Atsumi Masahiko, Tanaka Atsushi, Kawarabayashi Takahiko, Nishio Shunji, Sakamoto Hikaru, Hasegawa Takanori, Kitaguchi Masataka
Department of Neurology, Baba Memorial Hospital, 4-244 Higashi, Hamaderafunao-cho, Sakai, Osaka 592-8555, Japan.
No To Shinkei. 2004 Feb;56(2):163-7.
A 65-year-old man with previous history of congestive heart failure and genetically proven Becker muscular dystrophy (BMD) was suddenly suffered from aphasia and right hemiplegia. Physical examination showed severe motor aphasia, right hemiplegia, and signs of left heart failure. An echocardiogram before the onset of aphasia showed markedly dilated left ventricle and decreased ventricular contraction. Intracardiac thrombus was not detected. Although his electrocardiogram on admission showed sinus rhythm, atrial fibrillation was noted at the time of neurological deterioration. MRI of the brain revealed acute infarction in the territory of the left middle cerebral artery and the left anterior inferior cerebellar artery. MR angiography showed vascular occlusion at the left M2 segment. Cerebral embolism due to atrial fibrillation associated with BMD-related DCM was diagnosed. While an administration of anti-coagulant, diuretics, and dopamine relieved his respiratory distress and right hemiplegia, severe motor aphasia persisted. Cerebral embolism may be a notable complication in patients with BMD presenting with late-life expression of skeletal muscular weakness and antecedent cardiac involvement.
一名65岁男性,既往有充血性心力衰竭病史且基因检测证实患有贝克型肌营养不良(BMD),突然出现失语和右侧偏瘫。体格检查显示严重运动性失语、右侧偏瘫以及左心衰竭体征。失语发作前的超声心动图显示左心室明显扩张且心室收缩减弱。未检测到心内血栓。尽管他入院时的心电图显示窦性心律,但在神经功能恶化时发现了房颤。脑部MRI显示左侧大脑中动脉和左侧小脑前下动脉供血区域急性梗死。磁共振血管造影显示左侧M2段血管闭塞。诊断为与BMD相关的扩张型心肌病(DCM)伴发房颤导致的脑栓塞。在给予抗凝剂、利尿剂和多巴胺后,他的呼吸窘迫和右侧偏瘫得到缓解,但严重运动性失语仍持续存在。脑栓塞可能是BMD患者出现骨骼肌无力的晚期表现和先前心脏受累时的一个显著并发症。