PT  - JOURNAL ARTICLE
AU  - Amit KJ Mandal
AU  - Angad Ryatt
AU  - Kim O'Hare
AU  - Constantinos G Missouris
TI  - Lessons of the month: HLA-B27-associated syndrome and spontaneous intracranial hypotension resulting in behavioural variant frontotemporal dementia
AID  - 10.7861/clinmed.2020-0062
DP  - 2020 May 01
TA  - Clinical Medicine
PG  - e10--e11
VI  - 20
IP  - 3
4099  - http://www.rcpjournals.org/content/20/3/e10.short
4100  - http://www.rcpjournals.org/content/20/3/e10.full
SO  - Clin Med2020 May 01; 20
AB  - Spontaneous 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.