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Neuropsychological Deficits and Sleep in Myotonic Dystrophy

Published online by Cambridge University Press:  18 September 2015

R. Broughton*
Affiliation:
Division of Neurology, Ottawa General Hospital and University of Ottawa, Ottawa, Ontario
D. Stuss
Affiliation:
Division of Neurology, Ottawa General Hospital and University of Ottawa, Ottawa, Ontario
M. Kates
Affiliation:
Division of Neurology, Ottawa General Hospital and University of Ottawa, Ottawa, Ontario
J. Roberts
Affiliation:
Division of Neurology, Ottawa General Hospital and University of Ottawa, Ottawa, Ontario
W. Dunham
Affiliation:
Division of Neurology, Ottawa General Hospital and University of Ottawa, Ottawa, Ontario
*
Division of Neurology, Ottawa General Hospital, 501 Smyth Road, Ottawa, Canada K1H 8L6
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Abstract:

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Eight patients with myotonic dystrophy underwent comprehensive neuropsychological testing and overnight polysomnography to assess whether the waking cognitive impairment might be attributable to sleep structure abnormalities or to sleep-related respiratory problems. Patients showed substantial neuropsychological deficits, fragmented nocturnal sleep and, in half the patients, sleep apnea and/or hypopnea both mainly of central type. There was no statistically significant correlation between the degree of daytime cognitive deficit and the degree of sleep fragmentation or of respiratory problems at night. It was concluded that the neuropsychological deficit in mytonic dystrophy cannot be attributed to a secondary effect of nocturnal sleep apnea or sleep disruption but probably represents a direct effect of CNS lesions.

Type
Original Articles
Copyright
Copyright © Canadian Neurological Sciences Federation 1990

References

REFERENCES

1.Woodward, JB, Heaton, RK, Simon, DB, et al. Neuropsychological findings in myotonic dystrophy. J Clin Neuropsychol 1982; 4: 335342.Google ScholarPubMed
2.Bird, TD, Follett, C, Griep, E, Cognitive and personality function in myotonic dystrophy. J Neurol Neurosurg Psychiatry 1983; 46: 971980.CrossRefGoogle Scholar
3.Stuss, D, Kates, MH, Poirier, CA, et al. Evaluation of information processing speed and neuropsychological functioning in patients with myotonic dystrophy. J Clin Exp Neuropsychol 1987; 9: 131146.CrossRefGoogle ScholarPubMed
4.Coccagna, G, Marinelli, P, Lugaresi, E, Sleep and alveolar hypoventilation in myotonic dystrophy. Acta Neurol Belg 1982; 82: 185194.Google ScholarPubMed
5.Coccagna, G, Mantovani, M, Parchi, C, et al. Alveolar hypoventilation and hypersomnia in myotonic dystrophy. J Neurol Neurosurg Psychiatry 1975; 38: 977984.CrossRefGoogle ScholarPubMed
6.Goldenberg, F, Perrier, M, Harf, A, et al. Sleep and respiratory patterns in myotonic dystrophy. In: Koella, WP, Levin, P, eds. Sleep 1976 Basel: Karger. 1977; 439442.Google Scholar
7.Leygonie-Goldenberg, F, Perrier, M, Duizabo, PL, et al. Troubles de la vigilance, du sommeil et de la fonction respiratoire dans la maladie de Steinert. Rev Neurol (Paris) 1977; 133: 255270.Google Scholar
8.Guilleminault, C, Cummiskey, J, Motta, J, et al. Respiratory and hemodynamic study during wakefulness and sleep in myotonic dystrophy. Sleep 1978; 1: 1931.CrossRefGoogle ScholarPubMed
9.Cummiskey, J, Lynne-Davies, P, Guilleminault, C, Sleep study and respiratory function in myotonic dystrophy. In: Guilleminault, C, Dement, W, eds. Sleep Apnea Syndromes. New York: Liss 1978; 295308.Google Scholar
10.Cirignotta, F, Mondini, S, Zuccini, M, et al. Sleep-related breathing impairment in myotonic dystrophy. J Neurol 1987; 235: 8085.Google ScholarPubMed
11.Berry, DT, Webb, WB, Block, AJ, et al. Nocturnal hypoxia and neuropsychological variables. J Clin Exp Neuropsychol 1986; 8: 229238.CrossRefGoogle ScholarPubMed
12.Telakivi, T, Kajaste, S, Partinen, M, et al. Cognitive function in middle-aged snorers and controls: roles of excessive daytime somnolence and sleep-related hypoxic events. Sleep 1988; 2: 454462.Google Scholar
13.Kales, A, Caldwell, AB, Cadieux, RJ, et al. Severe obstructive sleep apnea - II: associated psychopathology and psychosocial consequences. J Chron Dis 1985; 38: 427434.Google ScholarPubMed
14.Greenberg, GD, Watson, RK, Deptula, D, Neuropsychological dysfunction in sleep apnea. Sleep 1987; 10: 254262.CrossRefGoogle ScholarPubMed
15.Besset, A, Tafti, M, Billiard, M, Is cognitive impairment in OSAS related to cerebral hypoxia or to sleep fragmentation? Presented at the 10th Congress of the European Sleep Research Society France: Strasbourg May 1990.Google Scholar
16.Findlay, LJ, Fabrizio, MJ, Knight, H, et al. Driving simulator performance in patients with sleep apnea. Am Rev Respir Dis 1989; 140: 529530.Google Scholar
17.Derderian, SS, Bridenbaugh, RJ, Rajogopal, KR, Neuropsychological symptoms in obstructive sleep apnea improve after treatment with nasal continuous positive airway pressure. Chest 1988; 94: 10231027.CrossRefGoogle ScholarPubMed
18.Bundy, S, Clinical evidence for heterogenecity in myotonic dystrophy. J Med Genet 1982; 19: 341348.Google Scholar
19.Rechtschaffen, A, Kales, A, eds. A Manual of Standardized Terminology, Techniques and Scoring Systems for Sleep Stages of Human Subjects Washington D.C.: National Institutes of Health Publication 1968; no. 204.Google Scholar
20.Guilleminault, C, eds. Sleeping and Waking Disorders: Indications and Techniques: Menlo Park. CA: Addison-Wesley 1982.Google Scholar
21.Wechsler, D, WAIS-R Manual. Wechsler Adult Intelligence Scale Revised: New York: Harcourt Brace Jovanovich 1981.Google Scholar
21.Army Individual Test Battery. Manual of directions and scoring. Washington D.C.: U.S.: Army War Department, Adjutant General's Office.Google Scholar
23.Stroop, JR, Studies of interference in serial verbal reactions. J Exp Psychol 1935; 18: 643662.Google Scholar
24.Spreen, O, Benton, AL, Neurosensory Center comprehensive examination for aphasia: Victoria, B.C.: Neuropsychology Laboratory, Department of Psychology, University of Victoria.Google Scholar
25.Wechsler, D, A standardized memory scale for clinical use. J Psychol 1945; 19: 8795.CrossRefGoogle Scholar
26.Glass, GV, Stanley, JC, Statistical methods in education and psychology: Englewood Cliff, N.J.: Prentice-Hall 1970.Google Scholar
27.Lugaresi, E, Medori, R, Montagna, P, et al. Fatal insomnia and dysautonomia with selective degeneration of thalamic nuclei. N Engl J Med 1986; 315: 9971003.CrossRefGoogle ScholarPubMed
28.Culebras, A, Feldman, RG, Merk, FB, Cytoplasmic inclusion bodies within neurones of the thalamus in myotonic dystrophy: a light and electron microscope study. J Neurol Sei 1973; 19: 319329.CrossRefGoogle ScholarPubMed