Abstract
A quantitative study of neurons in the dorsal raphe nucleus (DRN) and the superior central nucleus (SCN) was performed in seven patients with myotonic dystrophy (MyD), five of whom showed hypersomnia, and in eight age-matched controls. The densities of neurons in the DRN and the SCN were significantly lower in MyD patients with hypersomnia than in MyD patients without hypersomnia and control subjects. There was an appreciable positive correlation in the density of neurons between the DRN and the SCN in all MyD patients. These data suggest that the neuronal loss of the DRN and the SCN is associated with the presence of hypersomnia in MyD.
This is a preview of subscription content, log in via an institution to check access.
Similar content being viewed by others
References
Azmitia EC, Segal M (1978) An autoradiographic analysis of the differential ascending projections of the dorsal and median raphe nuclei in the rat. J Comp Neurol 179: 641–667
Baker KG, Halliday GM, Halasz P, Hornung JP, Geffen LB, Cotton RG, Törk I (1990) Cytoarchitecture of serotonin-synthesizing neurons in the pontine tegmentum of the human brain. Synapse 7: 301–320
Coccagna G, Mantovani M, Parchi C, Mironi F, Lugaresi E (1975) Alveolar hypoventilation and hypersomnia in myotonic dystrophy. J Neurol Neurosurg Psychiatry 38: 977–984
Ebinger G, Bruyland M, Martin JJ, Herregodts P, Cras P, Michotte Y, Gommé L (1987) Distribution of biogenic amines and their catabolites in bains from patients with Alzheimer's disease. J Neurol Sci 77: 267–283
Halliday GM, McCann HL, Pamphelett R, Brooks WS, Creasey H, McCusker E, Cotton RGH, Broe GA, Harper CG (1992) Brain stem serotonin-synthesizing neurons in Alzheimer's disease: a clinicopathological correlation. Acta Neuropathol 84: 638–650
Hansotia P, Frens D (1981) Hypersomnia associated with alveolar hypoventilation in myotonic dystrophy. Neurology 31: 1336–1337
Harper PS (1970) Myotonic dystrophy. Saunders, Philadelphia
Jouvet M (1972) The role of monoamines and acetylcholinecontaining neurons in the regulation of sleep-waking cycle. Ergeb Physiol 64: 166–307
Kitamura J, Kuboki Y, Tsuruta T, Kurihara T, Matsukawa S (1989) A new family with Joseph disease in Japan. Homovanillic acid, magnetic resonance, and sleep apnea studies. Arch Neurol 46: 425–428
Kohon NN, Faires JS, Rodman T (1964) Unusual manifestations due to involvement of involuntary mucsle in dystrophia myotonica. N Engl J Med 271: 1179–1183
Mayeux R, Stern Y, Cote L, Williams JB (1984) Altered serotonin metabolism in depressed patients with Parkinson's disease. Neurology 34: 642–646
Moore RY, Halaris AE, Jones BE (1978) Serotonin neurons of the midbrain raphe: ascending projections. J Comp Neurol 180: 417–438
Olszewski J, Baxter D (1982) Cytoarchitecture of the human brain stem, 2nd edn. Karger, Basel
Phemister JC, Small JM (1961) Hypersomnia in dystrophia myotonica. J Neurol Neurosurg Psychiatry 24: 173–175
Tabaton M, Schenone A, Romagnoli P, Mancardi GL (1985) A quantitative and ultrastructural study of substantia nigra and nucleus centralis superior in Alzheimer's disease. Acta Neuropathol (Berl) 58: 218–223
Ungerstedt U (1971) Stereotaxic mapping of the monoamine pathways in the rat brain. Acta Physiol Scand [Suppl] 367: 1–48
Van der Meché FGA, Boogaard JM (1987) Central sleepiness in myotonic dystrophy. J Neurol [Suppl] 235: 66
Van der Meché FGA, Boogaard JM, Van der Berg B (1986) Treatment of hypersomnolence in myotonic dystrophy with a CNS stimulant. Muscle Nerve 9: 341–344
Van Hilten JJ, Kerkhof GA, van Dijk JG, Dunnewold R, Wintzen AR (1993) Distribution of sleep-wake rhythmicity and daytime sleepiness in myotonic dystrophy. J Neurol Sci 114: 68–75
Wiklund L, Léger L, Persson M (1981) Monoamine cell distribution in the cat brain stem. A fluorescence histochemical study with quantification of indolaminergic and locus coeruleus cell group. J Comp Neurol 203: 613–647
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Ono, S., Kanda, F., Takahashi, K. et al. Neuronal cell loss in the dorsal raphe nucleus and the superior central nucleus in myotonic dystrophy: a clinicopathological correlation. Acta Neuropathol 89, 122–125 (1995). https://doi.org/10.1007/BF00296355
Received:
Revised:
Accepted:
Issue Date:
DOI: https://doi.org/10.1007/BF00296355