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Biomedical subjects

T Shimpo

Publications and source records attributed to T Shimpo.

At least 19 recordsLinked to original sources

[Somatic mosaicism of p(CTG)n expansion in a case of myotonic dystrophy with parotid tumor].

Myotonic dystrophy (MD) is an autosomal dominant systemic disorder with an unstable expansion of the CTG triplet repeat in the 3'-untranslated region of the gene encoding myotonine protein kinase (DMPK) which maps to chromosome 19q13.3. Somatic mosaicism of CTG repeats in MD has been reported; and it has been observed that CTG repeats in tumor tissues associated with MD are more expanded than the other tissues. It is not rare that parotid tumors are found in patients with MD. We performed Southern blot analysis for tissues from the parotid tumor, the normal parotid gland, the skeletal muscles, and the leukocyte from a 60-year-old patient with MD. CTG repeat was most expanded in the parotid tumor, and the normal parotid gland had longer expansion of CTG repeat than the skeletal muscles. The leukocyte had the shortest expansion of CTG repeat. The expansion of CTG repeat in the parotid tumor may be related to active cell division and may underlie the occurrence of tumors in MD.

Adenoma, Pleomorphic↗

[Pure word deafness after cerebral hemorrhage in the left temporal lobe: a case report].

We report a patient with pure word deafness after subcortical hemorrhage in the left temporal lobe. Repetition and auditory comprehension were severely impaired, while reading and visual comprehension of the same material were almost normal. He did not show hearing loss, but speech discrimination and melody recognition was poor. On the speech discrimination test, his score was low especially in the right ear. The threshold on the directional hearing test was mildly elevated. There was no temporal summation by click sounds. CT and MRI disclosed a subcortical hematoma in the left superior temporal gyrus. PET demonstrated hypoperfusion in the surrounding area, which was not activated by hearing a story. It was considered that pure word deafness in this case was due to the interruption of auditory inputs to Wernicke's area from both hemisphere by the hematoma. After 5 months, auditory comprehension recovered so that he did not have difficulty in conversation. Speech discrimination improved in both ears, probably due to the recovery of two auditory pathways; the ipsilateral pathway through the left auditory radiation and the contralateral pathway through the right auditory radiation and the corpus callosum. This case suggests that in pure word deafness due to a unilateral lesion, the improvement in speech discrimination during follow-up period may provide a clue as to the site of the responsible lesion and its recovery.

Aged↗

[Retrocollis and oculogyric crisis in association with bilateral putaminal hemorrhages].

A 50-year-old man with a history of hypertension developed right hemiparesis in February, 1985. Four years later, he noted tremor and involuntary extension of the neck (retrocollis) which was aggravated by walking or emotional stimuli. In addition to retrocollis, which was most pronounced upon turning the head to the left or backward, there also was upward deviation of the eyes (oculogyric crisis) and contraction of the left orbicularis oculi muscle. Magnetic resonance imaging study revealed one small old hematoma in the left posterior putamen and two in the right lenticular nucleus (one in the posterior putamen and the other in the globus pallidus). The findings in this case and in other reported cases of symptomatic retrocollis suggest that bilateral lesions of the putamen are associated with this type of focal dystonia.

Basal Ganglia↗

Onset and offset of electromyographic (EMG) silence in asterixis.

The onset and offset of electromyographic (EMG) silence were studied physiologically by silent period locked averaging method (SPLA) combined with a computer-assisted method for detecting EMG changes in 11 patients with asterixis of various aetiologies. The onset followed the EMG discharge which was closely associated with a sharp wave probably generated by the motor cortex in three patients. No EEG activity could be shown to be related to the offset of EMG silence in every patient. Jerky movement of asterixis was temporally related to the offset of EMG silence rather than the onset.

Adult↗

Magneto-electrical stimulation of central motor pathways compared with percutaneous electrical stimulation.

The central motor conduction to the relaxed muscles was studied in 30 normal volunteers using magneto-electrical stimulation (MES) of the central motor pathways. The results were compared with those obtained by the percutaneous electrical stimulation technique (PES) described previously. None of the cortical and spinal latencies (Lcor and Lsp, respectively) and the central motor conduction time were different between MES and PES in the upper limb muscles. In some lower limb muscles, however, the Lsps of MES were significantly shorter than those of PES. This was probably because the magnetic stimulation over the lumbar spinal column activated the motor roots at their exit from the spinal canal rather than the level of conus medullaris, at which activation occurs in the electrical stimulation.

Adult↗

[A case of chronic inflammatory demyelinating polyradiculoneuropathy with orthostatic hypotension].

A 27-year-old man was admitted to our hospital for his legs' numbness of subacute onset and discomfort while standing. No specific previous history was found and his family history was non-contributory. On admission, his general status was unremarkable except for arterial hypertension and mild tachycardia. Moderate impairment of superficial sensations and dysesthesia were noted in the distal extremities, tongue, oral cavity, and lips. Deep sensation was moderately impaired in the lower legs. Romberg sign was positive. He had mild weakness in the proximal muscles of the lower extremities. Hyporeflexia was noted in all extremities, but Achilles reflexes were absent. Pathologic reflexes were not noted. He fainted after two minute standing. On laboratory examination, serum IgM, C3, and C4 were mildly elevated. CSF protein level was prominently high without CSF pleocytosis. MCV was mildly decreased, and F wave conduction velocity was prominently decreased in the posterior tibial nerve, SCV was also mildly decreased in the right sural nerve. Needle electromyography showed mild neuropathic changes. Left sural nerve biopsy showed no abnormal finding in the myelinated and unmyelinated fibers. A 60 degree head-up tilting test caused a hypotensive attack, and Valsalva ratio was decreased. However, hand grip test and cold pressor test were normal. The response to noradrenaline infusion test and CVR-R were also normal. Muscle sympathetic activity (MSA) was recorded from the tibial nerve using a tungsten microelectrode (Iwase, et al.). His basic activity was higher and responsiveness was lower than age-matched normal controls. The regression line existed above the normal range.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

Physiological analysis of asterixis: silent period locked averaging.

Asterixis was studied in nine patients, using a new electrophysiological technique: silent period locked averaging (SPLA). There were two types of electromyographic (EMG) silence in the movements clinically judged as asterixis. The jerky movement in one of the two types might be caused by the silent period after the subclinical cortical myoclonus. SPLA would be useful for studying asterixis as well as other EMG silences.

Cerebral Cortex↗

Physiologic analysis of central motor pathways--simultaneous recording from multiple relaxed muscles.

The central motor pathways were studied with the technique of percutaneous electrical stimulation of the central nervous system in 40 normal volunteers. There were no significant differences between the right and left sides, or between males and females. A linear correlation between the cortical latency (Lcor) or the spinal latency (Lsp) and body height was observed, however, there was no correlation between the central conduction time (CCT) (Lcor-Lsp) and body height. Consequently, the CCT appears to provide a reliable estimate of the function of central motor pathways. We have established normal values for eight muscles at rest that would be required for localizing a very small intraspinal lesion.

Adult↗

[Magneto-electrical stimulation (MES)--compared with percutaneous electrical stimulation (PES)].

The central motor conduction was studied in 30 normal volunteers using a recently developed magneto-electrical stimulation technique (MES). The results were compared with those obtained by percutaneous electrical stimulation technique (PES) described previously. We made a magnetic stimulator similar to that of Barker et al. To stimulate the motor cortex, the magnetic coil was placed over the head. It was placed over the seventh cervical spinous process (C7) for cervical stimulation, and the first lumbar spinous process (L1) for lumbar stimulation. Cortical stimulation was performed when the subjects were at rest, and also at during weak voluntary contraction in some of them. Recordings were made from the deltoid (Del), biceps brachii (Bi), extensor carpi radialis (ECR), thenar, quadriceps femoris (Quad), tibialis anterior (TA) and flexor hallucis brevis (FHB) muscles with a pair of surface electrodes. The cortical and spinal latent periods (Lcor and Lsp, respectively) were measured. The central conduction time (CCT) was obtained by subtracting Lsp from Lcor for each muscle. In all subjects, responses were readily obtained by cortical, cervical and lumbar stimulations without discomfort in all the muscles examined. The cortical responses with amplitudes of more than 1mV could be recorded even in the lower limb muscles. There were no significant differences in Lsp and CCT between MES and PES, in all the upper limb muscles examined. The Lcors of the lower limb muscles obtained by MES were not different from those obtained by PES. However, the Lsps obtained by MES were significantly shorter than those by PES in the Quad and TA muscles.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

Central motor conduction in cerebrovascular disease and motor neuron disease.

Conduction in the central motor pathways was studied in 9 patients with cerebrovascular disease (CVD), 13 with amyotrophic lateral sclerosis (ALS) and 3 with spinal progressive muscular atrophy (SPMA). Motor responses evoked in the limb by cortical, cervical and lumbar stimulations were recorded. The central conduction time (CCT) was calculated for each muscle. In patients with CVD, responses to cortical stimulation were unobtainable or delayed in the paretic limb muscles. In patients with ALS the abnormality of central motor conduction had significant correlation with the extensor plantar response. The CCTs were normal in patients with SPMA. This technique demonstrated a subclinical lesion in some patients. We conclude that the new technique of examining motor conduction along the corticospinal tract may be useful to detect a subclinical lesion in the corticospinal tract.

Amyotrophic Lateral Sclerosis↗

Central motor and sensory conduction in adrenoleukomyeloneuropathy, cerebrotendinous xanthomatosis, HTLV-1-associated myelopathy and tabes dorsalis.

Central motor and sensory conduction was studied by percutaneous electrical stimulation of brain and spinal cord and by somatosensory evoked potential techniques respectively, in patients with adrenoleukomyeloneuropathy, cerebrotendinous xanthomatosis, human T-cell lymphotropic virus-1-associated myelopathy and tabes dorsalis. The results were all consistent with clinical and neuropathological findings in these disorders. Conductions in the corticospinal tract and posterior column could be evaluated separately with these two techniques. Percutaneous electrical stimulation technique would be useful for investigating conduction in the corticospinal tract in patients with spinal cord disorders.

Adrenoleukodystrophy↗