Atypical electroclinical presentation of Landau Kleffner syndrome: a case report of an 8 year old Nigerian child

Chuks G. Nwala, Omokhuale M. Ejemierele
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Abstract

Epileptic seizures account for common neurologic presentations in paediatric neurology units in developing countries. Atypical presentation of syndromic epilepsy may be missed especially sensory, psychic or autonomic manifestations. An 8 year old male Nigerian child presented with recurrent vomiting and delayed motor milestones, regression of speech (acquired aphasia) with unremarkable birth history or any underlying medical or surgical condition. He had delayed gross motor development and continuous bilateral polyspike waves of slow sleep on EEG. Self-limited autonomic epileptic seizure syndrome which was a strong differential of autonomic seizures in early childhood was not suggestive as his EEG findings failed to demonstrate occipital spike wave pattern nor did photic stimulation evoke any epileptiform waves. Child made significant clinical improvement with anticonvulsants, neuro vitamins, and behavioural therapy, evidenced by regain of non-verbal communication, resolution of autonomic symptoms and temper tantrums. A high index of suspicion should be applied in patients with acquired aphasia with subclinical or recurrent autonomic symptoms.
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兰道-克莱夫纳综合征的非典型电临床表现:一名 8 岁尼日利亚儿童的病例报告
癫痫发作是发展中国家儿科神经科常见的神经系统疾病。综合征癫痫的非典型表现可能会被漏诊,尤其是感觉、精神或自主神经表现。一名8岁的尼日利亚男童因反复呕吐、运动发育迟缓、言语退化(获得性失语)而就诊,出生史或任何潜在的内外科疾病均无异常。他的大运动发育迟缓,脑电图显示双侧连续多棘波慢速睡眠。自限性自发性癫痫发作综合征是幼儿期自发性癫痫发作的一个强有力的鉴别指标,但由于他的脑电图检查结果没有显示枕骨尖波模式,光刺激也没有诱发任何癫痫样波,因此没有提示性意义。经过抗惊厥药、神经维生素和行为治疗后,患儿的临床症状明显好转,表现为非语言交流能力恢复、自律神经症状和脾气得到缓解。对于伴有亚临床或复发性自主神经症状的后天性失语症患者,应高度怀疑。
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