A 24-year-old woman presented with history of recurrent episodic right hemicranial headache and seizures of 3 years duration. She also conceded that she used to get vertiginous sensations before the onset of generalized tonic clonic seizures each time. This time she presented with right hemicranial headache of different character, which was more severe and associated with retro-orbital pain 15 days before admission. Ten days after the onset of headache with different character, she noticed double vision followed by progressive drooping of right eye lid. General examination showed asymmetrical upper limb pulses, left radial, and brachial pulse being feeble. Her blood pressure was 170/110 mm Hg on recording from the right upper limb, while on left it was 90/60 mm Hg. In addition, there was evidence for bilateral renal bruit (left more than right). Neurological examination revealed right third and fourth cranial nerve palsies, and the remainder of the examination was normal.
Routine urine analysis, complete blood counts, and serum biochemistry (liver and kidney functions, glucose level, and lipid panel) were normal, except for an elevated erythrocyte sedimentation rate (ESR) of 88 mm at the end of an hour. Vasculitic work up in the form of rheumatoid factor, antinuclear antibodies, anti-double stranded DNA antibody, antiphospholipid antibody, proteinase-3-antineutrophil cytoplasmic antibody (ANCA) and myeloperoxidase-ANCA were negative. Serology for syphilis (Venereal Disease Research Laboratory [VDRL] test), HIV, and hepatitis B and C was negative. Chest X-ray, serum calcium, and angiotensin-converting enzyme levels were normal. Tuberculin skin test as well as Quantiferon-TB test were negative. Cerebrospinal fluid (CSF) analysis showed 2 lymphocytes/mm3, protein 75 mg/dl, and glucose 78 mg/dl (serum glucose-112 mg/dl). Cytological examination of the CSF did not show any malignant cell. CSF microbiology in the form of gram staining, acid fast bacilli (AFB) staining, VDRL, panfungal antigen, and cultures for AFB and fungi did not yield positive results, so was polymerase chain reaction for mycobacterium tuberculosis.
Magnetic resonance imaging of the brain revealed abnormal, thickened enhancing pachymeninges in bilateral frontal and right temporal region extending to cavernous sinus [Figures 1 a and b]. In addition, there was T2 and FLAIR (Fluid Attenuated Inversion Recovery) hyperintensity in right temporal lobe [Figure 2]. In view of feeble left radial pulse and renal bruit, patient underwent conventional digtal subtraction angiography (DSA), for better characterization of the vascular system. DSA revealed stenosis of left subclavian artery distal to the origin of vertebral artery, osteal stenosis of left renal artery, and narrowing of abdominal aorta at the level of the origin of renal arteries with normal terminal aorta and bilateral common iliac arteries [Figure 1 c and d]. Transthoracic two-dimensional echocardiography revealed mild aortic regurgitation with normal left ventricular function. Electroencephalogram showed intermittent epileptiform discharges over the right temporal region. Meningeal biopsy could not be performed as the patient declined the procedure. A diagnosis of TA was considered as patient satisfied the American College of Rheumatology (ACR) criteria.
After starting prednisolone (1 mg/kg/day), there was complete recovery from right third and fourth cranial nerve palsy over a period of 3 weeks along with normalization of ESR. Her blood pressure was well controlled on atenolol, while seizures on 300 mg of phenytoin. However, asymptomatic blood pressure difference in upper limbs was persistent. She was continued on phenytoin, atenolol, and low-dose prednisolone (tapered over a period of 12 weeks to 10 mg alternate days). She remained asymptomatic on these medications at 1-year follow-up. A postcontrast computed tomography (CT) scan at 6 months follow-up was normal and there was no evidence for meningeal enhancement. Her laboratory evaluation including ESR and renal function tests were within normal limits at the last follow-up. As patient remained asymptomatic and did not show signs of vascular insufficiency, a repeat angiogram, being an invasive test, was not considered.