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Transient cerebral arteriopathy: a disorder recognized by serial angiograms in children with stroke
S Chabrier1, G Rodesch, P Lasjaunias
1Département de Pédiatrie, Hôpital de Bicêtre, Le Kremlin-Bicêtre, France.
Insights
Transient cerebral arteriopathy is a newly characterized condition causing ischemic strokes in children. This disorder involves temporary narrowing of brain arteries, often resolving spontaneously, leading to stroke recovery.
Area of Science:
- Neurology
- Pediatric Neurology
- Vascular Neurology
Background:
- Ischemic strokes in children can stem from various causes.
- Idiopathic strokes require detailed investigation into underlying vascular pathologies.
Observation:
- A retrospective study identified 9 children (26%) with transient cerebral arteriopathy among 34 pediatric ischemic stroke cases.
- Patients, previously healthy with a mean age of 6 years, presented with acute hemiplegia.
Findings:
- Cerebral imaging revealed small subcortical infarcts in the basal ganglia or internal capsule.
- Arteriography demonstrated multifocal arterial wall lesions, including stenosis, primarily in the carotid system's basal arteries.
- Follow-up arteriography showed initial worsening of lesions, followed by regression, improvement, or stabilization in most cases.
Implications:
- Transient cerebral arteriopathy is a distinct clinical entity in pediatric ischemic stroke.
- The condition appears to be self-limiting, with a potential for complete clinical recovery.
- Further research is warranted to elucidate the presumed inflammatory etiology of this arteriopathy.
Abstract:
Repeated clinical evaluation and cerebral arteriography during the evolution of ischemic strokes of idiopathic origin allowed us to characterize a transient cerebral arteriopathy. We retrospectively studied the clinical characteristics, course, and neuroimaging features of this disorder in nine children. Of 34 children with ischemic strokes seen consecutively between 1984 and 1995, 9 (26%) were diagnosed as having transient attack of the cerebral arterial wall, termed transient cerebral arteriopathy. All of these patients had previously been in good health. The mean age at the time of the first stroke was 6 years (range, 2 9/12 years to 13 4/12 years). All children presented with acute hemiplegia. A recurrence of the stroke took place 3 months at the latest after the initial infarct in three children (mean clinical follow-up 2 7/12 years). Cerebral imaging in all the patients showed small subcortical infarcts located in basal ganglia or internal capsule. Arteriography revealed multifocal lesions of the arterial wall (focal stenosis or segmental narrowing), mostly located in the initial parts of basal arteries of the carotid system. Longitudinal arteriographic follow-up showed initial worsening of these arterial lesions (n = 5) for a maximum duration of 7 months followed by complete regression (n = 2), improvement (n = 5), or stabilization of the lesions (n = 2). Five patients had a complete clinical recovery. Further studies are necessary to confirm a presumed inflammatory cause of this arteriopathy.