Sturge-Weber syndrome with late onset hemiplegic migraine-like attacks and progressive unilateral cerebral atrophy
Cephalalgia. 2013-09-17; 34(1): 73-77
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1. Cephalalgia. 2014 Jan;34(1):73-7. doi: 10.1177/0333102413505237. Epub 2013 Sep
Sturge-Weber syndrome with late onset hemiplegic migraine-like attacks and
progressive unilateral cerebral atrophy.
Planche V(1), Chassin O, Leduc L, Regnier W, Kelly A, Colamarino R.
(1)Service de Neurologie, CH Jacques Lacarin, France.
BACKGROUND: Sturge-Weber syndrome (SWS) is an uncommon etiology of hemiplegic
migraine-like (HM-like) attacks, associated with epilepsy and mental retardation.
CASE: We report the case of a 40-year-old woman with SWS who has been suffering
from HM-like episodes since she was 24, with no history of seizure or mental
retardation. Susceptibility weighted imaging (SWI)-MRI and CT scans have shown
bilateral calcifications of the choroidal plexuses, a developmental venous
anomaly with dilated transmedullary veins and a left parieto-occipital
leptomeningeal angioma. (18)F-Fluorodeoxyglucose (FDG)-PET/CT revealed a diffuse
left-hemisphere hypometabolism. The comparison between the MRI performed at the
age of 24 and the one performed at the age of 40 highlighted a progressive
unilateral fronto-temporo-parietal atrophy. Surprisingly, even now, cognitive
functions of this patient are relatively preserved. Lamotrigine permitted an
improvement of HM-like attacks.
DISCUSSION: Explanations for this minimally symptomatic form of SWS may be the
absence of seizure, the importance of her deep venous drainage, the absence of
cortical calcification and white matter impairment in the affected hemisphere,
and, paradoxically, the severely asymmetric cortical metabolism. Furthermore,
this case reinforces the hypothesis that alteration of cerebral hemodynamics
could precipitate the cortical spreading depression giving rise to migraine with
CONCLUSION: We propose to consider SWS as a cause of apparently isolated
hemiplegic migraine and lamotrigine as a preventive medication in HM-like
PMID: 24045571 [Indexed for MEDLINE]