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1.
Intern Med ; 61(24): 3739-3741, 2022 Dec 15.
Artigo em Inglês | MEDLINE | ID: mdl-36198594

RESUMO

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. Her neurological symptoms improved two days after methylprednisolone pulse therapy. Cerebrospinal fluid (CSF) was negative for SARS-CoV-2 reverse transcription-polymerase chain reaction, the CSF-to-serum albumin quotient was mildly elevated, and interleukin 6 and 8 levels were normal in serum but mildly elevated in CSF. Omicron variant infection may increase blood-brain barrier permeability and intrathecal inflammation, causing autoimmune encephalitis.


Assuntos
Doenças Autoimunes do Sistema Nervoso , COVID-19 , Feminino , Humanos , Pessoa de Meia-Idade , SARS-CoV-2 , COVID-19/complicações , Esteroides
2.
J Neuroimmunol ; 361: 577748, 2021 12 15.
Artigo em Inglês | MEDLINE | ID: mdl-34653948

RESUMO

We report two patients with meningoencephalomyelitis without evidence of extra central nervous system (CNS) involvement. Brain MRI showed linear perivascular radial gadolinium enhancement patterns and spinal cord MRI showed longitudinal extensive T2-hyperintensity lesions. Pathological findings from brain biopsies were angiocentric T-cell predominant lymphoid infiltrates that lacked Epstein-Barr virus-positive atypical B cells. The patients were initially suspected to have isolated CNS-lymphomatoid granulomatosis (LYG). Thereafter, glial fibrillary acidic protein (GFAP)-immunoglobulin G were detected in their cerebrospinal fluid. This finding suggested autoimmune GFAP astrocytopathy. We speculate there is a link between isolated CNS-LYG and autoimmune GFAP astrocytopathy.


Assuntos
Astrócitos/patologia , Doenças Autoimunes do Sistema Nervoso/diagnóstico , Proteína Glial Fibrilar Ácida/imunologia , Granulomatose Linfomatoide/diagnóstico , Corticosteroides/uso terapêutico , Idoso , Especificidade de Anticorpos , Astrócitos/imunologia , Autoanticorpos/líquido cefalorraquidiano , Doenças Autoimunes do Sistema Nervoso/líquido cefalorraquidiano , Doenças Autoimunes do Sistema Nervoso/imunologia , Doenças Autoimunes do Sistema Nervoso/patologia , Encéfalo/diagnóstico por imagem , Encéfalo/imunologia , Encéfalo/patologia , Diagnóstico Diferencial , Humanos , Imunoglobulina G/líquido cefalorraquidiano , Imunoglobulinas Intravenosas/uso terapêutico , Imageamento por Ressonância Magnética , Masculino , Meningoencefalite/etiologia , Pessoa de Meia-Idade , Mielite/etiologia , Neuroimagem , Estudos Retrospectivos , Medula Espinal/diagnóstico por imagem , Medula Espinal/imunologia , Medula Espinal/patologia
3.
Rinsho Shinkeigaku ; 61(8): 522-529, 2021 Aug 30.
Artigo em Japonês | MEDLINE | ID: mdl-34275949

RESUMO

In Kyudo (Japanese archery), there are four disorders that hinder an archer's performance: Hayake (releasing the bow too early), Motare (unable to release the bow when intended), Biku (jerking when aiming), and Yusuri (shaking when drawing the bow, or aiming). These disorders are similar to Yips, a psycho-neuromuscular movement disorder, recognized in various sports, but few studies have examined yips in Kyudo. This study examined the frequency, classification, and risk factors of yips in Kyudo among medical students. The results showed that 41 of 65 students (63.1%) experienced at least one disorder. The frequency of Hayake was the highest (35 patients; 85.3%). An experience of playing was associated with the increased risk of yips in Kyudo. Motare was the only disorder that appeared on its own, and without complications from other disorders. Based on its characteristics, it was suspected that task-specific focal dystonia involved in Motare.


Assuntos
Distúrbios Distônicos , Transtornos dos Movimentos , Esportes , Humanos , Japão/epidemiologia , Prevalência
5.
Prion ; 14(1): 109-117, 2020 12.
Artigo em Inglês | MEDLINE | ID: mdl-32178563

RESUMO

The clinical characteristics of genetic Creutzfeldt-Jakob disease (gCJD) with a V180I mutation in the PRNP gene (V180I gCJD) are unique: elderly-onset, gradual progression, sporadic fashion, and cortical oedematous hyper-intensity on diffusion-weighted MRI (DW-MRI). This phenotype may become a potential target of future clinical therapeutic trials. The average disease duration of V180I gCJD patients is 23-27 months; however, considerably long-term survivors are also reported. The factors influencing survival and the clinicopathological characteristics of long-term survivors remain unknown. Herein, we report clinicopathological findings of a long-term survivor of V180I gCJD. A 78-year old woman was admitted to our hospital due to dementia and left hand tremor approximately 1.5 months after symptom onset. Neurological examination revealed dementia, frontal signs, and left hand tremor at admission. She had no family history of dementia or other neurological disease. DW-MRI revealed cortical oedematous hyper-intensities in the bilateral frontal lobes and the right temporal and parietal lobes. PRNP gene analysis indicated a V180I mutation with methionine homozygosity at codon 129. The symptoms gradually progressed, and she died of aspiration pneumonia 61 months after symptom onset. Neuropathological examination revealed severe cerebral atrophy with moderate to severe gliosis, but the brainstem was well preserved. Various-sized and non-confluent vacuole type spongiform changes were extensively observed in the cerebral cortices. Prion protein (PrP) immunostaining revealed weak and synaptic-type PrP deposits in the cerebral cortices. We consider that long-term tube feeding, and very mild brainstem involvement may be associated with the long-term survival of our V180I gCJD patient.


Assuntos
Síndrome de Creutzfeldt-Jakob/genética , Síndrome de Creutzfeldt-Jakob/patologia , Mutação/genética , Sobreviventes , Idoso , Envelhecimento/patologia , Síndrome de Creutzfeldt-Jakob/diagnóstico por imagem , Imagem de Difusão por Ressonância Magnética , Endopeptidase K/metabolismo , Evolução Fatal , Feminino , Humanos , Inflamação/patologia , Proteínas Priônicas/genética
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