Brainstem encephalopathy with stimulus-sensitive myoclonus leading to respiratory arrest, but with recovery: A description of two cases and review of the literature

Authors

  • Dr. D. M. Kullmann,

    Corresponding author
    1. National Hospital for Neurology and Neurosurgery and University Department of Clinical Neurology, Institute of Neurologe, London, England
    • University Department of Clinical Neurology, Institute of Neurology, Queen Square, London WC1N 3BG, U.K.
    Search for more papers by this author
  • R. S. Howard,

    1. National Hospital for Neurology and Neurosurgery and University Department of Clinical Neurology, Institute of Neurologe, London, England
    Search for more papers by this author
  • D. H. Miller,

    1. National Hospital for Neurology and Neurosurgery and University Department of Clinical Neurology, Institute of Neurologe, London, England
    Search for more papers by this author
  • N. P. Hirsh,

    1. National Hospital for Neurology and Neurosurgery and University Department of Clinical Neurology, Institute of Neurologe, London, England
    Search for more papers by this author
  • P. Brown,

    1. National Hospital for Neurology and Neurosurgery and University Department of Clinical Neurology, Institute of Neurologe, London, England
    Search for more papers by this author
  • C. D. Marsden

    1. National Hospital for Neurology and Neurosurgery and University Department of Clinical Neurology, Institute of Neurologe, London, England
    Search for more papers by this author

Abstract

Two unrelated patients developed bulbar symptoms, followed within several weeks by spontaneous myoclonus and painful, generalized, stimulus-sensitive jerks triggered by unexpected noises and cutaneous stimuli. They progressed to respiratory arrest and required mechanical ventilation, but both patients subsequently made an almost full recovery. These cases stress the importance of persevering with supportive treatment despite rapid progression of this severe generalized movement disorder. The relationship of brainstem reflex myoclonus to hyperekplexia, progressive encephalomyelities with rigidity, and the stiff-man syndrome is discussed.

Ancillary