Background

Alternating hemiplegia of childhood often manifests severe or extreme behavioral problems, the nature of which remains to be fully characterized.

Methods

We analyzed 39 consecutive patients with alternating hemiplegia of childhood for occurrence of behavioral problems and categorized those by severity: mild (not requiring intervention), moderate (requiring intervention but no risk), severe (minor risk to self, others, or both), and extreme (major risk). We then analyzed behavioral manifestations, concurrent morbidity, and medication responses in patients with severe or extreme symptoms.

Results

Two patients had mild behavioral problems, five moderate, 10 severe, six extreme, and 16 none. Extreme cases exhibited disruptive behaviors escalating to assaults. Triggers, when present, included peer-provocation, low frustration tolerance, limits set by others, and sleep disruption. Reversible psychotic symptoms occurred in two patients: in one triggered by infection and trihexyphenidyl, and in another triggered by sertraline. Of the 16 patients with severe or extreme symptoms, 13 had concurrent neuropsychiatric diagnoses. Occurrence of severe or extreme symptoms did not correlate with age, puberty, severity of intellectual disability, or mutation status (P > 0.05). A multidisciplinary team including mental health professionals comanaged all patients with severe or extreme symptoms with either behavioral therapy, medications, or both. When considering medications prescribed to more than four patients, medicines that demonstrated efficacy or partial efficacy in more than 50% of patients were alpha-adrenergic agonists and selective-serotonin-reuptake-inhibitors.

Conclusions

Patients with alternating hemiplegia of childhood (41%) often experience severe or extreme behavioral problems and, rarely, medication-triggered psychotic symptoms. These observations are consistent with current understanding of underlying alternating hemiplegia of childhood brain pathophysiology. Increasing awareness of these behavioral problems facilitates alternating hemiplegia of childhood management and anticipatory guidance.

中文翻译：

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儿童交替性偏瘫的严重和极端行为问题的特征。

背景

儿童的交替性偏瘫通常表现出严重或极端的行为问题，其性质尚待充分表征。

方法

我们分析了39例儿童交替性偏瘫的连续患者的行为问题的发生，并按严重程度将其分类：轻度（不需要干预），中度（需要干预但无风险），重度（对自己和他人的风险较小，和极端（重大风险）。然后，我们分析了具有严重或极端症状的患者的行为表现，并发发病率和用药反应。

结果

2例患者有轻度的行为问题，5例中度，10例严重，6例极端，16例没有。极端案件显示出破坏性行为升级为袭击。引发时，包括同伴挑衅，沮丧挫折忍耐力，他人设定的极限以及睡眠中断。两名患者出现了可逆的精神病症状：一名是由感染和三己基苯丙胺引发的，另一名是由舍曲林引发的。在16位有严重或极端症状的患者中，有13位同时进行了神经精神病学诊断。严重或极端症状的发生与年龄，青春期，智力残疾的严重程度或突变状态无关（P > 0.05）。一个由精神卫生专业人员组成的跨学科团队，通过行为疗法，药物或两者，共同管理所有患有严重或极端症状的患者。当考虑为超过四名患者开出处方药时，在50％以上的患者中显示出疗效或部分疗效的药物为α-肾上腺素能激动剂和选择性5-羟色胺再摄取抑制剂。

结论

患有儿童交替性偏瘫的患者（41％）经常会遇到严重或极端的行为问题，很少有药物触发的精神病症状。这些观察结果与当前对儿童脑病理生理的潜在交替性偏瘫的理解相一致。对这些行为问题的认识的提高促进了儿童管理和预期指导的交替偏瘫。