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Table of Contents    
Year : 2012  |  Volume : 60  |  Issue : 4  |  Page : 424-426

Hypertrophic pachymeningitis and cerebral infarction resulting from ANCA-associated vasculitis

Department of Neurology, Zhongshan Hospital, Fudan University, Shanghai, China

Date of Web Publication6-Sep-2012

Correspondence Address:
Xin Wang
Department of Neurology, Zhongshan Hospital, Fudan University, Shanghai
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0028-3886.100711

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How to cite this article:
Peng W, Wang X. Hypertrophic pachymeningitis and cerebral infarction resulting from ANCA-associated vasculitis. Neurol India 2012;60:424-6

How to cite this URL:
Peng W, Wang X. Hypertrophic pachymeningitis and cerebral infarction resulting from ANCA-associated vasculitis. Neurol India [serial online] 2012 [cited 2023 Sep 30];60:424-6. Available from:


Involvement of the nervous system is relatively rare in idiopathic antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (AAV), and only sporadic case reports of hypertrophic pachymeningitis (HP) and involvement of cerebral parenchyma have been documented. The clinical features of this systemic disease have not been fully recognized. [1],[2]

A 58-year-old man was admitted with recurrent headaches and double vision of a nine-month duration. He received prednisone treatment twice, with transient alleviation of symptoms, but the symptoms recurred on discontinuation of prednisone. Physical examination revealed incomplete bilateral III, right VI, and bilateral IX and X palsy, mild motor weakness in both the lower limbs, left side ataxia, meningeal signs, and tenderness of the gastrocnemius. Brain magnetic resonance imaging (MRI) demonstrated diffuse thickening of the dura, with enhancement after gadolinium administration [Figure 1]a,b and c, infarcts [Figure 2]a,b and MRI showing significantly diminished dural incrassation [Figure 1] d. A complete blood picture showed white blood cell: 13.7 x 10 9 / L, platelet: 495 x 10 9 / L, eosinophilic granulocyte: 0.79 x 10 9 / L (0.05 - 0.5 x 10 9 / L), and hemoglobin: 95 g / L. Serum albumin was 26 g / L (35 - 52 g / L) and g-globin was 30.7% (11.8 - 18.8%). Erythrocyte sedimentation rate was 114 mm / h, C-reactive protein was 6.8 mg / L (0 - 3.0 mg / L), and the rheumatoid factor was 266 IU / L (< 14 IU / ml), respectively. p-ANCA was positive with high, 98.5 RU / ml (< 20RU / ml), myeloperoxidase (MPO). Other autoimmune antibodies, such as, c-ANCA, antinuclear antibody, anticardiolipin antibody, dsDNA, SSA, and SSB, were negative. Investigations for tuberculosis and fungal infections were negative. Opening cerebrospinal fluid (CSF) pressure was 210 mmH 2 O and CSF analysis showed a red cell count of 2 / mm 3 , white cell count of 8 / mm 3 , protein of 0.94 (0.15 - 0.45 g / L), normal glucose and chloride, negative cultures for bacteria, acid-fast bacilli and fungi, and absent tumor cells. Chest computed tomography (CT) showed inflammatory disease in the bilateral lungs. Electromyogram (EMG) findings of the lower limb muscles were suggestive of inflammatory muscle disease. Whole body positron emission tomography (PET) / CT study did not reveal any tumor. Furthermore, the shapes of both kidneys were increscent and Fluorine-18 fluorodeoxyglucose (FDG) metabolism was diffusely elevated in both the renal cortices. Meningeal biopsy showed thickened dura with dense collagen fibrous tissue, with focal collapse and granular necrosis [Figure 3]a. There were foci of cell infiltrates with lymphocytes, macrophages, and a few neutrophils [Figure 3]b. No granulomatosis was found. Silver methenamine and Periodic acid-Schiff (PAS) staining were negative for fungus ([Figure 3]c and d). A repeat brain MRI showed a subacute infarct adjacent to the left lateral ventricle [Figure 1]c, [Figure 2]a and b.

The laboratory and imaging data and the meningeal biopsy features were suggestive of AAV. The PET / CT findings could go with renal involvement and post-biopsy MRI findings could suggest cerebral involvement. The patient was given methylprednisolone pulse therapy (500 mg / day x 4 days) and subsequent oral prednisone (60 mg / day), followed by tapering. Cyclophosphamide was used for three months (0.8 per month). Oral prednisone and methotrexate (15 mg weekly) were maintained at discharge. The patient had been followed up for 24 months and the symptoms of headache, double vision, gait disturbance, and pain in bilateral legs subsided, but signs of cranial nerve palsies still partly remained. The erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), perinuclear antineutrophil cytoplasmic antibodies (p-ANCA), myeloperoxidase (MPO), blood regular test, and serum albumin, returned to normal. The brain MRI showed significant reduction in dural incrassation [Figure 1]d.
Figure 1: (a) Diffuse thickening of cerebral dura maters (T1W imaging, the arrows point to thickened cerebral falx and tentorium of the cerebellum, respectively) (b) Diffuse dural enhancement (axial T1W post-contrast MRI) (c) A subacute infarction beside the left lateral ventricle (axial DWI imaging) (d) Dural incrassation significantly diminished (T1W imaging)

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Figure 2: (a) An infarct beside the left lateral ventricle (axial T2W imaging) (b) An infarct beside the left lateral ventricle (axial Flair imaging)

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Figure 3: (a) Dense collagen fibrous tissue with pathological changes of focal collapse and granular necrosis, (H and E staining, × 400); (b) A focus infiltrated by many inflammatory cells, (H and E staining, × 400); (c, d) Special stains (silver staining and PAS staining, × 400) for detecting fungi were negative

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ANCA-associated vasculitis (AAV) is a systemic disease with very complex clinical manifestations involving multiple organs and there are no well-established diagnostic criteria for the diagnosis of AAV. Thus, it is often difficult to confirm the diagnosis when the presentation is not so typical. The kidneys, lungs, skin, and joints are the most commonly and initially affected organs in 70-80% of the cases. [3] Presentation with neurological features as the initial manifestation is not so common and in such a scenario the differentials include meningitis of infective etiology, the Tolosa-Hunt syndrome, dural carcinomatosis, cavernous sinus diseases, orbital apex syndromes, and intracranial hypotension. [4],[5] Cerebral infarction secondary to AAV is also fairly rare. In cases of AAV, the distal branches of the arteries are mostly involved and the infarcts are multiple, mainly affecting the white matter; sometimes hemorrhagic lesions can also be seen. A cerebral angiography is often negative. In this patient the lesion seen in the post-biopsy MRI was an infarct secondary to AAV, because of the size and the location in the white matter. Rapid disease remission can be achieved by any of the treatment strategies that include methylprednisolone pulse therapy, cyclophosphamide, plasma exchange, and rituximab, which contribute to rapid remission. [6],[7] These patients need maintenance treatment with prednisone and other immunosuppressive agents such as methotrexate and azathioprine for a long period, to avoid relapses. The disease usually has a chronic relapsing and remitting course. Poor prognosis is associated with age-associated comorbidities and early renal and pulmonary involvement. [8]

 » References Top

1.Ozkul A, Tataroglu C, Kiylioglu N, Akyol A, Tataroglu C. Microscopic polyangiitis presenting with medullary infarct. J Neurol Sci 2011;300:173-5.  Back to cited text no. 1
2.Ghinoi A, Zuccoli G, Pipitone N, Salvarani C. Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis involving the central nervous system: Case report and review of the literature. Clin Exp Rheumatol 2010;28:759-66.  Back to cited text no. 2
3.Hoffman GS, Kerr GS, Leavitt RY, Hallahan CW, Lebovics RS, Travis WD, et al. Wegener granulomatosis: An analysis of 158 patients. Ann Intern Med 1992;116:488-98.  Back to cited text no. 3
4.Shobha N, Mahadevan A, Taly AB, Sinha S, Srikanth SG, Satish S, et al. Hypertrophic cranial pachymeningitis in countries endemic for tuberculosis: Diagnostic and therapeutic dilemmas. J Clin Neurosci 2008;15:418-27.  Back to cited text no. 4
5.Muthukumar N, Senthilbabu S, Usharani K. Idiopathic hypertrophic cranial pachymeningitis masquerading as Tolosa-Hunt syndrome. J Clin Neurosci 2005;12:589-92.  Back to cited text no. 5
6.Hamour S, Salama AD, Pusey CD. Management of ANCA-associated vasculitis: Current trends and future prospects. Ther Clin Risk Manag 2010;6:253-64.  Back to cited text no. 6
7.Stone JH, Merkel PA, Spiera R, Seo P, Langford CA, Hoffman GS, et al. Rituximab versus cyclophosphamide for ANCA-associated vasculitis. N Engl J Med 2010;363:221-32.  Back to cited text no. 7
8.Bosman T, Simonin C, Launay D, Caron S, Destée A, Defebvre L, et al. Idiopathic hypertrophic cranial pachymeningitis treated by oral methotrexate: A case report and review of literature. Rheumatol Int 2008;28:713-8.  Back to cited text no. 8


  [Figure 1], [Figure 2], [Figure 3]

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