Symptomatic, radiological and pathological involvement of the hypothalamus in neuromyelitis optica
- 1Department of Clinical Neurology, John Radcliffe Hospital, Oxford, UK
- 2Neuroscience Group, Weatherall Institute of Molecular Medicine, John Radcliffe Hospital, Oxford, UK
- 3Department of Clinical Neuropathology, John Radcliffe Hospital, Oxford, UK
- 4Department of Clinical Neuroradiology, John Radcliffe Hospital, Oxford, UK
- Dr J Palace, University Department of Clinical Neurology, John Radcliffe Hospital, Headington, Oxford OX2 6HE, UK; jacqueline.palace{at}clneuro.ox.ac.uk
- Received 16 July 2008
- Revised 22 September 2008
- Accepted 7 November 2008
Abstract
This study describes a young girl who presented with involuntary weight loss, spontaneous vomiting and behavioural change. Imaging confirmed hypothalamic and brainstem involvement. Routine investigations (including cerebrospinal fluid analysis and neuromyelitis optica IgG) were unhelpful. Biopsy of the hypothalamic lesion implicated an aggressive inflammatory aetiology. There was a response to conventional immunosuppression, while a further relapse responded to plasma exchange. She died 21 months after presentation. Postmortem examination was highly suggestive of neuromyelitis optica, which was subsequently confirmed following the identification of aquaporin 4 antibodies.
Footnotes
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Funding: SV is funded by a Patrick Berthoud Clinical Fellowship. PW is supported by Oxford Biomedical Research.
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Competing interests: AV and her department receive royalties and payments for aquaporin 4 antibodies.
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Patient consent: Obtained from the parents.
