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.

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PubMed1983

The peripheral nerve involvement in spinocerebellar degenerations.

We studies peripheral nerves electrophysiologically in 15 cases of spinocerebellar degenerations (SCD). Three patients showed abnormalities in 40% or more of the 13 items examined in this study. Two patients showed abnormalities in 20--40% of the items. Ten patients showed abnormalities in 20% or less of the items; six of them showed no abnormalities in any of the items. The incidence of abnormalities was slightly higher in the sensory nerve than in the motor nerve and was slightly higher in the peripheral areas than in the proximal areas in both the motor and sensory nerves. A high incidence of abnormalities was observed in cases of olivo-ponto-cerebellar atrophy (OPCA), the longer the history of the disease, the higher the incidence of abnormalities. This finding supports the idea that OPCA, not being only a single system, will develop into multiple system atrophy in a broad sense through rigidity and peripheral nerve disorders during a long course of the disease. Two patients with talipes cavus with muscular atrophy had peripheral nerve disorders.

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PubMed1983

[Myotonic dystrophy associated with hyperthyroidism].

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Adult↗