PT - JOURNAL ARTICLE AU - Maja Christensen AU - Mathew Wallis AU - Peter Jessup AU - Irène Lemelle AU - Dean L Jones TI - Cryopyrin-associated periodic syndrome: a treatable genetic inflammatory condition AID - 10.1136/practneurol-2021-002992 DP - 2021 Jun 09 TA - Practical Neurology PG - practneurol-2021-002992 4099 - http://pn.bmj.com/content/early/2021/06/09/practneurol-2021-002992.short 4100 - http://pn.bmj.com/content/early/2021/06/09/practneurol-2021-002992.full AB - A 20-year-old man presented with recurrent subdural haemorrhages on a background of progressive sensorineural hearing loss, juvenile idiopathic arthritis and intracranial hypertension of unknown cause. His mother had a similar previous history. They both had a persistently mildly elevated serum C reactive protein. Repeat lumbar punctures identified persistently elevated intracranial pressure and mild pleocytosis. A dural biopsy showed necrotising pachymeningitis with granulomatous vasculitis. The underlying cause in both patients was a cryopyrin-associated periodic syndrome. We discuss its varied phenotype and how clinicians need to be aware of this treatable genetic condition to facilitate early treatment and to prevent accumulation of disability.