Waragai M, Ogawara K, Takaya Y, Hayashi M
Department of Neurology, Kofu City Hospital.
Rinsho Shinkeigaku. 1997 Jul;37(7):587-94.
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.
促甲状腺激素释放激素（TRH）疗法常用于治疗脊髓小脑变性（SCD），其疗效已得到证实。然而，其有效性被认为因疾病类型、严重程度以及评估临床改善的方法而异。我们对23例SCD患者进行了酒石酸促甲状腺激素（TRH-T）疗效的研究，这些患者包括小脑型（皮质小脑萎缩（CCA）和遗传性皮质小脑萎缩（H-CCA））以及多系统型（多系统萎缩（MSA）和遗传性橄榄脑桥小脑萎缩（H-OPCA））。每天静脉注射2毫克TRH-T，持续20天。通过评估患者在躺卧、坐立、站立和行走时平衡功能的变化来评估TRH疗法的效果，这些变化可能反映SCD患者日常生活活动（ADL）中的运动功能。还使用声学分析对言语功能进行定性评估。在治疗前后测量脑脊液中的胺代谢产物（高香草酸（HVA）和5-羟吲哚乙酸（5-HIAA）），其可能反映中枢神经系统中的去甲肾上腺素和血清素代谢。虽然23例患者中有16例在TRH治疗过程中平衡功能有轻度或中度改善，但小脑型（CCA和H-CCA）患者与MSA患者相比改善更为显著。16例患者中有9例在TRH治疗后效果持续了很长时间（平均3.8个月），这9例患者中有8例（88.9%）为小脑型SCD。与MSA和H-OPCA患者相比，CCA患者脑脊液中HVA和5-HIAA的水平也有所升高。在16例通过TRH治疗平衡功能得到改善的患者中，有14例（87.5%）治疗前疾病严重程度为轻度或中度；有可能无需支撑行走，或偶尔需要支撑。综合考虑这些结果，TRH疗法可能对疾病严重程度为轻度的小脑型SCD患者有效，并且可能推荐用于支持小脑型SCD患者的ADL。
