Machado Célia, Amorim José M, Rodrigues Margarida, Cerqueira João, Lourenço Esmeralda, Pinho João
*Neurology Department †Neurorradiology Department, Hospital de Braga, Braga, Portugal.
Neurologist. 2015 May;19(5):132-4. doi: 10.1097/NRL.0000000000000025.
Paroxysmal dystonia is a rare manifestation of multiple sclerosis (MS).
A 41-year-old man presented to our Emergency Department with sudden and repeated episodes of left upper limb flexion and lower limb extension. His medical history included an episode of left facial palsy a year earlier. Neurological examination demonstrated only brisk deep tendon reflexes on the left upper limb. Routine blood and urine analyses were normal. Computed tomography of the brain and cervical Doppler were normal. Aspirin and sodium valproate were started, without improvement. Video-EEG monitoring revealed no electrographic abnormality synchronous with these paroxysmal events, excluding epileptic nature. Cerebral magnetic resonance imaging showed multiple T2 white matter lesions at the midbrain, right diencephalon, corpus callosum, cervical, and thoracic spinal cord. The right diencephalic lesion enhanced with gadolinium. Complete basic and immunologic analysis and serological studies were normal or negative. Oligoclonal bands were positive in cerebrospinal fluid (negative in serum). Methylprednisolone (1 g/d for 5 d) was started without clinical improvement. Carbamazepine (400 mg/d) was promptly effective, and discontinued after 1 month without recurrence.
The patient met the criteria for the diagnosis of MS according to the 2010 McDonald criteria. The timely and accurate diagnosis of MS requires the recognition of its varied and atypical clinical manifestations.
阵发性肌张力障碍是多发性硬化症(MS)的一种罕见表现。
一名41岁男性因左上肢屈曲和下肢伸展的突发反复发作出现在我们的急诊科。他的病史包括一年前的一次左侧面神经麻痹发作。神经系统检查仅显示左上肢的深腱反射亢进。血常规和尿常规分析正常。脑部计算机断层扫描和颈部多普勒检查正常。开始使用阿司匹林和丙戊酸钠,但病情无改善。视频脑电图监测未发现与这些阵发性事件同步的脑电图异常,排除了癫痫性质。脑磁共振成像显示中脑、右间脑、胼胝体、颈部和胸段脊髓有多个T2白质病变。右间脑病变钆增强。完整的基础和免疫学分析以及血清学研究正常或为阴性。脑脊液中寡克隆带阳性(血清中阴性)。开始使用甲泼尼龙(1 g/d,共5天),但临床症状无改善。卡马西平(400 mg/d)迅速起效,1个月后停药且无复发。
根据2010年麦克唐纳标准,该患者符合MS的诊断标准。MS的及时准确诊断需要认识到其多样和非典型的临床表现。