DOI:10.2214/AJR.05.0859
AJR 2006; 186:1181-1183
© American Roentgen Ray Society
Radiologic-Pathologic Conference of the Massachusetts General
Hospital |
Radiologic-Pathologic Conference of the Massachusetts General Hospital
Rheumatoid Meningitis: Radiologic and Pathologic Correlation
Stephen E. Jones1,
Nicole A. Belsley2,
Theresa C. McLoud1 and
Mark E. Mullins1
1 Department of Radiology, Massachusetts General Hospital, FND 216, 55 Fruit
St., Boston, MA 02114-2698.
2 Department of Pathology, Massachusetts General Hospital, Boston, MA
02114-2698.
Received May 23, 2005;
accepted after revision August 22, 2005.
Address correspondence to S. E. Jones
(sejones{at}partners.org).
Keywords: neuroimaging • rheumatoid arthritis • rheumatoid meningitis
Six months before admission to our hospital, a 58-year-old woman with a
6-year history of fibromyalgia noticed new rapid onset of weakness and
numbness in her left lower extremity that spread to the left upper extremity
and face, initially lasting only a few minutes. According to the report, MR
images obtained at another hospital showed no abnormalities. Subsequent
episodes of similar weakness and numbness increased in both frequency and
duration, necessitating an inpatient workup 1 month after the initial MR
examination. The workup revealed decreased sensation to pinprick in the left
lateral aspect of the thigh and the left lateral aspect of the calf
accompanied by episodic low-grade fevers. The findings of the rest of the
physical examination, the mental status examination, and
electroencephalography were normal. The initial clinical differential
diagnosis included viral meningitis and CNS carcinomatosis. Findings of a
metastatic workup were normal. The episodes of weakness and numbness persisted
despite a treatment course of acyclovir. The symptoms progressed to include
emotional lability and balance problems. Three days before admission to our
hospital, 6 months after the onset of symptoms, the patient began to
experience slurred speech.
MRI performed when the patient arrived at our hospital showed partial loss
of gray-white matter differentiation in the right frontal and parietal lobes,
effacement of the right-sided sulci due to subtle local mass effect, and
enhancement of the adjacent pachymeninges and leptomeninges (Figs.
1A,
1B,
1C, and
1D). Diffusion-weighted imaging
showed restricted diffusion in the adjacent subarachnoid space. This finding
was most consistent with the presence of viscous or dense material such as pus
or proteinaceous debris.
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Fig. 1A —58-year-old woman with rheumatoid meningitis. Unenhanced axial
T1-weighted image shows increased asymmetric signal within right
frontoparietal sulci (arrow). Subjacent loss of gray-white
differentiation and effacement of right-sided sulci due to subtle local mass
effect are evident.
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Fig. 1C —58-year-old woman with rheumatoid meningitis. Axial FLAIR image
shows relative right-sided increase of normal low signal intensity in
frontoparietal CSF (subarachnoid) spaces (arrows) most consistent
with infiltration of less aqueous material, such as subacute accumulation of
blood, pus, or proteinaceous material.
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Fig. 1D —58-year-old woman with rheumatoid meningitis. Axial
diffusion-weighted image shows increased signal intensity over right-sided
convexities (arrows) again involving right frontoparietal
subarachnoid space. This region coincides with low signal intensity on
apparent diffusion coefficient map (not shown) and therefore indicates
restricted diffusion. This finding is consistent with presence of viscous
material, such as pus or proteinaceous material.
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CSF analysis showed a reactive process and no evidence of carcinoma or
bacterial infection. MR-guided stereotactic brain biopsy showed fibrinoid
necrosis surrounded by histiocytes and a dense infiltrate of plasma cells in
the subarachnoid space (Figs.
1E and
1F). Some of the plasma cells
featured prominent cytoplasmic eosinophilic globules, known as Russell bodies,
resulting from an accumulation of immunoglobulin. Occasional Mott cells, or
plasma cells with a collection of Russell bodies, also were seen. Rare
multinucleated giant cells also were present. Staining results for bacteria,
acid-fast bacilli, fungi, and spirochetes were negative. All culture results
also were negative. Immunoglobulin studies revealed a polyclonal population of
plasma cells. The erythrocyte sedimentation rate was elevated at 78 mm/hr, and
alkaline phosphatase level was elevated at 102 U/L. The rheumatoid factor
level was extremely high at 432 U/mL (normal, < 30 U/mL). The antinuclear
antibody result was mildly positive. A gallium scan revealed no abnormal
findings.
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Fig. 1E —58-year-old woman with rheumatoid meningitis. Low-power micrograph
of leptomeninges shows necrosis with occasional scattered multinucleated giant
cells and dense lymphoplasmacytic infiltrate around small vessels within
subarachnoid space.
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The patient subsequently noticed an increase in morning stiffness and
swelling involving the metacarpophalangeal joints, wrists, shoulders, and
knees. Symptoms previously ascribed to fibromyalgia, including difficulty
sleeping at night and increased trigger points for pain, increased. Physical
examination showed marked synovitis involving the joints of the hands and the
wrists. Radiographs of both knees, hands, and shoulders showed mild
degenerative changes. MRI of the wrists and hands showed erosive changes most
consistent with inflammatory arthritis. The clinical findings suggested a
diagnosis of rheumatoid arthritis (RA), and the patient was treated with
cyclophosphamide (Cytoxan, Bristol-Myers Squibb) and prednisone. The patient
was given a presumptive diagnosis of rheumatoid leptomeningitis and
pachymeningitis, and her symptoms resolved with IV chemotherapy and
steroids.
Discussion
RA is a chronic inflammatory disease primarily involving joints; however,
extraarticular involvement is common. The annual incidence of RA is
approximately 30 cases per 100,000 persons with an age at onset typically
between 30 and 55 years. The disease affects more women than men. The onset is
usually insidious with joint pain, stiffness, and swelling. Diagnosis is based
on a constellation of clinical signs, radiographic correlates, and laboratory
values. Diagnostic criteria have been developed and validated by the American
College of Rheumatology
[1].
Nervous system involvement with RA is uncommon. Typical neurologic sequelae
are usually secondary to musculoskeletal involvement. An example is mass
effect on the spinal cord or peripheral nerves due to synovitis, pannus, or
articular subluxation. Atypical sequelae directly involving the CNS include
parenchymal and meningeal vasculitis, rheumatoid nodules, and meningitis (both
pachymeningitis and leptomeningitis)
[2]. Rarer complications
include organic brain syndrome and progressive multifocal leukoencephalopathy.
CNS involvement can occur without typical extracranial patterns of RA.
Neurologic symptoms of rheumatoid meningitis include cranial nerve
dysfunction, seizure, mental status change, and hemiparesis or paraparesis
[3-5].
Results of laboratory analysis of CSF usually are abnormal, showing an
elevated protein level with occasional pleocytosis and a depressed glucose
level. The diagnosis is one of exclusion, and all other causes of
leptomeningitis and pachymeningitis must be considered
(Table 1). Diagnosis is aided
by a clinical diagnosis of RA, positive serologic results for rheumatoid
factors, and the pathologic visualization of rheumatoid nodules.
Thickened dura representing pachymeningitis contains a nonspecific
infiltrate of mononuclear cells, particularly plasma cells. Less frequently
seen are areas of necrosis and multinucleated giant cells. Although they may
be present in 60% of cases, rheumatic nodules often do not cause symptoms. The
presence of epithelioid granulomas typically in the cranial meninges or
choroid plexus confirms the diagnosis of rheumatoid meningitis but is not a
specific finding. In addition to meningeal effects, CNS symptoms are caused by
CNS vasculitis related to a lymphoplasmacytic infiltrate in the vessel walls.
This infiltrate involves both parenchymal and meningeal vessels, although it
tends to spare large vessels, such as the middle cerebral artery.
References
- Arnett FC, Edworthy SM, Bloch DA, et al. The American Rheumatism
Association 1987 revised criteria for the classification for rheumatoid
arthritis. Arthritis Rheum 1988;31
: 315-324[Medline]
- UpToDate Web site. Piecyk ML, Schur PH. Neurologic
manifestations of rheumatoid arthritis. Available at:
patients.uptodate.com/topic.asp?file=rheumart/11368.
Updated March 14, 2005; accessed January 6, 2006
- Bathon JM, Moreland LW, DiBartolomeo AG. Inflammatory central
nervous system involvement in rheumatoid arthritis. Semin Arthritis
Rheum 1989; 18:258
-266[CrossRef][Medline]
- Cellerini M, Gabbrielli S, Maddali Bongi S, Cammelli D. MRI of
cerebral rheumatoid pachymeningitis: report of two cases with follow-up.
Neuroradiology 2001;43
: 147-150[Medline]
- Kato T, Hoshi K, Sekijima Y, et al. Rheumatoid meningitis: an
autopsy report and review of the literature. Clin
Rheumatol 2003; 22:475
-480[Medline]
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