Final Diagnosis -- Rheumatoid Meningitis (RM)
DIAGNOSIS
Rheumatoid meningitis (RM).
DISCUSSION
Rheumatoid arthritis (RA) is a chronic multisystem disease with many extra-articular manifestations. Typical neurologic sequelae of RA are usually secondary to musculoskeletal involvement. Atypical sequelae directly involving the central nervous system include parenchymal and meningeal vasculitis, rheumatoid nodules, and meningitis3. From these complications, RM is therefore a rare but one of the most severe, with only 56 cases described in the literature between 1954 and 2014 (Supplementary Data). Various symptoms are seen in RM: altered mental status, cranial neuropathies, hemiparesis, gait imbalance, memory loss, seizures and headaches3,6. Diagnosis of RM is therefore difficult. CSF analysis is generally characterized by non-specific findings with mild pleiocytosis and an elevated protein level5. Our case matches this description, with protein level at 59 mg/dl and presence of numerous lymphocytes and plasmocytes. Some authors report hypoglycorrhachia as a characteristic of RM3,9, but on the basis of a literature overview, the level of glucose is variable. Introduction of MRI examination of the brain has resulted in enhanced early diagnosis of RM, and significantly improved the prognosis of these patients. Because use of immunosuppressive therapy is the gold standard in RA, a biopsy is required to exclude infectious causes of chronic meningitis7. Pathological findings of RM are characterized by vasculitis, rheumatoid nodules and infiltration of mononuclear cells such as lymphocytes and plasma cells on leptomeninges4. All these criteria are met in our case. Differential diagnosis of granulomatous meningitis are infectious causes (bacterial as tuberculosis, fungal, parasites or viral as VZV) or non infectious causes such as sarcoidosis, systemic lupus erythematosus, neoplastic meningitis, granulomatous angiitis, idiopathic chronic pachymeningitis or Wegener's disease. To exclude infectious causes, special stains such as PAS, Ziehl-Neelsen, Grocott and Giemsa are required on biopsy material or centrifuged CSF sediment 1,2,8.
In conclusion, our case illustrates that RM is a difficult diagnosis, often established by exclusion on biopsy material. The diagnosis of RM should be considered during the differential diagnosis stage in patient with chronic meningitis.
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Contributed by Caroline Koopmansch, Nicky D’Haene, Delfyne Hastir, Niloufar Sadeghi, Calliope Maris, Isabelle Salmon