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Acute hemiparesis in a child as a presenting symptom of hemispheric cerebral proliferative angiopathy
1Division of Child Neurology, Department of Neurosciences, University of California, San Diego and Rady Children's Hospital, 8010 Frost Street, Suite 400, San Diego, CA 92123, USA.
Insights
A pediatric case of cerebral proliferative angiopathy presented with stroke-like symptoms including headaches and unsteadiness. Prompt diagnosis and management led to complete symptom resolution within 24 hours, highlighting this rare condition.
Area of Science:
- Neurology
- Vascular Malformations
- Pediatric Neurology
Background:
- Cerebral proliferative angiopathy (CPA) is a rare vascular malformation.
- It is distinct from classic arteriovenous malformations (AVMs).
- CPA can present with stroke-like symptoms in children.
Purpose of the Study:
- To report a case of cerebral proliferative angiopathy in a child.
- To describe the diagnostic process and clinical presentation.
- To emphasize the importance of recognizing CPA as a differential diagnosis in pediatric stroke mimics.
Main Methods:
- A 9-year-old girl presented with worsening headaches and unsteadiness.
- Neurologic examination revealed right hemiparesis and right homonymous hemianopsia.
- Imaging studies including CT, CT angiogram, and MRI were performed.
Main Results:
- CT showed an engorged sagittal and straight sinus with prominent cortical veins.
- CT angiogram confirmed a left hemispheric vascular malformation consistent with CPA.
- MRI showed no evidence of infarct, and symptoms resolved within 24 hours.
Conclusions:
- Cerebral proliferative angiopathy can mimic other neurological emergencies in children.
- Early diagnosis through advanced imaging is crucial.
- Prompt management can lead to rapid symptom resolution.
Abstract:
A 9-year-old girl with a several-month history of unilateral intermittent headaches presented to the hospital with worsening headaches and unsteadiness. Neurologic exam was positive for a mild right hemiparesis and right homonymous hemianopsia. Noncontrast computed tomography revealed an engorged sagittal and straight sinus with prominent cortical veins concerning an arteriovenous malformation and the patient was admitted to the pediatric intensive care unit. Computed tomography angiogram demonstrated a left hemispheric vascular malformation, without evidence of dural arteriovenous fistula on conventional angiogram consistent with a diagnosis of cerebral proliferative angiopathy. There was no evidence of infarct on magnetic resonance imaging, and the patient's symptoms were completely resolved within 24 hours. Cerebral proliferative angiopathy is a rare but important vascular malformation distinct from classic arteriovenous malformations that may present with stroke-like symptoms in childhood.
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