Rheumatoid meningitis is a rare extra-articular manifestation of rheumatoid arthritis, often with non-specific symptoms. In most cases brain MRI shows a patchy lepto- and pachymeningeal enhancement, but the diagnosis currently relies on examination of a meningeal biopsy with presence of plasma cells and rheumatoid noduli. Presence of IgM rheumatic factor (RF) has been found in several cases and recently four cases have shown high titer anti-cyclic citrullinated peptide (anti-CCP) in CSF, suggesting this as a potential marker for rheumatoid meningitis. We present a 62 year-old woman with sero-positive (IgM RF and anti-CCP) rheumatoid arthritis, presenting with headache and gait impairment. Brain MRI revealed the classical patchy meningeal enhancement and the diagnosis of rheumatoid meningitis was confirmed by neuropathological examination of a meningeal biopsy. Analysis of the CSF revealed positive IgM RF (92.7 IU/mL) and strongly positive anti-CCP (19,600 IU/mL) and CXCL-13 (>500 ng/L). After treatment with high-dose steroid and Rituximab the clinical symptoms resolved. A 6 month follow-up analysis of CSF showed a dramatic decrease in all these markers with negative IgM RF and a decrease in both anti-CCP (64 IU/mL) and CXCL-13 (<10 ng/L). Our case further underlines the potential use of CSF anti-CCP and IgM RF in the diagnosis of RM and the use of these markers and CXCL-13 in evaluation of treatment response. A case review of 48 cases of rheumatoid meningitis published since 2010, including, symptoms, serum, and CSF findings, treatment, and outcome is provided.

Introduction: Isolated unilateral hypoglossal nerve palsy is an infrequent condition that usually appears in connection to an underlying disease. Rarely, the cause cannot be determined, and there is no symptom remission. Case description: We report two cases of male patients who developed persistent idiopathic isolated unilateral hypoglossal nerve palsy. The younger patient complained of involuntary movements of the tongue, while the older patient developed dysarthria. In both cases, the symptoms lasted for several weeks before the initial clinical examination, and after comprehensive clinical, laboratory, and imaging investigations, no underlying cause was detected. Both patients did not show remission of symptoms on the follow-up examinations done after several months. Discussion: Idiopathic isolated unilateral hypoglossal nerve palsy is an underreported condition, most likely due to the expected reversible nature of the disease. In the case of persistence of symptoms, extensive medical assessment is needed before postulating a diagnosis of persistent idiopathic isolated unilateral hypoglossal nerve palsy. There are no treatment guidelines for this condition, but oral steroids were reported as a treatment option. Therefore, physicians should use a systematic approach to exclude serious underlying pathology or identify a treatable condition.

Funding information and disclosures are provided at the end of the article. Full disclosure form information provided by the authors is available with the full text of this article at Neurology.org/cp.