ABSTRACT
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.
Subject(s)
Action Potentials/physiology , Cerebral Infarction/pathology , Cerebral Infarction/physiopathology , Motor Neurons/physiology , Muscle, Skeletal/pathology , Aged , Aged, 80 and over , Electric Stimulation/methods , Female , Humans , Magnetic Resonance Imaging/methods , Male , Middle Aged , Muscle, Skeletal/physiopathology , Severity of Illness Index , Statistics, NonparametricSubject(s)
Brain Edema/pathology , Corpus Callosum/pathology , Diffusion Magnetic Resonance Imaging/standards , Encephalitis/pathology , Adult , Brain Edema/etiology , Brain Edema/physiopathology , Contrast Media/pharmacokinetics , Corpus Callosum/physiopathology , Diffusion , Encephalitis/physiopathology , Female , Humans , Nerve Fibers, Myelinated/pathology , Neural Pathways/pathology , Neural Pathways/physiopathology , Predictive Value of TestsABSTRACT
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.
Subject(s)
Cerebral Infarction/physiopathology , Hand/physiopathology , Motor Cortex/physiopathology , Motor Neurons/physiology , Muscle, Skeletal/physiopathology , Pyramidal Tracts/physiopathology , Action Potentials/physiology , Aged , Aged, 80 and over , Cerebral Infarction/pathology , Electric Stimulation , Electromyography , Excitatory Postsynaptic Potentials/physiology , Hand/innervation , Humans , Magnetic Resonance Imaging , Middle Aged , Motor Cortex/pathology , Muscle, Skeletal/innervation , Nerve Degeneration/etiology , Nerve Degeneration/physiopathology , Neural Conduction/physiology , Paresis/etiology , Paresis/physiopathology , Peripheral Nerves/physiopathology , Predictive Value of Tests , Pyramidal Tracts/pathology , Synaptic Transmission/physiology , Time Factors , Tomography, X-Ray ComputedABSTRACT
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.
