Elsevier

Sleep Medicine

Volume 8, Issues 7–8, November 2007, Pages 784-786
Sleep Medicine

Case Report
REM behavior disorder (RBD) can be one of the first symptoms of childhood narcolepsy

https://doi.org/10.1016/j.sleep.2006.11.018Get rights and content

Abstract

More than one in three adult patients suffering from narcolepsy-cataplexy experience rapid eye movement (REM) behavior disorder (RBD), while RBD in childhood is extremely rare. We present the cases of two girls (aged 9 and 7 years old) with narcolepsy-cataplexy, in whom RBD was one of the first symptoms of the disease. The coincidence of RBD was seen by nocturnal video-polysomnography (v-PSG), and narcolepsy was diagnosed from short sleep latency and multiple sleep onset REMs (SOREMs) during a multiple sleep latency test (MSLT). Both girls were human leukocyte antigen (HLA)-DQB1∗0602 positive, and their cerebrospinal fluid (CSF) hypocretin level (Hcrt-1) was extremely low.

Introduction

Rapid eye movement (REM) behavior disorder (RBD) is clinically characterized by dream-related abnormal motor behavior during REM sleep with polysomnographic (PSG) evidence of intermittent loss of REM atonia or excessive phasic muscle twitch activity of the submental and/or limb electromyogram (EMG) during REM sleep [1]. More than one in three adult patients suffering from narcolepsy-cataplexy experience RBD symptoms [2]. There is a younger age of onset and nearly equal sex distribution as distinct from idiopathic cases. To the best of our knowledge, only very few cases of RBD in children with concurrent narcolepsy have so far been described in the literature [3], [4].

Over the past two years, five new childhood cases of narcolepsy have been observed at the Department of Neurology, 1st Medical Faculty of Charles University in Prague. Four of them, all born in July, were diagnosed with narcolepsy-cataplexy; the remaining one was found to have narcolepsy without cataplexy. Diagnosis was established from short sleep latency and multiple sleep onset REM periods (SOREMs) during multiple sleep latency test (MSLT); all five children were also human leukocyte antigen (HLA)-DQB1∗0602 positive. In two girls with narcolepsy-cataplexy (aged 9 and 7 years old) clinical history pointed to RBD as one of the first symptoms of the disease. The diagnosis was corroborated by nocturnal video-polysomnography (v-PSG).

Section snippets

Case 1

This 9-year-old girl was admitted for imperative sleepiness lasting 2 months and for sporadic cataplectic attacks. At the same time, restless, nocturnal sleep with harmful behaviour and sleep-talking appeared. She would aggressively attack her sister and kick and strike the wall repeatedly. Objective neurologic findings were normal, and magnetic resonance imaging (MRI) revealed no pathology. MSLT demonstrated repeatedly shortened mean sleep latency (the first examination 0.3 min with three

Discussion

Unlike non-REM (NREM) parasomnias, RBD as a REM parasomnia is a extremely rare condition in childhood [5]. Sheldon and Jacobson [4] described the clinical and PSG characteristics of five children who met the criteria for RBD. One of their patients had well-documented narcolepsy. Three more cases of childhood narcolepsy accompanied by features of RBD were described by Schenck and Mahowald [3]. In a series of 17 patients, diagnosed according to established criteria for narcolepsy and for RBD,

Acknowledgement

This work was supported by a grant from the Ministry of Health (Czech Republik) 8086/NR 2004 and MSM 0021620849.

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