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Brain, Vol. 110, No. 4, 1061-1079, 1987
© 1987 Guarantors of Brain

research-article

THE SLOW CHANNEL SYNDROME

TWO NEW CASES

H. J. G. H. OOSTERHUIS1, J. NEWSOM-DAVIS2,6, J. H. J. WOKKE3, P. C. MOLENAAR4, T. V. WEERDEN1, B. S. OEN4, F. G. I. JENNEKENS3, H. VELDMAN3, A. VINCENT2, D. W. WRAY5, C. PRIOR5 and N. M. F. MURRAY6

1Department of Neurology, Academic Hospital Groningen, The Netherlands 2Department of Neurological Science, Royal Free Hospital School of Medicine London 3Laboratory for Neuromuscular Diseases, Department of Neurology Academic Hospital, Utrecht 4Pharmacological Laboratory, Medical Faculty University of Leiden London 5Department of Pharmacology, Royal FreeHospital School of Medicine London 6Institute of Neurology, National Hospital Queen Square, London

Correspondence to: Correspondence to: Dr H. J. G. H. Oosterhuis, Department of Neurology, Academic Hospital, Postbus 31.001, RB 9700 Groningen, The Netherlands.

Two patients are described with a myasthenic syndrome that presented in early adult life. One patient had 2 asymptomatic first degree relatives with similar electrophysiological findings. Both patients had abnormal fatiguability, arm weakness being prominent; neither of them responded to anti-cholinesterase medication. An abnormal decrement at 3 Hz stimulation was present, and a single stimulus evoked a repetitive response. Electrophysiological studies on biopsied intercostal muscle showed miniature endplate potentials of normal amplitudes but with prolonged rise and decay times. Anticholinesterase staining (Case 1) was not reduced, and showed elongation of some endplates. Ultrastructural studies (Case 2) showed degeneration of junctional folds and diffusely thickened endplate basal lamina. Calcium deposits were not observed and myopathic changes were slight. The findings are consistent with a prolonged open time of the ACh-induced ion channel.

Received July 8, 1986. Revised November 6, 1986. Accepted November 20, 1986.


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