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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.

Original publication

DOI

10.1136/jnnp.2008.157693

Type

Journal article

Journal

J Neurol Neurosurg Psychiatry

Publication Date

06/2009

Volume

80

Pages

679 - 682

Keywords

Adolescent, Aquaporin 4, Autoantibodies, Biopsy, Brain Stem, Diagnosis, Differential, Disease Progression, Fatal Outcome, Female, Humans, Hypothalamic Diseases, Hypothalamus, Magnetic Resonance Imaging, Necrosis, Neurologic Examination, Neuromyelitis Optica, Optic Nerve, Spinal Cord, Third Ventricle