Intracranial fibromuscular dysplasia in a six-year-old child: A rare causeof childhood stroke

Intracranial fibromuscular dysplasia is a nonatheromatous angiopathy that m ost commonly affects adult women and is rarely recognized in children. Symp toms include stroke and headache, although the vasculopathy may be asymptom atic. Diagnosis is based on angiographic appearance, commonly described as a "string of beads." The etiology of intracranial fibromuscular dysplasia i s not known, although possible causes include genetic predisposition, traum a, and underlying connective tissue disease. Treatment of intracranial fibr omuscular dysplasia is largely supportive once symptoms become manifest. We report a 6-year-old girl who presented to our center for further evaluatio n of a large left middle cerebral artery distribution infarction. The patie nt was previously healthy, without known risk factors for stroke. Initial s ymptoms consisted of a dense global aphasia and a right hemiparesis. On arr ival, the patient's aphasia had improved but she continued to have signific ant deficits in both receptive and expressive language as well as residual right hemiparesis. Magnetic resonance imaging and conventional angiographic studies demonstrated characteristic beading of the distal portion of the l eft internal carotid artery, as well as the proximal middle cerebral artery . Laboratory evaluation, echocardiogram, and renal ultrasound were normal. The renal vasculature did not demonstrate evidence of intracranial fibromus cular dysplasia. In conclusion, intracranial fibromuscular dysplasia should be considered in the differential diagnosis of childhood stroke. When reco gnized, other sites of vascular involvement should be sought, and considera tion of underlying disorders is important, as connective tissue disorders h ave been associated with a propensity to develop this vascular abnormality. Careful follow-up is warranted, due to possible progression of disease.