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Pediatric Oncall Journal

Takayasu Arteritis with Status Epilepticus 01/09/2014 00:00:00

Takayasu Arteritis with Status Epilepticus

Prakash C. Vaghela, Naresh B. Gohel, Rajenddra Kabaria.
Kalarav Children Hospital, Kushal Neonatal Nursery, Bhavnagar, Gujarat, India.

Dr Prakash C Vaghela, Neonatal intensivist.
Twelve years old female child fourth in birth order born of 2 nd degree consanguineous marriage presented with headache and four episodes of right sided focal seizures with secondary generalization last 2 hours. She had similar episode before 1 month, was hospitalized and was on regular anticonvulsant valproate. There is no contact with tuberculosis. On examination, weight was 37kg, height 110cm, the right and left radial, brachial pulses were absent. On auscultation, bruit was present over carotid arteries. The right and left carotid, femoral, and other pulses were palpable. Non-invasive blood pressure on right upper limb was 130/100/109 (systolic/ diastolic/mean mm of Hg), right lower limb 146/112/126, left upper limb139/91/112 and left lower limb 155/106/119. Ophthalmic examination revealed hypertensive retinopathy grade III. She had hepatosplenomegaly.

Her hemogram, blood sugar, renal function tests, liver function tests, urine analysis, CSF were normal. CT brain showed subarachnoid hemorrhage in left temporoparietal region. Tuberculin test was negative after 48 hours. USG chest and abdomen show bilateral pleural effusion and mild ascitis .Doppler study showed narrowing of both proximal subclavian arteries with loss of triphasic waveform distally in upper limb and narrowing of left main renal artery. CT angiography showed diffuse narrowing & irregularity of subclavian & axillary artery starting from just after origin of subclavian artery. Both renal arteries had stenosis and dilation.

Takayasu's arteritis (TA) is the third most common form of childhood vasculitis in Asian and Indian population, common in female after 10 years. Takayasu's arteritis or the aortic arch syndrome or Pulseless disease is a chronic, idiopathic, inflammatory disease primarily affecting large vessels such as aorta and its branches.

Takayasu's arteritis was first described by the Japanese ophthalmologist, Takayasu. Etiopathogenesis is unclear but autoimmune, genetic predisposition, Tuberculosis may be associated with TA. In pre-Pulseless phase, manifestations are low-grade fever, anorexia, weight loss, fatigue, myalgia, arthritis. Most of the patients present in Pulseless phase with hypertension and its complication. Cerebrovascular accident like ICH is an unusual complication of TA. Here patient presented with status epileptics due to SAH. Diagnosis is confirmed by CT angiography. Management of hypertension and its complication, steroid (prednisolone), immunosuppressive drugs like methotrexate or cyclophosphamide may be used. Intervention radiological techniques like percutaneous transluminal angioplasty or vascular surgery are mainstay of therapy.
Compliance with Ethical Standards
Funding None
Conflict of Interest None
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Cite this article as:
Vaghela P C, Gohel N B, Kabaria R. Takayasu Arteritis with Status Epilepticus. Pediatr Oncall J. 2008;5: 84.
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