RESUMO
We examined the relationship between the degree to which motor unit number estimates (MUNEs) decrease in association with the clinical features of patients with the infarction. Using a multiple-point stimulation technique, we obtained the MUNE of the hypothenar muscle group in 13 age-matched control subjects and 30 patients with cerebral infarction. In all patients, we obtained the Japan Stroke Scale (JSS) and head MR images. In 8 patients with acute cerebral infarction, admitted within 24 h after onset, we also obtained head MR angiograms and single-photon emission CT. There was a decrease in the MUNE of the hypothenar muscle group on the affected side of 24 patients with cerebral infarction and hand weakness. The decrease in the MUNE started from 4 to 30 h after the infarction, when T1-weighted MR images of the brain involved were normal. The degree to which the MUNE decreased correlated with the part of the JSS showing the upper extremity weakness. A decrease in the MUNE of the hypothenar muscle group within 30 h after cerebral infarction may be due to transsynaptic inhibition of the spinal alpha motor neurons innervating this muscle.
Assuntos
Potenciais de Ação/fisiologia , Infarto Cerebral/patologia , Infarto Cerebral/fisiopatologia , Neurônios Motores/fisiologia , Músculo Esquelético/patologia , Idoso , Idoso de 80 Anos ou mais , Estimulação Elétrica/métodos , Feminino , Humanos , Imageamento por Ressonância Magnética/métodos , Masculino , Pessoa de Meia-Idade , Músculo Esquelético/fisiopatologia , Índice de Gravidade de Doença , Estatísticas não ParamétricasAssuntos
Edema Encefálico/patologia , Corpo Caloso/patologia , Imagem de Difusão por Ressonância Magnética/normas , Encefalite/patologia , Adulto , Edema Encefálico/etiologia , Edema Encefálico/fisiopatologia , Meios de Contraste/farmacocinética , Corpo Caloso/fisiopatologia , Difusão , Encefalite/fisiopatologia , Feminino , Humanos , Fibras Nervosas Mielinizadas/patologia , Vias Neurais/patologia , Vias Neurais/fisiopatologia , Valor Preditivo dos TestesRESUMO
BACKGROUND: The mechanism of the decrease in motor unit number estimates (MUNEs) after cerebral infarction has not been studied systematically. We examined the relationship between the degree to which MUNEs decreased and the other clinical features of patients with the infarction. METHODS: Using a multiple point stimulation technique, we obtained the MUNE of the hypothenar muscle group in 13 age-matched control subjects and 30 patients with cerebral infarction. In all patients, we obtained the Japan Stroke Scale (JSS) and head MR images. In eight patients with acute cerebral infarction, admitted within 24 h after onset, we also obtained head MR angiograms and single-photon emission CT. FINDINGS: There was a decrease in the MUNE of the hypothenar muscle group on the affected side of 24 patients with cerebral infarction and hand weakness. The decrease in the MUNE started from 4 to 30 h after the infarction, when T1-weighted MR images of the brain involved were normal. The degree to which the MUNE decreased correlated with the part of the JSS showing the upper extremity weakness. INTERPRETATIONS: A decrease in the MUNE of the hypothenar muscle group within 30 h after cerebral infarction may be due to trans-synaptic inhibition of the spinal alpha motor neurons innervating this muscle.
Assuntos
Infarto Cerebral/fisiopatologia , Mãos/fisiopatologia , Córtex Motor/fisiopatologia , Neurônios Motores/fisiologia , Músculo Esquelético/fisiopatologia , Tratos Piramidais/fisiopatologia , Potenciais de Ação/fisiologia , Idoso , Idoso de 80 Anos ou mais , Infarto Cerebral/patologia , Estimulação Elétrica , Eletromiografia , Potenciais Pós-Sinápticos Excitadores/fisiologia , Mãos/inervação , Humanos , Imageamento por Ressonância Magnética , Pessoa de Meia-Idade , Córtex Motor/patologia , Músculo Esquelético/inervação , Degeneração Neural/etiologia , Degeneração Neural/fisiopatologia , Condução Nervosa/fisiologia , Paresia/etiologia , Paresia/fisiopatologia , Nervos Periféricos/fisiopatologia , Valor Preditivo dos Testes , Tratos Piramidais/patologia , Transmissão Sináptica/fisiologia , Fatores de Tempo , Tomografia Computadorizada por Raios XRESUMO
Autosomal dominant leukodystrophy is an extremely rare disease. Here we report on a dominantly inherited disease in a Japanese family with slowly progressive clinical course. Their symptoms and signs started in early childhood and very slowly progressed. In most patients spastic gait was the initial symptom. Neurological manifestations were characterized by pyramidal signs, ataxia, and mental deterioration. In addition to these neurological signs, the skeletal anomalies such as scoliosis and congenital hip dislocation were also present. MR images showed no abnormality in the early stage, but T2-weighted images revealed high intensity areas in the cerebral and cerebellar white matter, and the dentate nucleus in the advanced stage. Proton MR spectroscopy showed decrease of N-acetylaspartate/creatine ratio and increase of choline/creatine ratio in the advanced stage. Proton MR spectroscopy revealed normal N-acetylaspartate/creatine ratio and increase of choline/creatine ratio in the early stage. We suggested that these patients had abnormality in the white matter when MRI was still normal. We considered that intracranial demyelination was gradually progressed as the symptoms got aggravated.
