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Table of Contents
CASE REPORT
Year : 2013  |  Volume : 16  |  Issue : 3  |  Page : 411-413
 

Chronic meningitis with multiple cranial neuropathies: A rare initial presentation of Wegener's granulomatosis


Department of Neurology, Sawai Man Singh Medical College, Jaipur, Rajasthan, India

Date of Submission02-Apr-2012
Date of Decision29-Apr-2012
Date of Acceptance05-Aug-2012
Date of Web Publication26-Aug-2013

Correspondence Address:
Anjani Kumar Sharma
36, Ram Gali No 7, Raja Park, Jaipur, Rajasthan - 302 004
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0972-2327.116920

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   Abstract 

Wegener's granulomatosis (WG) is a systemic necrotizing vasculitis that affects the small blood vessels. It mainly affects the upper and lower respiratory tract and kidneys. Central nervous system (CNS) involvement is rare, and has been reported only in about 8% of cases during the course of illness. Initial presentation with neurologic affection, particularly chronic hypertrophic meningitis is very unusual. We report the case of a 34 year old male who presented with chronic hypertrophic meningitis and multiple cranial nerve involvement as the initial manifestation, without respiratory and renal symptoms. This case highlights the difficulties in diagnosing a rare disease with rarer presentation, and at the same time illustrates that Wegener's granulomatosis should be considered in the differential diagnosis of chronic meningitis.


Keywords: Chronic meningitis, cranial neuropathies, Wegener′s granulomatosis


How to cite this article:
Gupta V, Sharma AK, Sureka RK, Bhuyan SK, Singh PK. Chronic meningitis with multiple cranial neuropathies: A rare initial presentation of Wegener's granulomatosis. Ann Indian Acad Neurol 2013;16:411-3

How to cite this URL:
Gupta V, Sharma AK, Sureka RK, Bhuyan SK, Singh PK. Chronic meningitis with multiple cranial neuropathies: A rare initial presentation of Wegener's granulomatosis. Ann Indian Acad Neurol [serial online] 2013 [cited 2019 Jun 15];16:411-3. Available from: http://www.annalsofian.org/text.asp?2013/16/3/411/116920



   Introduction Top


Wegener's granulomatosis (WG) is a systemic necrotizing vasculitis that mainly affects the upper and lower respiratory tract and kidneys. [1] Central nervous system (CNS) involvement is rare, and has been reported only in about 8% of cases during the course of illness. [2] Initial presentation with CNS affection, particularly chronic hypertrophic meningitis is even rarer. Very few cases of Wegener's granulomatosis have been reported with such initial presentation.


   Case Report Top


A 34 year old male presented to our department with two months duration of headache, right earache, and vomiting. Two weeks later, he developed right facial numbness, inability to close the right eye, deviation of the mouth to left, loss of taste sensation in the right half of tongue, slurring of speech with nasal intonation, and difficulty in swallowing with nasal regurgitation of liquids. There was no history of weakness or sensory impairment in limbs, blurring of vision, hearing impairment, cough, fever or loss of appetite. There was no history of ear discharge, joint pains or rashes. He had a history of recurrent mild sinusitis since last 10 years; however, it was never serious enough to warrant medical intervention. He had no history of hypertension, diabetes, tuberculosis, or any other chronic illness.

His vitals and general physical examination were normal. Fundus examination did not reveal any abnormality. He had right 5 th and 7 th , and bilateral 9 th , 10 th cranial nerve palsies. There were no signs of meningeal irritation. Rest of the neurological examination was unremarkable. Systemic examination, including respiratory system was normal. He had undergone evaluation elsewhere prior to admission with us. His hemogram, fasting blood sugar, renal functions, liver functions and serum electrolytes were within the normal limits. Chest radiograph did not reveal any abnormality. Cerebrospinal fluid (CSF) analysis showed lymphocytic pleocytosis, with normal sugar and proteins. Magnetic resonance imaging (MRI) brain showed an inflammatory duromeningeal process with asymmetric and prominent enhancement in the right hemisphere suggestive of hypertrophic pachymeningitis [Figure 1]. He was treated with anti tubercular therapy (ATT) and oral steroids; however, there was no improvement in the response.

After hospitalization, we re-evaluated him to diagnose an alternative etiology. His erythrocyte sedimentation rate (ESR) was 50 mm/h (normal- 0-15 mm/h). The test results for antinuclear antibodies (ANA), Human immunodeficiency virus (HIV), hepatitis B surface antigen (HBsAg), Venereal Disease Research Laboratory (VDRL) and brucella serology were negative. Serum Angiotensin Converting Enzyme (ACE) level was within normal limit. Urine examination revealed microscopic hematuria with mild proteinuria. Repeat CSF analysis showed lymphocytic pleocytosis with moderately raised proteins and no atypical (malignant) cells were found. Bacterial antigen test, gram staining, Acid-fast bacilli (AFB) staining, India ink preparation and culture for bacteria and fungus were negative. CSF tuberculosis polymerase chain reaction (TB PCR)and VDRL test were negative. CSF ACE level was normal.
Figure 1: Magnetic resonance imaging brain of the patient in the study showing dural thickening and abnormal enhancement

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Computed Tomography (CT) thorax was planned to look for possible evidence of pulmonary tuberculosis and sarcoidosis. It showed multiple cavitating nodular opacities in the right lung field [Figure 2]. The otolaryngologist assessed him for earache, and advised CT scan of the temporal bones and paranasal sinuses, which then revealed bilateral mastoiditis and chronic rhino-sinusitis [Figure 3] and [Figure 4]. With these radiologic findings, a strong possibility of WG was suspected, which was further confirmed by positive Cytoplasmic antineutrophil cytoplasmic antibodies (c-ANCA) staining and nasopharyngeal biopsy which revealed an ulcer with chronic granulomatous inflammation [Figure 5]. He was put on oral cyclophosphamide, and the dosage of prednisolone was increased. Headache, vomiting, and earache disappeared within days. On follow up, the patient has displayed significant clinical improvement.
Figure 2: Computed Tomography scan of the thorax of the patient in the study showing multiple cavitating nodular opacities in right lung field

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Figure 3: Non Contrast Computed Tomography scan of the temporal bone of the patient in the study showing bilateral opaque mastoid air cells and tympanic cavities

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Figure 4: Non Contrast Computed Tomographyscan the paranasal sinuses of the patient in the study showing chronic rhino-sinusitis

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Figure 5: Nasopharyngeal biopsy of the patient in the study showing chronic granulomatous inflammation

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   Discussion Top


WG is a rare disease. It was initially described as a granulomatous affection of the upper and lower respiratory tract and kidney. Currently it is recognized as a necrotising, small vessel vasculitis that can involve nearly every organ or tissue in the body. [3] More than 90% of the patients initially present with upper or lower airway symptoms or both. [2] Neurological involvement during the course of the disease is usually in the form of mononeuritis multiplex and cranial neuropathy. Headache, cerebritis, seizures, stroke, meningitis, diabetes insipidus, hydrocephalus, external ophthalmoplegia, myelopathy, and myopathy are other manifestations which are seen less frequently. [4] CNS involvement usually occurs during the later stages of the disease, [3] and is seen in about 8% of the cases. Three mechanisms are involved in the pathogenesis of the different CNS manifestations seen in WG: a) vasculitis of the nervous system; b) contiguous invasion of granuloma from extracranial sites; c) and, remote intracranial granuloma. [4] Our patient presented with chronic hypertrophic pachymeningitis with multiple cranial neuropathies without any classical symptoms of WG. This unusual presentation delayed the diagnosis. He was initially given ATT considering the possibility of neurotuberculosis, which is endemic in our country and may present with similar features. [5] However, in absence of appropriate response he was investigated further, and eventually the diagnosis of WG was established. Meningeal involvement at any stage of disease is rare (1%). [2],[6] In a large case series of 324 patients of WG, meningitis was found only in 2 patients. [6] In another series of 158 patients, none of the patients was found to have meningitis. [2]

Untreated WG universally has a fatal course. Treatment with a combination of glucocorticoids and cyclophosphamide leads to a marked symptomatic improvement in more than 90% of patients, and 75% of patients achieve complete remission. [1] We also found excellent response to this combination treatment in our patient. In WG with chronic meningitis, an early diagnosis and proper treatment may prevent irreversible cranial nerve dysfunction and other sequelae, like communicating hydrocephalus. [4]


   Conclusion Top


Chronic meningitis with multiple cranial neuropathies may be the initial presenting feature of Wegener's granulomatosis. Such presentation is rare and needs a high degree of suspicion as early diagnosis and treatment can prevent morbidity and mortality.

 
   References Top

1.Langford CA, Fauci AS. The Vasculitis Syndromes. Harrison's Principles of Internal Medicine. 17 th ed, Vol 2. New York: The McGraw-Hill Companies, Inc.; 2008. p. 2121-4.  Back to cited text no. 1
    
2.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. 2
[PUBMED]    
3.Dörr J, Elitok S, Dieste FJ, Vogel HP, Schneider W, Wuerfel J, et al. Treatment-resistant chronic headaches and focal pachymeningitis in a 46-year-old man: A rare presentation of Wegener's granulomatosis. Lancet Neurol 2008;7:368-72.  Back to cited text no. 3
    
4.Jinnah HA, Dixon A, Brat DJ, Hellmann DB. Chronic meningitis with cranial neuropathies in Wegener's granulomatosis. Case report and review of the literature. Arthritis Rheum 1997;40:573-7.  Back to cited text no. 4
[PUBMED]    
5.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. 5
[PUBMED]    
6.Nishino H, Rubino FA, DeRemee RA, Swanson JW, Parisi JE. Neurological involvement in Wegener's granulomatosis: An analysis of 324 consecutive patients at the Mayo Clinic. Ann Neurol 1993;33:4-9.  Back to cited text no. 6
[PUBMED]    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]


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