Ponsford S, Pye I F, Elliot E J
Leicester Royal Infirmary, UK.
J R Soc Med. 1993 Oct;86(10):597-9.
Lafora body disease is a rare neurometabolic disorder of autosomal recessive inheritance. Symptoms begin in the second decade with progressive myoclonic epilepsy and survival is unusual beyond the age of 30. We report an electroencephalographic study in four cases of histologically proven Lafora body disease. Posterior epileptiform discharges were found even in the early stages of the disease and may assist in early diagnosis.
拉福拉体病是一种罕见的常染色体隐性遗传神经代谢障碍疾病。症状始于第二个十年，表现为进行性肌阵挛性癫痫，30岁以后存活情况罕见。我们报告了4例经组织学证实为拉福拉体病患者的脑电图研究。即使在疾病早期也发现了后部癫痫样放电，这可能有助于早期诊断。
