[Efficacy of TRH-T for spinocerebellar degeneration--the relation between clinical features and effect of TRH therapy].

Thyrotropin releasing hormone (TRH) therapy has been frequently attempted for the treatment of spinocerebellar degeneration (SCD) and its efficacy has been confirmed. However, effectiveness is considered to differ depending on disease type, severity and the method of evaluating clinical improvement. We investigated the efficacy of thyrotropin releasing hormone-tartrate (TRH-T) in 23 patients with SCD consisting of cerebellar form (cortical cerebellar atrophy (CCA) and hereditary cortical cerebellar atrophy (H-CCA)), and multiple system form (multiple system atrophy (MSA) and hereditary olivopontocerebellar atrophy (H-OPCA)). TRH-T, 2 mg per day, was given intravenously for 20 days. The effect of TRH therapy was evaluated by assessing changes in balance function while lying, sitting, standing and walking, that may reflect the movement functions in active daily life (ADL) for the patients with SCD. The speech function was also evaluated qualitatively using acoustic analysis. The amine metabolites (HVA and 5-HIAA) in cerebrospinal fluid possibly reflecting the noradrenaline and serotonin metabolism in the central nervous system were measured before and after treatment. Although mild or moderate improvement of the balance function during the course of TRH therapy was seen in 16 of the 23 patients, patients with cerebellar forms (CCA and H-CCA) improved significantly as compared to patients with MSA. The effect persisted for a long time (mean; 3.8 months) after TRH therapy in nine of the 16 patients, and eight (88.9%) of the nine had the cerebellar form of SCD. The levels of HVA and 5-HIAA in CSF also increased in patients with CCA as compared to patients with MSA and H-OPCA. The disease severity before the treatment in 14 (87.5%) of 16 patients who showed improvement of balance functions by TRH therapy was mild or moderate; possible of walking without support, or occasionally with support. Considering these results together, TRH therapy may be effective in patients with the cerebellar form of SCD, whose illness severity is mild, and may be recommended for support of ADL in patients with the cerebellar form of SCD.