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Table of Contents    
Year : 2011  |  Volume : 59  |  Issue : 6  |  Page : 915-916

Idiopathic hypertrophic pachymeningitis successfully treated with intravenous cyclophosphamide

1 Department of Neurology, Changzhou Second People's Hospital, Nanjing Medical University, Changzhou, China
2 Department of Neurology, The First Affiliated Hospital with Nanjing Medical University, Nanjing, Jiangsu, China

Date of Submission13-Nov-2011
Date of Decision14-Nov-2011
Date of Acceptance17-Nov-2011
Date of Web Publication2-Jan-2012

Correspondence Address:
Ding Xinsheng
Department of Neurology, The First Affiliated Hospital with Nanjing Medical University, Nanjing, Jiangsu
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0028-3886.91384

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How to cite this article:
Zhuoyou C, Chuanzhong Q, Xinsheng D. Idiopathic hypertrophic pachymeningitis successfully treated with intravenous cyclophosphamide. Neurol India 2011;59:915-6

How to cite this URL:
Zhuoyou C, Chuanzhong Q, Xinsheng D. Idiopathic hypertrophic pachymeningitis successfully treated with intravenous cyclophosphamide. Neurol India [serial online] 2011 [cited 2020 May 25];59:915-6. Available from:


Idiopathic hypertrophic pachymeningitis (IHP) is a rare chronic inflammatory disorder that causes fibrous thickening of the dura mater, and chronic headache and multiple cranial neuropathies are the most common clinical manifestations. [1] Medical treatment of IHP remains controversial. We report a patient with IHP who responded to intravenous cyclophosphamide.

A 60-year-old man presented with headache, hearing and visual impairment and right facial paralysis. His symptoms began one year before with episodic headache which subsequently increased in severity and frequency. Neurological examination revealed bilateral papilledema with decreased visual acuity on the right side and hearing loss and facial palsy on the right side. Contrast magnetic resonance imaging (MRI) revealed 10 to 12 mm thick markedly enhancing dura over the right cerebral hemisphere extending to the tentorium [Figure 1]. He underwent right frontal craniotomy and biopsy of the dura mater. Histological examination showed dense fibrous tissue, numerous lymphocytes and few plasma cells [Figure 2]. Erythrocyte sedimentation rate (ESR) during the first hour was 115 mm and c-reactive protein (CRP) was 61.4 mg/dl. Lumbar puncture revealed an opening pressure of >40 cmH 2 O and total protein was 7.23 g/dl. Workup for other causes of pachymeningitis was negative. Patient was treated initially with high-dose corticosteroid, 0.5 g methylprednisolone per day for five days, followed by 1 mg/kg oral daily prednisone with gradual tapering over a period of five months. There was a dramatic improvement of headache and focal neurological deficits. Within one week of stopping steroids, patient was hospitalized for exacerbation of neurological deficits. Repeat MRI showed no change in the size and thickness of the previous lesion. This time we decided to try a low-dose intravenous cyclophosphamide schedule, 400 mg twice weekly for two weeks first. The patient showed improvement in neurological deficits within one week of the first dose of cyclophosphamide. We tapered intravenous cyclophosphamide over a six-month period: for two months with 400 mg weekly and for four months with 400 mg every two weeks. MRI performed two months after having initiated the weekly intravenous cyclophosphamide schedule showed that the dural lesion had become thinner in size and contrast enhancement had markedly reduced [Figure 3]. During the course of the treatment the patient reported minimal side-effects. The neurological deficits improved steadily.
Figure 1: Pretreatment pachymeningeal thickening is hypointense or normointense on T1-, and hyperintense on T2-weighted sequences. The gadolinium-enhanced T1-weighted sequences demonstrate a marked enhancement of the dural lesions, extending to the tentorium on the right

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Figure 2: Photomicrograph showing hypertrophic collagenised tissue with infiltration by lymphocytes and plasma cells. a) H and E, x40, b) H and E, x100

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Figure 3: MRI performed two months after having initiated the weekly intravenous cyclophosphamide scheme, shows that the dural lesion has become thinner and enhancement has markedly reduced

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Very interesting and imaginative treatment approaches have been proposed in the treatment of IHP. However, steroid therapy is the most widely used pharmacological treatment. High-dose corticosteroid is usually effective and clinical improvement is excellent initially but in some cases there may be recurrences or progression of the disease. Additional therapy with azathioprine, cyclophosphamide or methotrexate may be useful and radiation therapy has also been given. [1],[2],[3] Cyclophosphamide is an anticancer (antineoplasticnone ) agent and it also has an immunosuppressive effect. Intravenous cyclophosphamide pulse therapy is an effective treatment for various autoimmune diseases such as systemic vasculitides, [4] rapidly progressive glomerulonephritis, systemic lupus erythematosus (SLE), [5] systemic rheumatoid vasculitis, [6] and multiple sclerosis. [7] The intermittent intravenous route is believed to be less toxic than oral daily treatment. In our experience with this case, this treatment schedule is effective and well tolerated, and may induce complete remission of the disease.

Nevertheless, the low frequency of IHP precludes designing a large randomized controlled trial to prove the safety and efficacy of cyclophosphamide at different doses and through different routes of administration. This communication should be considered only as a hypothesis-generating work waiting for systematic confirmation of the efficacy and safety of cyclophosphamide in IHP.

  References Top

1.Kupersmith MJ, Martin V, Heller G, Shah A, Mitnick HJ. Idiopathic hypertrophic pachymeningitis. Neurology 2004;62:686-94.  Back to cited text no. 1
2.Mamelak AN, Kelly WM, Davis RL. Idiopathic hypertrophic cranial pachymeningitis: Report of three cases. J Neurosurg 1993;79:270-6.  Back to cited text no. 2
3.Masson C, Henin D, Hauw JJ, Rey A, Raverdy P, Masson M. Cranial pachymeningitis of unknown origin: A study of seven cases. Neurology 1993;43:1329-34.  Back to cited text no. 3
4.Fort JG, Abruzzo JL. Reversal of progressive necrotizing vasculitis with intravenous pulse cyclophosphamide and methylprednisolone. Arthritis Rheum 1988;31:1194-8.  Back to cited text no. 4
5.Belmont HM, Storch M, Buyon J, Abraham S. New York university hospital for joint disease experience with intravenous cyclophosphamide treatment: Efficacy in steroid unresponsive lupus nephritis. Lupus 1995;04:104-8.  Back to cited text no. 5
6.Scott DG, Bacob PA. Intravenous cyclophosphamide plus methylprednisolone in the treatment of systemic rheumatoid vasculitis. Am J Med 1984;76:377-84.  Back to cited text no. 6
7.Killian JM, Bressler RB, Arustrong RM, Huston DP. Controlled pilot trial of monthly intravenous cyclophosphamide in mutiple sclerosis. Arch Neurol 1980;45:27-30.  Back to cited text no. 7


  [Figure 1], [Figure 2], [Figure 3]

This article has been cited by
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