Effects of Smile Training on Gait Disturbance in Parkinson's Disease Patient with Neuropsychiatric Symptoms: A Single Case Design.

Objective: To verify the efficacy of smile training in improving gait disturbances in patients with Parkinson's disease (PD) exhibiting neuropsychiatric symptoms.

Methods: A single-case BAB design with three intervention periods (B1, A1, and B2) was used. During periods B1 and B2, 10 min of smile training (facial muscles training and positive thinking training) was performed before the usual exercise therapy.

Gait Video-Based Prediction of Severity of Cerebellar Ataxia Using Deep Neural Networks.

Background: Pose estimation algorithms applied to two-dimensional videos evaluate gait disturbances; however, a few studies have used this method to evaluate ataxic gait.

Objective: The aim was to assess whether a pose estimation algorithm can predict the severity of cerebellar ataxia by applying it to gait videos.

Methods: We video-recorded 66 patients with degenerative cerebellar diseases performing the timed up-and-go test.

Genetic Creutzfeldt‒Jakob disease with 5-octapeptide repeats presented as frontotemporal dementia.

The N-terminus of the PRNP gene normally contains a 5-octapeptide repeat (R1-R2-R2-R3-R4), and insertions at this locus can cause hereditary prion diseases. In the present study, we found a 5-octapeptide repeat insertion (5-OPRI) in a sibling case of frontotemporal dementia. Consistent with previous literature, 5-OPRI rarely met the diagnostic criteria for Creutzfeldt‒Jakob disease (CJD).

Executive dysfunction in patients with spinocerebellar ataxia type 3.

The aim of this study was to assess the cognitive functions of patients with spinocerebellar ataxia type 3(SCA3). We examined 15 patients with genetically confirmed SCA3 and 15 healthy control subjects matched for age, years of education, and intellectual ability. We administered verbal memory (word recall and word recognition) and executive function tasks (word fluency test, forward and backward digit and visual span tests, Kana Pick-out Test, Trail Making Test, and conflicting instructions and a Go/NoGo task from the Frontal Assessment Battery).

A case of pure autotopagnosia following Creutzfeldt-Jakob disease.

A 69-year-old male (N.A.) with Creutzfeldt-Jakob disease showed pure autotopagnosia.

Cognitive dysfunction in patients with spinocerebellar ataxia type 6.

The aim of this study was to assess the cognitive functions of patients with spinocerebellar ataxia type 6 (SCA6). We examined 13 patients with genetically confirmed SCA6 and 13 healthy control subjects matched for age, years of education, global cognitive status, and intellectual ability. We administered verbal memory (word recall and word recognition), executive function (digit span, category and letter fluency, Frontal Assessment Battery, and Trail Making Test-A and B), and visuospatial construction (figure copying) tests.

Analysis of resources assisting in coping with swallowing difficulties for patients with Parkinson's disease: a cross-sectional study.

Background: Malnutrition induced by swallowing difficulties (SD) impairs the quality of life and gives rise to SD-related costs in Parkinson's disease (PD) patients. With results of a swallowing difficulty questionnaire and data of resources specifically obtained such as SD-related costs, caregivers, and dietary therapies, this study is to suggest statistically supported ideas for improvements in arrangements for how participants cope with SD and maintain general well-being.

Methods: We interviewed 237 PD patients.

A Cross-Sectional Study on Socioeconomic Systems Supporting Outpatients With Parkinson's Disease in Japan.

Objectives: We conducted a cross-sectional study to evaluate the socioeconomic systems supporting outpatients with Parkinson's disease (PD) in Japan.

Methods: The study was performed in 2013 at two private hospitals and one clinic in Hokkaido Prefecture, Japan. A survey was conducted with 248 consecutive PD patients, and the data from 237 PD outpatients were analyzed after excluding 11 patients who did not meet inclusion criteria.

Prospective 10-year surveillance of human prion diseases in Japan.

We analysed the epidemiological data and clinical features of patients with prion diseases that had been registered by the Creutzfeldt-Jakob Disease Surveillance Committee, Japan, over the past 10 years, since 1999. We obtained information on 1685 Japanese patients suspected as having prion diseases and judged that 1222 patients had prion diseases, consisting of definite (n=180, 14.7%) and probable (n=1029, 84.

[Effects of low-dose pergolide therapy on cardiac valves in patients with Parkinson's disease].

Background: Pergolide mesilate is widely used to treat Parkinson's disease in both the USA and Japan, but the maintenance dose is distinctly different between the USA (usually more than 1.5 mg/day) and Japan (usually less than 1.5 mg/day).

[Validity and clinical applicability of the Japanese version of amyotrophic lateral sclerosis--assessment questionnaire 40 (ALSAQ-40)].

We studied validity and clinical applicability of the Japanese version of amyotrophic lateral sclerosis (ALS) assessment questionnaire 40 (ALSAQ-40). The original version contains forty questions measuring five areas (domains) of health status: Physical Mobility, ADL/Independence, Eating and Drinking, Communication and Emotional Functioning. Data were obtained from 39 ALS patients and from their physicians at 15 centers in Japan.

Late onset ataxia phenotype in dentatorubro-pallidoluysian atrophy (DRPLA).

ABSTRACT We clinically and genetically studied three patients in a family with dentatorubro-pallidoluysian atrophy (DRPLA). The proband patient had 58/24 CAG repeat alleles of the DRPLA gene (normal < or = 34 repeats). Cerebellar ataxia first developed in the 6-7th decades and was the predominant feature for more than 10 years in all three, after which two of them manifested dementia and choreiform movements in the advanced stage.

[The advance in the research and therapeutic trials of amyotrophic lateral sclerosis].

The research concerning with the pathogenesis of amyotrophic lateral sclerosis (ALS) has been in steady progress in the last 10 years, including discovery of SOD mutation in familial ALS. Riluzole, by its inhibiting excitatory amino acid release, is the only drug, which has been demonstrated the neuroprotective activity in the randomised double-blind placebo-controlled clinical trials in patients with ALS, although many other clinical therapeutic trials for ALS patients has been carried out. We discussed the clinical trials being the under way, especially SR57746A, (1-[2-(naphth-2-yl)ethy]-4-(3-trifluoromethyl phenyl)-1, 2, 5, 6-tetrahydro-pyridine, hydrochloride), a non-peptide compound which has been shown to exhibit a wide range of neurotrophic effects both in vitro and in vivo, and its phase II study in Japan and two kinds of phase III studies ongoing in the United States, Canada and Europe.