A fatal case of ‘primary’ headache

We describe a case of a 33 year old caucasian male with a subacute history of headaches presenting to the medical team with pyrexia, confusion and hallucinations. He subsequently became disorientated, agitated and initially needed sedation and ventilation after becoming acutely hypoxic with autonomic instability. He was subsequently found to have dense, pre–geniculate blindness and generalised arreflexia.
Magnetic resonance imaging demonstrated normal brain parenchyma but diffuse nodular thickening and enhancement of the basal leptomeninges in the brain and throughout the entire spinal cord. Lumbar puncture revealed markedly xanthochromic CSF with opening pressures that were persistently raised above 40 cm CSF. CSF protein was elevated at 8–9 g/l; the CSF white cell count …