• Clin Med (Lond) · May 2020

    Case Reports

    Lessons of the month: HLA-B27-associated syndrome and spontaneous intracranial hypotension resulting in behavioural variant frontotemporal dementia.

    • Amit Kj Mandal, Angad Ryatt, Kim O'Hare, and Constantinos G Missouris.
    • Wexham Park Hospital, Slough, UK.
    • Clin Med (Lond). 2020 May 1; 20 (3): e10e11e10-e11.

    AbstractSpontaneous intracranial hypotension is uncommon and results from a cerebrospinal fluid (CSF) leak. We describe the case of a marathon runner who presented with postural headache attributable to CSF venous fistulation originating from a lower thoracic nerve root cyst. Subsequent investigations confirmed a unifying de novo diagnosis of human leukocyte antigen B27-associated syndrome. With unmitigated CSF loss over the following 3 months, the patient became bedbound and developed rapidly progressive behavioural variant frontotemporal dementia. Behavioural changes were somewhat reversible on restoration of CSF volume after surgical intervention.© Royal College of Physicians 2020. All rights reserved.

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