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Cerebellar Cognitive Affective Syndrome in Children With Acute Postinfectious Cerebellar Ataxia.
Pediatric Neurology ( IF 3.2 ) Pub Date : 2020-04-08 , DOI: 10.1016/j.pediatrneurol.2020.03.019
Lars Evald 1 , Jane Evald 2 , Dorthe Hansen 1 , Nadja Lindberg Bonne 3 , Jonas Kjeldbjerg Hansen 4
Affiliation  

BACKGROUND Acute postinfectious cerebellar ataxia is the most common cause of acute ataxia in childhood. One previous case study has suggested that cerebellar cognitive affective syndrome may be comorbid with acute postinfectious cerebellar ataxia, but this was not confirmed by formal assessments. METHODS Children aged three to 15 years with a confirmed diagnosis of acute postinfectious cerebellar ataxia were invited to participate. Three patients were included and assessed by a pediatrician, neuropsychologist, and logopedist at the subacute stage (less than 14 days post-onset) and after six months and one year of follow-up. RESULTS All three children complied with the diagnostic criteria of cerebellar cognitive affective syndrome. The cognitive and affective symptoms persisted longer than the motor symptoms. Child A (girl, aged three years and eight months) was most severely affected with slow progression of motor cerebellar symptom; the cerebellar cognitive affective symptoms had not entirely remitted at one-year follow-up. Child B (boy, aged four years and four months) had more subtle motor cerebellar symptoms that swiftly remitted within the first week; the cerebellar cognitive affective symptoms were also more subtle. Child C (boy, aged seven years and eleven months) was considerably affected by motor cerebellar symptoms but showed marked improvement within the first month; the cerebellar cognitive affective symptoms had not entirely remitted at one-year follow-up. CONCLUSION Cognitive affective cerebellar syndrome may be an overlooked complication of acute postinfectious cerebellar ataxia. The severity of cerebellar cognitive affective symptoms seemed to correspond to the severity of the cerebellar motor symptoms, but the improvement was remarkably slower.

中文翻译:

小儿急性感染后小脑共济失调的小脑认知情感综合症。

背景技术急性感染后小脑共济失调是儿童急性共济失调的最​​常见原因。先前的一项案例研究表明,小脑认知情感综合症可能与急性感染后小脑共济失调并存,但尚未得到正式评估的证实。方法邀请确诊为急性感染性小脑共济失调的3至15岁儿童参加。在亚急性期(发病后少于14天)以及六个月零一年的随访后,由三名患者入选并由儿科医生,神经心理学家和专科医生进行评估。结果三个孩子均符合小脑认知情感综合症的诊断标准。认知和情感症状持续时间长于运动症状。孩子A(女孩,3岁零8个月大)的运动小脑症状进展缓慢,受影响最为严重;在一年的随访中,小脑认知情感症状并未完全缓解。儿童B(男孩,年龄为4岁零4个月)有较细微的运动小脑症状,在第一周内迅速缓解。小脑的认知情感症状也较微妙。儿童C(男7岁11个月大)受小脑运动症状的影响很大,但在第一个月内表现出明显的改善;在一年的随访中,小脑认知情感症状并未完全缓解。结论认知情感性小脑综合征可能是急性感染后小脑共济失调的一个被忽略的并发症。
更新日期:2020-04-08
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