Keisuke Arasaki

Blood brain barrier destruction in hyperglycemic chorea in a patient with poorly controlled diabetes

A case of hemichorea in a patient with poorly controlled diabetes is reported. T1-weighted magnetic resonance imaging (MRI) showed an unusual homogeneous high-intensity area in the corpus striatum. Of interest in the case was the fact that the globus pallidus, which was enhanced with gadolinium at the onset of hemichorea, showed homogeneous high-intensity on a subsequent T1-weighted image. This indicated that blood brain barrier destruction preceded the signal intensity change in the basal ganglia. As far as the authors could determine, this is the first reported case showing such enhancement during the course of diabetic hemichorea.

Reduction in the motor unit number estimate (MUNE) after cerebral infarction

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.

The pattern of antiganglioside antibody reactivities producing myelinated nerve conduction block in vitro

We studied the pattern of human antiganglioside antibody reactivities causing an acute conduction block in rat myelinated nerve fibers, using an in vitro preparation of the sciatic-tibial nerve. With the aid of complements, IgM antibodies reacting with the terminal disaccharide of galactose (beta1-3)N-acetylgalactosamine produced the block. These findings may help us to understand the mechanism in which the conduction block occurs in neuropathies associated with antiganglioside antibodies.

Validity of electromyograms and tension as a means of motor unit number estimation

The purpose of this study was to validate three different techniques for obtaining motor unit number estimates of the rat medial gastrocnemius muscle. These consisted of two electromyographic techniques using unprocessed and digitally averaged unitary muscle action potentials, and one mechanical technique. We also injected subunit B of cholera toxin into this muscle and counted the number of spinal motor neurons labeled by the toxin. Our results revealed that a motor unit number estimate obtained by using the unprocessed unitary muscle action potential was statistically different from the actual number of motor neurons. The other two motor unit number estimates, however, were not statistically different from the actual motor neuron number. These two methods thus seem more appropriate than the first electromyographic method for obtaining an accurate motor unit number estimate.

A loss of functional spinal alpha motor neurons in amyotrophic lateral sclerosis

We obtained motor unit number estimates (MUNEs) of the hypothenar and the extensor digitorum brevis muscles in ALS patients by our new technique. One year after symptom onset, the MUNEs had decreased to ±30% of normal. Accordingly, we suggest that 70% of functional spinal alpha motor neurons are lost in the first post-onset year in ALS.

Longitudinal study of functional spinal alpha motor neuron loss in amyotrophic lateral sclerosis

Using a microstimulation technique for obtaining motor unit number estimates (MUNEs) of the hypothenar and extensor digitorum brevis (EDB) muscles, we performed a longitudinal study on the natural course of change in the clinical rating scale (Appel score) and of loss of functional spinal alpha motor neurons in amyotrophic lateral sclerosis. The Appel score increased to about 150% of normal at 12 months after onset, about 225% at 18 months after onset, and about 370% at 24 months after onset. By contrast, MUNEs decreased to about 27% of normal at 12 months after onset, about 12% at 18 months after onset, and about 5% at 24 months after onset. The relative merits of these different approaches in detecting changes in the disease process in its early phase are discussed.

Maximal and minimal motor nerve conduction velocities in amyotrophic lateral sclerosis

In 15 patients with amyotrophic lateral sclerosis (ALS) and 20 age-matched control subjects, we measured the maximal and minimal motor nerve conduction velocities of the ulnar nerve as well as the action potential amplitude of the abductor digiti minimi muscle. Both maximal and minimal motor nerve conduction velocities in ALS patients were significantly lower than those in the control group. However, the difference between the maximal and minimal conduction velocities in each ALS patient was not statistically different from that in each control subject. The maximal action potential amplitude of the muscle in the ALS patients was significantly smaller than that in the control group.

Autosomal dominant childhood onset slowly progressive leukodystrophy—a Japanese family with spastic paraparesis, ataxia, mental deterioration, and skeletal abnormality

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.

Reduction in the motor unit number estimate (MUNE) after cerebral infarction.

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.