Rosman N Paul, Bergia Berta M
1Departments of Pediatrics and Neurology, Division of Pediatric Neurology, Boston University School of Medicine, Boston Medical Center, Boston, MA, USA.
J Child Neurol. 2013 Dec;28(12):1587-98. doi: 10.1177/0883073812472391. Epub 2013 Jan 22.
Childhood disintegrative disorder, a rare, relentlessly progressive neurologic disorder, first described by Heller in 1908, remains a condition of great interest. It has long been debated whether it is a discrete disorder or simply a late-onset variant of childhood autism. We have studied 6 cases of childhood disintegrative disorder, collected over 8 years, and followed for 2.5 to 22 years (mean 8.6 years). Childhood disintegrative disorder begins later in life than autism, and following a period of entirely normal development; the regression is more global and more severe than in autism; seizures are more frequent than in autism, yet demonstrable organicity in childhood disintegrative disorder is decidedly rare. Lastly, the prognosis is usually much worse than in autism, but in those cases with neither seizures nor epileptiform activity on electroencephalography (EEG), the outcome may be more favorable. Childhood disintegrative disorder should be viewed as a condition distinct from childhood autism.
儿童期崩解性障碍是一种罕见的、呈进行性发展的神经疾病，于1908年由海勒首次描述，至今仍是备受关注的病症。长期以来，一直存在争论，即它是一种独立的疾病，还是仅仅是儿童自闭症的迟发型变体。我们研究了8年间收集的6例儿童期崩解性障碍病例，并随访了2.5至22年（平均8.6年）。儿童期崩解性障碍起病时间比自闭症晚，且此前有一段完全正常的发育时期；其退化比自闭症更广泛、更严重；癫痫发作比自闭症更频繁，但儿童期崩解性障碍中可证实的器质性病变却极为罕见。最后，其预后通常比自闭症差得多，但在那些脑电图（EEG）既无癫痫发作也无癫痫样活动的病例中，预后可能更乐观。儿童期崩解性障碍应被视为一种与儿童自闭症不同的病症。
