Publications by authors named "Nobuaki Yoshikura"

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.
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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.
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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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Article Synopsis
  • Two cases of anti-N-methyl-d-aspartate receptor (NMDAR) encephalitis following prolonged meningitis are discussed, highlighting a gap in existing literature.
  • The patients experienced meningitis for 60 days and 22 days, which improved with treatments like intravenous methylprednisolone and plasmapheresis.
  • The study suggests that anti-NMDAR encephalitis should be considered when diagnosing patients with long-lasting meningitis, especially since the patients had particularly lengthy episodes of meningitis.
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Despite the clinical impact of dysphagia in myasthenia gravis (MG), a standard protocol for diagnosing dysphagia reliably has not yet been established. High-resolution manometry (HRM) provides precise information on pharyngeal pressure. We hypothesized that swallowing pressure assessment using HRM during the edrophonium chloride (EC) test could identify mild bulbar symptoms with no abnormalities on videoendoscopic (VE) and videofluorographic (VF) examination of swallowing, and we tested this hypothesis on a 72-year-old female patient diagnosed with ocular MG who developed slight pharyngeal discomfort over 3 months.

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Anti-metabotropic glutamate receptor 1 (mGluR1) encephalitis is a rare autoimmune disorder manifesting with cerebellar syndrome. Patients with mGluR1 encephalitis have been treated with immunomodulatory therapies; however, little is known about the efficacy of this therapy. A 58-year-old Japanese woman presented with dizziness when walking and standing up.

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In the field of hereditary spastic paraplegia (HSP), progress in molecular diagnostics needs to be translated into robust phenotyping studies to understand genetic and phenotypic heterogeneity and to support interventional trials. ZFYVE26-associated hereditary spastic paraplegia (HSP-ZFYVE26, SPG15) is a rare, early-onset complex HSP, characterized by progressive spasticity and a variety of other neurological symptoms. While prior reports, often in populations with high rates of consanguinity, have established a general phenotype, there is a lack of systematic investigations and a limited understanding of age-dependent manifestation of symptoms.

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We encountered a 55-year-old woman with possible autoimmune encephalitis associated with the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) Omicron variant. She was not vaccinated against coronavirus disease 2019 (COVID-19). Consciousness disturbance, myoclonic-like movements and gait disturbance occurred 10 days after the COVID-19 symptom onset.

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Idiopathic sporadic ataxia (ISA) is the clinical term for nonfamilial ataxia with adult-onset and a slowly progressive course. However, immune-mediated cerebellar ataxia cannot be completely excluded from ISA. The current study investigated the neuropil antibodies against cell-surface antigens and clarified the clinical features and neuroimaging findings of patients with these antibodies.

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Progressive supranuclear palsy (PSP) with predominant frontal presentation (PSP-F) is a clinical phenotype of PSP that is characterized by frontal cognitive impairment and behavioral changes. Here, we report on a patient with pathologically diagnosed PSP-F in whom we were able to observe temporal changes of the clinical manifestations. A 77-year-old right-handed man developed progressive nonfluent aphasia (PNFA) at the age of 69 years, festinating gait, and clumsiness of his left arm at age 75, disinhibition at age 76, and unprovoked falls at age 77.

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Cerebellar ataxia, neuropathy, vestibular areflexia syndrome (CANVAS) is a late-onset, slow-progressing multisystem neurodegenerative disorder. Biallelic AAGGG repeat expansion in RFC1 has been identified as causative of this disease, and repeat conformation heterogeneity (ACAGG repeat) was also recently implied. To molecularly characterize this disease in Japanese patients with adult-onset ataxia, we accumulated and screened 212 candidate families by an integrated approach consisting of flanking PCR, repeat-primed PCR, Southern blotting and long-read sequencing using Sequel II, GridION or PromethION.

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Background: Neurological manifestations of coronavirus disease 2019 (COVID-19) are increasingly recognized and include encephalopathy, although direct infection of the brain by SARS-CoV-2 remains controversial. We herein report the clinical course and cytokine profiles of a patient with severe SARS-CoV-2-related encephalopathy presenting aphasia.

Case Presentation: An 81-year-old man developed acute consciousness disturbance and status epileptics several days after SARS-CoV-2 infection.

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Article Synopsis
  • The text discusses two patients diagnosed with meningoencephalomyelitis, which did not show involvement of other areas outside the central nervous system (CNS).
  • MRI scans revealed specific patterns of enhancement in the brain and significant lesions in the spinal cord, with brain biopsies showing T-cell lymphoid infiltrates without Epstein-Barr virus presence.
  • The initial diagnosis was isolated CNS-lymphomatoid granulomatosis, but detection of GFAP-immunoglobulin G in cerebrospinal fluid indicated a possible autoimmune condition, suggesting a connection between the two diagnoses.
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We present the case of a 72-year-old woman with slowly progressive spastic paraplegia and painful muscle spasms of the lower limbs. Spastic paraplegia began in the left lower extremity and extended to the right lower extremity 4 months later. We considered the diagnosis of amyotrophic lateral sclerosis (ALS) because of the left-dominant spastic paraplegia of bilateral lower limbs and due to the presence of fasciculation, hyperreflexias, and pathological reflexes.

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We report a case of primary central nervous system vasculitis (PCNSV) with longitudinally extensive transverse myelitis. A 47-year-old woman presented with malaise, progressive cognitive impairment, and lower limb spasticity. Diffuse hyperintense areas in the deep cerebral white matter on the diffusion-weighted image and T2-weighted images were observed during brain magnetic resonance imaging.

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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.

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Various neurological syndromes are associated with autoimmune encephalitis. Anti-mGluR1 antibody encephalitis presents mainly as subacute cerebellar ataxia, while behavioral changes and involuntary movements also occur. Anti-IgLON5 disease presents mainly as a sleep disorder.

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What Is Known And Objective: Ifosfamide, an alkylating agent, is widely used in the treatment of malignant diseases. However, these treatments are often limited due to the incidence of neuropsychiatric symptoms such as delirium, seizures, hallucinations and agitation. In this study, we examined risk factors for neuropsychiatric symptoms in patients receiving ifosfamide-based chemotherapy.

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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.

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Since autonomic dysfunction is closely associated with autoimmune encephalitis (AE), the objective of this study was to determine the autonomic symptoms and the prevalence of anti-α3 subunit of the ganglionic-type nicotinic acetylcholine receptor (gAChRα3) antibodies in the patients with AE. We reviewed the clinical features of 19 AE patients, and specifically analyzed sera for anti-gAChRα3 antibodies using the luciferase immunoprecipitation system (LIPS) assay. Cardiovascular autonomic symptoms were found to be common in patients with AE, and hypersalivation was seen only in patients with NMDAR encephalitis.

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Recently, the diagnostic criteria for idiopathic cerebellar ataxia (IDCA) have been proposed in Japan as a diagnosis to replace the clinical concept of cortical cerebellar atrophy, which was originally described as a neuropathological disorder. However, IDCA proposed in Japan may include various diseases such as multiple system atrophy with early stage, rare hereditary ataxias, and autoimmune-mediated cerebellar ataxia. We tackled this significant clinical challenge by detecting anti-cerebellar autoantibodies in patients' sera and identifying their target antigens.

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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.

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Article Synopsis
  • A 71-year-old man with left finger extension issues was initially thought to have Dupuytren contracture due to nodular lesions in his palm.
  • Further neurological exams showed muscle weakness and related abnormalities linked to C7-Th1 lesions, indicating another condition.
  • The final diagnosis combined distal-type cervical spondylolisthesis with Dupuytren contracture, highlighting the importance of detailed assessments to distinguish between overlapping symptoms.
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Article Synopsis
  • * The ear infections stemmed from issues like nasopharyngeal reflux and weakened eustachian tube muscles due to her condition and body position.
  • * It's important for doctors to understand that ALS patients often struggle to communicate pain and may overlook brain abscesses, emphasizing the need for better awareness and responsive care.
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