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Rheumatoid meningitis (RM) is a rare extra-articular manifestation of rheumatoid arthritis (RA) that has been increasingly recognized by neurologists. However, the diversity of its clinical manifestations makes its diagnosis difficult. RM does not have a unified diagnostic standard, and its link with RA needs to be studied further. Here we report two cases of RM without a history of RA. The first patient, an 80-year-old woman, presented with sudden unilateral limb weakness, with brain MR showing abnormal signals in the leptomeningeal of the right frontal parietal. Subarachnoid hemorrhage was excluded after imaging examination, and infectious meningitis was ruled out after cerebrospinal fluid (CSF) examination. The patient was diagnosed as having RM, she had increased levels of CCP and AKA, the markers of RA, but no history of the disease or other clinical manifestations of it. Another case, a 65-year-old man, was hospitalized with Bell's palsy. We found that he had intracranial imaging changes highly consistent with those characteristic of RM during his routine examination. Except for the left peripheral facial palsy, the patient had no other neurological signs or symptoms and no RA history. After a careful physical examination, we found no joint or other manifestations or serological abnormalities consistent with RA (RF, CCP, AKA, etc.). However, after excluding infection meningitis and considering the patient's unique imaging results, we diagnosed him as having RM. We report these two cases as references for clinical diagnosis and treatment of RM, providing a discussion of our rationale.

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INTRODUCTION: Rheumatoid meningitis (RM) is an extremely rare extra-articular complication of rheumatoid arthritis (RA), with approximately 165 cases reported world-wide. RM exhibits a broad range of symptoms, with stroke-like episodes and seizures being the most common manifestations. The primary differential diagnoses include vascular and infectious diseases. The influence of immunomodulatory medications on the pathophysiology of RM remains unclear. There are no consensus guidelines on therapeutic regimen. METHODS: We present four patients with prior history of RA that developed different neurological syndromes in correlation to radiological leptomeningitis. Clinical presentations, comorbid conditions, supplementary diagnostic assessments, treatments, and prognosis are provided. A literature review of recent immunosuppressive management in RM patients was performed. RESULTS: Three patients presented to hospital with recurrent focal seizures. Only two suffered meningism, reporting headache and fever. Magnetic resonance imaging (MRI) showed different grades of leptomeningitis across all cases. Notably, three cases demonstrated bilateral involvement extending to the pachymeninges. Two patients exhibited pronounced CSF mononuclear inflammation while extended microbiological evaluations yielded negative results. Two patients required biopsy for confirmation. The initiation of immunosuppressive therapy marked a turning point for three patients who previously exhibited progressive deterioration. Mortality was absent in all cases. CONCLUSIONS: Our experience remarks the elusive nature of RM. Rigorous exclusionary diagnostics are imperative to differentiate RM from mimicking conditions. Clinical manifestations oscillate between transient episodes and progressive neurological impairments, punctuated by frequent epileptic seizures. In scenarios where clinical worsening persists or where clinical and radiological evaluations are inconclusive, aggressive immunosuppressive therapy is recommended.

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BACKGROUND: The objective is to analyze and review the clinical, laboratory, and neuroimaging characteristics of rheumatoid meningitis (RM) in six patients with known rheumatoid arthritis (RA). METHODS: We performed a retrospective review of patients diagnosed with RM from August 2012 to June 2023. To identify the cases, we used medical term search engines and the hospital´s radiology case database. Clinical information and laboratory findings were gathered from the medical records. A neuroradiologist with five years of experience reviewed and analyzed the RM to determine the characteristics findings of RM. RESULTS: Six patients with RM are included. Seizures along with headaches were among the clinical signs that were documented. All the patients had high levels of rheumatoid factor (RF) and anti-cyclic citrullinated peptides (ACPA) in the peripheral blood. Biopsy in two cases confirmed typical rheumatoid nodules. Leptomeningeal enhancement was found bilaterally in all cases and was predominantly found in the frontoparietal region. "Mismatch DWI/FLAIR" was found in five patients. Bilateral subdural collections could be found in two patients. Brain PET scan revealed increased metabolism in two cases. CONCLUSION: Rheumatoid meningitis is a rare complication of rheumatoid arthritis (RA) with challenging clinical diagnosis due to non-specific symptoms. This study highlights the importance of MR in detecting characteristic neuroimaging patterns, including "mismatch DWI/FLAIR", to aid in early diagnosis. Increased awareness of this condition may facilitate timely intervention and improve prognosis. These results still need to be verified by large studies.

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Rheumatoid meningitis (RM) is a rare but serious extra-articular manifestation of rheumatoid arthritis. Due to the absence of specific biomarkers, imaging findings, or guidelines for its detection, the diagnosis of RM is difficult. This report describes a patient of RM diagnosed with an open biopsy and discusses the utility of anticyclic citrullinated peptide antibodies (ACPA) levels in the serum and cerebrospinal fluid (CSF), and contrast-enhanced (CE) fluid-attenuated inversion recovery (FLAIR) images for screening and monitoring RM. A 65-year-old woman presented with a 2-month history of headaches. Imaging studies showed asymmetric meningeal and leptomeningeal involvement seen on brain magnetic resonance imaging (MRI). An open biopsy of the meninges and leptomeninges depicted palisaded and necrotizing granulomatous inflammation, which suggests rheumatoid nodules. Treatment with prednisolone and tocilizumab led to symptom improvement and reduced lesion intensity on follow-up MRI. Throughout the treatment, the ACPA index in her serum and CSF, and the findings of CE-FLAIR images, rather than the CE T1WI, reflected disease activity. For 6 months, the patient has been stable without symptom recurrence. The ACPA index and the CE-FLAIR images were useful for the diagnosis and monitoring of RM. To validate these findings, further studies are necessary.

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Rheumatoid meningitis (RM) is an extra-articular complication of rheumatoid arthritis (RA). Although reports of RM sine arthritis exist, most patients with this presentation were diagnosed with RA within one year of RM onset. There are no established biomarkers reflecting the disease activity of RM. This case report highlights the elevation of matrix metalloprotease (MMP)-9 levels during the acute phase of RM and decline during remission. Additionally, this is the first case report of RA diagnosed three years after the onset of RM. It is important to further validate the utility of MMP-9 and conduct long-term follow-up of RM sine arthritis.

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Rheumatoid meningitis is a rare neurological manifestation that is difficult to diagnose. Here we report a 77-year-old woman who developed a left hemiparesis because of an arachnoid cyst with rheumatoid meningitis. The patient had a 13 year history of rheumatoid arthritis, which had been successfully treated. She developed left hemiparesis and visited our hospital. MRI showed a high signal intensity lesion in the right frontal subarachnoid space and an arachnoid cyst on Fluid attenuated inversion recovery (FLAIR). A gadolinium enhanced T1 weighted image revealed associated abnormal enhancement. We removed the arachnoid cyst and diagnosed the patient as having rheumatoid meningitis. Her symptoms and MRI findings improved rapidly by methylprednisolone pulse therapy. Rheumatoid meningitis is rare, but a case of arachnoid cyst associated with rheumatoid meningitis was extremely rare. This is the first report of such a case.

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Objective: To describe the clinical and neuroimaging characteristics of rheumatoid meningitis (RM) in Chinese patients. Methods: The patients admitted to our hospital with the diagnosis of RM in the past 8 years were retrospectively analyzed. Results: Six patients with RM were identified among 933 patients admitted with rheumatoid arthritis (RA). The symptoms of meningitis occurred after onset of arthritis in five patients and before onset in one. Headache (n=6), hyperacute focal neurological deficits (n=4) and seizures (n=3) were the most prevalent symptoms. The nadir modified Rankin Scale score was ≥3 in five patients. Rheumatoid factor was elevated in all patients, and interleukin-6 levels in cerebrospinal fluid were dramatically elevated in three of four tested patients. Magnetic resonance imaging of the brain revealed that the meninges were affected in all patients and the cerebral parenchyma was affected in one patient. The lesions were generally located in the frontoparietal region and showed restricted diffusion along the adjacent subarachnoid space. RM occurred during disease-modifying therapy in four patients. In the acute episode, three patients improved on tocilizumab and the other three improved on pulse corticosteroids. For maintenance therapy, two patients received combined therapy of tocilizumab and other immunosuppressive agents, one received adalimumab and methotrexate, and two received low-dose oral corticosteroids with an immunosuppressive agent. Five patients had a good outcome, and one died of Pneumocystis jirovecii pneumonia after stabilization of his neurologic conditions. No relapse of RM occurred on immunotherapy during follow-up. Conclusions: Chinese patients with RM share some remarkable clinical and neuroimaging features and respond well to appropriate immunotherapy. Tocilizumab could be a treatment option for this severe complication of RA.

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Background: A special type of meningioma is known to have infiltrated inflammatory cells within the tumor, associated with peritumoral inflammation. However, there have been no reports of meningioma with inflammatory response only around the tumor, without inflammatory cells within the tumor itself. Case Description: A 70-year-old woman presented with transient right hemiparesis due to an extra-axial tumor on the left frontal convexity. The tumor appeared hypointense on T1-weighted magnetic resonance images and hyperintense on T2-weighted images without peritumoral edema, and was homogenously enhanced associated with the peritumoral leptomeningeal enhancement. Cerebrospinal fluid examination showed an increase in the number of inflammatory cells with a predominance of mononuclear cells. During the following 1 month, the tumor size was unchanged, but the peritumoral leptomeningeal enhancement was remarkably enlarged with uncontrolled focal seizures. The tumor was subtotally removed and semisolid substances in the subarachnoid space were biopsied. Pathological examination with immunostaining revealed angiomatous meningioma: the tumor had no inflammatory cell infiltration within it, but was associated with the infiltration of immunoglobulin G4-negative lymphocytes into the border zone between the tumor and the dura mater, as well as numerous neutrophils and fibrinous exudates in the peritumoral subarachnoid space. The tumor removal rapidly improved the leptomeningeal enhancement and inflammatory reactions. Conclusion: The authors reported the first case of angiomatous meningioma associated with massive peritumoral inflammation without inflammatory infiltrates within the tumor itself.

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A 66-year-old woman in treatment for rheumatoid meningitis was found to be positive for anti-N-methyl-D-aspartate receptor (NMDAR) antibodies in the cerebrospinal fluid, and intravenous immunoglobulin improved her psychiatric symptoms. The co-existence of NMDAR antibodies should be considered in cases of poor response to treatments or atypical symptoms in rheumatoid meningitis.

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Rheumatoid meningitis (RM) is a rare complication of rheumatoid arthritis that can manifest as stroke-like episodes. We present the case of a 63-year-old woman with a past history of overlap syndrome and clinical manifestations suggestive of amyopathic dermatomyositis, rheumatoid arthritis, and systemic lupus erythematosus. She presented to the emergency department with sudden onset right-sided clumsiness and numbness, as well as a 2-week history of left hemicranial headache. Laboratory workup revealed positive serum antinuclear antibodies, anti-Ro antibodies, anti-citrullinated peptide antibodies (ACPA), and elevated rheumatoid factor. Lymphocytic pleocytosis, positive ACPA and anti-Ro antibodies with passive diffusion pattern, and negative microbiological studies were demonstrated in the CSF. Brain magnetic resonance imaging showed predominant left fronto-parieto-occipital leptomeningeal and pachimeningeal enhancement. She was diagnosed with RM and received methylprednisolone IV mg/kg once daily. Stroke-like episodes in the setting of a patient with lymphocytic pleocytosis in the cerebrospinal fluid (CSF) and meningeal enhancement should raise suspicion of RM. In this context, serum rheumatoid factor and ACPA levels should always be measured and ACPA should also be measured in CSF. To our knowledge, this is the first reported case of RM in the context of an overlap syndrome. ACPA levels in CSF could be a relevant diagnostic clue in the setting of central nervous system disturbance and overlapping autoimmune conditions that include rheumatoid arthritis. In our case, the presence of a suggestive clinical scenario of RM reinforces the probable pathogenic role of ACPA when it is present in the central nervous system, even without intrathecal synthesis evidence.

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Rheumatoid meningitis (RM) is a rare extra-articular manifestation of rheumatoid arthritis, usually with non-specific symptoms. In most cases, head magnetic resonance imaging (MRI) shows lamellar enhancements in leptomeninges and pachymeninges, but definitive diagnosis relies on meningeal biopsies. Here, we reported a 43-year-old RM patient without a previous history of rheumatoid arthritis. He came to seek medical assistance because of fever and headache. The head MRI showed bilateral enhancements in leptomeninges and pachymeninges, and blood tests showed that serum IgM rheumatoid factor (RF) (1010.0 IU/ml) and anti-cyclic citrullinated peptide (CCP) antibody (654.24 RU/ml) became positive with a further increase with the progression of the disease. After treatment with steroids, clinical symptoms were relieved. We also reviewed previous history, symptoms, and serum, cerebrospinal fluid and imaging findings in 15 RM cases without a history of rheumatoid arthritis published since 2010. Consistent with previous reported cases, the current case suggests importance of meningeal biopsies and increases in serum RF and anti-CCP antibody in diagnosis of RM. In addition, previous joint symptoms and chronic headaches, and leptomeningeal and pachymeningeal lesions on head MRI are also of great significance for the diagnosis.

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As a rare complication of rheumatoid arthritis (RA) in the central nervous system (CNS), rheumatoid meningitis (RM) mainly affects the meninges and has various clinical symptoms. The diagnostic and treatment approaches currently used are not practical. RM cases with positive NMDAR antibodies (Abs) have never been reported. In the present study, a 66-year-old man with a 1-year history of RA presented recurrent left lower limb weakness during activities for 1 month. The results showed that rheumatoid factor (RF) and anti-cyclic citrullinated peptide antibody (ACPA) were positive in the serum, and NMDAR Abs were present in cerebrospinal fluid (CSF). Hyperintensity was observed in the leptomeninges of the right frontal and parietal lobes, and subtle hyperintensity was observed in the left frontal and parietal lobes, as indicated by brain MRI. A meningeal biopsy revealed non-specific inflammation with the absence of rheumatoid nodules. The patient was given IVIg on day 7 after admission. The clinical symptoms were relieved, the lesions were alleviated, and abnormal biochemical indicators were gradually recovered 1 week after initiation of the treatment, while NMDAR Abs were present in CSF even after treatment. After 5 months of follow-up, the patient's serum and CSF ACPA and IL-6 levels were still high. The findings showed that brain MRI was adequate for the diagnosis of RM. ACPA and IL-6 might be the specific biomarkers for disease activity in RM. IVIg was effective as induction therapy for RM. Further studies should explore whether the presence of NMDAR Abs is associated with RM.

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Rheumatoid meningitis (RM) is a rare but often aggressive neurological complication of rheumatoid arthritis. The diagnosis of RM, besides the clinical, radiological, and laboratory criteria, usually requires a cerebral biopsy. Based on the two cases presented in this paper, we propose a new laboratory marker. Cerebrospinal fluid and serum anti-cyclic citrullinated peptide (CCP) IgG were measured, and the intrathecal synthesis of anti-CCP antibodies (anti-CCP antibody index) was calculated using the hyperbolic function. The anti-CCP antibody index was positive in both cases at first diagnosis and progressively decreased after treatments. Together with clinical and radiological criteria, the calculation of the anti-CCP intrathecal synthesis, more than the simple measurement of serum or cerebrospinal fluid anti-CCP antibody titers, may represent a useful tool for RM diagnosis and, possibly, for treatment response.

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Normal-pressure hydrocephalus (NPH) is a common cause of gait apraxia, cognitive impairment, and urinary incontinence in the elderly. It is usually a primary idiopathic disorder but can be secondary. We present a case of secondary NPH due to biopsy-confirmed rheumatoid meningitis initially refractory to intravenous (IV) immunotherapy. Our patient reported an excellent response right after shunting. Her gait remains normal one and a half years later. We searched PubMed for similar cases of rheumatoid meningitis with gait abnormality for additional clinicopathologic discussion. The patient's movement disorder initially improved with steroid taper. However, she developed progressive symptoms, later on, refractory to IV solumedrol and rituximab. She underwent ventriculoperitoneal shunting (VPS) and reported an outstanding outcome. This is the first reported biopsy-confirmed case of rheumatoid meningitis causing NPH to undergo shunting for immediate improvement. Previous cases of rheumatoid meningitis-associated Parkinsonism have improved with steroid induction. Although our patient's rheumatoid arthritis is now controlled, her case illustrates that NPH in autoinflammatory conditions may not recover with immune suppression alone. VPS is an option for a faster response in secondary NPH due to rheumatoid meningitis or other inflammatory disorders with progressive symptoms despite standard induction therapy.

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Rheumatoid arthritis (RA) is a chronic inflammatory disease characterized mainly by arthritis, with the possible occurrence of extra-articular manifestations. We report the case of a patient who developed leptomeningitis as the first sign of RA, one year before the diagnosis of RA. Methylprednisolone 1000 mg was given intravenously. Because of the onset of seizures and cognitive impairment, rituximab was started. After three cycles of rituximab (1000 mg on day 0 and 1000 mg on day 15, every 6 months), neurological clinical examination, MRI and electroencephalogram findings were significantly improved. LEARNING POINTS: Rheumatoid meningitis may occur in the disease course of rheumatoid arthritis with varied and non-specific symptoms.Cerebrospinal fluid examination, MRI and tests for rheumatoid factor or anti-citrullinated protein antibodies in serum or in cerebrospinal fluid are key examinations for diagnosing rheumatoid meningitis.Rituximab has good efficacy in rheumatoid meningitis.

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Rheumatoid meningitis is a rare complication of long-standing rheumatoid arthritis. We present the case of a 39-year-old Bolivian woman with a history of seropositive rheumatoid arthritis of 12 years of evolution without extra-articular manifestations that develops a severe headache with vomiting. The diagnosis of rheumatoid meningitis was performed based on clinical history, blood count and biochemistry results, cerebrospinal fluid analysis and cranial magnetic resonance imaging findings. High-dose intravenous glucocorticoids were started, followed by rituximab. After treatment, a significant clinical improvement was observed and repeat magnetic resonance imaging scan confirmed an improvement of the meningeal lesions.

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Rheumatoid meningitis (RM) is a rare but treatable central nervous system (CNS) manifestation of rheumatoid arthritis (RA) with various clinical presentations and atypical cerebrospinal fluid (CSF) findings. There are no established biomarkers for RM, making diagnosis a challenge. Herein, we present three cases of RM: two patients with RA diagnosis and one without. CSF analysis showed pleocytosis in only one case. In contrast, CSF neopterin levels were elevated in all three cases and decreased after steroid therapy. This study suggests that CSF neopterin levels may be a useful biomarker for diagnosing and therapeutically monitoring CNS inflammation in patients with RM.

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We report a 62-year-old female with rheumatoid meningitis. She presented with mental disorder, loss of consciousness, generalized seizures, and cognitive impairment. Brain MRI demonstrated high intensity lesions and abnormal enhancement along the left frontal and parietal sulci. Her serum and cerebrospinal fluid were positive for anti-cyclic citrullinated peptides (CCP) antibody, and the antibody index of cerebrospinal fluid anti-CCP antibody increased, which led us to suspect rheumatoid meningitis. Her symptoms improved immediately by methylpredonisolone pulse therapy and anti-CCP antibody turned negative in cerebrospinal fluid. However, she revealed arthritis with the reduction of betamethasone and was diagnosed as rheumatoid arthritis. We suggest that the elevation of antibody index of cerebrospinal fluid anti-CCP antibody is useful in the diagnosis of rheumatoid meningitis preceding neurological symptoms without arthritis, and anti-CCP antibody in cerebrospinal fluid may be helpful as the evaluation of the treatment.

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Rheumatoid meningitis (RM) is a rare central nervous system (CNS) manifestation of rheumatoid arthritis (RA) with a wide spectrum of symptoms. We present a review of the literature with a rare illustrative case of a 61-year-old man with a history of seropositive rheumatoid arthritis (RA) who presented headaches, stroke-like symptoms and seizures. MRI revealed the leptomeningeal enhancement in the right hemisphere. As cerebromeningeal fluid showed increased level of protein and was positive for Candida mannan, the initial clinical diagnosis was fungal meningitis. Despite the antifungal treatment the patient's clinical condition did not improve. Detailed laboratory, radiologic and histopathological diagnostics enabled the diagnosis of RM. In conclusion is worth to highlight that presentation of RM is variable and complex, diagnosing it is a big dilemma which is why it must be considered in the differential in a patient with long-standing seropositive RA.