A5086153164- Peripheral Neuropathies and Disorders
- Hereditary Neurological Disorders
- Amyotrophic Lateral Sclerosis Research
- Neurogenetic and Muscular Disorders Research
- Myasthenia Gravis and Thymoma
- Neurological disorders and treatments
- Platelet Disorders and Treatments
- Nerve injury and regeneration
- Multiple Myeloma Research and Treatments
- Botulinum Toxin and Related Neurological Disorders
- Muscle activation and electromyography studies
- Parkinson's Disease Mechanisms and Treatments
- Pain Mechanisms and Treatments
- Peripheral Nerve Disorders
- Amyloidosis: Diagnosis, Treatment, Outcomes
- Autoimmune Bullous Skin Diseases
- Neuroscience and Neural Engineering
- Autoimmune Neurological Disorders and Treatments
- Advanced Neuroimaging Techniques and Applications
- Eosinophilic Disorders and Syndromes
- Facial Nerve Paralysis Treatment and Research
- Transcranial Magnetic Stimulation Studies
- Healthcare professionals’ stress and burnout
- Long-Term Effects of COVID-19
- Muscle Physiology and Disorders
Chiba University
2016-2025
Chiba University Hospital
2016-2023
Transthyretin amyloidosis (ATTR amyloidosis) is a rare, life-threatening disease caused by the accumulation of variant or wild-type (ATTRwt transthyretin amyloid fibrils in heart, peripheral nerves, and other tissues organs.Established 2007, Amyloidosis Outcomes Survey (THAOS) largest ongoing, global, longitudinal observational study patients with ATTR amyloidosis, including both inherited disease, asymptomatic carriers pathogenic TTR mutations. This descriptive analysis examines baseline...
<h3>Objective</h3> Vascular endothelial growth factor (VEGF) plays an essential role in the pathophysiology of polyneuropathy, organomegaly, endocrinopathy, M-protein and skin changes (POEMS) syndrome. Anti-VEGF antibody (bevacizumab) appears to be attractive therapeutic option. The aim this study is investigate effects bevacizumab for patients with POEMS <h3>Methods</h3> We reported six treated reviewed literature. <h3>Results</h3> serum VEGF levels decreased immediately after...
: This study aimed to reveal the diagnostic utility of Gold Coast (GC) criteria in Japanese patients with amyotrophic lateral sclerosis (ALS) by comparing sensitivity/specificity revised El Escorial (R-EE) and Awaji criteria, because its has not been studied Asian ALS.
ABSTRACT POEMS syndrome is a multisystemic disease associated with monoclonal plasma cell disorders. Although the presence of bone lesions included in diagnostic criteria, their precise manifestations remain unknown. Here, we retrospectively analyzed patients and evaluated clinical features. Clinical data lesion information by computed tomography (CT) imaging were obtained from 114 syndrome. Regardless sclerotic lesions, divided into two groups according to (lytic group: n = 17, 14.9%) or...
Guillain-Barré syndrome (GBS) is an acute, self-limited, immune-mediated peripheral neuropathy. Current treatments for GBS include intravenous immunoglobulin (IVIg) and plasma exchange, which may not sufficiently benefit severely affected patients. This study evaluated the efficacy safety of eculizumab add-on therapy to IVIg (standard-of-care treatment) in patients with severe GBS.
Background and purpose Cranial nerve palsy is occasionally present in patients with chronic inflammatory demyelinating polyneuropathy (CIDP), but its prevalence, characteristics relations the CIDP subtypes have rarely been investigated. The aim of this study was to systematically assess cranial involvement typical atypical CIDP. Methods Clinical data were reviewed 132 consecutive CIDP, including ( n = 89), multifocal acquired sensory motor neuropathy (MADSAM) 31), distal symmetric (DADS) 9)...
Transthyretin amyloidosis (ATTR amyloidosis) is primarily associated with a cardiac or neurologic phenotype, but mixed phenotype increasingly described. This study describes the cohort in Amyloidosis Outcomes Survey (THAOS). THAOS an ongoing, longitudinal, observational survey of patients ATTR amyloidosis, including both hereditary (ATTRv) and wild-type disease, asymptomatic carriers pathogenic transthyretin variants. Baseline characteristics (at enrollment reclassified during follow-up) are...
Abstract Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes ( POEMS ; C row– F ukase) syndrome is a devastating disorder related to plasma cell dyscrasia, characterized by polyneuropathy, edema/effusion, M ‐protein changes. Novel therapeutic approaches can substantially improve the prognosis. Diagnosis of often challenging because combination clinical presentations. To facilitate precise early diagnosis, comprehensive systemic survey essential, we propose...
The presence of antibodies against muscle-specific receptor tyrosine kinase (MuSK) appears to define a subgroup patients with myasthenia gravis (MG) characterized by weakness predominant in bulbar, facial and neck muscles compared anti-acetylcholine (AChR) antibody-positive MG. To investigate the patterns severity neuromuscular transmission failure different MuSK-positive MG, we performed single fiber electromyography (SFEMG) (frontalis) limb (extensor digitorum communis, EDC) three...
Abstract Background and purpose Muscle ultrasonography has been increasingly recognized as a useful tool for detection of fasciculations. Separately, concordance between dominant hand onset side reported in amyotrophic lateral sclerosis (ALS). The aim this study was to reveal the distribution fasciculations whole body, focusing on handedness. Methods In 106 consecutive patients with ALS, muscle systematically performed 11 muscles (the tongue, bilateral biceps brachii, 1st dorsal interosseous...
POEMS syndrome is a rare monoclonal plasma cell disorder, with unique symptoms distinct from those of other neoplasms, including high serum VEGF levels. Because the prospective isolation clones has not yet been successful, their real nature remains unclear. Herein, we performed single-cell RNA-Seq BM cells patients and identified that had Ig λ light chain (IGL) sequences (IGLV1-36, -40, -44, -47) amino acid changes specific to syndrome. The proportions in were markedly smaller than multiple...
<b>Objective:</b> In patients with hemifacial spasm (HFS), abnormal muscle responses (AMR) are frequently present. The objective of this study was to investigate whether the afferent input AMR is mediated by antidromic facial nerve stimulation or orthodromic trigeminal stimulation. <b>Methods:</b> in orbicularis oris were recorded 28 HFS. When present, they after subthreshold and weak delivered skin. <b>Results:</b> recordable 24 (86%) patients, usually consisted early constant component...
Abstract Background Previous studies have shown that increased motor axonal excitability is associated with shorter survival in amyotrophic lateral sclerosis patients. Aim To study the properties related functional decline. Methods A total of 60 patients were prospectively evaluated at baseline, 3 months and 6 months. Motor function was revised rating scale. Multiple indices, such as strength‐duration time constant (a measure persistent sodium currents), supernormality threshold...
A previous study using traditional paired-pulse TMS methods (amplitude-tracking) has reported differences in resting motor threshold (RMT) and short-interval intracortical inhibition (SICI) between healthy subjects of Caucasian Han Chinese backgrounds, probably due to the skull shape. The amplitude-tracking method delivers stimuli with constant intensity causes substantial variabilities motor-evoked potential amplitudes. To overcome this variability, tracking transcranial magnetic...