Roy Krishnendu, Talukdar Arunansu, Ray Sayantan, Pal Partha
Department of General Medicine, Medical College, Kolkata, India.
BMJ Case Rep. 2012 Sep 11;2012:bcr2012006669. doi: 10.1136/bcr-2012-006669.
Authors describe the case of a 16-year-old girl who presented with fever, tonic-clonic seizures, unequal arm blood pressures and pulselessness in the left upper limb. On examination, there was a systolic bruit over umbilical region, a pansystolic murmur of mitral regurgitation was found. Neurological examination was normal except for an asymmetry in brain hemicircumference one side compared with the other. She has borderline intelligence (IQ 70) according to Wechsler Adult Performance Intelligence Scale. Magnetic resonance imaging (MRI) of brain revealed atrophic of left cerebral hemisphere with mildly ventricular dilatation, prominent paranasal and mastoid air cells, suggestive of Dyke-Davidoff-Masson syndrome (DDMS). Conventional angiography showed narrowed left internal carotid artery. There was also stenosed brachial artery, absent left renal artery with narrowed infrarenal abdominal aorta. The patient was put on antihypertensive drugs. We hypothesise that Takayasu arteritis and related vascular occlusion is the cause of her acquired cerebral changes.
作者描述了一名16岁女孩的病例，该女孩出现发热、强直阵挛性癫痫发作、双臂血压不等以及左上肢无脉。检查发现，脐周区域有收缩期杂音，存在二尖瓣反流的全收缩期杂音。除了一侧脑半球周长与另一侧不对称外，神经系统检查正常。根据韦氏成人智力量表，她的智力处于临界水平（智商70）。脑部磁共振成像（MRI）显示左侧大脑半球萎缩，伴有轻度脑室扩张、鼻窦和乳突气房明显，提示戴克 - 戴维多夫 - 马森综合征（DDMS）。传统血管造影显示左侧颈内动脉狭窄。肱动脉也有狭窄，左肾动脉缺如，肾下腹主动脉狭窄。该患者接受了降压药物治疗。我们推测，高安动脉炎及相关血管闭塞是其获得性脑部病变的原因。
