Abstract

A 41-year-old man was admitted to hospital with a 1-week history of fever, headache and vomiting. He was known to have Evans’ syndrome (immune thrombocytopenia with autoimmune haemolytic anaemia), which had been treated by splenectomy 3 years prior to presentation. He had declined the relevant vaccinations. On arrival in the Emergency Department, he complained of headache, was confused and non-compliant with examination. He had neck stiffness, but was apyrexial and cardiovascularly stable. His Glasgow Coma Scale (GCS) was 13 (Eyes 3, Motor 6, Verbal 4), but there were no localizing signs. Inflammatory markers were elevated [C-reactive protein (CRP) 367 mg/l] and neutrophilia was detected [white cell count (WCC) 27 × 109/l, neutrophils 25 × 109/l]. Platelets were 51 × 109/l consistent with Evans’ syndrome. A computed tomography (CT) head scan revealed moderate hydrocephalous, but no other abnormality. The cerebrospinal fluid (CSF) was turbid and the protein concentration was grossly elevated (17 g/l). CSF glucose was undetectable (<0.1 mmol/l). CSF WCC could not be performed as the sample clotted, but Streptococcus pneumoniae urine antigen was positive and subsequently both blood and CSF cultures grew S. pneumoniae . Treatment was commenced with intravenous ceftriaxone (2 g bis die) at presentation and he received a 4-day course of dexamethasone [10 mg quater die sumendus (qds)]. Clinical improvement was noted; by the time steroids were withdrawn, there was mild …

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