Childhood arterial ischemic stroke due to mineralizing angiopathy: an 18-year single-center experience
Carolina Gorodetsky1, Elizabeth Pulcine1, Pradeep Krishnan2
1Department of Pediatrics, Division of Neurology, The Hospital for Sick Children, Toronto, ON, Canada.
Insights
Mineralizing angiopathy, a childhood stroke syndrome, presents with basal ganglia infarction and calcifications. This study expands its known characteristics, revealing later onset and diverse neurological deficits in affected children.
Area of Science:
- Neurology
- Pediatric Neurology
- Neuroradiology
Background:
- Mineralizing angiopathy is an age-specific stroke syndrome in children, often linked to basal ganglia infarction and calcification following minor head trauma.
- The pathophysiology, clinical course, and outcomes of this rare condition remain poorly understood.
- Existing diagnostic approaches may overlook this entity, leading to misdiagnosis.
Purpose of the Study:
- To describe the clinical and radiographic features of a cohort of children with mineralizing angiopathy.
- To compare these findings with previously published literature.
- To expand the understanding of the phenotype and potential diagnostic challenges of mineralizing angiopathy.
Main Methods:
- A single-center, consecutive cohort of 14 children diagnosed with mineralizing angiopathy between January 2002 and January 2020 was analyzed.
- Clinical data, including stroke onset, neurological deficits, and follow-up, were collected.
- Radiographical findings were reviewed, with a focus on basal ganglia lesions and calcifications.
Main Results:
- Previously unreported findings included later stroke onset (in 8 children >18 months), basal ganglia hemorrhage (in 4), multifocal infarcts (in 3), additional non-basal ganglia calcifications (in 3), and thrombophilia (in 1).
- Seven children experienced moderate-to-severe neurological deficits.
- No symptomatic stroke recurrence was observed during a mean follow-up of 3 years and 7 months.
Conclusions:
- This study expands the known phenotype of mineralizing angiopathy, highlighting variations in onset age and presentation.
- The findings underscore the importance of considering non-contrast computed tomography alongside MRI for detecting calcifications in pediatric stroke.
- Most children with mineralizing angiopathy experience significant, long-term neurological sequelae.
Abstract:
Mineralizing angiopathy is a unique, age-specific stroke syndrome characterized by basal ganglia infarction and lenticulostriate calcification after minor head injury in early childhood. There is limited understanding of the pathophysiology, course, and clinical outcome of this syndrome. We describe the clinical and radiographical phenotype of a single-center, consecutively enrolled cohort of children with mineralizing angiopathy from January 2002 to January 2020 and provide a comparative analysis to previously published literature. Fourteen children were identified. Previously unreported findings include: stroke onset in eight children older than 18 months; presence of basal ganglia hemorrhage in four; multifocal basal ganglia infarcts in three; presence of additional non-basal ganglia calcifications in three; and presence of thrombophilia in one. Seven children had moderate-to-severe neurological deficits. There was no symptomatic stroke recurrence (mean follow-up 3y 7mo, SD 1y 7mo). Our expanded phenotype highlights distinct characteristics of mineralizing angiopathy in children and has the potential to inform future research. What this paper adds Children with mineralizing angiopathy are often misdiagnosed as having a limb fracture despite normal x-rays. A magnetic resonance imaging-only approach may miss this entity. Non-contrast computed tomography, in addition to MRI is recommended to identify calcifications in idiopathic arterial ischemic stroke. Most children have moderate-to-severe neurological sequela.
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