Narcolepsy, like RBD, is a prominent disorder of REM sleep dysregulation. Narcolepsy has a very strong association with HLA class II genes, with the DQB 1*0602 (DQwl group) allele being expressed in nearly all cases. Our center performed HLA class II antigen phenotyping in a group of 25 Caucasian males who had RBD but not narcolepsy: 84% (n = 21) were DQwl
(DQB 1*05,06) positive [and 28% (n = 7) were DR2 positive]; DQB 1*0501 (n = 9) and DQB 1*0602 (n = 7) were the most common phenotypes (Schenck et al., 1996b). The 84% DQwl rate in RBD was significantly greater (p = .015) than the 56% DQwl rate found in a local Caucasian comparison group (n = 66), and was greater than the 39-66% DQwl rates in 12 published Caucasian groups (n = 40-418 per group). In contrast to the nearly 100% DQwl-DR2 linkage in narcolepsy, only 28% of RBD patients in this report were DR2-positive. The strong dissociation between DQwl and DR2 in RBD can be contrasted with the very strong DQwl-DR2 association in narcolepsy. Narcolepsy and RBD, therefore, have strikingly convergent (DQwl) and divergent (DR2) HLA findings.
The recognition of RBD has demonstrated multiple physiological and clinical relationships; has shed additional scientific light on the "bumps in the night"; has opened up new areas of research on sleep and neurological disorders, particularly extrapyramidal disorders and narcolepsy; and has underscored the vital link between basic research and clinical medicine.
This work was supported in part by a grant from Hennepin Faculty Associates.
Arnulf, I., Bonnet, A.-M., Damier, P., Bejjani, B.-P., Seilhean, D., Derenne, J.-P., and Agid, Y. (2000). Hallucinations, REM sleep, and Parkinson's disease: a medical hypothesis. Neurology 55:281-288.
Blatt, I., Peled, R., Gadoth, N., and Lavie, P. (1991). The value of sleep recording in evaluating somnambulism in young adults. Electroencephalogr Clin. Neurophysiol. 78:407-412.
Boeve, B. F„ Silber, M. H„ Petersen, R. C., Kokmen, E., Parisi, J. E., and Olson, E. J. (1997). REM sleep behavior disorder and degenerative dementia with or without parkinsonism: A syndrome predictive of Lewy body disease? Neurology 48:A358-A359.
Boeve, B. F., Silber, M. H., Ferman, T. J., et al. (1998). REM sleep behavior disorder and degenerative dementia: an association likely reflecting Lewy body disease. Neurology 51:363-370.
Cornelia, C. L., Tanner, C. M., and Ristanovic, R. K. (1993). Polysomnographic sleep measures in Parkinson's disease patients with treatment-induced hallucinations. Ann. Neurol. 34:710-714.
Cox, S., Risse, G., Hawkins, J., Schenck, C., and Mahowald, M. (1990). Neuropsychological data in 34 patients with REM sleep behavior disorder (RBD). Sleep Res 19:206.
Datta, S., Dossi, R. C., Pare, D., Oakson, G., and Steriade, M. (1991). Substantia nigra reticulata neurons during sleep-waking states: Relation with ponto-geniculo-occipital waves. Brain Res. 566:344-347.
D'Cruz, O'N. F., and Vaughn, B.V. (1997). Nocturnal seizures mimic REM behavior disorder. Am. J. Electroneurodiagnostic Technol. 37:258-264.
Eisensehr, I., Linke, R., Noachtar, S., Schwarz, J., Gildehaus, F. J., and Tatsch, K. (2000). Reduced striatal dopamine transporters in idiopathic rapid eye movement sleep behaviour disorder: comparison with Parkinson's disease and controls. Brain 123:1155 -1160.
Ferman, T. J., Boeve, B. F„ Smith, G. E., Silber, M. H„ Kokmen, E., Petersen, R. C., and Ivnik, R. J. (1999). REM sleep behavior disorder and dementia: cognitive differences when compared with AD. Neurology 52:951-957.
Garcia-Rill, E. (1991). The pedunculopontine nucleus. Prog. Neurobiol. 36:363-389.
Guilleminault, C., and Silvestri, R. (1982). Disorders of Arousal and Epilepsy during Sleep, In Sleep and Epilepsy, M. B. Sterman, N. M. Shouse, and P. Passouant, eds., p. 513. New York: Academic Press.
Hendricks, J. C., Morrison, A. R., and Mann, G. L. (1982). Different behaviors during paradoxical sleep without atonia depend on pontine lesion site. Brain Res. 239:81-105.
Henley, K., and Morrison, A. R. (1974). A re-evaluation of the effects of lesions of the pontine tegmentum and locus coeruleus on phenomena of paradoxical sleep in the cat. Acta Neurobiol. Exp. 34:215-232.
ICSD—International classification of sleep disorders (1990). In Diagnostic and Coding Manual. (Diagnostic Classification Steering Committee, M. J. Thorpy Chairman). Rochester, MN: American Sleep Disorders Association.
Iranzo, A., and Santamaria, J. (1998). Slow wave sleep in REM sleep behavior disorder. J. Sleep Res. 7(2): 126.
Jellinger, K. A. (1991). Pathology of Parkinson's disease: changes other than the nigrostriatal pathway. Mol. Chem. Neuropathol. 14:153-197.
Jouvet, M., and Delorme, F. (1965). Locus coeruleus et sommeil paradoxal. C. R. Soc. Biol. 159:895 - 899.
Jouvet, M., Sastre, J.-P., and Sakai, K. (1981). Toward an Etho-ethnology of Dreaming, In Psychophysiological Aspects of Sleep, I. Karacan, ed., pp. 204-214. Park Ridge, N.J.: Noyes Publishers.
Kavey, N. B., Whyte, J., Resor, S. R., and Gidro-Frank, S. (1990). Somnambulism in adults. Neurology 49:749-152.
Kavey, N. B., and Whyte, J. (1993). Somnambulism associated with hallucinations. Psychosomatics 34:86-90.
Koller, W. C. (1992). How accurately can Parkinson's disease be diagnosed? Neurology 42(1):6—16.
Kushida, C. A., Clerk, A. A., Kirsch, C. M., Hotson, J. R„ and Guilleminault, C. (1995). Prolonged confusion with nocturnal wandering arising from NREM and REM sleep: A case report. Sleep 18:757-764.
Lai, Y. Y., and Siegel, J. M. (1990). Muscle tone suppression and stepping produced by stimulation of midbrain and rostral pontine reticular formation. J. Neurosci. 10:2727-2734.
Lai, Y. Y., and Siegel, J. M. (1997). Brainstem-mediated locomotion and myoclonic jerks. I. Neural substrates. Brain Res. 745:257 - 264.
Lapierre, O., and Montplaisir, J. (1992). Polysomnographic features of REM sleep behavior disorder: Development of a scoring method. Neurology 42:1371 -1374.
Lugaresi, E., and Cirignotta, F. (1981). Hypnogenic paroxysmal dystonia: Epileptic seizure or a new syndrome? Sleep 4:129-138.
Mahowald, M. W., and Ettinger, M. G. (1990). Things that go bump in the night: The parasomnias revisited./ Clin. Neurophysiol. 7:119-143.
Mahowald, M. W„ and Schenck, C. H. (1990). REM Sleep Behavior Disorder, In Handbook of Sleep Disorders, M. J. Thorpy, ed., p. 567. New York: Marcel Dekker.
Mahowald, M. W., Bundlie, S. R., Hurwitz, T. D„ and Schenck, C. H. (1990). Sleep violence-forensic implications: Polygraphic and video documentation. J. Forensic Sci. 35:413-432.
Mahowald, M. W„ Schenck, C. H„ Rosen, G. M„ and Hurwitz, T. D. (1992). The role of a sleep disorder center in evaluating sleep violence. Arch. Neurol. 49:604- 607.
Mahowald, M. W., and Schenck, C. H. (1993). Parasomnia Purgatory: The Epileptic/Non-Epileptic Parasomnia Interface, In Non-Epileptic Seizures, A. J. Rowan and J. Gates, eds., pp. 123-139. Boston: Butterworth-Heinemann.
Mahowald, M. W., and Schenck, C. H. (1994). REM Sleep Behavior Disorder, In Principles and Practice of Sleep Medicine, 2nd ed., M. H. Kryger, T. Roth, and W. C. Dement, eds., pp. 574-588. Philadelphia: W. B. Saunders.
Mayer, G., and Meier-Ewert, K. (1993). Motor dyscontrol in sleep of narcoleptic patients (a lifelong development?). J. Sleep Res. 2:143-148.
Morrison, A. R. (1979). Brain-Stem Regulation of Behavior during Sleep and Wakefulness, In Progress in Psychobiology and Physiological Psychology, Vol. 8, J. M. Sprague and A. N. Epstein, eds., pp. 91 -131. New York: Academic Press.
Nalamalapu, U., Goldberg, R., DiPhillipo, M., and Fry, J. M. (1996). Behaviors simulating REM behavior disorder in patients with severe obstructive sleep apnea. Sleep Res. 25:311.
Negro, P. J., and Faber, R. (1996). Lewy body disease in a patient with REM sleep disorder. Neurology 46:1493-1494.
Olson, E. J., Boeve, B. F., and Silber, M. H. (2000). Rapid eye movement sleep behaviour disorder: demographic, clinical and laboratory findings in 93 cases. Brain 123:331-339.
Pareja, J. A., Caminero, A. B., Masa, J. F., and Dobato, J. L. (1996). A first case of progressive supranuclear palsy and pre-clinical REM sleep behavior disorder presenting as inhibition of speech during wakefulness and somniloquy with phasic muscle twitching during REM sleep. Neurologia 11:304-306.
Plazzi, G., Corsini, R., Provini, F., Pierangeli, G., Martinelli, P., Montagna, P., Lugaresi, E., and Cortelli, P. (1997). REM sleep behavior disorders in multiple system atrophy. Neurology 48:1094-1097.
Pompeiano, O. (1976). Mechanisms Responsible for Spinal Inhibition during Desynchronized Sleep: Experimental Study, In Advances in Sleep Research, Vol. 3, Narcolepsy. C. Guilleminault, W. C. Dement, and P. Passouant, eds., pp. 411 -449. New York: Spectrum Press.
Rye, D. B., Johnston, L. H., Watts, R. L., and Bliwise, D. L. (1999). Juvenile Parkinson's disease with REM sleep behavior disorder, sleepiness, and daytime REM onset. Neurology 53:18681870.
Sakai, K., Sastre, J.-P., and Danamori, N. (1981). State-Specific Neurons in the Ponto-Medullary Reticular Formation with Special Reference to the Postural Atonia during Paradoxical Sleep in the Cat, In Brain Mechanisms of Perceptual Awareness and Purposeful Behavior, O. Pompeiano and C. A. Marsan, eds., pp. 405-429. New York: Raven Press.
Sastre, J.-P, and Jouvet, M. (1979). Le comportement onirique du chat. Physiol. Behav. 22:979-989.
Schenck, C. H., Bundlie, S. R„ Ettinger, M. G„ and Mahowald, M. W. (1986). Chronic behavioral disorders of human REM sleep: A new category of parasomnia. Sleep 9:293-308.
Schenck, C. H., Bundlie, S. R., Patterson, A. L., and Mahowald, M. W. (1987). Rapid eye movement sleep behavior disorder: A treatable parasomnia affecting older adults. JAMA 257:1786-1789.
Schenck, C. H„ Milner, D. M„ Hurwitz, T. D., Bundlie, S. R., and Mahowald, M. W. (1989a). A polysomnographic and clinical report on sleep-related injury in 100 adult patients. Am. J. Psychiatry 146:1166-1173.
Schenck, C. H., Milner, D. M„ Hurwitz, T. D„ Bundlie, S. R„ and Mahowald, M. W. (1989b). Dissociative disorders presenting as somnambulism: Polysomnographic, video and clinical documentation (8 cases). Dissociation 2:194-204.
Schenck, C. H., and Mahowald, M. W. (1990). A polysomnographic, neurologic, psychiatric and clinical outcome report on 70 consecutive cases with REM sleep behavior disorder (RBD): Sustained clonazepam efficacy in 89.5% of 57 treated patients. Clev. Clin. J. Med. 57(Suppl.): 10-24.
Schenck, C. H., and Mahowald, M. W. (1992a). Does REM sleep behavior disorder protect against obstructive sleep apnea? Sleep Res. 21:257.
Schenck, C. H., and Mahowald, M. W. (1992b). Motor dyscontrol in narcolepsy: Rapid-eye-movement (REM) sleep without atonia and REM sleep behavior disorder. Ann. Neurol. 32:3-10.
Schenck, C. H., Hopwood, J., Duncan, E., and Mahowald, M. W. (1992). Preservation and loss of REM-atonia in human idiopathic REM sleep behavior disorder (RBD): Quantitative polysomnographic (PSG) analyses in 17 patients. Sleep Res. 21:16.
Schenck, C. H., Hurwitz, T. D., and Mahowald, M. W. (1993). REM sleep behaviour disorder: An update on a scries of 96 patients and a review of the world literature. J. Sleep Res. 2:224.
Schenck, C. H„ and Mahowald, M. W. (1996a). REM sleep parasomnias. Neurol. Clin. 14:697-720.
Schenck, C. H., and Mahowald, M. W. (1996b). Long-term, nightly benzodiazepine treatment of injurious parasomnias and other disorders of disrupted nocturnal sleep in 170 adults. Am. J. Med. 100:548-554.
Schenck, C. H., and Mahowald, M. W. (1996c). Delayed emergence of a parkinsonian disorder in 38% of 29 older males initially diagnosed with idiopathic REM sleep behavior disorder. Neurology 46:388-393.
Schenck, C. H„ Garcia-Rill, E., Skinner, R. D„ Anderson, M. L., and Mahowald, M. W. (1996a). A case of REM sleep behavior disorder with autopsy-confirmed Alzheimer's disease: post-mortem brainstem histochemical analyses. Biol. Psychiatry 40:422-425.
Schenck, C. H., Garcia-Rill, E., Segall, M„ Noreen, H., and Mahowald, M. W. (1996b). HLA class II genes associated with REM sleep behavior disorder. Ann. Neurol. 39:261-263.
Schenck, C. H., Mahowald, M. W., Anderson, M. L., Silber, M. H., Boeve, B. F., and Parisi, J. E. (1997a). Lewy body variant of Alzheimer's disease (AD) identified by postmortem ubiquitin staining in a previously reported case of AD associated with REM sleep behavior disorder. Biol. Psychiatry 42:527-528.
Schenck, C. H., Boyd, J. L., and Mahowald, M. W. (1997b). A parasomnia overlap disorder involving sleepwalking, sleep terrors, and REM sleep behavior disorder in 33 polysomnographically confirmed cases. Sleep 20:972-981.
Sforza, E., Zucconi, M., Petronelli, R., Lugaresi, E., and Cirignotta, R. (1988). REM sleep behavioral disorders. Eur. Neurol. 28:295-300.
Sforza, E., Krieger, J., and Petiau, C. (1997). REM sleep behavior disorder: clinical and physiopatho-logical findings. Sleep Med. Rev. 1:57-69.
Shouse, M. N., and Siegel, J. M. (1992). Pontine regulation of REM sleep components in cats: Integrity of the pedunculopontine tegmentum (PPT) is important for phasic events but unnecessary for atonia during REM sleep. Brain Res. 571:50-63.
Silber, M. H., and Ahlskog, J. E. (1992). REM sleep behavior disorder in Parkinsonian syndromes. Sleep Res. 21:313.
Silber, M. H„ Dexter, D. D„ Ahlskog, J. E„ Hauri, P. J., and Shepard, J. W. (1993). Abnormal REM sleep motor activity in untreated Parkinson's disease. Sleep Res. 22:274.
Tachibana, N., Yamanaka, K., and Kaji, R. (1994). Sleep bruxism as a manifestation of subclinical rapid eye movement sleep behavior disorder. Sleep 17:555.
Tachibana, N„ Kimura, K„ Kitajima, K„ Shinde, A., Kimura, J., and Shibasaki, H. (1997). REM sleep motor dysfunction in multiple system atrophy: With special emphasis on sleep talk as its early clinical manifestation. J. Neurol. Neurosurg. Psychiatry 63:678-681.
Tan, A., Salgado, M., and Fahn, S. (1996). Rapid eye movement sleep behavior disorder preceding Parkinson's disease with therapeutic response to levodopa. Mov. Disord. 11:214-216.
Thorpe, M. J. (1990). Rhythmic Movement Disorder, In Handbook of Sleep Disorders, M. J. Thorpe, ed„ pp. 609-629. New York: Marcel Dekker.
Tison, F., Wenning, G. K., Quinn, N. P., and Smith, S. J. M. (1995). REM sleep behaviour disorder as the presenting symptom of multiple system atrophy. J. Neurol. Neurosurg. Psychiatry 58:379-380.
Turner, R. S„ Chervin, R. D., Frey, K. A., Minoshima, S„ and Kuhl, D. E. (1997). Probable diffuse Lewy body disease presenting as REM sleep behavior disorder. Neurology 49:523-527.
Uchiyama, M., Isse, K., Tanaka, K., Yokota, N., Hamamoto, M., Aida, S., Ito, Y., Yoshimura, M., and Okawa, M. (1995). Incidental Lewy body disease in a patient with REM sleep behavior disorder. Neurology 45:709-712.
Whyte, J., Kavey, N. B., and Gidro-Frank, S. (1991). A self-destructive variant of jactatio capitis nocturna. J. Nerv. Ment. Dis. 179:49-50.
Wright, B. A., Rosen, J. R., Buysse, D. J., Reynolds, C. F„ and Zubenko, G. S. (1990). Shy-Drager syndrome presenting as a REM behavioral disorder. J. Geriatr. Psychiat. Neurol. 3:110-113.
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