MacIntosh D S, Camfield P R, Camfield C S
Department of Pediatrics, Dalhousie University Medical School, IWK Grace Health Centre, Halifax, Nova Scotia, Canada.
J Child Neurol. 1998 Aug;13(8):372-6. doi: 10.1177/088307389801300802.
We examined the prognosis for children with epilepsy not conforming to a genetic syndrome, who also had close relatives with epilepsy. Probands, with no identified cause for epilepsy except a first-degree relative with epilepsy, were identified from a population-based cohort of 504 children in Nova Scotia, with onset of seizures between 1977 and 1985. The primary outcome measure was seizure remission after an average of 15 years follow-up for probands (n = 27) and 26 years for their affected relatives (n = 32). Of probands, 92% were seizure free for 3 or more years at the end of follow-up, compared with 76% of relatives. When seizures began before age 12 years, 96% of probands and 94% of affected relatives were seizure free at the end of follow-up. There was little concordance for the details of the clinical course between probands and affected family members. This high level of remission was considerably better than for similar patients from the original Nova Scotian cohort (P < .02). We conclude that children with epilepsy not conforming to a well-defined genetic syndrome, but with an affected first-degree relative, have a remarkably good prognosis.
我们研究了不符合遗传综合征的癫痫患儿的预后情况，这些患儿还有患癫痫的近亲。先证者是从新斯科舍省504名儿童的人群队列中确定的，他们癫痫发作的起始时间在1977年至1985年之间，除了有癫痫的一级亲属外，没有其他明确的癫痫病因。主要结局指标是先证者平均随访15年（n = 27）及其受影响亲属随访26年（n = 32）后的癫痫缓解情况。在随访结束时，92%的先证者有3年或更长时间无癫痫发作，而亲属的这一比例为76%。当癫痫发作始于12岁之前时，96%的先证者和94%的受影响亲属在随访结束时无癫痫发作。先证者和受影响家庭成员之间临床病程的细节一致性很低。这种高缓解率明显优于新斯科舍省原始队列中的类似患者（P <.02）。我们得出结论，不符合明确遗传综合征但有患癫痫的一级亲属的癫痫患儿预后非常好。
