Indian Journal of Sleep Medicine

Register      Login

SEARCH WITHIN CONTENT

FIND ARTICLE

Volume / Issue

Online First

Archive
Volume  19 (2024)
Volume  18 (2023)
Volume  17 (2022)
Volume  16 (2021)
Volume  15 (2020)
Volume  14 (2019)
Volume  13 (2018)
Volume  12 (2017)
Volume  11 (2016)
Volume  10 (2015)
Volume  9 (2014)
Volume  8 (2013)
Volume  7 (2012)
Volume  6 (2011)
Volume  5 (2010)
Volume  4 (2009)
Volume  3 (2008)
Volume  2 (2007)
Volume  1 (2006)
Related articles

VOLUME 10 , ISSUE 2 ( April-June, 2015 ) > List of Articles

ORIGINAL ARTICLE

Clinical Profile of Narcolepsy in Children in India

Anand Kumar, Arun Grace Roy, Siby Gopinath, Dinesh Singh, Sheela Nampoodiri, Vinayan K P, Mali A. Einen, Emmanuel Mignot

Keywords : Hyper somnolence, Sleep Attacks, Cataplexy, Narcolepsy, Hypnagogic-hallucination

Citation Information : Kumar A, Roy AG, Gopinath S, Singh D, Nampoodiri S, P VK, Einen MA, Mignot E. Clinical Profile of Narcolepsy in Children in India. Indian Sleep Med 2015; 10 (2):74-78.

DOI: 10.5958/0974-0155.2015.00011.X

License: NA

Published Online: 01-06-2013

Copyright Statement:  NA


Abstract

Objective/Background: 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). Materials and Methods: 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. Inclusion criteria: 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. Exclusion criteria: Children with day time somnolence due to causes other than narcolepsy were excluded (Hypothyroidism, Obstructive sleep apnea, systemic diseases). Results/Conclusion: 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.

PDF Share
  1. Paul C. Peterson, Aatif M. Husain: Pediatric narcolepsy. Brain & Development 30 2008; 609–23.
  2. AASM. International classification of sleep disorders. 2nd ed. Westchester IL: American Academy of Sleep Medicine; 2005.
  3. Challamel MJ, Mazzola ME, Nevsmalova S, Cannard C, Louis J, Revol Ml. Narcolepsy in children. Sleep 1994;17(Suppl. 8):S17–20.
  4. McKenna C, McNicholas F. Childhood onset narcolepsy. A case report. Eur Child adolesc Psychiatry 2003; 12:43–7.
  5. Ohayon MM, Ferini-Strambi L, Plazzi G, Smirne S, Castronovo V. Frequency of narcolepsy symptoms and other sleep disorders in narcoleptic patients and their first-degree relatives. J Sleep Res 2005;14:437–45.
  6. Stores G. The protean manifestations of childhood narcolepsy and their misinterpretation. Dev Med Child Neurol 2006;48(4):307–10.
  7. Adi Aran, Mali Einen, Ling Lin, Guiseppe Plazzi, Seiji Nishino. Clinical and therapeutic aspects of childhood narcolepsy-cataplexy: a retrospective study of 51 children. Sleep 2010 ;33(11): 1457–64.
  8. Challamel MJ, Mazzola ME, Nevsimalova S, Cannard C, Louis J, Revol M. Narcolepsy in children. Sleep. 1994;17(8 Suppl):S17–20.
  9. Overeem S, Mignot E, van Dijk JG, Lammers GJ. Narcolepsy: Clinical features, new pathophysiologic insights and future perspectives. J Clin Neurophysiol 2001; 18(2):78–105.
  10. Thorpy M. Current concepts in the etiology, diagnosis and treatment of narcolepsy. Sleep Med 2001;2(1):5–17.
  11. Sona Nevsimalova. Narcolepsy in childhood. Sleep Medicine Reviews 2009;13: 169–80.
  12. Stores G, Montgomery P, Wiggs L. The psychological problems of children with narcolepsy and those with excessive daytime sleepiness of uncertain origin. Pediatrics 2006; 118:1116– 23.
  13. Okun ML, Lin L, Pelin Z, Hong S, Mignot E. Clinical aspects of narcolepsy–cataplexy across ethnic groups. Sleep 2002; 25(1):27–35.
  14. KokSW, OvereemS, Visscher TL, Lammers GJ, Seidell JC, Pijl H,Meinders A E. Hypocretin deficiency in narcoleptic humans is associated with abdominal obesity. Obes Res 2003; 11(9):1147–54.
  15. Lammers GJ, Pijl H, Iestra J, Langius JA, Buunk G, Meinders AE. Spontaneous food choice in narcolepsy. Sleep 1996; 19(1):75–6.
  16. Schuld A, Beitinger PA, Dalal M, Geller F, Wetter TC, AlbertED, et al. Increased body mass index (BMI) in male narcoleptic patients, but not in HLA-DR2-positive healthy male volunteers. Sleep Med 2002; 3(4):335–9.
  17. Kotagal S, Krahn LE, Slocumb N. A putative link between childhood narcolepsy and obesity. Sleep Med 2004;5(2):147–50.
  18. Crocker A, Espana RA, Papadopoulou M, Saper CB, Faraco J, Sakurai T, et al. Concomitant loss of dynorphin, NARP, and orexin in narcolepsy. Neurology 2005;65(8):1184–8.
  19. Nishino S, Ripley B, Overeem S, Nevsimalova S, Lammers GJ, Vankova J, et al. Low cerebrospinal ûuid hypocretin (orexin) and altered energy homeostasis in human narcolepsy. Ann Neurol 2001; 50:381–8.
  20. Chabas D, Foulon C, Gonzales J, Nasr M, Caen OL, Willer JC, et al. Eating disorder and metabolism in narco leptic patients. Sleep 2007;30:1267–73.
  21. Guilleminault C, Pelayo R. Narcolepsy in prepubertal children. Ann Neurol 1998;43(1):135–42.
  22. Kotagal S, Goulding PM. The laboratory assessment of daytime sleepiness in childhood. J Clin Neurophysiol 1996;13:208–18.
  23. Huang YS, Tafti M, Guilleminault C. Daytime sleepiness with and without cataplexy in ChineseeTaiwanese patients. Sleep Med 2006;7:454–7.
  24. Kadotani H, Faraco J, Mignot E. Genetic studies in the sleep disorder narcolepsy. Genome Res September 5, 2007;8:427–34.
  25. Kanbayashi T, Yano T, Ishiguro H, Kawanishi K, Chiba S, Aizawa R, et al. Hypocretin (orexin) levels in human lumbarCSF in different age groups: infants to elderly persons. Sleep 2002;25:337–9.
  26. Kubota H, Kanbayashi T, Tanabe Y, Ito M, Takanashi J, Kohno Y, et al. Decreased cerebrospinal ûuid hypocretin-1 levels near the onset of narcolepsy in 2 pre-pubertal children. Sleep 2003; 26:55–57.
  27. Greenhill LL, Pliszka S, Dulcan MK, Bernet W, Arnold V,Beitchman J, et al. Practice parameter for the use of stimulantmedications in the treatment of children, adolescents, and adults. J Am Acad Child Adolesc Psychiatry 2002;41(Suppl. 2):26–49.
  28. Merrill S.Wise, Jennifer Lynch.. Narcolepsy in children. Seminars in Pediatric Neurology 2001; Vol 8, (4) :198– 206
  29. Young D, Zorick F, Wittig R, Roehrs T, Roth T. Narcolepsy in a pediatric population. Am J Dis Child. 1988 Feb; 142(2):210–3.
PDF Share
PDF Share

© Jaypee Brothers Medical Publishers (P) LTD.