Chronic meningitis with multiple cranial neuropathies: A rare initial presentation of Wegener's granulomatosis
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.\nHis vitals and general physical examination were normal. Fundus examination did not reveal any abnormality. He had right 5th and 7th, and bilateral 9th, 10th 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 []. He was treated with anti tubercular therapy (ATT) and oral steroids; however, there was no improvement in the response.\nAfter 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.\nComputed 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 []. 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 [Figures and ]. 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 []. 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.