An unusual case of inflammatory meningitis in a young man with systemic lupus erythematosus

Our patient, a 24-year-old man, initially presented with inflammatory arthritis and Raynaud’s, and was found to be anti-ribonucleoprotein antibody (anti-RNP) and anti-nuclear antibody positive. He responded well to methotrexate and sulfasalazine. After two years of stability, he developed fevers, lymphadenopathy and synovitis and was treated with 30 mg prednisolone daily. Within two weeks he presented with episodes of dysarthria, dysphasia and unilateral upper limb weakness. Each episode lasted up to 20 minutes with speech disturbance followed by sensory symptoms in the form of spreading paraesthesia affecting the left arm. There was no associated headache. The patient would make a complete recovery with no residual deficit. Magnetic resonance imaging (MRI) including standard T2, diffusion weighted, perfusion weighted, post-contrast T1 weighted imaging and magnetic resonance angiography (MRA) of the brain were all normal. Cerebrospinal fluid (CSF) analysis showed a protein of 0.97 g/litre. Serology showed evolution into a classical systemic lupus erythematosus picture with positive anti-double stranded DNA antibodies, crithidia, anti-RNP and anti-Smith antibodies, and low complement. Mycophenolate mofetil (MMF) replaced methotrexate and sulfasalazine and 30 mg prednisolone daily was continued.

One month later, our patient presented with dysphasia, right-sided hemiparesis, facial weakness, confusion and episodes of vomiting. Temperature was 40℃ and Glasgow Coma Scale 11. Cefotaxime and aciclovir were started empirically. MMF was discontinued. MRI brain showed extensive diffuse dural and leptomeningeal enhancement, with normal T2 and diffusion weighted imaging, compatible with a meningitic process (Figures 1 and 2). CSF analysis showed a white cell count of 70 × 10⁶/litre with 80% lymphocytes and 10% polymorphs, and a glucose level of 2.9. Of note was the protein level of 5.39 g/litre. After two days there was clinical and radiological improvement; repeat MRI showed regression of the leptomeningeal enhancement.

OPEN IN VIEWER

Figure 1

Normal T2 magnetic resonance imaging showing extensive diffuse dural and leptomeningeal enhancement.

OPEN IN VIEWER

Figure 2

Diffusion weighted magnetic resonance imaging showing extensive dural and leptomeningeal enhancement.

On day seven of antimicrobials, our patient deteriorated with left-sided weakness, left sensory inattention and a left homonymous hemianopia. He had a witnessed tonic-clonic seizure. Temperature was 38.1℃. MRI and MRA brain showed no abnormalities. CSF analysis again showed a high protein at 5.05 g/litre. Treatment with 100 mg intravenous methylprednisolone and 1 g levetiracetam twice daily was commenced, and MMF restarted. There was a prompt clinical improvement. After nine days, the methylprednisolone was switched to 60 mg prednisolone daily. The following day our patient developed expressive dysphasia and a right inferior quadrantonopia. Bloods were indicative of macrophage activation syndrome. Methylprednisolone was recommenced at 1 g daily, and intravenous immunoglobulins at 1 g/kilogram were administered over two days. The patient improved, and the improvement was sustained on switching back to 60 mg prednisolone.

The CSF picture was not typical for bacterial or tuberculous meningitis, and there was no evidence of infective organisms in the CSF. Histological examination showed lymphocytic pleocytosis suggestive of a chronic inflammatory cell response. A final diagnosis of inflammatory meningitis related to SLE was made. We presume the episodes of focal neurological deficit at presentation were due to simple partial seizures, although the spreading sensory disturbance was more suggestive of a migrainous phenomenon. Since discharge the patient has had rituximab, is off steroids and is neurologically intact.

The American College of Rheumatology has described 19 neuropsychiatric manifestations of SLE; ¹ aseptic meningitis is one of the least common. Ten cases are reported in the literature between 1966 and 2000, ² most attributed to drugs and only a minority directly to SLE. ³ In one case series ² the CSF results varied greatly with some showing increased white cell count, and protein levels ranging from 0.21 to 2.93 g/l. Our case is unique because although some features were in keeping with aseptic meningitis, the MRI appearances were suggestive of a pachymeningitis, and the level of CSF protein (above 5 g/l), was exceptionally high. In conclusion we describe unusual neurological manifestations of SLE characterised by pachymeningitis, aseptic meningitis and encephalitis (seizures, focal neurological deficit) with grossly elevated CSF protein, responding to immunosuppression.