Badoe E V
Department of Child Health, University of Ghana Medical School, Ghana.
West Afr J Med. 2011 Mar-Apr;30(2):140-4.
Alternating hemiplegia of children is a rare neurological disorder that in its characteristic form has few differential diagnosis. The diagnosis of intractable seizures is difficult to avoid for physicians not aware of the disease.
To describe the clinical characteristics of Alternating Hemiplegia of Childhood (AHC), and response to various drugs
A Ghanaian child with AHC was followed up for three years at the Neurology Clinic, Korle Bu Teaching Hospital, Accra. Her characteristics including EEG and MRI findings were documented. She was severely unsuccessfully treated as an epileptic. Further clinical re-evaluation provided clues to the diagnosis of alternating hemiplegia of childhood.
The child, a female patient, was seen within the first week of life. The initial complaints were abnormal eye movements, and subsequently recurrent hemiplegic episodes, that started at age two and lasted hours to days. Attacks occurred at a frequency of about three per month and lasted from several hours to three days. An established trigger was bathing with cold water. Sleep relieved symptoms. The child had evidence of global developmental delay and neurological abnormalities including ataxia. EEG and MRI were both reported as abnormal. She experienced recurrent seizures. Topiramate and several anti-convulsants were not helpful but aripiprazole reduced the frequency of attacks.
The case highlights the fact that AHC starts very early in life and is commonly misdiagnosed as epilepsy .It can coexist with epilepsy and abnormal MRI findings. Aripiprazole appears effective in its treatment.
儿童交替性偏瘫是一种罕见的神经系统疾病,其典型形式的鉴别诊断较少。对于不了解该疾病的医生来说,很难避免将其误诊为难治性癫痫。
描述儿童交替性偏瘫(AHC)的临床特征以及对各种药物的反应。
一名患有AHC的加纳儿童在阿克拉科尔勒布教学医院神经科门诊接受了三年的随访。记录了她的特征,包括脑电图和磁共振成像(MRI)检查结果。她曾被作为癫痫患者进行治疗,但治疗效果不佳。进一步的临床重新评估为儿童交替性偏瘫的诊断提供了线索。
该患儿为女性,出生后第一周就被发现。最初的症状是异常的眼球运动,随后从两岁开始出现反复发作的偏瘫发作,持续数小时至数天。发作频率约为每月三次,持续数小时至三天。确定的诱因是用冷水洗澡。睡眠可缓解症状。该患儿有全面发育迟缓及包括共济失调在内的神经功能异常的证据。脑电图和MRI检查均报告异常。她有反复发作的癫痫。托吡酯和几种抗惊厥药物均无效,但阿立哌唑减少了发作频率。
该病例突出了这样一个事实,即AHC在生命早期就开始发病,常被误诊为癫痫。它可与癫痫及MRI异常表现同时存在。阿立哌唑似乎对其治疗有效。