Prognostic factors of subacute comprehensive encephalitis: a retrospective study.

Purpose: The etiology of encephalitis is unknown in 40%-50% of cases, so a comprehensive examination of encephalitis would be significant and meaningful. The short-term outcomes in appropriately managed patients are also unknown. Short-term clinical outcomes following onset can provide clinicians with clues regarding the clinical course in the immediate future.

Clinical Characteristics of Patients With Becker Muscular Dystrophy Having Pathogenic Microvariants or Duplications.

Background And Objectives: Becker muscular dystrophy (BMD) is an allelic disorder of Duchenne muscular dystrophy (DMD) in which pathogenic variants in cause progressive worsening of motor dysfunction, muscle weakness and atrophy, and death due to respiratory and cardiac failure. BMD often has in-frame deletions that preserve the amino acid reading frame, but there are some cases with microvariants or duplications. In recent years, the importance of therapeutic development and care for BMD has been emphasized.

Effect of zonisamide on sleep and rapid eye movement sleep behavioral disorders in patients with Parkinson's disease: A randomized control trial.

Introduction: Zonisamide is a medication developed in Japan that is effective for motor symptoms and wearing off in Parkinson's disease (PD). Zonisamide has properties that may improve sleep disorders. The aim of this study is to verify the safety and efficacy of zonisamide for sleep disorders and rapid eye movement (REM) sleep behavioral disorders (RBD) using a mobile two-channel electroencephalography /electrooculography recording system in patients with PD.

Urinary titin reflects the severity of walking ability, muscle strength, and muscle and cardiac damage in patients with Becker muscular dystrophy.

Background: Becker muscular dystrophy (BMD) is a dystrophinopathy caused by a pathological variant of the DMD gene. Urinary titin, a degradation product of the giant protein titin present in muscle sarcomeres, has been used as a biomarker to reflect muscle degradation in Duchenne muscular dystrophy, a more severe dystrophinopathy. However, the clinical significance of urinary titin levels in BMD remains unclear.

Changes in brain functional connectivity between on and off states and their relationship with cognitive impairment in Parkinson's disease.

Parkinson's disease (PD) is characterized by motor and non-motor symptoms. Cognitive decline is crucial in disease progression and affect quality of life; however, their underlying mechanisms in PD remain unclear. We explored the relationship between cognitive impairment and functional connectivity (FC) using resting-state functional magnetic resonance imaging in 26 patients with sporadic PD, focusing on the changes in FC between on and off states.

History and Perspective of LAMP-2 Deficiency (Danon Disease).

Danon disease, an X-linked dominant vacuolar cardiomyopathy and skeletal myopathy, is caused by a primary deficiency of lysosome-associated membrane protein-2 (LAMP-2). This disease is one of the autophagy-related muscle diseases. Male patients present with the triad of cardiomyopathy, myopathy, and intellectual disability, while female patients present with cardiomyopathy.

Early-morning OFF in Parkinson's disease: A systematic literature review and current therapeutics.

Objective: Early morning OFF (EMO) is one of the first motor complications to manifest and frequently signals the onset of additional motor complications in Parkinson's Disease (PD). Although EOM are frequently observed in patients with PD and many caregivers must help with their motor inability, the treatment is still unsatisfactory. The majority of research that has been conducted on the wearing-off state of patients with PD has focused on daytime symptoms; evening and early morning symptoms have received much less attention.

Impact of sex, age at onset, and anti-cN1A antibodies on sporadic inclusion body myositis.

Background: Inclusion body myositis (IBM) is a progressive myopathy occurring in patients over 45 years of age, with heterogeneous and variable clinical features. This study aimed to determine the influence of autoantibodies, gender, and age of onset on the clinical features of IBM.

Methods: Medical records and muscle histology findings of 570 participants with suspected IBM were reviewed.

Reduction in presynaptic dopamine transporter may be associated with future problematic delusion.

Objectives: Psychosis, especially in delusions, greatly impairs the quality of life of patients with Parkinson's disease (PD) and their caregivers. Few objective risk indicators of the association between psychosis and clinical features has been reported. It is unclear whether the reduction in DAT binding represents the underlying mechanism of delusion or its association.

[Dermatomyositis].

Dermatomyositis (DM) is distinguished from other idiopathic inflammatory myopathies by the characteristic skin rashes, muscle pathology, and muscle symptoms. Five myositis-specific autoantibodies have been identified in DM, and the correlation between each antibody and the clinical picture is clear. Pathological analysis has also identified DM as a type I interferonopathy of the skeletal muscle.

Compound muscle action potential of whole-forearm flexors: A clinical biomarker for inclusion body myositis.

Objective: This study aimed to investigate the potential of whole-forearm flexor muscle (WFFM) compound muscle action potential (CMAP) as a quantitative biomarker for inclusion body myositis (IBM) pathology.

Methods: We prospectively enrolled 14 consecutive patients (10 men and 4 women) diagnosed with IBM based on muscle biopsies. We evaluated the baseline-to-peak amplitude of the WFFM CMAP and other quantitative parameters, including grip and pinch strength, Inclusion Body Myositis Functional Rating Scale (IBMFRS) score, and other routine muscle CMAP amplitudes.

A Rare Case of Overlapping Durvalumab-induced Myositis, Takotsubo-like Morphological Changes Caused by Myocarditis, and Myasthenia Gravis.

Immune checkpoint inhibitors can cause a range of immune-related adverse events, including myositis, Takotsubo cardiomyopathy, and myasthenia gravis. We herein report a rare case of a 78-year-old man with concurrent durvalumab-induced myositis, Takotsubo-like morphological changes caused by myocarditis, and myasthenia gravis. The patient initially required invasive ventilation and exhibited symptoms of myasthenia gravis after treatment with high-dose steroids.

Daily light exposure profiles and the association with objective sleep quality in patients with Parkinson's disease: The PHASE study.

Study Objectives: Light information crucially influences sleep initiation and continuity. The purpose of this study was to compare daily light exposure between patients with Parkinson's disease (PD) and non-PD older adults and evaluate the association of daily light exposure with objective sleep measures in patients with PD.

Methods: In this cross-sectional study of 189 outpatients with PD and 1101 community-dwelling older adults (controls), daily light exposure was measured using wrist light meters during the daytime and light meters set in the bedrooms during the nighttime, and objective sleep quality was measured by wrist actigraphy.

Electromyography varies by stage in inclusion body myositis.

Introduction: Inclusion body myositis (IBM) is a chronic inflammatory muscle disease that is characterized by mixed myogenic and neurogenic electromyography (EMG) findings. We investigated the association between EMG findings and the IBM stage.

Methods: We included consecutive patients diagnosed with IBM based on muscle biopsy and had needle EMG performed within 1 month of biopsy.

Identification of three distinct cell populations for urate excretion in human kidneys.

In humans, uric acid is an end-product of purine metabolism. Urate excretion from the human kidney is tightly regulated by reabsorption and secretion. At least eleven genes have been identified as human renal urate transporters.

[Biological phase separation in neuromuscular diseases].

Biological phase separation refers to the liquid-liquid phase separation of biomolecules such as proteins in cells. Phase separation is driven by low-complexity domains of phase-separating proteins and strictly controlled by regulatory factors. Phase separation has also been found to be disrupted by genetic abnormalities.

Lidocaine as a potential therapeutic option for super-refractory status epilepticus: A case report.

New-onset refractory status epilepticus (NORSE) is a rare and devastating condition and the prognosis is often poor, with half to two-thirds of survivors experiencing drug-resistant epilepsy, residual cognitive impairment, or functional disability, and the mortality rate is 16% to 27% for adults. We describe a patient with cryptogenic NORSE and favorable recovery from drug-resistant super-refractory SE after the use of intravenous lidocaine. The patient experienced fever and presented with refractory generalized tonic-clonic seizures.

Bradykinesia and rigidity modulated by functional connectivity between the primary motor cortex and globus pallidus in Parkinson's disease.

The mechanisms underlying motor fluctuations in patients with Parkinson's disease (PD) are currently unclear. Regional brain stimulation reported the changing of motor symptoms, but the correlation with functional connectivity (FC) in the brain network is not fully understood. Hence, our study aimed to explore the relationship between motor symptom severity and FC using resting-state functional magnetic resonance imaging (rsfMRI) in the "on" and "off" states of PD.

Challenges in evaluating forearm muscle activity based on the compound muscle action potential of the flexors of the whole forearm.

Objective: Muscle strength, which correlates with the compound muscle action potential (CMAP), can also be estimated by measuring the CMAP. Therefore, we evaluated the CMAP of the flexor muscles of the whole forearm to identify their muscle strength.

Methods: Fourteen healthy volunteers were enrolled.

Pathogenic role of anti-cN1A autoantibodies in sporadic inclusion body myositis.

Background: Sporadic inclusion body myositis (sIBM) is an intractable muscle disease that frequently affects elderly people. Autoantibodies recognising cytosolic 5'-nucleotidase 1A (cN1A) were found in the sera of patients with sIBM. However, the pathogenic role of the autoantibodies remained unknown.