Shen Guangxun, Nan Guangxian, Shin Chae-Won, Park Hyeyoung, Lee Kwee-Yum, Jeon Beomseok
Department of Neurology, China-Japan Union Hospital of Jilin University, Changchun, China.
Department of Neurology, SNU-SMG Boramae Medical Center, Seoul, Republic of Korea.
BMC Neurol. 2016 Nov 17;16(1):228. doi: 10.1186/s12883-016-0745-6.
Myoclonus is a clinical sign characterized by sudden, brief jerky, shock-like involuntary movements of a muscle or group of muscles. Dystonia is defined as a syndrome of sustained muscle contractions, frequently causing twisting and repetitive movements or abnormal postures. Cases of myoclonus or dystonia secondary to a structural lesion in the cerebellum have been reported. However, there has never been a reported case of combined myoclonus and dystonia secondary to a cerebellar lesion.
Herein, we report a 22-year-old female patient with sudden-onset myoclonic jerks, dystonic posture and mild ataxia in the right upper extremity. At age 19, she experienced sudden headache with vomiting. The neurological examination showed ataxia, myoclonus and dystonia in the right upper extremity. Brain images demonstrated a hemorrhage in the right cerebellar hemisphere secondary to a cavernous malformation. After resection of the hemorrhagic mass, headache with vomiting disappeared and ataxia improved, but myoclonus and dystonia persisted.
It is the first report of combined focal myoclonus and dystonia secondary to a cerebellar lesion.
肌阵挛是一种临床体征,其特征为一块肌肉或一组肌肉突然、短暂、急促、类似电击样的不自主运动。肌张力障碍被定义为一种持续肌肉收缩的综合征,常导致扭曲和重复性运动或异常姿势。继发于小脑结构性病变的肌阵挛或肌张力障碍病例已有报道。然而,从未有过继发于小脑病变的肌阵挛和肌张力障碍合并出现的报道。
在此,我们报告一名22岁女性患者,其右上肢突发肌阵挛性抽搐、肌张力障碍姿势及轻度共济失调。19岁时,她突发头痛伴呕吐。神经系统检查显示右上肢共济失调、肌阵挛和肌张力障碍。脑部影像显示继发于海绵状畸形的右小脑半球出血。切除出血性肿块后,头痛伴呕吐消失,共济失调改善,但肌阵挛和肌张力障碍持续存在。
这是首例继发于小脑病变的局灶性肌阵挛和肌张力障碍合并出现的报道。