Central hypoventilation as the presenting symptom in Hu associated paraneoplastic encephalomyelitis

Central hypoventilation is usually caused by ischaemic or neoplastic lesions of the medulla and upper cervical spinal cord. An autoimmune disorder is not usually considered in the differential diagnosis of this syndrome. We retrospectively identified 14 patients from our database of 202 patients wit...

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Bibliographic Details
Published in:Journal of neurology, neurosurgery and psychiatry Vol. 78; no. 10; pp. 1143 - 1145
Main Authors: Gómez-Choco, Manuel J, Zarranz, Juan J, Saiz, Albert, Forcadas, María I, Graus, Francesc
Format: Journal Article
Language:English
Published: London BMJ Publishing Group Ltd 01-10-2007
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Summary:Central hypoventilation is usually caused by ischaemic or neoplastic lesions of the medulla and upper cervical spinal cord. An autoimmune disorder is not usually considered in the differential diagnosis of this syndrome. We retrospectively identified 14 patients from our database of 202 patients with Hu antibodies who presented with brainstem symptoms. Three were admitted to hospital because of central hypoventilation. All underwent intubation and mechanical ventilation. They could breathe properly while they were awake but suffered deep apnoeas during sleep. Two died, but one is still alive requiring ventilatory assistance during sleep. Autopsy was performed in one of the patients which showed severe inflammatory infiltrates and neuronal loss in the medulla. All patients had normal brain imaging studies and the cause of central hypoventilation was an unsolved problem until Hu antibodies were determined.
Bibliography:PMID:17878194
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local:jnnp;78/10/1143
ArticleID:jn117994
href:jnnp-78-1143.pdf
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ISSN:0022-3050
1468-330X
DOI:10.1136/jnnp.2007.117994