Just A, Canaple S, Joly H, Piussan C, Rosa A
Service de Neurologie, CHU Nord, Amiens.
Rev Neurol (Paris). 1996 Oct;152(10):634-6.
A 39 year old caucasian man was admitted in 1994 to the neurological department with a left pure motor hemiplegia that appeared suddenly. This patient showed typical features of Werner's syndrome. He had a hoarse voice, a diffuse muscle weakness and atrophy in the upper and lower limbs with chronic ulcers on the legs. His scalp and public hair were sparse. Cranial MRI revealed several lesions in the white matter, low signal intensity on T1 weighted images and high signal on T2 weighted images. Cerebrospinal fluid (CSF was inflammatory with hypercytosis and proteinorachia was 0.50 g/l with synthesis of IgG. Sural nerve biopsy revealed muscle atrophy and the loss of myelinated fibers. Thus, central and peripheral nervous systems were affected in this case.
一名39岁的白种男性于1994年因突发左侧纯运动性偏瘫入住神经科。该患者表现出典型的沃纳综合征特征。他声音嘶哑，上下肢弥漫性肌无力和萎缩，腿部有慢性溃疡。他的头皮和阴毛稀少。头颅磁共振成像显示白质有多处病变，T1加权像上呈低信号强度，T2加权像上呈高信号。脑脊液有炎症，细胞增多，蛋白-脑脊液比值为0.50 g/l，有IgG合成。腓肠神经活检显示肌肉萎缩和有髓纤维丧失。因此，该病例中中枢和周围神经系统均受到影响。
