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Papers of the Week

2020 May

Clin Med (Lond)



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


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 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.