Home Print this page Email this page Small font sizeDefault font sizeIncrease font size
Users Online: 544


Home  | About Us | Editors | Search | Ahead Of Print | Current Issue | Archives | Submit Article | Instructions | Subscribe | Contacts | Login 

Table of Contents
Year : 2013  |  Volume : 3  |  Issue : 1  |  Page : 88-90

Wegener's granulomatosis with subdural hematoma as the initial manifestation

Department of Medicine and Neurosurgery, University College of Medical Sciences and GTB Hospital, New Delhi, India

Date of Web Publication22-Mar-2013

Correspondence Address:
Sarathi Kalra
Research Assistant, University of Texas - MD Anderson Cancer Center and School of Public Health, Houston, TX - 77030, USA

Login to access the Email id

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/2229-5151.109430

Rights and Permissions

Wegener's granulomatosis (WG) is a systemic vasculitis, which can involve any organ system in the body; however, involvement of central nervous system at presentation is very uncommon. Dural involvement in WG has been infrequently reported; however, presentation with subdural hematoma as the initial manifestation is extremely rare. We present a case of WG presenting as subdural hematoma, which resolved on cytotoxic therapy without surgical evacuation.

Keywords: Hemiparesis, meningitis, subdural hematoma, vasculitis, Wegener′s granulomatosis

How to cite this article:
Kalra S, Yadav A, Agarwal S, Kumar S. Wegener's granulomatosis with subdural hematoma as the initial manifestation. Int J Crit Illn Inj Sci 2013;3:88-90

How to cite this URL:
Kalra S, Yadav A, Agarwal S, Kumar S. Wegener's granulomatosis with subdural hematoma as the initial manifestation. Int J Crit Illn Inj Sci [serial online] 2013 [cited 2022 Dec 7];3:88-90. Available from: https://www.ijciis.org/text.asp?2013/3/1/88/109430

   Introduction Top

Wegener's granulomatosis is an uncommon systemic disease characterized by necrotizing vasculitis of small blood vessels with granuloma formation. It commonly involves the upper and lower respiratory tracts and kidney. The involvement of central nervous system in WG is rare. Central nervous system manifestations of WG include cranial neuritis, mononeuritis multiplex, cerebral vasculitis, and meningeal involvement. Subdural hematoma as a manifestation of WG is extremely rare and till date, only one such case has been reported. Here, we present a patient of WG presenting with hemiparesis and subdural hematoma. Hematoma resolved and patient improved on cytotoxic therapy without surgical evacuation.

   Case Report Top

A 42-year-old male presented with complaints of low-grade fever, diffuse headache, and joint pains for 8 weeks. There was no history of head injury. Patient gave history of pain and swelling of knees, ankle, wrist and elbow joints, not associated with morning stiffness. There was history of decreased appetite and loss of 6 kg body weight. He was examined by general practitioner. His physical examination revealed blood pressure - 90/60 mmHg and temperature - 38°C. Systemic examination was normal. Laboratory investigations revealed hemoglobin-14 g/dL, total leukocyte count- 13,200/cmm, and platelet count 4,53,000/cmm. Urine albumin was negative, and urine microscopic examination was normal. Tests for renal, liver, and thyroid function were within normal limits. Peripheral smear for malarial parasite and HIV serology were negative. Blood and urine cultures were sterile. Chest radiography and ultrasound examination of abdomen were normal. Brain MRI revealed subdural hematoma (subacute stage) in left fronto-parietal region [Figure 1] and [Figure 2]. The neurosurgeon advised surgical evacuation of hematoma, but the patient did not give consent. He was given empirical antibiotics and cerebral decongestants. Non-contrast CT scan of head revealed subacute subdural hematoma in the left fronto-parietal region. On 50 th day of his illness, patient developed right hemiparesis, and repeat CT scan of head showed infarct of left basal ganglia, which extended up to the parietal region.
Figure 1: MRI brain T1-weighted image showing left fronto-parietal subdural hematoma with effacement of left side gyri and sulci

Click here to view
Figure 2: MRI brain T2-weighted image showing left fronto-parietal subdural hematoma

Click here to view

The patient presented to the emergency room of our hospital with weakness of right half of body for 7 days. In addition, he gave history of fever, headache, and joint pains for 3 months. There was no history suggestive of chronic upper respiratory symptoms, except for 4-5 episodes of epistaxis about 6 months back, which subsided without any treatment. Patient was conscious and oriented, and power of right upper and lower limbs was grade 3-4/5 with hyper-reflexia. Urinalysis and renal function tests were normal. Detailed investigations for fever were undertaken. Urine and blood cultures were sterile, and malarial parasite was negative. X-ray chest, liver biopsy, and bone marrow biopsy were normal. Ultrasound abdomen revealed mild hepatomegaly with fatty liver and multiple hypoechoic lesions in the substance of spleen. Contrast-enhanced CT scan of abdomen showed irregular wedge-shaped hypodense lesions having attenuation value of 6 × 35 HU in spleen. Patient was empirically put on anti-tubercular treatment; however, he did not respond. Five weeks later, contrast-enhanced CT scan of chest revealed thin-walled cavitary lesions in both upper lobes with parenchymal mass [Figure 3]. Patient also developed evidence of episcleritis. At this stage, cytoplasmic anti-neutrophil cytoplasmic antibody (cANCA) levels were found to be raised. Twenty-four hour urine protein excretion was 2.1 g/day. Blood urea was 52 mg/dL, and serum creatinine was 2.1 mg/dL. Renal biopsy showed evidence of pauci-immune crescentic glomerulonephritis.
Figure 3: CT scan showing cavities in both upper lobes associated with parenchymal lesion

Click here to view

A diagnosis of WG was made, and the patient was treated with 3 doses of intravenous methylprednisolone followed by oral prednisolone and oral cyclophosphamide. Fever subsided within 10 days of initiation of immunosuppressants, and renal functions improved to normal level within 2 weeks. Cyclophosphamide was replaced by mycophenolate mofetil after 6 months, and dose of prednisolone was tapered to 10 mg per day. MRI brain repeated after 8 months of follow-up did not show any subdural hematoma. The patient is on regular follow-up and for last 1 year; he is largely asymptomatic, except for mild residual right hemiparesis.

   Discussion Top

This patient initially presented with fever, headache, and joint pains and had fronto-parietal subdural hematoma on imaging. Organ systems commonly involved in WG such as paranasal sinuses, lungs, kidneys, and skin were not involved. Subdural hematoma is an exceedingly rare manifestation of WG. We could find only one case report of WG presenting as subdural hematoma. [1] In this case, MRI scan was suggestive of subdural hematoma or empyema, hence subdural drainage was done, which revealed pus-like fluid. The diagnosis of WG in this case was established retrospectively. Shiotani et al. reported a case that presented with fever and severe headache, and CT and MRI of head revealed subdural masses. [2] The diagnosis in this case was established by needle biopsy of nasal polyps and positive cANCA. WG presenting as meningitis has also been reported but is also very rare. [3],[4] It has been reported that meningeal involvement usually develops in patients with localized disease who are more likely to have destructive lesions of upper airways. [5] In our case, the diagnosis of WG was somewhat delayed on account of atypical presentation. The diagnosis was made after detection of characteristic finding on CT scan of chest and test for cANCA. In our patient, the development of cerebral infarct and subdural hematoma appear to be a manifestation of CNS vasculitis due to WG.

WG is a devastating disease; hence, high index of suspicion and timely treatment is warranted. In patients with subdural hematoma without history of head injury, CNS vasculitis due to WG, though very rare, may be considered as one of the possibilities.

   References Top

1.Yokote H, Terada T, Nakai K, Itakura T. Subdural and meaningful involvement related to Wegener's granulomatosis: Case report. Neurosurgery 1997;40:1071-3.  Back to cited text no. 1
2.Shiotani A, Mukobayashi C, Oohata H, Yamanishi T, Hara T, Itoh H, . Wegener's granulomatosis with dural involvement as the initial clinical manifestation. Intern Med 1997;36:514-8.  Back to cited text no. 2
3.Al Dhanhani A, Macaulay R, Maloney B, Hanly JG. Meningeal involvement in Wegener's granulomatosis. J Rheumatol 2006;33:364-7.  Back to cited text no. 3
4.Yasuhara T, Fukuhara T, Nakagawa M, Terai Y, Yoshino K, Mizobuchi K, et al. Wegener granulomatosis manifesting as meningitis. Case report. J Neurosurg 2002;97:1229-32.  Back to cited text no. 4
5.Ayala de la Cruz Mdel C, González Díaz R, López Lara ND. Wegener's granulomatosis. Report of a pediatric case and review of the literature. Rev Alerg Mex 2003;50:71-5.  Back to cited text no. 5


  [Figure 1], [Figure 2], [Figure 3]

This article has been cited by
1 Lessons of the month: ANCA-associated vasculitis – granulomatosis with polyangiitis: ‘the great mimic’
James Hesford,Andrew RL Medford,Harsha Gunawardena
Clinical Medicine. 2021; 21(2): e231
[Pubmed] | [DOI]
2 Untypical Manifestations of Granulomatosis with Polyangiitis—A Review of the Literature
Joanna Milkowska-Dymanowska,Paulina Laskowska,Mikolaj Rzuczkowski,Adam J. Bialas,Wojciech J. Piotrowski,Pawel Górski
SN Comprehensive Clinical Medicine. 2019;
[Pubmed] | [DOI]
3 Hypertrophic pachymeningitis and subdural haematoma in an otitis media with ANCA-associated vasculitis patient successfully treated with intravenous cyclophosphamide
Misaki Yasutake,Naoichiro Yukawa,Yoshifumi Iwahashi,Masakazu Fujimoto,Kayoko Tabata,Ryo Matsumiya,Katsunori Tanaka,Takao Fujii
Modern Rheumatology Case Reports. 2019; : 1
[Pubmed] | [DOI]
4 Rheumatoid meningitis can present MRI findings that mimic chronic subdural haematoma
Kaoru Yagita,Akiyo Shinde,Toshihiko Suenaga
BMJ Case Reports. 2019; 12(8): e229642
[Pubmed] | [DOI]
5 Eosinophilic Granulomatosis with Polyangiitis and Spinal Subdural Hematoma: A Case-Based Review of the Literature
Saeedeh Shenavandeh,Mehdi Eazadnegahdar,Mohammad Ali Nazarinia,Shiva Najafzadeh,Fariba Zarei
Iranian Red Crescent Medical Journal. 2018; In Press(In Press)
[Pubmed] | [DOI]
6 Neurological Manifestations of Rheumatic disorders
Svetlana Lvovich,Donald P. Goldsmith
Seminars in Pediatric Neurology. 2016;
[Pubmed] | [DOI]
7 Subdural Hematoma Mimickers: A Systematic Review
Dragos Catana,Alex Koziarz,Aleksa Cenic,Siddharth Nath,Sheila Singh,Saleh A. Almenawer,Edward Kachur
World Neurosurgery. 2016; 93: 73
[Pubmed] | [DOI]
8 Granulomatosis with polyangiitis: Indian experience
Ashok Kumar,Anil Abrol
Indian Journal of Rheumatology. 2015; 10: S59
[Pubmed] | [DOI]
Saloomeh Saati,Srinivas R. Sadda
Retinal Cases & Brief Reports. 2014; 8(4): 236
[Pubmed] | [DOI]


    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
    Access Statistics
    Email Alert *
    Add to My List *
* Registration required (free)  

  In this article
   Case Report
    Article Figures

 Article Access Statistics
    PDF Downloaded90    
    Comments [Add]    
    Cited by others 9    

Recommend this journal