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NEUROLOGY 1996;46:228-231
© 1996 American Academy of Neurology

Neuronal loss in the medullary reticular formation in myotonic dystrophy

A clinicopathological study

S. Ono, MD, F. Kanda, MD, K. Takahashi, MD, Y. Fukuoka, MD, K. Jinnai, MD, H. Kurisaki, MD, S. Mitake, MD, T. Inagaki, MD and K. Nagao, MD

From the Departments of Neurology (Dr. Ono) and Surgical Pathology (Dr. Nagao), Teikyo University School of Medicine, Ichihara Hospital, Ichihara; the Department of Neurology (Drs. Kanda, Takahashi, Fukuoka, and Jinnai), National Sanatorium Hyogo Chuo Hospital, Sanda; the Department of Neurology (Dr. Kurisaki), Tokyo National Hospital, Kiyose; and the Department of Neurology (Drs. Mitake and Inagaki), Nagoyashi-Kosein Geriatric Hospital, Nagoya, Japan.
Received January 5, 1995. Accepted in final form April 25, 1995.
Address correspondence and reprint request to Dr. S. Ono, Department of Neurology, Teikyo University School of Medicine, Ichihara Hospital, 3426-3, Anesaki, Ichihara City, Chiba 299-01, Japan.

Article abstract-Respiratory insufficiency occurs frequently in patients with myotonic dystrophy (MyD). We have performed a quantitative study of neurons linked to respiratory function in the dorsal central medullary nucleus (DCMN), the ventral central medullary nucleus (VCMN), and the subtrigeminal medullary nucleus (SMN) in seven patients with MyD and eight age-matched controls. Alveolar hypoventilation of the central type occurred in three of the MyD patients but not in the remaining MyD patients or controls. The densities of neurons of the DCMN, the VCMN, and the SMN in MyD patients with hypoventilation were significantly lower than in MyD without hypoventilation and controls. These data suggest that the neuronal loss of the DCMN, VCMN, and SMN is associated with the presence of hypoventilation in MyD and may be an important feature of MyD.

NEUROLOGY 1996;46: 228-231




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