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Takayasu arteritis: a treatable cause of stroke in infancy
M H Kohrman1, P R Huttenlocher
1Department of Pediatrics, University of Chicago, Pritzker School of Medicine, Illinois.
Insights
Early diagnosis and immunosuppression significantly improved outcomes for a 6-month-old infant with Takayasu arteritis, a rare and severe aortic disease. Prompt treatment led to neurological recovery and arterial healing.
Area of Science:
- Pediatric Cardiology
- Vascular Inflammation
- Neurology
Background:
- Takayasu arteritis is a rare, life-threatening arteritis affecting the aorta and its branches.
- Untreated, Takayasu arteritis has a high mortality rate, particularly in pediatric cases.
- Cerebral and cardiac manifestations can be severe, leading to significant morbidity.
Observation:
- A 6-month-old infant presented with stroke-like symptoms, including hemiparesis and decreased pulses.
- Imaging revealed widespread arterial abnormalities, including carotid and aortic involvement, consistent with Takayasu arteritis.
- The patient exhibited an elevated erythrocyte sedimentation rate, indicating active inflammation.
Findings:
- Cerebral angiography showed carotid artery dilatation and middle cerebral artery stenosis.
- Cardiac angiography revealed aortic root dilatation, carotid artery origin stenosis, and descending aorta beading.
- Treatment with prednisone and azathioprine led to normalization of inflammatory markers and neurological improvement.
Implications:
- Early diagnosis and aggressive immunosuppressive therapy are crucial for improving outcomes in pediatric Takayasu arteritis.
- Vigorous treatment can lead to arterial healing and resolution of neurological deficits.
- This case highlights the potential for successful management of this rare pediatric vascular disease.
Abstract:
Takayasu disease is an arteritis of unknown etiology involving the aorta and its major branches. Untreated, 75% of patients die within two years. A 6-month-old black female presented with a right focal seizure, a flaccid right hemiparesis, decreased pulses in the right arm, a large left frontoparietal hypodense area on computed tomography, and an elevated erythrocyte sedimentation rate. Cerebral angiography demonstrated irregular dilatation of both carotid arteries and narrowing of the left middle cerebral artery. Aneurysm of the right sinus of Valsalva, dilatation of the aortic root, narrowing of the origins of both carotid arteries, and beading of the descending aorta were demonstrated by cardiac angiography. The patient was treated with prednisone (2 mg/kg/day) and azathioprine (1 mg/kg/day). There was gradual return of the erythrocyte sedimentation rate to normal over the ensuing 3-10 months, resolution of the hemiparesis, and acquisition of normal developmental milestones. Digital subtraction angiography revealed improvement in the appearance of the descending aorta and of the common carotid arteries with the disappearance of the arterial wall irregularities. Early diagnosis and vigorous immunosuppression may improve outcome in this rare and often fatal vascular disease in childhood.