Möbius-Syndrom und Narkolepsie View Full Text


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Article Info

DATE

2014-12

AUTHORS

S. Krämer, U. Goldammer, E. Sindern

ABSTRACT

BACKGROUND: Moebius syndrome is a rare neurological disease that has a frequent association with parasomnia. CASE REPORT: We report on a patient with Moebius syndrome and the clinical presentation of a narcolepsy cataplexy syndrome. With the hypoplasia of the brainstem in the cranial magnetic resonance imaging, we were able to show the morphological correlate of Moebius syndrome. Comorbidity was detected by cognitive tests, polysomnography and detection of hypocretin in the cerebrospinal fluid. Despite normal sleep onset latency and only one episode of sleep onset rapid eye movement (REM) in the multiple sleep latency test, where expressiveness is significantly reduced in cases of paralysis of horizontal eye movement, the diagnosis of parasomnia with narcolepsy cataplexy symptoms could be made. DISCUSSION: The hypocretin level of 132 pg/ml measured in the cerebro spinal fluid is compatible with this diagnosis and shows the relevance of a detailed diagnostic of parasomnia in patients with Moebius syndrome. More... »

PAGES

1569-1572

References to SciGraph publications

  • 2004-09. Moebius syndrome, narcolepsy and parasomnias: Report on two patients in SOMNOLOGIE
  • 2005-02. Cognitive evaluation in adult patients with Möbius syndrome in JOURNAL OF NEUROLOGY
  • 1979-01. Möbius syndrome in ACTA NEUROPATHOLOGICA
  • 2003-01. Cataplexy in association with Moebius syndrome in JOURNAL OF NEUROLOGY
  • Identifiers

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    http://scigraph.springernature.com/pub.10.1007/s00115-014-4174-8

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    PUBMED

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    51 schema:description BACKGROUND: Moebius syndrome is a rare neurological disease that has a frequent association with parasomnia. CASE REPORT: We report on a patient with Moebius syndrome and the clinical presentation of a narcolepsy cataplexy syndrome. With the hypoplasia of the brainstem in the cranial magnetic resonance imaging, we were able to show the morphological correlate of Moebius syndrome. Comorbidity was detected by cognitive tests, polysomnography and detection of hypocretin in the cerebrospinal fluid. Despite normal sleep onset latency and only one episode of sleep onset rapid eye movement (REM) in the multiple sleep latency test, where expressiveness is significantly reduced in cases of paralysis of horizontal eye movement, the diagnosis of parasomnia with narcolepsy cataplexy symptoms could be made. DISCUSSION: The hypocretin level of 132 pg/ml measured in the cerebro spinal fluid is compatible with this diagnosis and shows the relevance of a detailed diagnostic of parasomnia in patients with Moebius syndrome.
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