Neurological complications of Werner’s syndrome

Abstract.

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.