Publications by authors named "Takayoshi Shimohata"
Article Synopsis
- This study investigated how adenosine deaminase (ADA) levels in cerebrospinal fluid (CSF) relate to different neurological disorders.
- Five specific diseases showed significantly higher CSF ADA levels compared to a noninflammatory control group, with tuberculous meningitis (TBM) showing the highest increase.
- Strong positive correlations were found between CSF ADA levels and several other laboratory parameters, suggesting that elevated ADA levels might indicate T-cell hyperactivation in the central nervous system.
A 69-year-old woman with left-sided breast cancer developed elevated creatine kinase levels and muscle weakness in her extremities after treatment with pembrolizumab. The patient was diagnosed with immune checkpoint inhibitor (ICI)-related myositis. Although the patient had no symptoms of dysphagia, we evaluated her swallowing function because esophageal dysfunction is a known complication of idiopathic inflammatory myopathy.
View Article and Find Full Text PDFBackground: Despite advances in reperfusion therapies, ischemic stroke remains a major cause of long-term disability due to residual hypoxic lesions persisting after macrovascular reperfusion. These residual hypoxic lesions, caused by microvascular dysfunction, represent an important therapeutic target. We previously demonstrated that oxygen-glucose-deprived peripheral blood mononuclear cells (OGD-PBMCs) migrate to ischemic brain regions and promote functional recovery after stroke.
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- Leonardo da Vinci was not only a talented painter but also had a deep interest in medicine and studied the brain in hopes of finding the "seat of the soul."
- His unique skills, like mirror writing and possible ADHD, may have fueled his creativity but also led to incomplete projects.
- He likely died from a stroke, and speculation exists that nerve issues caused paralysis in his right arm, hindering his ability to paint later in life.
Article Synopsis
- A multicenter study in Japan began in 2014 to gather data on progressive supranuclear palsy (PSP) and corticobasal degeneration (CBD) to better understand their clinical features and identify potential biomarkers.
- Initial assessments of 349 patients focused on symptoms, clinical scores (like the PSPRS), and factors affecting disease progression over eight years.
- Results indicated that patients with Richardson's syndrome (RS) had better clinical scores compared to those with CBD, and cognitive dysfunction played a significant role in disease severity and progression.
Background: Advances in diagnostic procedures have led to an increasing rate of diagnosis of autoimmune encephalitis or paraneoplastic neurological syndrome (AE/PNS) among patients with progressive supranuclear palsy (PSP)-like manifestations.
Methods: In this narrative review, we first discuss the clinical characteristics of AE/PNS in comparison to those of PSP, followed by a discussion of diagnosis and treatment.
Results: The antibodies involved in these conditions include anti-IgLON5, -Ma2, and -Ri antibodies, each of which has a characteristic clinical presentation.
[Significance and Response to APOE Genetic Testing in the Anti-amyloid-β Therapy Era].
Brain Nerve
September 2024
Article Synopsis
- Traditionally, APOE genetic testing wasn't part of standard practices for diagnosing and managing Alzheimer's disease.
- Recent studies emphasize its significance, particularly in evaluating the safety of anti-amyloid-β treatments like lecanemab, which now recommend such testing.
- However, implementing this testing in clinical settings raises various clinical, ethical, legal, and economic challenges that need thorough discussion, especially in Japan.
Article Synopsis
- Hereditary spastic paraplegia (HSP) is a neurological condition with various forms, and SPG26 is a more complex type that involves difficulty with movement, cognitive issues, and other neurological symptoms due to mutations in the GM2S gene.
- This study identified a new genetic variant in a Japanese patient with SPG26, which led to the finding that their cells had impaired ganglioside expression, and laboratory tests confirmed that the variant protein lacked the expected enzyme activity.
- The research also discovered additional potentially harmful genetic variants through analysis of a population database, underscoring the need for further molecular studies on HSP26-related mutations in Japan.
Article Synopsis
- Contactin-associated protein 1 (Caspr1) is found throughout the peripheral and central nervous systems, but cases linking anti-Caspr1 antibodies to CNS symptoms have been rare.
- A 69-year-old man with polyneuropathy and memory loss tested positive for anti-Caspr1 antibodies in both his blood and cerebrospinal fluid, leading to a diagnosis of anti-Caspr1 nodopathy.
- Following treatment with rituximab, the patient's symptoms—including polyneuropathy and memory issues—significantly improved, highlighting the need to consider CNS effects of anti-Caspr1 antibodies.
Article Synopsis
- Two patients diagnosed with multiple system atrophy (MSA) exhibited symptoms of autoimmune cerebellar ataxia and responded positively to immunotherapy.
- Patient 1 had mild improvement and showed inflammatory markers in his cerebrospinal fluid, while Patient 2 had a more aggressive progression of symptoms.
- Anti-neuronal antibodies were identified in both patients, specifically targeting Purkinje cell cytoplasm, suggesting a link between autoimmune processes and MSA-like symptoms.
Article Synopsis
- - The study investigates how well conventional MRI can differentiate corticobasal degeneration (CBD) from its mimics due to similar clinical features.
- - Researchers analyzed the degree of brain atrophy and asymmetry in MRI images of 19 CBD patients and 16 patients with conditions that mimic CBD, like Alzheimer's and progressive supranuclear palsy.
- - Findings suggest that specific patterns of atrophy and the presence of white matter hyperintensity can be used as imaging biomarkers to help diagnose CBD more accurately.
Article Synopsis
- The case discusses chronic progressive autoimmune GFAP astrocytopathy, which is a condition affecting the brain's supportive cells (astrocytes).
- High-intensity signals were found in specific brain areas, indicating inflammation or damage, particularly in the white matter and certain lobes.
- The patient showed improvement after receiving four courses of intravenous methylprednisolone (IVMP) and one course of intravenous immunoglobulin (IVIg).
Complete nanopore repeat sequencing of SCA27B (GAA- ataxia) in Japanese.
J Neurol Neurosurg Psychiatry
November 2024
Article Synopsis
- This study investigated repeat expansion in patients with adult-onset cerebellar ataxia, focusing on the differences between Japanese and non-Japanese populations.
- The researchers used nanopore sequencing to analyze samples from 460 Japanese patients and various control groups, finding that different repeat motifs (GCA in Japanese vs. GGA in non-Japanese) affect pathogenicity.
- Ultimately, the study highlights how unique features of repeat expansion and genetic background contribute to the prevalence of the disease in different ethnic groups.
A 59-year-old man had developed visual abnormality, nausea, headache, and weight loss since three months before. The ophthalmologist found severe optic disc edema in both eyes, and referred him to our hospital. The patient had mild cerebellar ataxia.
View Article and Find Full Text PDFNutritional interventions targeting weight loss are useful for the treatment of amyotrophic lateral sclerosis (ALS). However, the changes in body composition after nutritional intervention remain unclear. We herein present a patient with ALS who experienced an increased weight and muscle mass owing to nutritional therapy and physical exercise.
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- - Paraneoplastic disorders of the peripheral nervous system are immune-related conditions that occur alongside tumors, with various clinical symptoms.
- - The most recognized symptom is sensory neuronopathy, resulting from damage to the dorsal root ganglia, which often helps in diagnosing underlying tumors.
- - Detection of antineuronal antibodies in the blood or cerebrospinal fluid can occur, and early tumor removal along with immunotherapy may help alleviate symptoms and improve patient health.
Article Synopsis
- Multiple system atrophy (MSA) is a challenging, incurable neurodegenerative disease, causing neurologists significant difficulty in delivering diagnoses due to its life-threatening implications, especially the risk of sudden death.
- A study involving 194 neurologists in Japan found that 92.3% found it difficult to explain MSA diagnoses, while 82.8% struggled with discussing sudden death risks.
- Factors contributing to these challenges include the emotional burden on neurologists, complexity of conveying crucial information, the family’s role in treatment decisions, and the inherent difficulties in diagnosing MSA.
Anti-IgLON5 disease as a differential diagnosis of multiple system atrophy.
Parkinsonism Relat Disord
July 2024
Article Synopsis
- - Anti-IgLON5 disease is a rare autoimmune condition that can mimic symptoms of various neurodegenerative disorders, notably progressive supranuclear palsy and multiple system atrophy (MSA).
- - In a study of 35 patients suspected of having MSA, anti-IgLON5 antibodies were found in three individuals who exhibited similar clinical features, such as parkinsonism and severe orthostatic hypotension, along with some atypical symptoms.
- - The research suggests that anti-IgLON5 disease should be considered when diagnosing MSA, emphasizing the need for thorough physical examinations to differentiate between these conditions effectively.
Article Synopsis
- A 54-year-old Japanese man developed headache and fever after receiving a SARS-CoV-2 vaccine, and within a week, he became deeply unconscious.
- MRI and cerebrospinal fluid tests revealed abnormal lesions in his brain, leading to a diagnosis of autoimmune GFAP-astrocytopathy (GFAP-A) with positive anti-GFAP antibodies.
- Despite treatment with steroids and immunoglobulin therapy, the patient's condition worsened, with further enlargement of brain lesions and atypical pathological findings showing inflammation and damage to neural cells.
Article Synopsis
- * Medical examinations revealed severe hypotension and issues with esophageal motility, including lower esophageal stricture and hypercontraction, while high-resolution manometry indicated premature contractions and reduced peristalsis.
- * The man's symptoms related to food stagnation and vomiting improved after endoscopic balloon dilation, highlighting that distal esophageal spasm (DES) can be a complication in MSA patients.
Article Synopsis
- A 26-year-old woman with polymyositis and receiving immunosuppressive therapy contracted COVID-19 (omicron strain) and developed fever followed by status epilepticus shortly after hospital admission.
- Brain imaging showed abnormalities, and despite normal CSF tests, IL-8 levels were significantly higher in the CSF compared to the serum, suggesting potential inflammation in the brain.
- The patient's condition deteriorated despite treatments like steroid therapy and remdesivir, leading to her death from sepsis, highlighting the serious risk of acute necrotizing encephalopathy (ANE) even in cases of omicron COVID-19.
Article Synopsis
- Anti-IgLON5 disease is an autoimmune encephalitis that often goes undiagnosed, characterized by diverse symptoms affecting sleep, movement, and other functions.
- A study of 87 patients revealed strong associations between the disease and specific HLA-DQ genotypes, indicating a genetic predisposition to its development.
- Experiments showed that modified IgLON5 peptides preferentially bind to these HLA-DQ receptors, suggesting that this interaction could trigger T-cell responses involved in initiating the disease.
This study aimed to retrospectively review the frequency and clinical features of 13 patients with progressive supranuclear palsy (PSP) and idiopathic normal pressure hydrocephalus (iNPH). All patients were found to have PSP-Richardson's syndrome (PSP-RS). Shunt surgery was effective in 5 of 11 patients (45.
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