A5016131935- Neurological disorders and treatments
- Botulinum Toxin and Related Neurological Disorders
- Parkinson's Disease Mechanisms and Treatments
- Genetic Neurodegenerative Diseases
- Amyotrophic Lateral Sclerosis Research
- Peripheral Neuropathies and Disorders
- Hereditary Neurological Disorders
- Neurogenetic and Muscular Disorders Research
- Neurological diseases and metabolism
- Transcranial Magnetic Stimulation Studies
- Pain Mechanisms and Treatments
- Neuroscience and Neural Engineering
- Acute Ischemic Stroke Management
- Muscle activation and electromyography studies
- Ion channel regulation and function
- Nerve injury and regeneration
- Cerebrovascular and Carotid Artery Diseases
- Stroke Rehabilitation and Recovery
- Peripheral Nerve Disorders
- Myasthenia Gravis and Thymoma
- Autoimmune Neurological Disorders and Treatments
- Parkinson's Disease and Spinal Disorders
- Lung Cancer Treatments and Mutations
- Mitochondrial Function and Pathology
- Neurological and metabolic disorders
Tokushima University
2015-2024
Utano Hospital
2003-2023
National Hospital Organization
2020-2023
Fukushima Medical University
2023
Kyoto Prefectural University of Medicine
2005-2023
Chiba University
2023
Niigata University
2023
Kanazawa Medical University
2023
Kyoto University
1996-2021
Tokushima University Hospital
2012-2021
© 2020 The Authors. Published by Elsevier B.V. on behalf of International Federation Clinical Neurophysiology. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
Multiple-system atrophy is an intractable neurodegenerative disease characterized by autonomic failure in addition to various combinations of parkinsonism, cerebellar ataxia, and pyramidal dysfunction. Although multiple-system widely considered be a nongenetic disorder, we previously identified multiplex families with this disease, which indicates the involvement genetic components.
Analysis of ALS patient iPSC-derived motor neurons indicates that Src/c-Abl inhibitors may have potential for treating ALS.
Highlights•Rapid, robust neuronal induction from human iPSCs to model AD drug responsiveness•iPSC-based screening of pharmaceutical compounds for Aβ phenotypes•A combination existing drugs synergistically improve phenotypes AD•Anti-Aβ cocktail decreases toxic levels in neurons derived patients' cellsSummaryIn the process development, vitro studies do not always adequately predict human-specific responsiveness clinical trials. Here, we applied advantage iPSC-derived neurons, which offer...
Abstract Patients with focal dystonia take advantage of certain cutaneous or proprioceptive sensory inputs to alleviate their symptoms (“sensory trick”). We examined the effects increasing muscle spindle activity by tonic vibration reflex maneuver and Decemberreasing it intramuscular injection lidocaine. The was applied palm tendon forearm muscles in 15 patients writer's cramp age‐matched normal subjects. In 11 patients, induced dystonic postures movements typical those seen during writing....
Writer's cramp, or focal hand dystonia, is characterized by involuntary coactivation of antagonist unnecessary muscles while writing performing other tasks. Although the mechanism underlying this muscle overactivation unknown, recent studies changes in cerebral blood flow during have demonstrated a reduction activation primary motor cortex (MC) and hyperactivity parts frontal non-primary areas. Therefore, any measures that decrease activities areas such as premotor (PMC) supplementary area...
Dystonia is a neurological syndrome characterized by sustained muscle contractions that produce repetitive twisting movements or abnormal postures. X-linked recessive dystonia parkinsonism (XDP; DYT3; Lubag) an adult-onset disorder manifests severe and progressive with high frequency of generalization. In search for the anatomical basis dystonia, we performed postmortem analyses functional anatomy basal ganglia based on striatal compartments (ie, striosomes matrix compartment) in XDP. Here,...
We report two patients with multifocal demyelinating motor neuropathy whose symptoms improved after immunoglobulin infusion. Clinical signs included atrophy of the tongue and limb muscles, closely resembling that neuron disease. Nerve conduction studies revealed multiple sites block without sensory abnormalities, suggesting selective involvement fibers. Careful documentation is required to diagnose this entity, which may be treated therapy.
Lower limb spasticity in post-stroke patients can impair ambulation and reduces activities of daily living (ADL) performance patients. Botulinum toxin type A (BoNTA) has been shown effective for upper spasticity. This study assesses the treatment lower a large placebo-controlled clinical trial. In this multicenter, randomized, double-blind, parallel-group, study, we evaluate efficacy safety one-time injections botulinum Japanese with One hundred twenty were randomized to single BoNTA 300 U...
Hereditary motor and sensory neuropathy with proximal dominant involvement (HMSN-P) is an autosomal-dominant neurodegenerative disorder characterized by widespread fasciculations, proximal-predominant muscle weakness, atrophy followed distal involvement. To date, large families affected HMSN-P have been reported from two different regions in Japan. Linkage haplotype analyses of previously new the use high-density SNP arrays further defined minimum candidate region 3.3 Mb chromosomal 3q12....
Glucocerebrosidase gene (GBA) variants that cause Gaucher disease are associated with Parkinson (PD) and dementia Lewy bodies (DLB). To investigate the role of GBA in multiple system atrophy (MSA), we analyzed a large case-control series.We sequenced coding regions flanking splice sites 969 MSA patients (574 Japanese, 223 European, 172 North American) 1509 control subjects (900 315 294 American). We focused solely on Gaucher-disease-causing variants.In Japanese series, found nine carriers...
The El Escorial criteria for the diagnosis of amyotrophic lateral sclerosis (ALS) were established 20 years ago and have been used as inclusion clinical trials. However, concerns raised concerning their use diagnostic in practice. Moreover, modern genetics shed new light on heterogeneity ALS close relationship between frontotemporal dementia (FTD) recognized, World Federation Neurology Research Group ALS/MND has initiated discussions to amend update criteria, while preserving essential...
<h3>Importance</h3> The effectiveness of currently approved drugs for amyotrophic lateral sclerosis (ALS) is restricted; there a need to develop further treatments. Initial studies have shown ultrahigh-dose methylcobalamin be promising agent. <h3>Objective</h3> To validate the efficacy and safety patients with ALS enrolled within 1 year onset. <h3>Design, Setting, Participants</h3> This was multicenter, placebo-controlled, double-blind, randomized phase 3 clinical trial 12-week observation...
Multiple nerve excitability measurements were used to investigate axonal membrane properties of patients diagnosed with multifocal motor neuropathy (MMN). Six selected, all evidence distal focal conduction block involving the median in forearm. In patients, was stimulated at wrist, just site block, and resulting compound muscle action potentials recorded from abductor pollicis brevis. Stimulus-response behaviour, strength--duration time constant, threshold electrotonus 100 ms polarizing...
In order to clarify further the characteristics of ictal direct current (DC) shifts in human epilepsy, we investigated them by subdural and scalp recording six three patients, respectively, both having mainly neocortical lobe epilepsy (five with frontal two parietal temporal epilepsy). By using electrodes made platinum, DC were observed 85% all recorded seizures (89 seizures) among they localized just one or at which conventional initial EEG change was also observed. They closely accompanied...