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Intracranial arteriopathy in tuberous sclerosis complex
Susana Boronat1, Elias A Shaaya2, Maria Auladell2
1Department of Neurology, Massachusetts General Hospital, Boston, MA, USA Department of Pediatric Neurology, Vall d'Hebron Hospital, Universitat Autònoma de Barcelona, Barcelona, Spain.
Journal of Child Neurology
|September 24, 2013
Summary
Intracranial aneurysms are more common in tuberous sclerosis complex patients than the general population. This suggests a potential link between neural crest cell dysfunction and brain vessel abnormalities in this condition.
Area of Science:
- Neurology
- Genetics
- Vascular Medicine
Background:
- Tuberous sclerosis complex (TSC) is a genetic disorder associated with benign tumor formation.
- Arterial aneurysms, particularly aortic and intracranial, have been anecdotally reported in TSC patients.
- The precise incidence and underlying mechanisms of intracranial aneurysms in TSC remain unclear.
Purpose of the Study:
- To investigate the prevalence of intracranial aneurysms in patients diagnosed with tuberous sclerosis complex.
- To explore the potential association between TSC and intracranial arteriopathy.
Main Methods:
- Retrospective review of brain magnetic resonance imaging (MRI) reports from 404 definite TSC patients and 16 probable/possible TSC patients.
- Detailed review of brain images for a subset of these patients (220 definite and 16 probable/possible TSC).
- Comparison of intracranial aneurysm prevalence in TSC patients with general population data.
Main Results:
- Intracranial aneurysms were identified in 3 out of 404 (0.74%) definite TSC patients, a higher rate than the general population (0.35%).
- Two of the affected TSC patients were children.
- A fourth aneurysm was found in a probable TSC patient with manifestations linked to neural crest derivatives.
Conclusions:
- The findings suggest an increased risk of intracranial aneurysms in individuals with tuberous sclerosis complex.
- The study hypothesizes a role for neural crest cell dysfunction in the development of intracranial arteriopathy in TSC.
- This dysfunction may affect the smooth muscle cells of forebrain vessels, which originate from neural crest cells.
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