Wegener's granulomatosis with subdural hematoma

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Case Report

DOI: 10.4103/2229-5151.109430 Quick Response Code:

Wegener’s granulomatosis with subdural hematoma as the initial manifestation Sarathi Kalra, Anil Yadav, Sunil Agarwal, Sunil Kumar

ABSTRACT 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. Key Words: Hemiparesis, meningitis, subdural hematoma, vasculitis, Wegener’s granulomatosis

INTRODUCTION 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 A 42-year-old male presented with complaints of lowgrade 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 88

Departments of Medicine and Neurosurgery, University College of Medical Sciences and GTB Hospital, New Delhi, India Address for correspondence: Dr. Sarathi Kalra, Research Assistant, University of Texas - MD Anderson Cancer Center and School of Public Health, Houston, TX, USA- 77030, E-mail: [email protected]

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 [Figures 1 and 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 50th 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. 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

International Journal of Critical Illness and Injury Science | Vol. 3 | Issue 1 | Jan-Mar 2013

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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 pauciimmune crescentic glomerulonephritis.

Figure 1: MRI brain T1-weighted image showing left fronto-parietal subdural hematoma with effacement of left side gyri and sulci

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 followup and for last 1 year; he is largely asymptomatic, except for mild residual right hemiparesis.

DISCUSSION

Figure 2: MRI brain T2-weighted image showing left fronto-parietal subdural hematoma

Figure 3: CT scan showing cavities in both upper lobes associated with parenchymal lesion

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, contrastenhanced CT scan of chest revealed thin-walled cavitary

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.


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Cite this article as: 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. Source of Support: Nil. Conflict of Interest: No.
