (from 1: strongly disagree to 7: strongly agree) were used to examine the differences in “confidence in ability to handle difficult patients” and “learning motivation to handle difficult patients”. The two items examined by both pre- and post-seminar questionnaires, were analyzed by a paired t-test. The residents were also surveyed on their satisfaction with the seminar, acquisition of new knowledge, and impressions and comments (free-text answers).Results: The findings of the questionnaire showed a significant post-seminar increase in confidence (3.1±1.6 to 4.0±1.5 [p

Background: Acquired idiopathic generalized anhidrosis (AIGA) leads to heat intolerance due to the loss or reduction in thermoregulatory sweating over an extensive area of the body. The pathomechanism of AIGA is still unclear but is believed to be autoimmune. Objectives: We investigated the clinical and pathological features of inflammatory AIGA (InfAIGA) and noninflammatory AIGA (non‐InfAIGA) within the skin. Methods: We compared anhidrotic and normohidrotic skin samples from 30 patients with InfAIGA and non‐InfAIGA, as well as skin samples of melanocytic nevus as a negative control. We conducted morphometric analysis and immunohistochemical analysis of cell types and expression of inflammatory molecules (TIA1, CXCR3 and MxA). MxA expression was used as a proxy for type 1 interferon activity. Results: We found that tissue samples from patients with InfAIGA exhibited inflammation within the sweat duct and atrophy of the sweat coil, whereas patients with non‐InfAIGA exhibited only atrophy of the sweat coil. Cytotoxic T lymphocyte infiltration and MxA expression were only observed in the sweat ducts of patients with InfAIGA. Conclusions: InfAIGA is associated with increased sweat duct inflammation and sweat coil atrophy, whereas non‐InfAIGA is only associated with sweat coil atrophy. These data suggest that inflammation leads to epithelial destruction of sweat ducts associated with the sweat coil atrophy and subsequent loss of function. Non‐InfAIGA may be regarded as a postinflammatory state of InfAIGA. These observations indicate the contribution of both type 1 and type 2 interferons to sweat gland injury. The mechanism involved is similar to the pathomechanism of alopecia areata (AA). [ABSTRACT FROM AUTHOR]

J Neurol Neurosurg Psychiatry 2004;75:287-291 Objective: To define the nature of micturition disturbance in patients with acute idiopathic autonomic neuropathy (AIAN). Methods: Micturitional symptoms were observed during hospital admissions and [...]

Background: Constipation is a prominent lower gastrointestinal tract dysfunction that occurs frequently in Parkinson's disease (PD). Objective: To investigate colonic transport and dynamic rectoanal behaviour during filling and defecation in [...]

Objectives: Urinary dysfunction and orthostatic hypotension are the prominent autonomic features in multiple system atrophy (MSA). A detailed questionnaire was given and autonomic function tests were performed in 121 patients with MSA concerning both urinary and cardiovascular systems.Methods: Replies to the questionnaire on autonomic symptoms were obtained from 121 patients including three clinical variants; olivopontocerebellar atrophy (OPCA) type in 48, striatonigral degeneration (SND) type in 17, and Shy-Drager type in 56. Urodynamic studies comprised measurement of postmicturition residuals, EMG cystometry, and bethanechol injection. Cardiovascular tests included head up tilt test, measurement of supine plasma noradrenaline (norepinephrine,NA), measurement of R-R variability (CV R-R), and intravenous infusions of NA and isoproterenol.Results: Urinary symptoms (96%) were found to be more common than orthostatic symptoms (43%) (p<0.01) in patients with MSA, particularly with OPCA (p<0.01) and SND (p<0.01) types. In 53 patients with both urinary and orthostatic symptoms, patients who had urinary symptoms first (48%) were more common than those who had orthostatic symptoms first (29%), and there were patients who developed both symptoms simultaneously (23%). Post-micturition residuals were noted in 74% of the patients. EMG cystometry showed detrusor hyperreflexia in 56%, low compliance in 31%, atonic curve in 5%, detrusor-sphincter dyssynergia in 45%, and neurogenic sphincter EMG in 74%. The cystometric curve tended to change from hyperreflexia to low compliance, then atonic curve in repeated tests. Bethanechol injection showed denervation supersensitivity of the bladder in 19%. Cardiovascular tests showed orthostatic hypotension below -30 mm Hg in 41%, low CV R-R below 1.5 in 57%, supine plasma NA below 100 pg/ml in 28%, and denervation supersensitivity of the vessels (alpha in 73%; beta2 in 60%) and of the heart (beta1 in 62%).Conclusion: It is likely that urinary dysfunction is more common and often an earlier manifestation than orthostatic hypotension in patients with MSA, although subclinical cardiovascular abnormalities appear in the early stage of the disease. The responsible sites seem to be central and peripheral for both dysfunctions. [ABSTRACT FROM AUTHOR]

Abstract: 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. [Copyright &y& Elsevier]

We report the design and the performance of the engineering model of the digital signal processing system called the Pulse Shape Processor (PSP) for the Soft X-ray Spectrometer (SXS) onboard the ASTRO-H satellite. The SXS employs an X-ray microcalorimeter system, in which X-ray photons are detected as a heat pulse due to photoelectric absorption. The pixelized HgTe absorbers are cooled down to 50 mK. The required energy resolution is 7 eV (FWHM) at 6 keV. Since the data link to the satellite data recorder is limited to 200 kbit s^-1, the onboard digital processor PSP plays a critical role in achieving the required resolution. The PSP is also the rate-limiting factor for other performance of the SXS, such as maximum count rate and energy range. In this paper, we show the design of the PSP, and show the performance based on a series of laboratory tests performed with the engineering models of the detector and the analog readout electronics. We found that (1) the PSP can register energy in the 0.07–18 keV band [energy range], (2) the energy resolution of the engineering model system, including the detector, analog electronics, and the PSP, is 4.8–5.7 eV at 5.9 keV [energy resolution], and (3) the PSP has sufficient processing power to handle a point-like source fainter than 0.3 Crab [maximum count rate]. These results are expected to be quite similar to those with the flight model, thus the results will be useful for the observation planning using the SXS. [ABSTRACT FROM AUTHOR]

Study design:Cross-sectional, observational, controlled study.Objective:High spinal cord injury (SCI) results in disruption of sympathetic vasomotor control. Vasodilatation as a response to local heating is a biphasic mechanism: the first phase (neurogenic) is mediated by the axon-reflex and is modulated by activity of sympathetic nerves. Our objective was to determine whether the response to heat provocation in trunk dermatomes may provide a measure of vasomotor sympathetic function in SCI.Setting:National Spinal Injuries Centre, Stoke Mandeville Hospital, Buckinghamshire, UK; Autonomic Unit, The National Hospital for Neurology and Neurosurgery, Queen Square, London, UK; Neurovascular Medicine Unit, Imperial College London at St Mary's Hospital, UK.Subjects:A total of 30 subjects were studied; 18 had chronic complete SCI (level C6–T11) and 12 were healthy controls.Methods:Recordings of skin blood flow (SkBF) were obtained with thermostatic laser Doppler probes placed in the upper trunk (at C4) and lower trunk (T10 or T12) dermatomes.Results:SkBF at baseline (SkBFbas) and SkBF at the first peak of vasodilatation (SkBFmax) showed no significant differences between SCI and controls either in upper or lower trunk dermatomes. However, the ratio of SkBFmax/SkBFbas was significantly different in lower trunk dermatomes in SCI at C6–T5 level (7.5±3.5 PU) compared to SCI at T6-T11 level (3.5±1.5 PU) (P<0.01).Conclusion:Measurement of SkBF in response to local heating may provide a safe, noninvasive method to assess integrity of sympathetic spinal pathways to the local vasculature. This may aid the classification of the SCI lesions, as the autonomic component currently is not included in the accepted American Spinal Injury Association scoring.Spinal Cord (2006) 44, 222–226. doi:10.1038/sj.sc.3101837; published online 20 September 2005 [ABSTRACT FROM AUTHOR]

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 investigated LUT function in DLB by evaluting clincal 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 postvoidresidual in all patients, uroflowmetry in fice, and electromyogrophy (EMG) cystometry in seven. Results: All patients had symptoms of LUT: ruinary inconfinence (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 voluem <100 ml. Decreased urinary flow was seen in all fice and detrusor overactivity 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 underactive detrusor (n=1), and detrvsor-sphincler dyssynergia (voiding phase) (n=1) were also seen; 2/3 patients who underwent motor unit potential analysis had neurogenic changes. Conclusion: LUT dysunction is a common feature in DLB, not only due to dementia and immobility, but also to central and peripheral types of somato-autonomic dysfunction. [ABSTRACT FROM AUTHOR]

Objectives: To assess muscarinic acetylcholine receptors (mAChRs) in the brains of patients with progressive supranuclear palsy and Parkinson's disease, and to correlate the cholinergic system with cognitive function in progressive supranuclear palsy and Parkinson's disease.Methods: Positron emission tomography (PET) and [11C]N-methyl-4-piperidyl benzilate ([11C]NMPB) was used to measure mAChRs in the brain of seven patients with progressive supranuclear palsy, 12 patients with Parkinson's disease, and eight healthy controls. All of the patients with progressive supranuclear palsy were demented. The Parkinson's disease group consisted of 11 non-demented patients and one demented patient. The mini mental state examination (MMSE) was used to assess the severity of cognitive dysfunction in all of the subjects. The modified Wisconsin card sorting test (WCST) was used to evaluate frontal cognitive function in the non-demented patients with Parkinson's disease and controls.Results: The mean K3 value, an index of mAChR binding, was significantly higher for the frontal cortex in the patients with Parkinson's disease than in the controls (p<0.01). By contrast, the patients with progressive supranuclear palsy had no significant changes in the K3 values of any cerebral cortical regions. The mean score of the MMSE in the progressive supranuclear palsy group was significantly lower than that in the control group. Although there was no difference between the Parkinson's disease and control groups in the MMSE, the non-demented patients with Parkinson's disease showed significant frontal lobe dysfunction in the WCST.Conclusions: The increased mAChR binding in the frontal cortex of the patients with Parkinson's disease may reflect denervation hypersensitivity caused by loss of the ascending cholinergic input to that region from the basal forebrain and may be related to frontal lobe dysfunction in Parkinson's disease. The cerebral cortical cholinergic system may not have a major role in cognitive dysfunction in progressive supranuclear palsy. [ABSTRACT FROM AUTHOR]

Striatal dopamine D1 receptors were investigated in 11 patients with Parkinson's disease (PD), five patients with striatonigral degeneration (SND) and six age-matched controls by positron emission tomography and carbon-11 labelled SCH23390. The SND patients showed mean 12%, 21%, and 31% declines in the ratios of radioactivity in the caudate, anterior putamen, and posterior putamen compared with that in the occipital cortex. These ratios were not significantly altered in the PD patients. The results may explain the different therapeutic responses to levadopa between SND and PD patients, and this technique might prove useful for their differentiation. [ABSTRACT FROM AUTHOR]

IgG anti-GQ1b antibody was present in a patient with acute ataxia and areflexia without ophthalmoplegia or elementary sensory loss. Sensory nerve conduction studies and somatosensory evoked potentials were normal, but postural body sway analysis showed dysfunction of the proprioceptive afferent system. The clinical presentation and laboratory results for this patient resemble those of Miller Fisher syndrome, except for the lack of ophthalmoplegia. This case may represent part of an IgG anti-GQ1b syndrome. [ABSTRACT FROM AUTHOR]