BACKGROUND: Stroke-like episodes associated with Sturge-Weber Syndrome have previously been attributed to thrombosis within the leptomeningeal malformation causing infarction. CASE REPORT: A 22-year-old woman with a right-sided port-wine nevus presented with acute left hemiparesis. Brain magnetic resonance imaging (MRI) demonstrated a vascular malformation of the right cerebral hemisphere consistent with Sturge-Weber Syndrome. There was mild edema of the right hemisphere cortex. While in hospital she developed recurrent complex partial seizures. Repeat brain MRI showed a diffusion abnormality affecting the right hemisphere with an increase in the degree of edema. There was no evidence of thrombosis on cerebral angiography. The patient's neurologic deficit and acute MRI changes subsequently resolved. CONCLUSIONS: Although cerebral angiography could potentially demonstrate thrombosis causing large vessel obstruction, it is unlikely to have the resolution to detect stasis at a microvascular level. Microcirculatory stasis with reversible ischemia is the likely pathogenic mechanism in this case.
BACKGROUND:Stroke-like episodes associated with Sturge-Weber Syndrome have previously been attributed to thrombosis within the leptomeningeal malformation causing infarction. CASE REPORT: A 22-year-old woman with a right-sided port-wine nevus presented with acute left hemiparesis. Brain magnetic resonance imaging (MRI) demonstrated a vascular malformation of the right cerebral hemisphere consistent with Sturge-Weber Syndrome. There was mild edema of the right hemisphere cortex. While in hospital she developed recurrent complex partial seizures. Repeat brain MRI showed a diffusion abnormality affecting the right hemisphere with an increase in the degree of edema. There was no evidence of thrombosis on cerebral angiography. The patient's neurologic deficit and acute MRI changes subsequently resolved. CONCLUSIONS: Although cerebral angiography could potentially demonstrate thrombosis causing large vessel obstruction, it is unlikely to have the resolution to detect stasis at a microvascular level. Microcirculatory stasis with reversible ischemia is the likely pathogenic mechanism in this case.
Authors: Alejandro J De la Torre; Aimee F Luat; Csaba Juhász; Mai Lan Ho; Davis P Argersinger; Kara M Cavuoto; Mabel Enriquez-Algeciras; Stephanie Tikkanen; Paula North; Craig N Burkhart; Harry T Chugani; Karen L Ball; Anna Lecticia Pinto; Jeffrey A Loeb Journal: Pediatr Neurol Date: 2018-04-18 Impact factor: 3.372
Authors: Warren Lo; Douglas A Marchuk; Karen L Ball; Csaba Juhász; Lori C Jordan; Joshua B Ewen; Anne Comi Journal: Dev Med Child Neurol Date: 2011-12-23 Impact factor: 5.449
Authors: Sara Sabeti; Karen L Ball; Sanjoy K Bhattacharya; Elena Bitrian; Lauren S Blieden; James D Brandt; Craig Burkhart; Harry T Chugani; Stephen J Falchek; Badal G Jain; Csaba Juhasz; Jeffrey A Loeb; Aimee Luat; Anna Pinto; Eric Segal; Jonathan Salvin; Kristen M Kelly Journal: Pediatr Neurol Date: 2021-05-06 Impact factor: 4.210