Rheumatoid meningitis [Neurosurgery Wiki]

This page is read only. You can view the source, but not change it. Ask your administrator if you think this is wrong.
====== Rheumatoid meningitis ======

Central nervous system involvement in [[rheumatoid arthritis]] (RA) is a rare and treatable, yet potentially fatal, condition that is frequently recognized only by [[autopsy]]
((Bathon JM, Moreland LW, DiBartolomeo AG.
Inflammatory central nervous system involvement
in rheumatoid arthritis. Semin Arthritis Rheum 1989;
18: 258-66.)).

Till 2018 approximately 25 [[case report]]s were published describing various clinical symptoms of this condition, including [[confusion]], [[focal neurological deficit]]s, [[seizure]]s, stroke-like episodes, and [[Parkinsonism]]
((Finkelshtein V, Lampl Y, Lorberboym M, Kanner A, Ben-Ami Raichman D, Dabby R,
Tanay A. Self-limited [[Rheumatoid Meningitis]] as a Presenting Symptom of Rheumatoid
Arthritis. Isr Med Assoc J. 2018 Apr;20(4):262-264. PubMed PMID: 29629737.)).


Rheumatoid [[meningitis]] (RM) is a rare [[complication]] of [[rheumatoid arthritis]] (RA) and has a high mortality rate. It can present as a first diagnosis of RA, in long-standing disease, or in active or well-controlled disease. Neurological manifestations vary widely.

RM must be considered in adult patients with or without RA diagnosis
((Villa E, Sarquis T, de Grazia J, Núñez R, Alarcón P, Villegas R, Guevara C. Rheumatoid meningitis: a systematic review and meta-analysis. Eur J Neurol. 2021 May 9. doi: 10.1111/ene.14904. Epub ahead of print. PMID: 33966315.)).

===== Epidemiology =====

RM affected adults with an average age of 62 years, with or without a previous diagnosis
((Villa E, Sarquis T, de Grazia J, Núñez R, Alarcón P, Villegas R, Guevara C. Rheumatoid meningitis: a systematic review and meta-analysis. Eur J Neurol. 2021 May 9. doi: 10.1111/ene.14904. Epub ahead of print. PMID: 33966315.)).

===== Clinical features =====
The most common clinical manifestations were transient focal neurological signs (64.6%), systemic symptoms (51.3%), episodic headache (50.4%), and neuropsychiatric alterations (47.7%). Joint manifestations were present in only 27.4% of cases
((Villa E, Sarquis T, de Grazia J, Núñez R, Alarcón P, Villegas R, Guevara C. Rheumatoid meningitis: a systematic review and meta-analysis. Eur J Neurol. 2021 May 9. doi: 10.1111/ene.14904. Epub ahead of print. PMID: 33966315.)).

===== Diagnosis =====

In a systematic review brain magnetic resonance imaging showed unilateral or bilateral involvement, predominantly supratentorial and frontoparietal. Both pachy- and leptomeninges were affected, the latter more frequently (82.88%). The laboratory findings included increased levels of rheumatoid factor (89.71%), anti-cyclic citrullinated peptide (89.47%), C-reactive protein (82.54%), and erythrocyte sedimentation rate (81.81%). Cerebrospinal fluid analysis showed an increase in the protein level (76.14%), with pleocytosis (85.19%) of mononuclear predominance (89.19%). Biopsy was performed in 72.52% of the patients
((Villa E, Sarquis T, de Grazia J, Núñez R, Alarcón P, Villegas R, Guevara C. Rheumatoid meningitis: a systematic review and meta-analysis. Eur J Neurol. 2021 May 9. doi: 10.1111/ene.14904. Epub ahead of print. PMID: 33966315.)).

MRI findings usually exhibit non-specific meningeal thickening and contrast enhancement 
((Cianfoni A, Falcone C, Faustini F, et al. Rheumatoid
leptomeningitis: magnetic resonance imaging and
pathologic findings – a case report. J Neuroimaging
2010; 20: 192-4.)).

CSF studies may be normal or show non-specific lymphocytic pleocytosis with protein elevation.
The significance of increased Rheumatoid factor (RF) in the CSF is unclear
((Kim HY, Park JH, Oh HE, Han HJ, Shin DI, Kim
MH. A case of rheumatoid meningitis: pathologic and magnetic resonance imaging findings. Neurol
Sci 2011; 32: 1191-4.)).

===== Treatment =====
Corticosteroid pulse therapy was the main induction therapy
((Villa E, Sarquis T, de Grazia J, Núñez R, Alarcón P, Villegas R, Guevara C. Rheumatoid meningitis: a systematic review and meta-analysis. Eur J Neurol. 2021 May 9. doi: 10.1111/ene.14904. Epub ahead of print. PMID: 33966315.)).


===== Outcome =====
RA activity and time with the disease were associated with a worse prognosis.

Disease relapse occurred in 31.17% of patients, while 54.54% had a full recovery.
((Villa E, Sarquis T, de Grazia J, Núñez R, Alarcón P, Villegas R, Guevara C. Rheumatoid meningitis: a systematic review and meta-analysis. Eur J Neurol. 2021 May 9. doi: 10.1111/ene.14904. Epub ahead of print. PMID: 33966315.)).

===== Systematic review and meta-analysis =====
Villa et al. from the Hospital Clínico Universidad de [[Chile]], [[Santiago]], aimed to describe the characteristics of the disease, including clinical, imaging, and laboratory findings, treatment, outcomes, and prognosis reported in the literature.

They included 103 studies with 130 cases. RM affected adults with an average age of 62 years, with or without a previous diagnosis. RA activity and time with the disease were associated with a worse prognosis. The most common clinical manifestations were transient focal neurological signs (64.6%), systemic symptoms (51.3%), episodic headache (50.4%), and neuropsychiatric alterations (47.7%). Joint manifestations were present in only 27.4% of cases. Brain magnetic resonance imaging showed unilateral or bilateral involvement, predominantly supratentorial and frontoparietal. Both pachy- and leptomeninges were affected, the latter more frequently (82.88%). The laboratory findings included increased levels of rheumatoid factor (89.71%), anti-cyclic citrullinated peptide (89.47%), C-reactive protein (82.54%), and erythrocyte sedimentation rate (81.81%). Cerebrospinal fluid analysis showed an increase in the protein level (76.14%), with pleocytosis (85.19%) of mononuclear predominance (89.19%). Biopsy was performed in 72.52% of the patients. Corticosteroid pulse therapy was the main induction therapy. Disease relapse occurred in 31.17% of patients, while 54.54% had a full recovery.

RM must be considered in adult patients with or without RA diagnosis. These findings may aid clinicians in timely RM diagnosis and treatment, thus improving its outcomes
((Villa E, Sarquis T, de Grazia J, Núñez R, Alarcón P, Villegas R, Guevara C. Rheumatoid meningitis: a systematic review and meta-analysis. Eur J Neurol. 2021 May 9. doi: 10.1111/ene.14904. Epub ahead of print. PMID: 33966315.)).

===== Case reports =====

A patient with a 30-year history of RA, well-controlled with [[methotrexate]] therapy, presented with new-onset [[seizure]]s. Magnetic resonance imaging showed leptomeningeal and pachymeningeal enhancement. A de novo workup resulted in a diagnosis of RM.

Cerebrospinal fluid findings for RM are nonspecific, typically lymphocytic pleocytosis; however, they can be neutrophilic, as in this case. Magnetic resonance imaging findings consist of leptomeningeal and pachymeningeal enhancement but can also involve the parenchyma. The diagnosis is typically confirmed with meningeal biopsy. Treatment involves high-dose corticosteroids or immunomodulatory therapy, or both. Long-term follow-up with radiologic surveillance typically ranges from improvement to resolution
((Parsons AM, Zuniga LA, Hoxworth JM, Lyons M, Aslam F, Goodman BP. Rheumatoid
Meningitis: A Case Review. Neurologist. 2018 May;23(3):83-85. doi:
10.1097/NRL.0000000000000158. PubMed PMID: 29722740.
)).
----
Finkelshtein V, Lampl Y, Lorberboym M, Kanner A, Ben-Ami Raichman D, Dabby R,
Tanay A. Self-limited [[Rheumatoid Meningitis]] as a Presenting Symptom of Rheumatoid
Arthritis. Isr Med Assoc J. 2018 Apr;20(4):262-264. PubMed PMID: 29629737.