Uncommon presentation of Takayasu Arteritis as a convulsive syncope

C.M. Santhosham*

Senior Consultant Intensivist, Programme Director – MEM SEMI CCT, Kauvery Hospital, Trichy, India

Introduction

Takayasu Arteritis (TA) is a chronic granulomatous vasculitis of the large vessels mainly involved in aorta and its main branches, is prevalent all around the world. It causes stenosis of large arteries and ischemic damage to target organs. There is usually a delay in recognizing Takayasu arteritis because of the rarity and unfamiliarity with the disease and nonspecific early symptoms.

Case Presentation

A 36- years-aged female presented to the ER with complaints of recurrent seizure-like activities for the past 2 years, along with headache and blurring of vision in the left eye, during sitting and standing positions. The patient had a history of upper limb BP not recordable for the past 1 year.

On examination in the ER, the patient had GCS: E4V5M6, bilateral pupil 2 mm RTL, afebrile, HR: 98 bpm (very feeble in both radial and brachial artery), BP: RUL-90/60, LUL- 90/50, RLL- 110/80, LLL- 100/80, SpO2: 98% RA, RR: 22 bpm

Neurological examination revealed no focal neurological deficit. Other systemic examinations also revealed no abnormality. MRI BRAIN and EEG have done outside were normal studies.

Laboratory results indicated normal blood counts, elevated ESR- 87, CRP- 35, S. Electrolytes were normal.

USG Doppler- bilateral carotid artery and bilateral upper limb revealed findings suggestive of aortoarteritis.

CT Carotid angiography:

Concentric mural thickening involving the arch and descending thoracic aorta, involving right subclavian artery, causing severe stenosis.
Complete occlusion of left subclavian artery from its origin.
Diffuse mural thickening of bilateral common carotid arteries with complete occlusion of left internal carotid artery

All were features suggestive of Takayasu arteritis.

ECHO- normal study. USG Abdomen- contracted right kidney.

Our patient was diagnosed as Takayasu arteritis, presenting with convulsive syncope, fulfilling three of the six criteria for diagnosis: onset of age less than 40 years, decreased brachial artery pulse, arteriographic narrowing or occlusion of aorta and its primary branches.

The patient started on T. Methotrexate 15 mg weekly once, orednisolone 60 mg once daily, and folic acid and calcium supplements.

Patient improved symptomatically and was discharged at Day 6 of admission with further follow-up.

Discussion

Takayasu arteritis is inflammatory arteritis of large vessels, predominantly affects young females and involves the aorta and its branches. Etiology is unknown, the underlying pathology is inflammation leading to stenosis, blockage or aneurysm formation. The disease has 3 stages, Stage 1: Pre-Pulseless stage, Stage 2: May be devoid of any signs of inflammation, and Stage 3: Stage of quiescence.

Diagnosis is based on six criteria: onset at age less than or equal to 40 years, claudication of an extremity, decreased brachial artery pulse, greater than 10 mmHg difference in systolic blood pressure between arms, a bruit over the subclavian arteries or the aorta and the arteriographic evidence of narrowing or occlusion of aorta and its primary branches.

Syncope/Seizure has an initial manifestation that has been reported but is rare. Treatment of TA includes immunomodulators like oral Steroids and Methotrexate, control of hypertension, and vascular intervention if needed.

Conclusion

We present this case in order to highlight two findings: the importance of a detailed clinical history and examination. Early diagnosis is paramount in managing Takayasu arteritis to prevent morbidity and reduce complications of the disease.

Reference

[1] Menon B, Himabindu A. Takayasu arteritis presenting as convulsive syncope which had been misinterpreted as epilepsy: a case report. J Med Case Rep. 2010;4:352.

[2] Pallangyo P, Misidai N, Hemed NR. Takayasu arteritis mistaken for epilepsy: A case presenting with convulsive syncope. J Med Cases. 2020 Feb;11(2):37-40.

Uncommon presentation of Takayasu Arteritis as a convulsive syncope