Miles D K, Holmes G L
Department of Neurology, Children's Hospital, Harvard Medical School, Boston, Massachusetts 02115.
J Clin Neurophysiol. 1990 Jul;7(3):369-79. doi: 10.1097/00004691-199007000-00004.
Benign neonatal seizures is a rare but increasingly recognized syndrome characterized by seizures in the neonatal or infantile period. Two forms are recognized: familial and nonfamilial. In both instances, the seizures may be quite severe, and status epilepticus is common. The nonfamilial form is characterized by idiopathic, self-limited seizures occurring in previously normal neonates. The seizures most commonly occur at day 5 and have been called "fifth-day fits" by some authors. Familial seizures most frequently have their onset during the first week of life, but onset may occur as late as early infancy. These seizures may recur for several months before resolving. No cause is found for the seizures, and the patient appears healthy during the interictal period. The family history reveals benign neonatal seizures in other family members. Although the prognosis is favorable in both syndromes, seizures may occasionally occur later in life in the familial form. The familial form of benign neonatal seizures is autosomal dominant, and the gene has been localized to chromosome 20.
良性新生儿惊厥是一种罕见但越来越被认可的综合征，其特征为新生儿期或婴儿期出现惊厥。已确认有两种类型：家族性和非家族性。在这两种情况下，惊厥可能相当严重，癫痫持续状态很常见。非家族性类型的特征是在先前正常的新生儿中出现特发性、自限性惊厥。惊厥最常发生在出生后第5天，一些作者将其称为“第五天惊厥”。家族性惊厥最常在出生后第一周内发病，但发病也可能晚至婴儿早期。这些惊厥可能会反复出现数月后才缓解。未发现惊厥的病因，患者在发作间期看起来健康。家族史显示其他家庭成员有良性新生儿惊厥。虽然两种综合征的预后都良好，但家族性类型的惊厥偶尔可能在以后的生活中再次出现。家族性良性新生儿惊厥为常染色体显性遗传，相关基因已定位到20号染色体。
