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Abstract #19673 Published in IGR 9-4
Transient exacerbation of hemiplegia following minor head trauma in Sturge-Weber syndrome
Zolkipli Z; Aylett S; Rankin PM; Neville BGRDevelopmental Medicine and Child Neurology 2007; 49: 697-699
Sturge-Weber syndrome (SWS) is a sporadic disorder characterized by naevus (port wine stain), a pial angioma, and glaucoma. The angioma comprises abnormal tortuous vessels on the leptomeninges with underlying brain gliosis, calcification, and atrophy. The cerebral angioma is commonly unilateral but may be bilateral. Hemiplegia usually follows recurrent hemiconvulsions and may be related to venous stasis. The hemiplegia can be static, progressive, or fluctuating. Transient worsening of the hemiplegia can be seen with seizures and episodes resembling hemiplegic migraine. We report five patients (four females, one male) with SWS who have had transient worsening of hemiplegia following minor head injuries, occurring between the ages of ten months and 12 years (median age four years six months). An additional pilot survey suggests that this may affect up to 20% of patients.
Dr. S. Aylett, Department of Paediatric Neurology, Great Ormond Street Hospital, UCL Institute of Child Health, Great Ormond Street, London WC1N 3JH, UK. ayletS@gosh.nhs.uk
Classification:
9.4.15 Glaucoma in relation to systemic disease (Part of: 9 Clinical forms of glaucomas > 9.4 Glaucomas associated with other ocular and systemic disorders)