Wegener's granulomatosis with subdural hematoma as the initial manifestation
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 [Figures and ]. 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.\nThe 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 []. 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.\nA 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.