Division of Pediatric Neurology and Developmental Medicine, Duke University, Durham, NC, USA.
Department of Psychiatry and Behavioral Sciences, Duke Pediatric Neuropsychology Program, Duke University, Durham, NC, USA.
Dev Med Child Neurol. 2020 Jul;62(7):820-826. doi: 10.1111/dmcn.14473. Epub 2020 Feb 7.
To evaluate presence and severity of social impairments in alternating hemiplegia of childhood (AHC) and determine factors that are associated with social impairments.
This was a retrospective analysis of 34 consecutive patients with AHC (19 females, 15 males; mean age: 9y 7mo, SD 8y 2mo, range 2y 7mo-40y), evaluated with the Social Responsiveness Scale, Second Edition (SRS-2).
SRS-2 scores, indicating level of social impairment, were higher than population means (75, SD 14 vs 50, SD 10, p<0.001). Of these, 27 out of 34 had high scores: 23 severe (>76), four moderate (66-76). All subscale domains, including social cognition, social communication, social awareness, social motivation, restricted interests, and repetitive behavior, had abnormal scores compared to population means (p<0.001). High SRS-2 scores were associated with the presence of autism spectrum disorder (ASD) and epilepsy (p=0.01, p=0.04), but not with other scales of AHC disease symptomatology. All nine patients who received formal evaluations for ASD, because they had high SRS-2 scores, were diagnosed with ASD.
Most patients with AHC have impaired social skills involving multiple domains. ASD is not uncommon. High SRS-2 scores in patients with AHC support referral to ASD evaluation. Our findings are consistent with current understandings of the pathophysiology of AHC and ASD, both thought to involve GABAergic dysfunction.
Most patients with alternating hemiplegia of childhood (AHC) have impaired social skills involving multiple domains. These impairments are significant compared to population means. Most patients with AHC have high Social Responsiveness Scale, Second Edition (SRS-2) scores. Patients with AHC with high SRS-2 scores are likely to have autism spectrum disorder.
评估儿童交替性偏瘫（AHC）中社会障碍的存在和严重程度，并确定与社会障碍相关的因素。
这是对 34 例连续 AHC 患者（19 名女性，15 名男性；平均年龄：9 岁 7 个月，标准差 8 岁 2 个月，范围 2 岁 7 个月至 40 岁）的回顾性分析，采用社会反应量表第二版（SRS-2）进行评估。
SRS-2 评分，表明社会障碍的程度，高于人群平均值（75，标准差 14 与 50，标准差 10，p<0.001）。其中，34 例中有 27 例得分较高：23 例严重（>76），4 例中度（66-76）。与人群平均值相比，所有子量表领域，包括社会认知、社会交流、社会意识、社会动机、受限兴趣和重复行为，均有异常得分（p<0.001）。高 SRS-2 评分与自闭症谱系障碍（ASD）和癫痫（p=0.01，p=0.04）的存在相关，但与 AHC 疾病症状的其他量表无关。所有 9 名因 SRS-2 评分高而接受 ASD 正式评估的患者均被诊断为 ASD。
大多数 AHC 患者的社交技能存在多个领域的障碍。ASD 并不少见。AHC 患者 SRS-2 评分高支持转诊进行 ASD 评估。我们的发现与当前对 AHC 和 ASD 病理生理学的理解一致，两者都被认为涉及 GABA 能功能障碍。
大多数儿童交替性偏瘫（AHC）患者的社交技能存在多个领域的障碍。与人群平均值相比，这些障碍是显著的。大多数 AHC 患者的社会反应量表第二版（SRS-2）评分较高。AHC 患者 SRS-2 评分高者可能患有自闭症谱系障碍。
