Conceição Isabel, De Carvalho Mamede
Department of Neurology and Neuromuscular Unit, Institute of Molecular Medicine, Faculty of Medicine, Hospital de Santa Maria, Av Prof Egas Moniz, 1649-028, Lisbon, Portugal.
Muscle Nerve. 2007 Jan;35(1):116-8. doi: 10.1002/mus.20644.
We compared early- with late-onset cases in 86 patients with familial amyloidotic polyneuropathy type I (FAP). Among these patients, 43 presented before age 50 (early-onset) and 43 after this age (late-onset). Sex and geographical distribution were similar, although a family history of the disorder was more frequent in early-onset cases. In late-onset disease, autonomic dysfunction was less frequent, but organ involvement and severe neuropathic pain were more frequent. Neurophysiological measurements were similar. FAP is a cause of neuropathy in elderly patients, in particular in those presenting with neuropathic pain.
我们比较了86例Ⅰ型家族性淀粉样多神经病（FAP）患者的早发和晚发病例。在这些患者中，43例在50岁之前发病（早发），43例在50岁之后发病（晚发）。性别和地理分布相似，尽管该疾病的家族史在早发病例中更为常见。在晚发病例中，自主神经功能障碍较少见，但器官受累和严重神经病理性疼痛更为常见。神经生理学测量结果相似。FAP是老年患者神经病变的一个病因，尤其是那些伴有神经病理性疼痛的患者。
