Transcript Rheumatoid arthritis meningitis

Rheumatoid arthritis in the
brain- a diagnosis of exclusion
Dr Suzanne O’Leary
SpR Neuroradiology
Frenchay Hospital
Bristol
UK
Bristol case
• 59 year old female patient
• Known diagnosis of Rheumatoid arthritis
• RA affecting joints only. Inactive at time of
this presentation
• Otherwise well
• No previous neurological condition
Presentation
• Severe headache that had persisted for 2
weeks
• Headache had been sudden in onset and
very severe.
• Initially a diagnosis of SAH was
considered
CT head
• NO ICH
• Bifrontal low
attenuation consistent
with oedema.
Callosal splenium
thickening.
• ? Intrinsic tumour
• ? Inflammatory
process
• ? infection
• MRI advised
Deterioration
• Patient becoming increasingly confused
and absent episodes.
• Acyclovir started
• ECG, CXR,FBC, MSU normal.
• No evidence of infection.
• ESR elevated
• ANA positive
• RhF positive
MRI
FLAIR
T1W
DWI
T2W
MRI- T1W + GAD
MRI findings
• Post contrast images show leptomeningeal enhance
ment either
side of the falx in the anterior interhemispheric fissu
re,
with enhancement of the dura over the falx at this
site.
There is reactive oedema in the adjacent anteromed
ial frontal lobes.
• Appearances are unusual but clearly demonstrate a
meningeal
process which may be inflammatory, reactive or mal
ignant.
Is there any abnormality on lumbar puncture?
CT angiogram
CTA
• The medial frontal lobes demonstrate
paucity of vessels.
• The vessels that are visible are irregular in
outline.
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Catheter angiogram
Catheter angiogram findings
• There is abnormality in the vasculature supplying
the medial frontal lobes bilaterally where there is slight
reduction in vascularity and evidence of vascular
irregularity including general stenosis with pruning and
beading in places. There is no arteriovenous shunting. T
here
does not appear to be involvement of any of the more
proximal vessels. No other territories appear to be
involved.
• The abnormal vasculature corresponds to the abnormality
on the CT and MRI. This
raises the possibility of a vasculitic process
accounting for the changes on the CT and in the anteri
or
cerebral artery distribution predominantly callosal marginal
.
Lumbar puncture
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Lymphocytosis
Protein increase
Glucose reduced
Gram stain, ZN stain, India ink,
mycobacterium and fungal cultures were
negative.
• Treated with Acyclovir on admission
Brain biopsy- Rheumatoid
meningitis and vasculitis
• Fibrinoid necrosis
• Surrounded by histiocytes
• Dense infiltrate of plasma cells within the
subarachnoid space
• Vasculitis of the leptomeninges and
underlying cortex
• Staining for bacteria, acid-fast bacillii,
fungi and spirochetes was negative.
Diagnosis
• Definite diagnosis of Rheumatoid
meningitis and vasculitis made
• Patient started on steroids and
immunosuppressants
• Clinical improvement within days
• Discharged home after 7 days.
Rheumatoid Arthritis
• Systemic inflammatory disorder, usually affecting the
joints.
• Extra-articular sites affected, including skin, lungs, eyes
and blood vessels.
• Blood vessels are involved, it is medium and small
vessels of the skin and peripheral nervous system which
are more commonly affected.
• There are case reports of it affecting the brain, causing a
cerebral vasculitis and rarely causes meningitis or
pachymeningitis.
• The neurological sequelae of RA cause death in 18.6%
of cases.
• This is often a diagnosis of exclusion, and brain biopsy
may be required.
Rheumatoid arthritis and the brain
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Nervous system involvement with RA is uncommon.
Neurologic complications are usually due to mass effect on the spinal
cord or peripheral nerves from synovitis, pannus formation or
subluxation
Direct involvement of the CNS includes pachymeningitis,
leptomeningitis and vasculitis.
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.
CSF analysis are usually abnormal, with an elevated protein level,
occasional pleocytosis and a low glucose level.
The diagnosis is one of exclusion, and all other causes of
leptomeningitis and pachymeningitis must be considered
Diagnosis is aided by a clinical diagnosis of RA, positive serologic
results for rheumatoid factors, and the pathologic visualization of
rheumatoid nodules.
Leptomeningitis/ Pachymeningitis
• Lepto/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.
• CNS symptoms can be caused by a CNS vasculitis due to a
lymphoplasmacytic infiltrate in the vessel walls. This involves
both parenchymal and meningeal vessels. Large vessels,
such as the middle cerebral artery, are usually spared.
Leptomeningitis/
Pachymeningitis
• Infection-TB, fungal, cysticercosis,
pyogenic
• Tumour- lymphoma, leukaemia,
carcinomatosis, meningioma
• Inflammation- sarcoid, Whipples, Behcets,
Sjogrens, Wegners, Temporal arteritis, RA
• Idiopathic pachymeningitis
• Intracerebral hypotension.
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Conclusion
• Rare complication of RA
• Need to exclude all other causes of
lepto/pachymeningitis.
• Brain biopsy may be necessary
• Poor prognosis- 6 months
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References
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1. Jones SE, Belsley NA, Mcloud TC,Mullins ME. Rheumatoid Meningitis: Radiologic and
Pathologic correlation. AJR 2006;186:1181-1183.
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2. Tan HJ,Raymond AA, Phadke PP, Rozman Z. Rheumatoid pachymeningitis. Singapore Med J
2004;45(7):337
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3. 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
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4.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.
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Thank you
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