Anderson Neil E, Haas Lindsay F
Neurology Dept, Auckland Hospital, Private Bag, 92024 Auckland, New Zealand.
J Neurol. 2003 Oct;250(10):1174-8. doi: 10.1007/s00415-003-0168-3.
Patients with Werner's syndrome have the appearance of premature ageing. Neurological complications are usually regarded as uncommon. The neurological manifestations in three patients with cardinal features of Werner's syndrome, including short stature, premature greying and baldness, thin arms and legs, cataracts and scleroderma-like skin changes, are presented. The neurological features included transient ischaemic attacks secondary to atherosclerosis in the common carotid arteries (one patient), sensory peripheral neuropathy (one patient) and peripheral neuropathy with a possible myelopathy (one patient). In one of these patients the diagnosis of Werner's syndrome was not recognised prior to neurological referral. Although neurological disease in patients with Werner's syndrome is uncommon, it may be under-recognised. Some of the neurological complications are secondary to premature cerebrovascular disease, but the pathogenesis of peripheral neuropathy and myelopathy in patients with Werner's syndrome is uncertain.
患有沃纳综合征的患者有早衰的外貌。神经并发症通常被认为不常见。本文介绍了三名具有沃纳综合征主要特征的患者的神经学表现,这些特征包括身材矮小、过早白发和秃头、四肢纤细、白内障以及硬皮病样皮肤改变。神经学特征包括颈总动脉动脉粥样硬化继发的短暂性脑缺血发作(一名患者)、感觉性周围神经病(一名患者)以及可能伴有脊髓病的周围神经病(一名患者)。在其中一名患者中,在神经科转诊之前未确诊沃纳综合征。虽然沃纳综合征患者的神经疾病不常见,但可能未得到充分认识。一些神经并发症继发于过早的脑血管疾病,但沃纳综合征患者周围神经病和脊髓病的发病机制尚不确定。