Narcolepsy-Cataplexy: Is Streptococcal Infection a Trigger?

Abstract

Narcolepsy-cataplexy is an uncommon sleep disorder which may present in childhood. We report a case of an 8-year-old presenting with narcolepsy-cataplexy following a streptococcal infection. Autoimmune etiology for narcolepsy has been suggested. In our patient increased anti-streptolysin O and antiDNAse B titers were noted. As suggested by recent cases, the streptococcal infection was likely a trigger for narcolepsy onset in this genetically predisposed child. The patient was initially diagnosed as having Sydenham chorea due to motor movements. However, these transient movements may be due to the narcolepsy onset. Narcolepsy in childhood may present with atypical symptoms; it might be diffi cult to obtain accurate history and can be misdiagnosed as in the reported case. A high index of clinical suspicion is needed to diagnose these patients. Keywords: Narcolepsy, streptococcal infection Citation: Natarajan N; Jain SV; Chaudhry H; Hallinan BE; Simakajornboon N. Narcolepsy-cataplexy: is streptococcal infection a trigger? J Clin Sleep Med 2013;9(3):269-270.http://dx.doi.org/10.5664/jcsm.2498 C a S E R E p o R t SN arcolepsy with hypocretin defi ciency occurs in approximately 1 out of 3,000 individuals. The incidence in children is not known. The loss of hypocretin-secreting neurons in hypothalamus is implicated in the disease process, which is believed to be autoimmune. Recently, post-infectious etiologies have been implicated. We report a case of an 8-year-old presenting with narcolepsy-cataplexy after a streptococcal infection. REpoRt oF CaSEAn 8-year-old biracial male presented to our clinic for a second opinion. Two months prior to presentation, he had a sore throat and fever of 102°F. Rapid strep test for Streptococcus pharyngitis was negative. Three weeks later, he had rapid onset of somnolence. He was sleeping more than 10 hours in night with 45 to 60-min naps every 3-4 hours. He had brief asynchronous jerks of all extremities prior to falling asleep and slurred speech after laughing. A video EEG and MRI brain were normal. Thyroid and liver function tests, and EBV, and Lyme titers were normal. Anti-streptolysin O (ASO) and anti-DNAse B (ADB) were elevated to 200 IU/mL (normal 0-100 IU/mL) and 587 U/mL (normal 0-170 U/mL), respectively. He was diagnosed with Sydenham chorea (SC) and started on penicillin.He continued to have increased sleepiness and was referred to us. He also had a decline in school performance and moodiness. He denied hallucinations, vivid dreams, or sleep paralysis. Mother noted increased jitteriness/shaking when he laughed. His Epworth Sleepiness Scale score was 19, and BMI was 19.7 kg/m 2 (> 90 th percentile). His physical examination was normal. There were no complex motor movements.Differential diagnosis included narcolepsy or hypersomnia associated with sleep (OSA, PLMD) or neurological disorders. A lumbar puncture showed normal cerebrospinal fl uid (CSF) protein, glucose, and cell count. The CSF neurotransmitter metabolites tetrahydrobiopterin and neopterin profi les were normal. CSF...

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