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Biomedical subjects

T Tabira

Publications and source records attributed to T Tabira.

At least 181 records · Page 10Linked to original sources

Marchiafava-Bignami disease, striatal degeneration, and other neurological complications of chronic alcoholism in a Japanese.

A Japanese man with a variety of neurological complications, had drunk Japanese rice wine (sake) daily for about 25 years. There was a progressive development of parkinsonism, cerebellar ataxia, and mental deterioration by the time he was 32. He died of pneumonia at age 50 and the autopsy revealed Marchiafava-Bignami disease (MBD), striatal degeneration, pseudolaminar sclerosis of Morel, atrophy of the corpus mamillare and pons, cortical cerebellar atrophy, pseudopellagra, and polyneuropathy. This is the first case of MBD in a Japanese related to the ingestion of Japanese "sake", and it is also a rare case in that almost all of the neurological complications seen with chronic alcoholism were apparent. Striatal degeneration seems to be a rare complication of chronic alcoholism.

Alcoholism↗

Cortical deafness in multiple sclerosis.

Cortical deafness in a patient with multiple sclerosis is reported. Complete recovery from total deafness was seen following stages of auditory agnosia and pure word deafness. The otological and neurophysiological studies suggested lesions in subcortical white matter. This report stresses the rarity of the condition, its subcortical origin and good prognosis.

Adult↗

Experimental allergic encephalomyelitis: suppression with SD-170, a new synthetic drug.

SD-170 is a new drug synthesized by Umezawa and associates. The clinical, pathological, and immunologic effects of this drug on acute EAE were investigated. In guinea pigs immunized with 5 micrograms MBP in CFA, SD-170 diminished the clinical severity as well as delaying the onset of death in acute EAE. All clinical observations were supported by histologic findings. On the contrary in animals immunized with spinal cord homogenate SD-170 did not suppress acute EAE. In SD-170 treated animals there seemed to be a lowered cellular response to MBP by the lymphocyte proliferative response test; there also seemed to be a lowered humoral antibody response to MBP. We conclude that SD-170 is an immunosuppressive of acute EAE.

Animals↗

[A case of neuronal ceroid-lipofuscinosis (Jansky-Bielshowsky type): morphological, biochemical and electrophysiological studies (author's transl)].

We reported a case of late infantile neuronal ceroid-lipofuscinosis. The patient was an 8-year-old boy presenting with marked psychomotor deterioration, progressive visual failure due to retinal degeneration and optic atrophy, startle reaction to auditory stimuli, frequent myoclonus and generalized convulsions. The routine laboratory examinations were all normal. EEG was markedly abnormal because of poorly organized background activity and frequent paroxysmal spike-and-wave complexes. CT scan showed evidence of severe atrophy of the cerebrum, cerebellum and brainstem. Electron microscopic examination of the biopsied rectum revealed fingerprint profiles in the neurons and pericytes beneath the muscularis mucosa. Cultured skin fibroblasts also contained electron dense inclusions, some of which showed fingerprint profiles. Urinary glycopeptides were normal. Lyscsomal enzyme activities in leukocytes and cultured fibroblasts were normal. Neurophysiological studies revealed giant cortical potentials evoked by the auditory as well as somatosensory stimulation. Simultaneous recording of the somatosensory evoked EEG and EMG potentials disclosed that the myoclonus in this patient was stimulus-sensitive and compatible with the cortical reflex myoclonus. With regard to hypothetical pathogenesis of this disease, we studied lipoperoxide in the blood before and after anti-oxidant therapy. We also measured vitamin A and carotene, since these substrates are related to retinoic acid. Although vitamin A and carotene were normal, lipoperoxide was slightly elevated. However, it was not influenced by the treatment with anti-oxidant. Significance of elevated lipoperoxide to the pathogenesis of this diseases has not solved.

Ceroid↗

A clinical comparative study of multiple sclerosis and neuro-Behçet's syndrome.

Clinical comparisons were made between Japanese patients with multiple sclerosis (66 cases) and neuro-Behçet's syndrome (23 cases). Those with neuro-Behçet showed marked male predominance, while those with multiple sclerosis showed slight female preponderance. Both showed encephalomyelopathy disseminated in time and space. Patients with multiple sclerosis, however, showed a more polyphasic course, whilst those with neuro-Behçet showed a more progressive one. In multiple sclerosis optic neuritis, acute transverse myelitis, painful tonic seizures, mental disturbance and internuclear ophthalmoplegia were common. On the other hand, in neuro-Behçet the main neurological manifestation was progressive pseudobulbar palsy. Serum and CSF showed more inflammatory changes in neuro-Behçet than in multiple sclerosis. Clinical estimation suggested that in multiple sclerosis the main lesions were in the optic nerve, tegmentum of the brain stem and spinal cord, whereas in neuro-Behçet they were in the basal parts of the brain stem.

Adolescent↗

E-PTA stains oligodendroglial surface membranes and microtubules in optic nerves during myelination.

Aldehyde fixed Xenopus tadpole and frog optic nerves were stained en bloc with ethanolic phosphotungstic acid (E-PTA). During rapid myelination, intense staining was observed on cytoplasmic faces of paranodal terminal loops and loosely wrapped oligodendroglial membranes found along inner and outer surfaces of compact myelin sheaths. Oligodendroglial microtubules also were heavily stained. Where stained cytoplasmic faces fused to form a lamella of compact myelin, the intense staining was reduced to a thinner, fainter line. In optic nerves of adult frogs, the staining was less dense but the pattern was similar. The staining distribution and available histochemical evidence indicate that E-PTA stains positively charged proteins non specifically. Since myelin basic protein is found in oligodendroglia during myelination, we suggest that it is being stained by E-PTA while being transported along microtubules to sites where it is inserted into developing myelin lamellae.

Animals↗

An experimental analysis of interlamellar tight junctions in amphibian and mammalian C.N.S. myelin.

The distribution of interlamellar tight junctions was examined in myelin sheaths of Xenopus tadpole optic nerve and rabbit epiretinal tissue fixed with aldehydes, postfixed with osmium ferrocyanide and embedded in a water-soluble medium, Durcupan. Intramyelinic zonulae occludentes were clearly formed by fusion of adjacent intraperiod lines which corresponded to the external leaflets of oligodendrocytes. These occurred in register with other tight junctions present within successive lamellae and appeared as a series of radial lines extending either partially or totally across the thickness of the myelin sheath. This distribution of zonulae occludentes corresponded with that of tight junctional particle strands observed in freeze-fracture replicas. Analysis of intramyelinic vacuolation induced by hexachlorophene (HCP) intoxication indicated that lamellar splitting was frequently limited by the tight junctions. The intramyelinic zonulae occludentes also restricted the diffusion of colloidal lanthanum which had penetrated the myelin intraperiod gap following in vivo perineural injection. The results of this study provide evidence favouring a correspondence between interlamellar tight junctions and the 'radial component' of myelin described earlier by other investigators. Furthermore, observations of swollen myelin sheaths, resulting from HCP intoxication, suggest that these junctions may play a major role in maintaining myelin sheath integrity and limiting the extent of breakdown during certain pathological conditions.

Animals↗

In vivo test for myelinotoxicity of cerebrospinal fluid.

A quantitative double blind procedure is described to test the myelinotoxicity of cerebrospinal fluid (SSF) by using optic nerves of Xenopus tadpoles as an in vivo model of a myelinated CNS tract. Only 0.5 ml of unconcentrated CSF is needed for a test and the result is known in 5 days. Groups of 8-10 Xenopus tadpoles received a subcutaneous injection of 12-13 muL of a coded CSF sample or of a saline control solution around the right optic nerve. After 48 h, whole mounts of the right optic nerves were prepared and the slides were randomized before using a differential-interference contrast microscope to count the myelin lesions. The myelinotoxicity of a CSF sample was considered positive (+) when it produced significantly higher (P less than 0.01) counts than the saline control. When P was less than 0.05, the counts were recorded as borderline (+/-) and it was negative (-) when P was greater than 0.05.

Animals↗

Multiple sclerosis cerebrospinal fluid produces myelin lesions in tadpole optic nerves.

To investigate the myelinotoxicity of cerebropsinal fluid in multiple sclerosis, we used an in vivo model of the myelinated central-nervous-system tract of tadpoles for quantitative double-blind tests of 46 cerebrospinal-fluid samples. Groups of xenopus tadpoles were injected with cerebrospinal fluid near the optic nerve. Forty-eight hours later, whole mounts of optic nerves were prepared, and a differential interference microscope was used to count myelin lesions. Cerebrospinal-fluid samples from 60 per cent of the patients with an acute attack of definite multiple sclerosis had myelinotoxic activity. This activity correlated best with the severity and duration of the disease, rather than with gamma-globulin or total protein concentrations. Activity was negative in 85 per cent of cerebrospinal-fluid samples from a control group with other neurologic diseases. This assay is a useful method for investigating myelinotoxic factors of cerebrospinal fluid in patients with multiple sclerosis, but was not helpful diagnostically.

Acute Disease↗

The penetration of fluorescein-conjugated and electrondense tracer proteins into Xenopus tadpole optic nerves following perineural injection.

The permeability of Xenopus tadpole optic nerves to macromolecules was studied in order to evaluate the usefulness of this system for studying mechanisms of serum-induced CNS demyelination in vivo. Single injections of either horseradish peroxidase (HRP), ferritin or fluorescein-conjugated human IgG were injected around the right optic nerve and tadpoles were then sacrificed between 15 min and 48 h. Each of the tracers had penetrated the nerve parenchyma by 30 min. Entry of HRP and ferritin occurred mainly via extracellular clefts between adjacent astrocytic endfeet in the glia limitans region. A similar mode of passage was suggested for IgG. Once within the nerve, the tracers became rapidly associated with myelinated axons. HRP was also seen in the periaxonal space but did not directly penetrate the myelin sheath. By 24 h, extracellular localization of tracer was virtually absent with nearly all of the tracer now being concentrated in vesicles within astrocytic processes and perikarya. The distribution of the tracers was not confined to the optic nerve on the injected side; some was seen in adjacent cranial peripheral nerves and surrounding extraocular musculature. Also, tracers eventually penetrated the pial sheath of the contralateral optic nerve. The results of this study indicate that tadpole optic nerves are permeable to a wide range of macromolecules. Furthermore, the distribution of these tracers to nearby cranial peripheral nerves may provide an important opportunity for testing the differential effect of various substances on central and peripheral myelin sheaths.

Animals↗

3H-thymidine autoradiography of CSF cells in primary reticulum cell sarcoma of the brain.

CSF cells in a case of primary reticulum cell sarcoma of the brain with diffuse subarachnoid spreading were examined by 3H-thymidine autoradiography. Immediately after lumbar puncture, the CSF withdrawn was incubated at 37 degrees C for 1 hr with an admixture of 3H-thymidine at a rate of 1 muCi/ml CSF. The cells were collected by centrifugation and the microautoradiographic procedure was performed. The labeling index (L.I.) of the total CSF cells was 10.5%, and when non-neoplastic cells, i.e. polymorphonuclear leukocytes, small lymphocytes, monocytes etc., were excluded, the real L.I. of the tumor cells in the CSF was supposed to be more than 14.4%. Referring to the results of various brain tumors reported in the literature, this belongs to the highest labeling group. The high L.I. of the tumor cells in this case was well consistent with the extremely rapid clinical course. It should be stressed that the examination of CSF cells by 3H-thymidine autoradiography in cases of brain tumors could be one of the valuable methods indicating the DNA synthesis of the tumor cells, which is an important parameter of malignancy.

Adult↗

Giant axonal neuropathy. A clinical entity affecting the central as well as the peripheral nervous system.

An 8-year-old girl had progressive muscle weakness and a unique posture of the lower limbs, areflexia, distal sensory impairment, and remarkably kinky hair. Histologic examination of the sural nerve showed giant axons filled with neurofilamentous masses. The clinical and histologic findings resembled those of recent cases reported as "giant axonal neuropathy." Our patient's precocious puberty, Babinski's sign, and electroencephalographic abnormalities suggested central nervous system involvement. Two cases previously reported and the present one appear to represent a new clinical entity that affects the central and the peripheral nervous system.

Axons↗