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Published on: May 23, 2016
Early-onset bilateral cerebral arteriopathies: Cohort study of phenotype and disease course
Amina Al-Yassin1, Dawn E Saunders1, Mark T Mackay1
1From the Neurosciences Unit (A.A.-Y., V.G.), UCL Institute of Child Health; the Radiology Department (D.E.S.), Great Ormond Street Hospital, NHS Foundation Trust, London, UK; and the Neurology Department (M.T.M.), Royal Children's Hospital, Melbourne, Australia.
Insights
Young children with bilateral cerebral arteriopathies, especially moyamoya (CASCADE 3A), face a severe prognosis with frequent strokes and disease progression. These findings highlight limitations in current classification systems for pediatric cerebrovascular disease.
Area of Science:
- Pediatric Neurology
- Cerebrovascular Diseases
- Neuroimaging
Background:
- Arterial ischemic stroke (AIS) in young children is often associated with underlying arteriopathies.
- Bilateral cerebral arteriopathies present unique challenges in diagnosis and management.
- Understanding the characteristics and outcomes of these conditions is crucial for early intervention.
Purpose of the Study:
- To characterize the clinical features, neuroimaging findings, and outcomes of young children diagnosed with AIS and bilateral cerebral arteriopathies.
- To evaluate the progression of arteriopathy and recurrence of ischemic events.
- To assess the utility of current classification systems, such as the Childhood Arterial Ischemic Stroke Standardized Classification and Diagnostic Evaluation (CASCADE) criteria.
Main Methods:
- Retrospective review of 31 children with AIS and bilateral cerebral arteriopathies.
- Analysis of clinical presentations, neurological course, and functional outcomes using the modified Rankin Scale.
- Detailed neuroimaging review to assess infarct patterns, arteriopathy characteristics (involvement, symmetry), and disease progression.
- Classification of arteriopathies using CASCADE criteria.
Main Results:
- The median age of affected children was 18 months, with common presentations including hemiparesis and seizures.
- A significant proportion experienced recurrent AIS or transient ischemic attacks (20/31), with progressive arteriopathy observed in 14/23 reimaged patients.
- Symmetric involvement of the carotid circulation was common (24/31), and CASCADE 3A (moyamoya) was associated with a significantly shorter time to recurrence.
- Poor functional outcomes were noted, with motor and cognitive impairments.
Conclusions:
- Young children with bilateral cerebral arteriopathies, particularly moyamoya (CASCADE 3A), exhibit a malignant clinical course characterized by frequent recurrences and progressive disease.
- Current classification systems may not fully capture the complexity of these pediatric cerebrovascular conditions.
- The symmetric and systemic nature of these arteriopathies suggests potential developmental or genetic etiologies.
Objective:
To describe characteristics of young children with arterial ischemic stroke (AIS) and bilateral cerebral arteriopathies.
Methods:
Retrospective review of clinical features, course, and outcome. Neuroimaging was analyzed for infarct pattern, cerebrovascular diagnosis (anatomic/Childhood Arterial Ischemic Stroke Standardized Classification and Diagnostic Evaluation [CASCADE] criteria), and disease progression.
Results:
In the 31 children (median age, 18 months), presentations included acute hemiparesis (n=23) and focal seizures (n=12). Seven had systemic arterial disease; 13 had cardiac abnormalities. Twenty had recurrent AIS or transient ischemic attack (after median of 3 months); 16 had >1 recurrence. Median modified Rankin Scale score was 3, with motor impairments in 20, cognitive impairments in 11, and seizures in 7. At presentation, 17 had old and acute infarcts. Twenty-five had high signal in white matter. A total of 13/23 reimaged patients accrued further infarcts over a median of 39 months. Arteriopathy involved the carotid circulation bilaterally in all; 6 had posterior circulation and 11 had extracranial involvement. Arteriopathy distribution was symmetric in 24/31. CASCADE categories were 3A in 19, 3B in 5, 3C in 5, and 7 in 2. After a median of 35 months, 14 had had progression of arteriopathy. Patients categorized as CASCADE 3A (moyamoya) had significantly shorter time to recurrence than other groups.
Conclusion:
Young children with bilateral cerebral arteriopathies (particularly meeting criteria for CASCADE 3A) have a malignant course, with frequent recurrent events, progressive disease, and poor outcomes. Current classifications are limited in characterizing disease in many cases. Symmetric involvement suggests these arteriopathies may be developmentally determined, while systemic involvement suggests potential genetic etiology.
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