Case report

A 52-year-old Caucasian man, previously healthy except for a sickle cell trait, developed severe night sweats followed 4 months later by priapism and then, over 1 week, a gradual painless binocular reduction in vision. His visual acuity was 0.98 (right) and 0.9 (left) logMAR. There was no vitreous activity and retinal examination was normal. Fluorescein angiography showed leakage from vasculature in the superficial choroid but vessel calibre was normal. Optical coherence tomography showed multiple serous retinal detachments. Inflammatory causes were high on the list of ophthalmological differential diagnoses, and he was treated with 3 days of 500 mg intravenous methylprednisolone followed by oral prednisolone 40 mg.

Two weeks later, he developed increasing low back pain with radiation to his thighs, followed by numbness of his perianal region, buttocks and posterior thighs, with urinary retention and faecal incontinence. Over the next 3 days he developed progressively ascending bilateral leg weakness. His pain became extremely difficult to control.

On examination, there were signs of a cauda equina syndrome. The weakness initially affected the muscles supplied by L5–S1 roots, with sensory disturbance in an S2–S5 distribution, but this rapidly progressed to a complete paraplegia with absent sensation to all modalities from L2 downwards. His legs were flaccid and areflexic. He had a swinging temperature up to 39°C.

MRI of the spine showed thickening of the lumbosacral nerve roots, which enhanced with contrast (figure 1). He had raised inflammatory markers (erythrocyte sedimentation rate 46 mm per first hour (<15) and serum C reactive protein of 100–120 mg/L (<10)), a mild microcytic anaemia and panhypopituitarism. His serum lactate dehydrogenase was markedly elevated at 3047 IU/L (266–500 IU/L). Autoantibodies, including perinuclear anti-neutrophil cytoplasmic antibody (pANCA), were negative. Repeated cerebrospinal fluid (CSF) examination showed a lymphocytosis of 27–32 white cells/µL (≤5), a markedly raised protein at 4.0–7.5 g/L (0.15–0.45), and a low glucose at 0.6−1.9mg/L. Flow cytometry and cytology …