Narcolepsy in childhood and adolescence

Reimão, Rubens; Lemmi, Hélio

doi:10.1590/S0004-282X1991000300005

Abstracts

The objective of this study was to evaluate clinical, polysomnography and the multiple sleep latency test (MSLT) features in young narcoleptics. We evaluated 14 patients with mean age of 13.6 years old (ranging from 6 to 18 years) ; 11 were males and 3 females. Daytime sleepiness was the main complaint in all cases and started at the ages of 6 to 17 years. Cataplexy was described in 10 cases and it was considered mild to moderate in all but one case. Sleep paralysis was present in 6 cases and hypnagogic hallucinations in 7 cases. The main polysomnography characteristics were the short sleep latency in 9 cases and the sudden onset of REM periods in 7 cases. The MSLT showed short or borderline sleep latencies in 13 cases, with a mean of 4.9 min; 2 or more REM periods were present in 13 cases. Clinical, polysomnographic and MSLT characteristics in the age bracket focused were remarkably similar to those of adult narcoleptics suggesting the stability of these psysiopa-thological markers.

O objetivo deste estudo foi analisar características clínicas, polissonográficas e do teste das latências múltiplas do sono (TLMS) em crianças e adolescentes narcolépticos. Avaliamos 14 pacientes com média de idade de 13,6 anos (variando de 6 a 18 anos); 11 eram do sexo masculino, 3 do feminino. Sonolência excessiva diurna foi a queixa principal em todos os casos e principiou entre 6 e 17 anos de idade. Cataplexia foi descrita em 10 casos sendo leve em 9 destes. Paralisia do sono estava presente em 6 oasos e alucinação hipnagógica em 7 casos. A polissonografia mostrou duas características principais, a saber, a latência do sono reduzida em 9 casos e o início súbito de sono REM no princípio da noite em 7 casos. O TLMS mostrou a latência do sono reduzida ou limítrofe em 13 casos, com média de 4,9 min. As características clínicas, polissonográficas e do TLMS na faixa etária aqui estudada foram semelhantes às do adulto, sugerindo a estabilidade de tais indicadores fisio-patológicos.

CONTENTS CONTEÚDO

Rubens Reimão; Hélio Lemmi

Sleep Disorders Center, Albert Einstein Hospital, São Paulo, Brazil ; Sleep Disorders Center, Baptist Memorial Hospital, Memphis, TN, USA

SUMMARY

The objective of this study was to evaluate clinical, polysomnography and the multiple sleep latency test (MSLT) features in young narcoleptics. We evaluated 14 patients with mean age of 13.6 years old (ranging from 6 to 18 years) ; 11 were males and 3 females. Daytime sleepiness was the main complaint in all cases and started at the ages of 6 to 17 years. Cataplexy was described in 10 cases and it was considered mild to moderate in all but one case. Sleep paralysis was present in 6 cases and hypnagogic hallucinations in 7 cases. The main polysomnography characteristics were the short sleep latency in 9 cases and the sudden onset of REM periods in 7 cases. The MSLT showed short or borderline sleep latencies in 13 cases, with a mean of 4.9 min; 2 or more REM periods were present in 13 cases. Clinical, polysomnographic and MSLT characteristics in the age bracket focused were remarkably similar to those of adult narcoleptics suggesting the stability of these psysiopa-thological markers.

RESUMO

O objetivo deste estudo foi analisar características clínicas, polissonográficas e do teste das latências múltiplas do sono (TLMS) em crianças e adolescentes narcolépticos. Avaliamos 14 pacientes com média de idade de 13,6 anos (variando de 6 a 18 anos); 11 eram do sexo masculino, 3 do feminino. Sonolência excessiva diurna foi a queixa principal em todos os casos e principiou entre 6 e 17 anos de idade. Cataplexia foi descrita em 10 casos sendo leve em 9 destes. Paralisia do sono estava presente em 6 oasos e alucinação hipnagógica em 7 casos. A polissonografia mostrou duas características principais, a saber, a latência do sono reduzida em 9 casos e o início súbito de sono REM no princípio da noite em 7 casos. O TLMS mostrou a latência do sono reduzida ou limítrofe em 13 casos, com média de 4,9 min. As características clínicas, polissonográficas e do TLMS na faixa etária aqui estudada foram semelhantes às do adulto, sugerindo a estabilidade de tais indicadores fisio-patológicos.

Texto completo disponível apenas em PDF.

Full text available only in PDF format.

Acknowledgement - We are indebt with Joseph Belluomini and Sharon Burt (BMH) for technical assistance and support.

Dr. Rubens Reimão - Rua Glicineas 128 - 04048 São Paulo SP - Brasil.

1. Association of Sleep Disorders Center. Diagnostic classification of sleep and arousal disorders. Sleep 1979, 2:1.
2. Broughton R, Ghanem Q, Hishikawa Y, Sugita Y, Nevrismalova S, Roth B. Life effects of narcolepsy: relationships to geographic origin (North American, Asian or European) and to other patient and illness variables. Can J Neurol Sci 1983, 10:100.
3. Carskadon MA. Ontogeny of human sleepiness as measured by sleep latency. In Dinges DF, Broughton RJ (eds): Sleep and Alertness: Chronobiological, Behavioral and Medical Aspects of Napping. New York: Raven Press, 1989.
4. Carskadon MA. The second decade. In Guilleminault C (ed): Disorders of Sleeping and Waking: Indications and Techniques. Menlo Park; Addison-Wesley Publ, 1982.
5. Carskadon MA, Dement WC, Mitler MM, Roth T, Westbrook PR, Keenan S. Guidelines for the multiple sleep latency test (MSLT) : a standard measure of sleepiness. Sleep 1986, 9:519.
6. Carskadon MA, Harvey K, Dement WC. Sleepiness in young adolescents. Sleep 1981, 4:229.
7. Carskadon MA, Harvey K, Dement WC. Multiple sleep latency test in the development of narcolepsy. West J Med 1981, 135:414.
8. Carskadon MA, Orav EJ, Dement WC. Evolution of sleep and daytime sleepiness in adolescents. In Guilleminault C, Lugaresi E (eds) : Sleep/Wake Disorders: Natural History, Epidemiology and Long-Term Evolution. New York: Raven Press, 1983.
9. Chisolm RC, Brook CJ, Harrison GF. Prepubescent narcolepsy in a 6-year-old child. Sleep Res 1985, 15:113.
10. Cowan R, Reimão R, Lemmi H, Vander Zwaag R. Narcolepsia e início súbito de períodos REM após despertares noturnos. Arq Neuro-Psiquiat (São Paulo) 1985, 43:229.
11. Dement WC, Carskadon MA, Ley R. The prevalence of narcolepsy. Sleep Res 1973, 2:147.
12. Hoed JVD, Kraemer H, Guilleminault C. Disorders of excessive daytime somnolence: polygraphic and clinical data for 100 patients. Sleep 1981, 4:23.
13. Kotagal S, Hartse KM, Walsh JK. Characteristics of narcolepsy in preteenaged children. Pediatrics 1990, 85:205.
14. Lemmi H, Reimão R, Cowan R, Appavoo S, Vander Zwaag R. Sleep onset REM periods after spontaneous awakenings and the diagnosis of narcolepsy. Sleep Res 1985, 14:186.
15. Mitler M. The multiple sleep latency test as an evaluation for excessive somnolence. In Guilleminault C (ed) : Disorders of Sleeping and Waking: Indications and Techniques. Menlo Park: Addison-Wesley Publ, 1982.
16. Mitler MM, Hoed JVD, Carskadon MA, Richarson G, Park R, Guilleminault C, Dement WC. REM sleep episodes during the multiple sleep latency test in narcoleptic patients. Electroenceph Clin Neurophysiol 1979, 46:479.
17. Nevrismalova S, Roth B, Zouhar A, Zemanova H. Narcolepsy-cataplexy and periodic hypersomnia in infancy. Electroenceph Clin Neurophysiol 1987, 67:21.
18. Nevrismalova-Bruhova S, Roth B. Heredofamilial aspects of narcolepsy and hypersomnia. Schw Arch Neurol Neurochir Psychiatr 1972, 110:45.
19. Rechtschaffen A, Kales A. Manual of Standardized Terminology, Techniques and Scoring for Sleep Stages of Human Subjects. Los Angeles: Brain Information Service, University of California, 1968.
20. Reimão R, Lemmi H, Belluomini J. Aspectos poligráficos dos movimentos periódicos do sono. Arq Neuro-Psiquiat (São Paulo) 1984, 42:313.
21. Richardson RS, Carskadon MA, Flagg W, Hoed JVD, Dement WC, Mitler MM. Excessive daytime sleepiness in man: multiple sleep latency measurement in narcoleptic and control subjects. Electroenceph Clin Neurophysiol 1978, 45:621.
22. Williams RL, Karacan I, Hursch CJ (eds) : EEG of Human Sleep. New York: John Wiley & Sons, 1974.
23. Wittig R, Zorick F, Roehrs T. Narcolepsy in a 7-year-old child. J Pediatr 1983, 102:725.
24. Yoss RE, Daly DD. Narcolepsy in children. Pediatrics 1960, 25:1025.
25. Young D, Zorick F, Wittig R, Roehrs T, Roth T. Narcolepsy in a pediatric population Am J Dis Childh 1988, 142:210.

Narcolepsy in childhood and adolescence

Narcolepsia na infância e adolescência

Publication Dates

Publication in this collection
22 Feb 2011
Date of issue
Sept 1991

This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.

[1] 1. Association of Sleep Disorders Center. Diagnostic classification of sleep and arousal disorders. Sleep 1979, 2:1.

[2] 2. Broughton R, Ghanem Q, Hishikawa Y, Sugita Y, Nevrismalova S, Roth B. Life effects of narcolepsy: relationships to geographic origin (North American, Asian or European) and to other patient and illness variables. Can J Neurol Sci 1983, 10:100.

[3] 3. Carskadon MA. Ontogeny of human sleepiness as measured by sleep latency. In Dinges DF, Broughton RJ (eds): Sleep and Alertness: Chronobiological, Behavioral and Medical Aspects of Napping. New York: Raven Press, 1989.

[4] 4. Carskadon MA. The second decade. In Guilleminault C (ed): Disorders of Sleeping and Waking: Indications and Techniques. Menlo Park; Addison-Wesley Publ, 1982.

[5] 5. Carskadon MA, Dement WC, Mitler MM, Roth T, Westbrook PR, Keenan S. Guidelines for the multiple sleep latency test (MSLT) : a standard measure of sleepiness. Sleep 1986, 9:519.

[6] 6. Carskadon MA, Harvey K, Dement WC. Sleepiness in young adolescents. Sleep 1981, 4:229.

[7] 7. Carskadon MA, Harvey K, Dement WC. Multiple sleep latency test in the development of narcolepsy. West J Med 1981, 135:414.

[8] 8. Carskadon MA, Orav EJ, Dement WC. Evolution of sleep and daytime sleepiness in adolescents. In Guilleminault C, Lugaresi E (eds) : Sleep/Wake Disorders: Natural History, Epidemiology and Long-Term Evolution. New York: Raven Press, 1983.

[9] 9. Chisolm RC, Brook CJ, Harrison GF. Prepubescent narcolepsy in a 6-year-old child. Sleep Res 1985, 15:113.

[10] 10. Cowan R, Reimão R, Lemmi H, Vander Zwaag R. Narcolepsia e início súbito de períodos REM após despertares noturnos. Arq Neuro-Psiquiat (São Paulo) 1985, 43:229.

[11] 11. Dement WC, Carskadon MA, Ley R. The prevalence of narcolepsy. Sleep Res 1973, 2:147.

[12] 12. Hoed JVD, Kraemer H, Guilleminault C. Disorders of excessive daytime somnolence: polygraphic and clinical data for 100 patients. Sleep 1981, 4:23.

[13] 13. Kotagal S, Hartse KM, Walsh JK. Characteristics of narcolepsy in preteenaged children. Pediatrics 1990, 85:205.

[14] 14. Lemmi H, Reimão R, Cowan R, Appavoo S, Vander Zwaag R. Sleep onset REM periods after spontaneous awakenings and the diagnosis of narcolepsy. Sleep Res 1985, 14:186.

[15] 15. Mitler M. The multiple sleep latency test as an evaluation for excessive somnolence. In Guilleminault C (ed) : Disorders of Sleeping and Waking: Indications and Techniques. Menlo Park: Addison-Wesley Publ, 1982.

[16] 16. Mitler MM, Hoed JVD, Carskadon MA, Richarson G, Park R, Guilleminault C, Dement WC. REM sleep episodes during the multiple sleep latency test in narcoleptic patients. Electroenceph Clin Neurophysiol 1979, 46:479.

[17] 17. Nevrismalova S, Roth B, Zouhar A, Zemanova H. Narcolepsy-cataplexy and periodic hypersomnia in infancy. Electroenceph Clin Neurophysiol 1987, 67:21.

[18] 18. Nevrismalova-Bruhova S, Roth B. Heredofamilial aspects of narcolepsy and hypersomnia. Schw Arch Neurol Neurochir Psychiatr 1972, 110:45.

[19] 19. Rechtschaffen A, Kales A. Manual of Standardized Terminology, Techniques and Scoring for Sleep Stages of Human Subjects. Los Angeles: Brain Information Service, University of California, 1968.

[20] 20. Reimão R, Lemmi H, Belluomini J. Aspectos poligráficos dos movimentos periódicos do sono. Arq Neuro-Psiquiat (São Paulo) 1984, 42:313.

[21] 21. Richardson RS, Carskadon MA, Flagg W, Hoed JVD, Dement WC, Mitler MM. Excessive daytime sleepiness in man: multiple sleep latency measurement in narcoleptic and control subjects. Electroenceph Clin Neurophysiol 1978, 45:621.

[22] 22. Williams RL, Karacan I, Hursch CJ (eds) : EEG of Human Sleep. New York: John Wiley & Sons, 1974.

[23] 23. Wittig R, Zorick F, Roehrs T. Narcolepsy in a 7-year-old child. J Pediatr 1983, 102:725.

[24] 24. Yoss RE, Daly DD. Narcolepsy in children. Pediatrics 1960, 25:1025.

[25] 25. Young D, Zorick F, Wittig R, Roehrs T, Roth T. Narcolepsy in a pediatric population Am J Dis Childh 1988, 142:210.