Safety and Selection Criteria of Single-joint Hybrid Assistive Limb for Upper Limb Paralysis After Stroke: A Phase I Trial.

Robotic rehabilitation is a high-intensity intervention for upper limb paralysis after a stroke. This study explored the safety and feasibility of using a single-joint hybrid assistive limb (HAL-SJ) exoskeletal device for upper limb paralysis in patients with acute stroke. In total, 11 patients with stroke (6 with moderate paralysis and 5 with severe paralysis) were enrolled between October 2021 and October 2023 in a stroke care unit.

Biological variation of 16 biochemical analytes estimated from a large clinicopathologic database of dogs and cats.

Background: Biochemical measurements are commonly evaluated using population-based reference intervals; however, there is a growing trend toward reassessing results with within-subject variation (CV).

Objectives: We aimed to estimate the CV of 16 biochemical analytes using a large database of dogs and cats, which refers to the results of routine health checkups.

Methods: Pairs of sequential results for 16 analytes were extracted from a database of adult patients.

The Evaluation of Benzodiazepine-induced Dysphagia Using High-resolution Manometry.

We evaluated the pathophysiology of dysphagia considered to be induced by benzodiazepine using high-resolution manometry (HRM). A 53-year-old man with Parkinson disease had had dysphagia for over 3 months. He had been taking several benzodiazepines for more than four years.

Between-day reliability and minimum detectable change of the Conventional Gait Model 2 and Plug-in Gait Model during running.

Background: The Plug-in Gait model (PiG) is commonly used in 3D motion analysis but has limited reliability. Although an improved version of PiG has been developed, called the Conventional Gait Model 2 (CGM2), there is limited evidence on its between-day reliability for running.

Research Question: What is the between-day intraclass correlation coefficient (ICC3,k) and minimum detectable change (MDC) of lower limb kinematics and kinetics for CGM2 during running and does reliability differ between CGM2 and PiG.

Clinical characteristics of intractable or persistent hiccups and nausea associated with herpes zoster.

Aim: Intractable or persistent hiccups and nausea (IHN) are rarely associated with herpes zoster (HZ-IHN). We aimed to identify the clinical characteristics of HZ-IHN by comparing them with those of neuromyelitis optica spectrum disorder associated with IHN (NMOSD-IHN).

Methods: We collected 8 patients with HZ-IHN and 12 patients with NMOSD-IHN diagnosed between 2002 and 2020 from medical databases.

Clinicopathological findings of a mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes/Leigh syndrome overlap patient with a novel m.3482A>G mutation in MT-ND1.

We report clinicopathological findings of a patient with mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes/Leigh syndrome (MELAS/LS) associated with a novel m.3482A>G mutation in MT-ND1. A 41-year-old woman had experienced multiple stroke-like episodes since age 16.

DNA methylation is reconfigured at the onset of reproduction in rice shoot apical meristem.

DNA methylation is an epigenetic modification that specifies the basic state of pluripotent stem cells and regulates the developmental transition from stem cells to various cell types. In flowering plants, the shoot apical meristem (SAM) contains a pluripotent stem cell population which generates the aerial part of plants including the germ cells. Under appropriate conditions, the SAM undergoes a developmental transition from a leaf-forming vegetative SAM to an inflorescence- and flower-forming reproductive SAM.

Autoimmune Glial Fibrillary Acidic Protein Astrocytopathy Presenting with Slowly Progressive Myelitis and Longitudinally Extensive Spinal Cord Lesions.

We report a 65-year-old man with autoimmune glial fibrillary acidic protein astrocytopathy (GFAP-A) who presented with gait disturbance that he had experienced for approximately half a year. On neurological examination, he displayed spastic paraplegia and autonomic dysfunctions including dysuria and constipation. Spinal cord magnetic resonance imaging showed longitudinally extensive spinal cord lesions (LESCLs) extending from the cervical to the thoracic cords.

Serial evaluation of swallowing function in a long-term survivor of V180I genetic Creutzfeldt-Jakob disease.

Swallowing function in long-term survivors with Creutzfeldt-Jakob disease (CJD) remains unknown. Herein, we demonstrated serial evaluation of swallowing function in a case with V180I genetic CJD (gCJD) using videofluoroscopic examination of swallowing (VF). A 69-year-old woman was admitted to our hospital because of bradykinesia and memory disturbances 4 months after the onset of symptoms.

[Clinical findings of a patient with hemiballism after superficial temporal artery-middle cerebral artery anastomosis for idiopathic middle cerebral artery stenosis].

A 32-year-old woman experienced several episodes of transient numbness on the left side of her face and body. MR angiography revealed severe stenosis in the right middle cerebral artery (MCA). Abnormal collateral vessel networks were not observed, and idiopathic MCA stenosis was diagnosed.

Functional evaluation of PDGFB-variants in idiopathic basal ganglia calcification, using patient-derived iPS cells.

Causative genes in patients with idiopathic basal ganglia calcification (IBGC) (also called primary familial brain calcification (PFBC)) have been reported in the past several years. In this study, we surveyed the clinical and neuroimaging data of 70 sporadic patients and 16 families (86 unrelated probands in total) in Japan, and studied variants of PDGFB gene in the patients. Variant analyses of PDGFB showed four novel pathogenic variants, namely, two splice site variants (c.

Novel PHOX2B mutations in congenital central hypoventilation syndrome.

Background: Congenital central hypoventilation syndrome (CCHS) is caused by mutation of paird-like homeobox 2B (PHOX2B). Approximately 90% of patients were found to carry polyalanine repeat expansion mutation (PARM), and the remaining 10% had non-PARM (NPARM). In PARM, the length of the polyalanine expansion correlates with clinical disease severity.

Inorganic phosphorus (Pi) in CSF is a biomarker for SLC20A2-associated idiopathic basal ganglia calcification (IBGC1).

Introduction: Idiopathic basal ganglia calcification (IBGC), also called Fahr's disease or recently primary familial brain calcification (PFBC), is characterized by abnormal deposits of minerals including calcium mainly and phosphate in the brain. Mutations in SLC20A2 (IBGC1 (merged with former IBGC2 and IBGC3)), which encodes PiT-2, a phosphate transporter, is the major cause of IBGC. Recently, Slc20a2-KO mice have been showed to have elevated levels of inorganic phosphorus (Pi) in cerebrospinal fluid (CSF); however, CSF Pi levels in patients with IBGC have not been fully examined.

Clinical findings of a probable case of MM2-cortical-type sporadic Creutzfeldt-Jakob disease with antibodies to anti-N-terminus of α-enolase.

We report the case of a 76-year-old woman presenting with 47-month history of progressive dementia and cortical blindness with no family history. Antibodies against thyroid glands and the N-terminus of α-enolase (NAE) were detected in her serum. Neurological examination revealed progressive dementia, frontal signs, visual disturbance, and exaggerated bilateral tendon reflexes in both legs.

Acute-Onset Severe Occipital Neuralgia Associated With High Cervical Lesion in Patients With Neuromyelitis Optica Spectrum Disorder.

Objective: To address occipital neuralgia in patients with neuromyelitis optica spectrum disorder (NMOSD).

Background: NMOSD is an inflammatory demyelinating disease that commonly presents with pain; however, headache symptoms have received little attention.

Methods: We presented three cases of NMOSD in which the patients experienced acute-onset, severe, and steroid-responsive occipital neuralgia.

Living with idiopathic basal ganglia calcification 3: a qualitative study describing the lives and illness of people diagnosed with a rare neurological disease.

Purpose: Idiopathic basal ganglia calcification (IBGC) is a rare, intractable disease with unknown etiology. IBGC3 is a familial genetic disease defined by genetic mutations in the major causative gene (). People with IBGC3 experience distress from the uncommon nature of their illness and uncertainty about treatment and prognoses.

Preserved regional cerebral blood flow in the occipital cortices, brainstem, and cerebellum of patients with V180I-129M genetic Creutzfeldt-Jakob disease in serial SPECT studies.

Creutzfeldt-Jakob disease (CJD) with a causative point mutation of valine to isoleucine at codon 180 (V180I) is one of the major types of genetic CJD (gCJD) in Japan. V180I gCJD is rarely accompanied by a family history, and its clinical characteristics include late-onset, long disease duration, and edematous cortical hyperintensity in diffusion, fluid attenuate inversion and T2-weighted MRI. We performed serial imaging with single-photon emission computed tomography (SPECT) and MRI in three V180I gCJD cases over long-term observation.

[A case of lithium intoxication induced by an antihypertensive angiotensin 1 subtype-specific angiotensin II receptor blocker in an elderly patient with bipolar disorder and hypertension].

Lithium carbonate is considered to be a first-line treatment for bipolar disorder; however, this drug has a narrow therapeutic window, and lithium intoxication is commonly induced by various drugs interaction and situations. We herein report a case of lithium intoxication induced by the administration of an antihypertensive agent targeting the angiotensin 1 (AT1) subtype of the angiotensin II receptor in a 65-year-old woman with a 40-year history of bipolar disorder type 1, and 1-year history of essential hypertension. Her bipolar disorder had been well-controlled with 600 mg/day of lithium carbonate for more than 10 years.

Reduction in the numbers of drugs administered to elderly in-patients with polypharmacy by a multidisciplinary review of medication using electronic medical records.

Aim: Polypharmacy is a major problem for elderly patients in developed countries. We investigated whether a multidisciplinary medication review using electronic medical records could reduce the number of drugs administered to elderly patients receiving polypharmacy.

Methods: The present study included 432 elderly patients (188 women, 244 men; 267 patients aged 65-74 years and 165 patients aged ≥75 years) who were admitted to and discharged from the Department of Neurology and Geriatrics, Gifu University Hospital, between 2004 and 2011; those who died at the hospital were excluded.

PAMAM Dendron Lipid Assemblies That Undergo Structural Transition in Response to Weakly Acidic pH and Their Cytoplasmic Delivery Capability.

Dendron lipids designed to consist of amine-terminated polyamidoamine G1 dendron and two octadecyl chains were used for the preparation of pH-responsive molecular assemblies having phase structures that are changed through their dynamic molecular shape. The dendron lipid contains two primary amines and two tertiary amines in the dendron moiety, changing its charged state in the pH region between pH 10 and pH 4. The assemblies were shown to take a vesicle structure at neutral and alkaline pHs, but their structure changed to a micelle-like structure below pH 6.

Evaluation of SLC20A2 mutations that cause idiopathic basal ganglia calcification in Japan.

Objective: To investigate the clinical, genetic, and neuroradiologic presentations of idiopathic basal ganglia calcification (IBGC) in a nationwide study in Japan.

Methods: We documented clinical and neuroimaging data of a total of 69 subjects including 23 subjects from 10 families and 46 subjects in sporadic cases of IBGC in Japan. Mutational analysis of SLC20A2 was performed.

[A case of nonsystemic vasculitic neuropathy with spondylosis deformans in an 84-year-old woman].

We report an 84-year-old woman with left lower limb muscle weakness and numbness who also had weakness in her right lower limb, which showed spontaneous partial improvement. Neurological examination revealed lower extremity weakness and sensory disturbance in all modalities, predominantly distally on the left side. Laboratory studies yielded normal results, except for a slightly high erythrocyte sedimentation rate.