Cookies on this website
We use cookies to ensure that we give you the best experience on our website. If you click 'Continue' we'll assume that you are happy to receive all cookies and you won't see this message again. Click 'Find out more' for information on how to change your cookie settings.

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 encephalomyelitis with rigidity, and the stiff-man syndrome is discussed.

Original publication

DOI

10.1002/mds.870110618

Type

Journal article

Journal

Mov Disord

Publication Date

11/1996

Volume

11

Pages

715 - 718

Keywords

Adult, Arousal, Brain Stem, Encephalomyelitis, Follow-Up Studies, Humans, Male, Myoclonus, Reflex, Abnormal, Reflex, Startle, Respiration, Artificial, Respiratory Insufficiency