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Biomedical subjects

T Tsubaki

Publications and source records attributed to T Tsubaki.

At least 73 records · Page 4Linked to original sources

Chronic recurrent demyelinating encephalomyelitis associated with hemophagocytic lymphohistiocytosis.

A man aged 31 with remittent fever, leukopenia, polyuria, and splenomegaly in addition to chronic and recurrent episodes of neurologic abnormalities was examined pathologically. A chronic type of perivenous encephalomyelitis was found in the CNS, and chronic inflammatory lesions were noticed in the infundibulo-hypophyseal system and also in the peripheral nerves. Moreover, hemophagocytic lymphohistiocytosis predominated in the reticuloendothelial system. This seemed to be the first adult case of demyelinating disease with hemophagocytic lymphohistiocytosis.

Adult↗

Controlled trial of thyrotropin releasing hormone tartrate in ataxia of spinocerebellar degenerations.

The clinical efficacy, dose-response relationship, and safety of TRH-T (thyrotropin releasing hormone tartrate) were assessed in 290 patients with spinocerebellar degeneration (SCD) in a 2-week, double-blind study using placebo as control. 254 patients satisfied the criteria for inclusion in evaluation of the drug efficacy. The patients were treated with TRH-T in an intramuscular dose of 2 mg, 0.5 mg or 0 mg (placebo) as TRH once a day for 2 weeks. Clinical responses to these treatments were evaluated 3 times: at the end of weeks 1 and 2 of treatment and a week after the end of treatment. The results of "global improvement rating" as well as those of "ataxia improvement rating" showed that both 2 mg and 0.5 mg TRH-T treatments were significantly superior to placebo treatment in patients with predominantly cerebellar form of SCD. The effect was well maintained a week after the end of the 2-week treatment in the patients who were given TRH-T in daily dose of 2 mg and showed improvement at the end of treatment. The results of "improvement rating of each symptom" revealed that 2 mg treatment was significantly more effective than placebo for disorders of standing, gait, speech and writing. In the patients who had no pyramidal involvement or disorder of deep sensation, the drug efficacy and dose-response relationship were evident. Adverse reactions to the drug such as headache, feeling febrile and nausea were observed in 50% of the patients on 2 mg treatment, in 38% of those on 0.5 mg treatment and in 21% of those on placebo patient, however, discontinued treatment because of adverse reactions.

Adult↗

Heat-evoked responses of dorsal horn nociceptive neurons in the monkey.

Locations and response characteristics of nociceptive neurons in the dorsal horn of the spinal cord were studied in urethane-chloralose anesthetized Japanese macaques. There were two general categories of nociceptive neurons. Neurons of the first type, nociceptive specific (NS) neurons, responded only to intense mechanical stimuli. Neurons of the second type, wide dynamic range (WDR) neurons, were activated by touch and pressure but responded maximally to noxious mechanical stimuli. NS neurons were located in laminae I and IIo of the superficial dorsal horn. WDR neurons were located not only in laminae I and IIo but also in laminae IV-VI. NS and WDR neurons in laminae I and IIo were spatially not segregated from each other. Projecting neurons were included in both categories of nociceptive neurons, but lamina IIo neurons were exclusively non-projecting neurons. Noxious heat stimulation of the peripheral receptive field as defined by mechanical stimuli, activated a significant fraction of NS and WDR neurons. Nearly all heat-sensitive WDR neurons in both the superficial layers and nucleus proprius received combined A delta- and C-fiber input. Heat-sensitive NS neurons received either exclusively A delta-fiber input or combined A delta- and C-fiber input. All the heat-sensitive neurons tested showed a monotonous increase of heat-evoked discharges between the threshold temperature and the peak temperature. In both categories of heat-sensitive neurons, noxious heat neurons predominated over warming-noxious neurons. Hence, the thermal threshold did not necessarily parallel the mechanical threshold.

Animals↗

The peripheral neuropathy in De Sanctis-Cacchione syndrome. Histological, ultrastructural, and morphometric studies.

Histological, ultrastructural, and morphometric studies were performed on nerve and muscle biopsies from three patients with de Sanctis-Cacchione syndrome. Sural nerves showed marked loss of the myelinated fibers, in proportion to decrease in nerve conduction velocities and in inverse proportion to the severity of the clinical symptoms, which were related to the survival length. The larger fibers were involved earlier and more markedly than the smaller. The unmyelinated fibers were also decreased in number. Electron-microscopic studies showed the presence of primary degeneration of myelin sheaths or Schwann cells. Muscle biopsies showed grouping of type I and type II fibers in all three patients. Therefore, peripheral nerve involvement in de Sanctis-Cacchione syndrome was suggested to result from chronic degeneration of the neuronal cells and Schwann cells.

Adolescent↗

Oculopharyngeal muscular dystrophy and distal myopathy. Intrafamilial difference in the onset and distribution of muscular involvement.

A family is reported which included a patient with a variant form of oculopharyngeal muscular dystrophy. The patient's son suffered from infantile muscular dystrophy with a distal distribution in the lower extremities and no oculopharyngeal symptoms. Case 1, the father, showed blepharoptosis, but no limitation of ocular movements. Case 2, the son, showed early onset of weakness and more rapid progression of muscle involvement than the father. In both patients EMG, muscle biopsies and elevated serum CPK indicated the myopathic nature of the disorder. A muscle biopsy specimen in Case 2 showed abundant rimmed vacuoles and abnormal filaments 13-19 nm in diameter in the sarcoplasm, usually reported to occur in inclusion body myositis. The findings indicate that oculopharyngeal muscular dystrophy and distal myopathy are related in their etiology and distal myopathy and inclusion body myositis are regarded as variant forms of the same disease.

Adult↗

Case-control studies of motor neuron disease: association with mechanical injuries.

Two case-control studies of motor neuron disease that involved 712 cases and 158 cases, respectively, showed that (1) mechanical injuries were two to three times more frequent in both sexes, heralding amyotrophic lateral sclerosis, progressive bulbar paralysis, and progressive muscular atrophy; (2) the head, neck, spine, and the extremities were more often traumatized; (3) traumatized parts were not correlated with the initial manifestation of the disease; and (4) more males were traumatized, but males still predominated among uninjured cases. These results suggested that mechanical injuries were not the cause, but probably one of the risk factors of the disease. No association was observed with smoking, drinking, residence, home space, drinking water, animals, experience as a war prisoner, stay on Guam, parental consanguinity, measles, polio, mumps, tuberculosis, rheumatism, prothesis of the total teeth, shell splinters retained in the body, occupational exposures to radiations, chemicals, or gases, atomic bombings, electric injuries, surgical operations, and occupations.

Amyotrophic Lateral Sclerosis↗

Reduced glycine receptor in the spinal cord in amyotrophic lateral sclerosis.

Transmitter receptor binding was estimated in the spinal cord of 6 subjects with amyotrophic lateral sclerosis (ALS) and 4 control subjects in assays using 3H-quinuclidinyl benzilate for muscarinic cholinergic receptors, 3H-strychnine for glycinergic receptors, 3H-spiroperidol for dopaminergic receptors, 3H-muscimol for GABAergic receptors, and 3H-dihydroalprenolol for beta-adrenergic receptors. In ALS, glycinergic receptor binding was greatly reduced in the anterior gray matter. This finding may be attributed to loss of large neurons in the anterior gray matter, known to be characteristic of ALS.

Adult↗

A new myopathy with tubulomembranous inclusions.

A muscle biopsy performed on a 16-year-old boy with progressive myopathy revealed hitherto unrecognized peculiar inclusions which consisted of 3 types of structures. The first type consisted of laminated tubulomembranous structures and most of the inclusions belonged to this type. The lamellae were regularly spaced with a periodicity of 8.5--9 nm and curving a little, and were observed as concentric lamellae according to the plane of sectioning. The second type of inclusions consisted of curvifilamentous material. The third type had the appearance of moderately electron-dense granular material surrounded by a single unit membrane. The origin and nature of these inclusions is obscure, but the diagnosis of some kind of storage myopathy was suspected in this case.

Adolescent↗