1Department of Neurology, Amrita Institute of Medical Sciences, Kochi, 682041, Kerala
2Department of Pediatric Genetics, Amrita Institute of Medical Sciences, Kochi, 682041, Kerala, India
3Stanford University Center For Narcolepsy, 3165 Porter Drive, Palo Alto, CA, 94304, USA
*Address for correspondence: A. G. Roy, Associate professor, Department of Neurology, AIMS, Ponnekkara lane, Kochi 682041, Kerala. Email address: arungraceroy@aims.amrita.edu
Online published on 7 October, 2016.
Narcolepsy is a chronic disorder characterized by excessive daytime somnolence (EDS), sleep paralysis, hypnagogic or hypnopompic hallucinations and cataplexy. Symptoms of narcolepsy typically start in childhood and young adult and clinical picture can be variable. In this article, the clinical picture, and investigations of 19 children with diagnosis of narcolepsy are described. The objective of this study is to describe the clinical profile, polysomnography (PSG) and multiple sleep latency test (MSLT) features and investigations in 19 children (<18years).
This is a descriptive retrospective study reporting on the clinical and biological features of children diagnosed with narcolepsy in the sleep center. All children underwent overnight PSG followed by MSLT. HLA analysis and CSF hypocretin assay were part of protocol but HLA analysis was done only in 13 children and CSF hypocretin assay in 6 cases.
Children less than 18 years satisfying the diagnostic criteria proposed by the International Classification of Sleep Disorders for narcolepsy with cataplexy (narcolepsy type 1) and narcolepsy without cataplexy (narcolepsy type 2) were taken.
Children with day time somnolence due to causes other than narcolepsy were excluded (Hypothyroidism, Obstructive sleep apnea, systemic diseases).
Nineteen children (14 males and 5 females) were there in cohort. All 19 children had excessive day time somnolence. Nine children (47.3%) had hypnagoic hallucinations, 7 (36.84%) had cataplexy and none had sleep paralysis. Overnight PSG showed shortened sleep latency in 15 children, while normal sleep latency was seen in 4 children. MSLT showed shortened sleep latency and more than two SOREM in all children. HLA DQB1*0602 was positive in 13 patients and CSF hypocretin levels were measured in 6 cases, values were below 100 pcg/ml. This is the largest case series of narcolepsy in children from India. Presentation of narcolepsy in children is similar to that reported in adults although typical cataplexy may not be present at the onset. Narcolepsy is a chronic disorder that remains under-diagnosed till adulthood with a significant impact on behavior and performance.
Hyper somnolence, Sleep Attacks, Cataplexy, Narcolepsy, Hypnagogic-hallucination
