Saft Carsten, von Hein Sarah Maria, Lücke Thomas, Thiels Charlotte, Peball Marina, Djamshidian Atbin, Heim Beatrice, Seppi Klaus
Department of Neurology, Huntington Centre NRW, Ruhr-University Bochum, St. Josef-Hospital, Bochum, Germany.
Department of Neuropaediatrics, University Children's Hospital, Ruhr University, Bochum, Germany.
J Huntingtons Dis. 2018;7(2):167-173. doi: 10.3233/JHD-170283.
Motor symptoms in Huntington's disease (HD) are heterogeneous with dystonia being described as a symptom with a very high prevalence not only in juvenile cases.
Treatment options for dystonia are limited. Cannabinoids have been described as a potential treatment for patients with dystonia of a different origin. Here, we present early onset HD patients with a marked improvement of motor symptoms mainly due to alleviation of dystonia due to treatment with cannabinoids. In addition we review the current literature concerning the use of cannabinoids in HD.
The Unified Huntington's Disease Rating Scale (UHDRS) motor score, including a chorea and dystonia subscore, was conducted before and after the start of cannabinoids in seven patients without any other changes in medication.
The UHDRS motor score and the dystonia subscore (±SD) improved from 70.9 (25.5) to 60.6 (26.9) with a mean change of 10.3 [95% CI 6.0-14.6] and from 12.3 (4.0) to 8.0 (3.6) with a mean change of 4.3 [95% CI 2.3-6.3], respectively (both p = 0.018).
Improvement of motor symptoms, mainly dystonia, led to several relevant improvements from a global clinical perspective such as improvement of care, gait and fine motor skills and weight gain. Moreover, we observed changes in behavior with less irritability and apathy, as well as less hypersalivation in some cases.
亨廷顿舞蹈症(HD)的运动症状具有异质性,肌张力障碍不仅在青少年病例中被描述为一种患病率很高的症状。
肌张力障碍的治疗选择有限。大麻素已被描述为对不同病因的肌张力障碍患者的一种潜在治疗方法。在此,我们报告了早期发作的HD患者,其运动症状有显著改善,主要是由于大麻素治疗减轻了肌张力障碍。此外,我们回顾了目前有关大麻素在HD中应用的文献。
对7例患者在开始使用大麻素之前和之后进行统一亨廷顿舞蹈症评定量表(UHDRS)运动评分,包括舞蹈症和肌张力障碍子评分,且药物治疗无其他任何变化。
UHDRS运动评分和肌张力障碍子评分(±标准差)分别从70.9(25.5)改善至60.6(26.9),平均变化为10.3 [95%可信区间6.0 - 14.6],以及从12.3(4.0)改善至8.0(3.6),平均变化为4.3 [95%可信区间2.3 - 6.3](两者p = 0.018)。
从整体临床角度来看,运动症状的改善,主要是肌张力障碍的改善,带来了一些相关的改善,如护理、步态和精细运动技能的改善以及体重增加。此外,我们观察到行为上的变化,易怒和冷漠减少,并且在某些情况下流涎过多的情况也有所减轻。