Primary Malignant Lymphoma of the Cauda Equina Diagnosed after Decompression for Lumbar Spinal Stenosis: A Case Report.

Primary malignant lymphoma confinement to the cauda equina is rare. Only 14 cases of primary malignant lymphoma in the cauda equina have been reported. In these cases, the clinical features were similar to those of lumbar spinal canal stenosis (LSCS).

Sortilin acts as an endocytic receptor for α-synuclein fibril.

Cell-to-cell spreading of misfolded α-synuclein (αSYN) is supposed to play a key role in the pathological progression of Parkinson's disease (PD) and other synucleinopathies. Receptor-mediated endocytosis has been shown to contributes to the uptake of αSYN in both neuronal and glial cells. To determine the receptor involved in αSYN endocytosis on the cell surface, we performed unbiased, and comprehensive screening using a membrane protein library of the mouse whole brain combined with affinity chromatography and mass spectrometry.

Epsin2, a novel target for multiple system atrophy therapy via α-synuclein/FABP7 propagation.

Multiple system atrophy (MSA) is a neurodegenerative disease characterized by the accumulation of misfolded α-synuclein (αSyn) and myelin disruption. However, the mechanism underlying αSyn accumulation in MSA brains remains unclear. Here, we aimed to identify epsin-2 as a potential regulator of αSyn propagation in MSA brains.

Plasma sphingolipid abnormalities in neurodegenerative diseases.

Background: In recent years, there has been increasing evidence that several lipid metabolism abnormalities play an important role in the pathogenesis of neurodegenerative diseases. However, it is still unclear which lipid metabolism abnormalities play the most important role in neurodegenerative diseases. Plasma lipid metabolomics (lipidomics) has been shown to be an unbiased method that can be used to explore lipid metabolism abnormalities in neurodegenerative diseases.

Mirror writing and cortical hypometabolism in Parkinson's disease.

Mirror writing (MW) is the production of individual letters, words, or word strings in the reverse direction. Parkinson's disease (PD) is a progressive neurodegenerative disorder, and high MW rates have been reported in patients with PD. Thus, the present study sought to identify the factors that cause MW in patients with PD.

Determination of language areas in patients with epilepsy using the super-selective Wada test.

The Wada test is the gold standard for determining language-dominant hemisphere. However, the precise determination of language areas in each patient requires more invasive methods, such as electrocortical stimulation. Some studies have reported the use of anesthetic injection into selective cerebral arteries to predict postoperative function.

Bedside evaluation of swallowing function to predict aspiration pneumonia in Duchenne muscular dystrophy.

Objective: Aspiration pneumonia is one of the leading causes of death in patients with muscular dystrophy; therefore, it is important to predict its occurrence in the clincal setting. We aimed to examine the usefulness of repeated saliva swallowing test (RSST), modified water swallowing test (MWST), and flexible endoscopic evaluation of swallowing (FEES) for evaluating the Hyodo score at the bedside, to predict the risk of aspiration pneumonia in patients with Duchenne muscular dystrophy (DMD).

Methods: In this retrospective cohort study involving 43 patients, we evaluated the swallowing function using the RSST, MWST, and FEES, and predicted the likelihood of aspiration pneumonia within 2 years after the assessment.

Destructive Spondyloarthropathy due to Congenital Insensitivity to Pain with Anhidrosis: A Case Report of Long-Term Follow-Up.

Congenital insensitivity to pain with anhidrosis (CIPA) is a rare autosomal-recessive hereditary neuropathy causing congenital loss of pain sensation, thermoception, and perspiration. CIPA sometimes causes destructive spondyloarthropathy, the so-called Charcot spine, because of insensitivity to pain stimuli. Herein, we report a case of CIPA with severe spinal destruction treated by multiple spinal reconstructive surgeries and over 15 years of follow-up.

Collective Expert Perspectives on the Use of Safinamide as Adjunctive Therapy for Parkinson's Disease: Online-Based Delphi Survey.

Background: Safinamide is a selective, reversible monoamine oxidase-B inhibitor with a sodium channel inhibitory effect. Published clinical evidence supports safinamide as an effective therapy for Parkinson's disease (PD) with wearing-off. However, to date, no consensus recommendations have been available to guide physicians in Asia on the optimal use of safinamide in clinical practice.

Effect of donepezil for dementia prevention in Parkinson's disease with severe hyposmia (The DASH-PD study): A randomized long-term placebo-controlled trial.

Background: Dementia greatly contributes to poor prognosis in patients with Parkinson's disease (PD). We previously reported that severe olfactory dysfunction may be a good predictor of Parkinson's disease dementia (PDD). In this trial, we investigated whether early administration of donepezil to patients with severe hyposmia can reduce the development of PDD.

Decreased sensitivity to loss of options in patients with Parkinson's disease.

Humans prefer to have many options when making decisions. When there is a threat of options disappearing, humans invest more to keep these options available, indicating that they are sensitive to the loss of options. This study examined whether patients with Parkinson's disease (PD), a disease characterized by dopamine depletion, try to keep options available when options are disappearing.

Phosphorylated alpha-synuclein in Iba1-positive macrophages in the skin of patients with Parkinson's disease.

Background: Increasing evidence suggests that alpha-synuclein (αSyn) accumulation in cholinergic and adrenergic fibers in the skin is a useful biomarker to diagnose idiopathic Parkinson's disease (IPD). It has been widely reported that phosphorylated αSyn (p-αSyn) deposits in autonomic fibers in IPD are a biomarker in the skin, but other tissue localizations have not been fully investigated.

Objective: It has been previously suggested that αSyn aggregates activate peripheral macrophages and that peripheral macrophages ingest pathological αsyn aggregates in aged rats or IPD patients.

[Parkinson's Disease with Intolerable Painful Dyskinesia: Standard Treatment and Subsequent Tips].

The pathogenesis of pain related to dyskinesia in Parkinson disease is thought to be based on hypersensitivity to pain. To relieve symptoms, it is essential to reduce dyskinesia and alleviate symptoms. Frequent small doses of levodopa, dopamine agonists, MAOB inhibitors, and amantadine are effective.

Tranilast for advanced heart failure in patients with muscular dystrophy: a single-arm, open-label, multicenter study.

Background: The transient receptor potential cation channel subfamily V member 2 (TRPV2) is a stretch-sensitive calcium channel. TRPV2 overexpression in the sarcolemma of skeletal and cardiac myocytes causes calcium influx into the cytoplasm, which triggers myocyte degeneration. In animal models of cardiomyopathy and muscular dystrophy (MD), TRPV2 inhibition was effective against heart failure and motor function.

Neurogenic lower urinary tract dysfunction in association with severity of degenerative spinal diseases: Short-term outcomes of decompression surgery.

Objectives: Cervical myelopathy (CM) and lumbar canal stenosis (LCS) are common degenerative spinal diseases among the elderly, and the major associated complaints include lower urinary tract symptoms (LUTS). The aim of this study was to investigate subjective and objective urological parameters of patients undergoing decompression surgery for CM and LCS.

Methods: We retrospectively reviewed patients who underwent evaluation by the International Prostate Symptom Score (IPSS) and uroflowmetry before decompression surgery for CM and LCS.

The characteristics of dysphagia and the incidence of pneumonia in Myotonic dystrophy type 1 patients especially concerning swallowing function evaluated by endoscopy.

Objective: The objectives of the study were to clarify the characteristics of dysphagia and the incidence of pneumonia in Myotonic dystrophy type 1 (DM1) patients, and to investigate the relationship between the development of pneumonia and the DM1 patient's background, especially concerning swallowing function evaluated by endoscopy.

Methods: The subjects were 88 DM1 patients who underwent swallowing function evaluation. The severity of disease in DM1patients was assessed based on the muscular impairment rating scale (MIRS), and the number of CTG repeats.

A prospective multicenter validation study of a machine learning algorithm classifier on quantitative electroencephalogram for differentiating between dementia with Lewy bodies and Alzheimer's dementia.

Background And Purpose: An early and accurate diagnosis of Dementia with Lewy bodies (DLB) is critical because treatments and prognosis of DLB are different from Alzheimer's disease (AD). This study was carried out in Japan to validate an Electroencephalography (EEG)-derived machine learning algorithm for discriminating DLB from AD which developed based on a database of EEG records from two different European countries.

Methods: In a prospective multicenter study, patients with probable DLB or with probable AD were enrolled in a 1:1 ratio.

Facet cysts in the subaxial cervical spine: Case series focused on radiological and histopathological findings with a scoping review.

Background: Facet cysts in the subaxial cervical spine are a relatively rare cause of neuropathy. This case series aimed to investigate the radiological and histopathological features and surgical results of these lesions, and provide possible mechanisms of cyst development.

Methods: Thirteen subaxial cervical facet cysts in 12 patients were diagnosed on the basis of magnetic resonance imaging and computed tomography with facet arthrography.

Effects of rasagiline on Parkinson's Disease Questionnaire (PDQ-39) emotional well-being domain in patients with Parkinson's disease: A post-hoc analysis of clinical trials in Japan.

Background: Identifying the factors that influence health-related quality of life (HRQoL) is of great scientific interest, but a potential causal relationship between treatment and HRQoL has yet to be fully elucidated. Japanese patients reported better HRQoL outcomes on the Parkinson's Disease Questionnaire (PDQ-39) emotional well-being domain, a 6-question subset of the PDQ-39 which is considered to reflect the emotional aspects of the disease-specific HRQoL, when treated with rasagiline, than placebo, in both a monotherapy clinical trial (NCT02337725) and an adjunctive therapy clinical trial in patients with wearing-off phenomena (NCT02337738).

Objective: To investigate how rasagiline exerts its effect on the PDQ-39 emotional well-being domain in Japanese patients with Parkinson's disease.

F-THK5351 Positron Emission Tomography Imaging in Neurodegenerative Tauopathies.

We aimed to determine whether tau deposits and monoamine oxidase B (MAO-B) detection using F-THK5351 positron emission tomography (PET) can assist in the differential distribution in patients with corticobasal syndrome (CBS), progressive supranuclear palsy (PSP), and Alzheimer's disease (AD) and whether F-THK5351 retention of lesion sites in CBS and PSP can correlate with clinical parameters. F-THK5351 PET was performed in 35 participants, including 7, 9, and 10 patients with CBS, PSP, and AD, respectively, and 9 age-matched normal controls. In CBS and PSP, cognitive and motor functions were assessed using the Montreal Cognitive Assessment, Addenbrooke's Cognitive Examination-Revised, and Frontal Assessment Battery, Unified Parkinson's Disease Rating Scale Motor Score, and PSP Rating Scale.

Impaired perception of illusory contours and cortical hypometabolism in patients with Parkinson's disease.

Neuroimaging evidence suggests that areas of the higher-order visual cortex, including the lateral occipital complex (LOC), are engaged in the perception of illusory contours; however, these findings remain unsubstantiated by human lesion data. Therefore, we assessed the presentation time necessary to perceive two types of illusory contours formed by Kanizsa figures or aligned line ends in patients with Parkinson's disease (PD). Additionally, we used F-fluorodeoxyglucose positron emission tomography (FDG-PET) to measure regional cerebral glucose metabolism in PD patients.

A sublethal ATP11A mutation associated with neurological deterioration causes aberrant phosphatidylcholine flipping in plasma membranes.

ATP11A translocates phosphatidylserine (PtdSer), but not phosphatidylcholine (PtdCho), from the outer to the inner leaflet of plasma membranes, thereby maintaining the asymmetric distribution of PtdSer. Here, we detected a de novo heterozygous point mutation of ATP11A in a patient with developmental delays and neurological deterioration. Mice carrying the corresponding mutation died perinatally of neurological disorders.