ABSTRACT Symptomatic rheumatoid pachymeningitis is a rare extra-articular manifestation of rheumatoid arthritis. Clinical symptoms are non-specific and diagnosis is frequently made by exclusion. We present a 61-year-old woman with a 9-year history of rheumatoid arthritis presenting with deafness and progressive disability over a two month duration. She was diagnosed as having rheumatoid pachymeningitis based on the cerebral magnetic resonance imaging findings.
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ABSTRACT: Rheumatoid arthritis is a systemic disease in which cerebral and eye involvement is neither common nor fully understood. Although it is rarely the cause of pachymeningitis and optic neuritis, rheumatoid arthritis should always be kept in mind in these two conditions. We present a 52-year-old male with an 8 month history of rheumatoid arthritis who was referred to the neurology department with headache and decreasing vision and was diagnosed as having rheumatoid pachymeningitis and optic neuritis on the basis of MRI findings.British Journal of Radiology 05/1999; 72(856):404-7. · 1.22 Impact Factor
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ABSTRACT: Focal rheumatoid inflammation of the dura and leptomeninges was found in an 89-year-old man who was neurologically asymptomatic and had clinically inactive rheumatoid disease. Previous reports of this entity have not stressed that rheumatoid meningitis can be virtually asymptomatic and can develop in persons with apparently quiescent joint disease. The presence of vasculitis of the meningeal or cerebral vessels is associated with symptoms. Intracranial rheumatoid lesions were associated with rheumatoid lesions in other organs, especially the heart and lungs.Archives of pathology & laboratory medicine 04/1983; 107(3):129-31. · 2.78 Impact Factor
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ABSTRACT: Rheumatoid pachymeningitis is a rare complication of rheumatoid arthritis. This disease was confined to the dura and pia-arachnoid of the lumbar cord in our patient. Her neurologic deficits responded to surgical decompression and corticosteroid therapy. Radiologic evidence and the differences in cell count, protein, and glucose content between lumbar and cisternal cerebrospinal fluid indicate that rheumatoid pachymeningitis can be localized to a discrete region of the central nervous system. Elevated immunoglobulins, IgM and IgG rheumatoid factors, low molecular weight IgM, and immune complexes were found in the cerebrospinal fluid and implicate an immune reaction in the pathogenesis of this disease, which is probably similar to inflammatory processes involving other organs in rheumatoid arthritis.Annals of internal medicine 06/1979; 90(5):786-9. · 13.98 Impact Factor
Singapore Med J 2004 Vol 45(7) : 337
H J Tan, A A Raymond, P P Phadke, Z Rozman
Faculty of Medicine
Jalan Yaacob Latif
Bandar Tun Razak
56000 Kuala Lumpur
H J Tan, MD,
Lecturer and Physician
A A Raymond, MMed,
P P Phadke, MBBS,
Z Rozman, MBBS
Dr Tan Hui Jan
Tel: (60) 39170 2530
Fax: (60) 39173 7829
Symptomatic rheumatoid pachymeningitis is a
rare extra-articular manifestation of rheumatoid
arthritis. Clinical symptoms are non-specific and
diagnosis is frequently made by exclusion. We
present a 61-year-old woman with a 9-year history
of rheumatoid arthritis presenting with deafness
and progressive disability over a two month
duration. She was diagnosed as having rheumatoid
pachymeningitis based on the cerebral magnetic
resonance imaging findings.
Keywords: internal auditory canals, magnetic
resonance imaging, rheumatoid arthritis, rheumatoid
pachymeningitis, sensorineural deafness
Singapore Med J 2004 Vol 45(7):337-339
Rheumatoid arthritis is a multisystem disease with
many extra-articular manifestations. The pathogenesis
of rheumatoid arthritis involves an immune process
characterised by immunoglobulins, IgM and IgG
rheumatoid factors. Part of the localised immune
process has been implicated in the development of
rheumatoid meningitis. Rheumatoid meningitis is
a pachymeningitis that is rarely encountered in
rheumatoid arthritis. This is the first case of rheumatoid
pachymeningitis presenting with bilateral sensorineural
deafness. Brain imaging revealed enhancement of the
tissues in both internal auditory canals.
A 61-year-old woman was hospitalised for progressive
disability of two months duration. She had a 9-year
history of rheumatoid arthritis involving the small
joints of her hands, wrists and ankles. She had
occasional disease exacerbation for which she was
prescribed analgesics and methotrexate. She had
initially complained of hearing problems to her family.
She slowly became forgetful and confused in the
following two months, and she was not able to
perform her routine daily activities. There was no
headache, fever, nausea or vomiting.
On physical examination, there were bilateral
symmetrical deforming polyarthropathies of her
hands. The neurological examination revealed
bilateral sensorineural deafness. The mini-mental
state examination could not be done, as the patient
did not understand the instructions. Her muscle bulk,
power and tendon reflexes were normal. Plantar
responses were flexor. The examination of the heart,
lungs and abdomen was normal.
The rheumatoid factor was positive. Antinuclear
antibodies and VDRL were negative. The cerebrospinal
fluid results were abnormal (Table I). The Gram stain,
Indian ink stain, Ziehl-Neelson stain and latex
agglutination tests were negative. Mycobacterium
and fungal cultures were negative. Brain magnetic
resonance (MR) imaging showed prominent
periventricular areas of hyperintensity in the parieto-
occipital and periaqueductal regions. There was
thick meningeal enhancement in the posterior
interhemispheric fissure and enhancement of both
internal auditory canals (Fig. 1).
Neurological manifestations in rheumatoid arthritis,
although not very common, causes death in up to
18.6% of cases(1). Documented nervous system
manifestations of rheumatoid arthritis include
spinal cord compression due to subluxation of the
cervical vertebrae, cerebral haemorrhage due to
Fig. 1 Enhanced (a, b) axial and (c) coronal (C) T1-W MR images
show enhancement in the convexity sulci (arrows in a) and the
interhemispheric fissure, and in both the internal auditory canals
(arrows in b & c).
C a s e R e p o r t
Singapore Med J 2004 Vol 45(7) : 338
serum hyperviscosity associated with the elevated
titres of circulating rheumatoid factors, vasculitis,
rheumatoid meningeal nodules, choroid plexus
infiltration, rheumatoid synovial infiltration of
the spinal dura and pachymeningitis(2). Other
causes of pachymeningitis include sarcoidosis,
tuberculosis, lymphoma, Wegener’s granulomatosis
and syphilis(1). Short of doing a brain biopsy, the
diagnosis of rheumatoid pachymeningitis is one of
exclusion. In this patient, the entire infective screen
Rheumatoid pachymeningitis is a rare occurrence
in rheumatoid arthritis, and previous reported
experience with this complication is limited to case
reports. Both fibrinoid deposits and rheumatoid
nodules cause pachymeningitis. Its clinical course
is one of longstanding, erosive and seropositive
disease with prominent extra-articular features. There
may or may not be clinical evidence of active synovitis
when the neurological disease becomes symptomatic.
Similarly, this patient who had long-standing rheumatoid
disease did not have active arthritis during her neurological
presentation. Although patients with pachymeningitis
may remain asymptomatic, several case reports have
described its clinical manifestations. They include
seizures(3), diffuse cerebral dysfunction(4), headaches,
meningitis, cortical blindness.
Our patient presented with confusion and
sensorineural deafness. This could be attributed to
the thick meningeal lesions, seen as areas of
hyperintensity on MR imaging, involving the
periaqueductal region, parieto-occipital gray and
white matter, and both internal auditory canals. The
diffuse meningeal involvement could be a combination
of pachymeningitis and vasculitis. The cerebrospinal
fluid may show some combination of high protein,
mild pleocytosis and hypoglycorrhachia, as seen in
our patient. Extremely low glucose content is a
well-known characteristic of rheumatoid effusions.
The mechanism of hypoglycorrachia in rheumatoid
meningitis is due to interference with glucose entry(5).
This differs from the non-rheumatoid meningeal
diseases, such as in infections and carcinomatous
meningitis where certain inflammatory products
interfere with the glucose transport system into
the cerebrospinal fluid. Cerebral MR imaging in
this patient showed focal hyperintensity in the
parieto-occipital region, periaqueductal region and
the internal auditory canals, with prominent
enhancement. Previous case reports evaluating
rheumatoid pachymeningitis with radiological
examination include gallium scintigraphy, technetium
scintigraphy, enhanced computed tomography and
Methotrexate has long been a recognised
treatment of rheumatoid vasculitis. The long-term
use of methotrexate in this patient may predispose
to accelerated nodulosis. Karam et al(6) reported
isolated rheumatoid nodulosis of the meninges in
a patient with well-controlled, non-deforming
rheumatoid arthritis during methotrexate therapy.
Although this patient did not have any rheumatoid
nodules, the possibility of methotrexate-induced
meningeal rheumatoid nodulosis could not be
excluded. Pathologically, the dura has single or
multiple rheumatoid nodules, or may show diffuse
inflammatory changes consisting of a central area of
fibrinoid necrosis surrounded by combination of
pallisading epitheloid cells, lymphocytes, plasma
cells and multinucleated giant cells. Vasculitis of
the leptomeninges and underlying cortex may
occasionally be present.
The diagnosis of rheumatoid pachymeningitis
is ominous as most patients die within six months
of onset of neurological symptoms. The use of
corticosteroids and immunosuppressants has not
been shown to have any significant effect on this
condition(7). In summary, rheumatoid pachymeningitis
should be considered as a diagnostic possibility in
Table 1. Summary of laboratory results.
White cell count4.0 x109
Platelet count133 x109
Urea 4.3(2.5-6.4) mmol/L
Sodium 127(135-150) mmol/L
Total bilirubin9 <23
Alkaline phosphatase103(32-104) U/L
Erythrocyte sedimentation rate10(1-20)mm/hour
– glucose 0.2 (2.2 -4.2)mmol/L
– microscopic examinationwhite blood
Singapore Med J 2004 Vol 45(7) : 339
the proper clinical setting, when evidence of abnormal
meningeal enhancement is found on enhanced
1. Agildere AM, Tutar NU, Yucel E, Coskun M. Case report.
Pachymeningitis and optic neuritis in rheumatoid arthritis: MRI
findings. Br J Radiol 1999; 72:404-7.
2. William TC, Drew JM, Rizzo M, Ryals TJ, Sato Y, Bell WE.
Evaluation of pachymeningitis by contrast-enhanced MR imaging in
a patient with rheumatoid disease. AM J Neuroradiol 1990; 11:1247-8.
3. Beck D, Corbett JJ. Seizures due to central nervous system rheumatoid
meningovasculitis. Neurology 1983; 33:1058-61.
4. Spurlock RG, Richman AV. Rheumatoid meningitis: a case report and
review of the literature. Arch Pathol Lab Med 1983; 107:129-31.
5. Markenson JA, McDougal JS, Tsairis P, Lockshin MD, Christian CL.
Rheumatoid meningitis: a localized immune process. Ann Int Med
6. Karam NE, Roger L, Hankins LL, Reveille JD. Rheumatoid nodulosis
of the meninges. J Rheumatol 1994; 21:1960-3.
7. Rosenbaum RB, Campbell SM, Rosenbaum JT. Clinical Neurology of
Rheumatic Diseases. Newton: Butterworth-Heinemann,1996.