Brainstem encephalopathy with stimulus‐sensitive myoclonus leading to respiratory arrest, but with recovery: A description of two cases and review of the literature
Identifieur interne : 005688 ( Main/Exploration ); précédent : 005687; suivant : 005689Brainstem encephalopathy with stimulus‐sensitive myoclonus leading to respiratory arrest, but with recovery: A description of two cases and review of the literature
Auteurs : Kullmann [Royaume-Uni] ; R. S. Howard [Royaume-Uni] ; D. H. Miller [Royaume-Uni] ; N. P. Hirsh [Royaume-Uni] ; P. Brown [Royaume-Uni] ; C. D. Marsden [Royaume-Uni]Source :
- Movement Disorders [ 0885-3185 ] ; 1996-11.
English descriptors
- KwdEn :
- Adult, Arousal (physiology), Brain Stem (physiopathology), Brainstem, Encephalomyelitis (physiopathology), Follow-Up Studies, Humans, Hyperekplexia, Male, Myoclonus, Myoclonus (physiopathology), Reflex, Abnormal (physiology), Reflex, Startle (physiology), Respiration, Artificial, Respiratory Insufficiency (physiopathology), Respiratory arrest, Startle.
- MESH :
- physiology : Arousal, Reflex, Abnormal, Reflex, Startle.
- physiopathology : Brain Stem, Encephalomyelitis, Myoclonus, Respiratory Insufficiency.
- Adult, Follow-Up Studies, Humans, Male, Respiration, Artificial.
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.
Url:
DOI: 10.1002/mds.870110618
Affiliations:
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S." last="Howard">R. S. Howard</name><affiliation wicri:level="2"><country>Royaume-Uni</country><placeName><region type="country">Angleterre</region></placeName><wicri:cityArea>National Hospital for Neurology and Neurosurgery and University Department of Clinical Neurology, Institute of Neurologe, London</wicri:cityArea></affiliation></author><author><name sortKey="Miller, D H" sort="Miller, D H" uniqKey="Miller D" first="D. H." last="Miller">D. H. Miller</name><affiliation wicri:level="2"><country>Royaume-Uni</country><placeName><region type="country">Angleterre</region></placeName><wicri:cityArea>National Hospital for Neurology and Neurosurgery and University Department of Clinical Neurology, Institute of Neurologe, London</wicri:cityArea></affiliation></author><author><name sortKey="Hirsh, N P" sort="Hirsh, N P" uniqKey="Hirsh N" first="N. P." last="Hirsh">N. P. Hirsh</name><affiliation wicri:level="2"><country>Royaume-Uni</country><placeName><region type="country">Angleterre</region></placeName><wicri:cityArea>National Hospital for Neurology and Neurosurgery and University Department of Clinical Neurology, Institute of Neurologe, London</wicri:cityArea></affiliation></author><author><name sortKey="Brown, P" sort="Brown, P" uniqKey="Brown P" first="P." last="Brown">P. Brown</name><affiliation wicri:level="2"><country>Royaume-Uni</country><placeName><region type="country">Angleterre</region></placeName><wicri:cityArea>National Hospital for Neurology and Neurosurgery and University Department of Clinical Neurology, Institute of Neurologe, London</wicri:cityArea></affiliation></author><author><name sortKey="Marsden, C D" sort="Marsden, C D" uniqKey="Marsden C" first="C. D." last="Marsden">C. D. Marsden</name><affiliation wicri:level="2"><country>Royaume-Uni</country><placeName><region type="country">Angleterre</region></placeName><wicri:cityArea>National Hospital for Neurology and Neurosurgery and University Department of Clinical Neurology, Institute of Neurologe, London</wicri:cityArea></affiliation></author></analytic><monogr></monogr><series><title level="j">Movement Disorders</title><title level="j" type="sub">Official Journal of the Movement Disorder Society</title><title level="j" type="abbrev">Mov. 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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.</div></front></TEI><affiliations><list><country><li>Royaume-Uni</li></country><region><li>Angleterre</li></region></list><tree><country name="Royaume-Uni"><region name="Angleterre"><name sortKey="Kullmann" sort="Kullmann" uniqKey="Kullmann" last="Kullmann">Kullmann</name></region><name sortKey="Brown, P" sort="Brown, P" uniqKey="Brown P" first="P." last="Brown">P. Brown</name><name sortKey="Hirsh, N P" sort="Hirsh, N P" uniqKey="Hirsh N" first="N. P." last="Hirsh">N. P. Hirsh</name><name sortKey="Howard, R S" sort="Howard, R S" uniqKey="Howard R" first="R. S." last="Howard">R. S. Howard</name><name sortKey="Marsden, C D" sort="Marsden, C D" uniqKey="Marsden C" first="C. D." last="Marsden">C. D. Marsden</name><name sortKey="Miller, D H" sort="Miller, D H" uniqKey="Miller D" first="D. H." last="Miller">D. H. Miller</name></country></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
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