Shinnar Shlomo, Rapin Isabelle, Arnold Susan, Tuchman Roberto F, Shulman Lisa, Ballaban-Gil Karen, Maw Myint, Deuel Ruthmary K, Volkmar Fred R
Department of Neurology, Albert Einstein College of Medicine, Bronx, NY, USA; Department of Pediatrics, Albert Einstein College of Medicine, Bronx, NY, USA; The Comprehensive Epilepsy Management Center at Montefiore Medical Center, Albert Einstein College of Medicine, Bronx, NY, USA; The Children's Evaluation and Rehabilitation Center at Rose F. Kennedy Center, Albert Einstein College of Medicine, Bronx, NY, USA.
Department of Neurology, Albert Einstein College of Medicine, Bronx, NY, USA; Department of Pediatrics, Albert Einstein College of Medicine, Bronx, NY, USA.
Pediatr Neurol. 2001 Mar;24(3):185-191. doi: 10.1016/S0887-8994(00)00266-6.
Language regression is observed both in autistic regression and as part of acquired epileptic aphasia (Landau-Kleffner Syndrome). We prospectively identified 177 children with language regression at four major medical centers, and their clinical characteristics were recorded. Their mean age at regression was 22.8 months. The mean time-to-specialist referral was 38 months of age. Most children (88%) met criteria for autism or manifested autistic features. Males (P = 0.02) and children less than 3 years of age who regressed (P = 0.016) had a higher probability of developing autistic behaviors. Seizures were more common in children who regressed after they reached 3 years of age (P < 0.001), and children with seizures were less likely to have associated autistic regression (P < 0.001). Electroencephalogram abnormalities were reported in 37% of patients and were more common in children with seizures (P < 0.001). At last follow-up, language function was impaired in 88% of the children, although some improvement was noted in 57%. We conclude that the loss of previously acquired language at any age, even if that language only includes a few words or communicative gestures, is often associated with a more global regression in cognition and/or behavior and has serious implications for future function. Early identification and referral of these children is necessary to allow for diagnosis and intervention.
在自闭症性退行和作为获得性癫痫性失语(兰道-克莱夫纳综合征)的一部分中均观察到语言倒退。我们在四个主要医疗中心前瞻性地确定了177名有语言倒退的儿童,并记录了他们的临床特征。他们倒退时的平均年龄为22.8个月。转诊至专科医生的平均时间为38个月龄。大多数儿童(88%)符合自闭症标准或表现出自闭症特征。男性(P = 0.02)和倒退的3岁以下儿童(P = 0.016)出现自闭症行为的可能性更高。癫痫发作在3岁后倒退的儿童中更常见(P < 0.001),有癫痫发作的儿童发生相关自闭症性退行的可能性较小(P < 0.001)。37%的患者报告有脑电图异常,且在有癫痫发作的儿童中更常见(P < 0.001)。在最后一次随访时,88%的儿童语言功能受损,尽管57%的儿童有一些改善。我们得出结论,在任何年龄丧失先前获得的语言,即使该语言仅包括几个单词或交流手势,通常都与更全面的认知和/或行为倒退相关,并对未来功能有严重影响。对这些儿童进行早期识别和转诊以便进行诊断和干预是必要的。