Focal inhibitory seizure with prolonged deficit in adult Sturge-Weber syndrome.

Jerome Aupy, Charlotte Bonnet, Jean-Simon Arnould, Philippe Fernandez, Cecile Marchal, Paolo Zanotti-Fregonara
Epileptic Disorders. 2015-09-01; 17(3): 328-331
DOI: 10.1684/epd.2015.0755

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1. Epileptic Disord. 2015 Sep;17(3):328-31. doi: 10.1684/epd.2015.0755.

Focal inhibitory seizure with prolonged deficit in adult Sturge-Weber syndrome.

Aupy J(1), Bonnet C(2), Arnould JS(3), Fernandez P(3), Marchal C(2),
Zanotti-Fregonara P(3).

Author information:
(1)Centre Hospitalier Universitaire de Bordeaux, Pôle de Neurosciences Cliniques,
Institut des Maladies Neurodégénératives, CNRS UMR 5293.
(2)Centre Hospitalier Universitaire de Bordeaux, Pôle de Neurosciences Cliniques.
(3)Centre Hospitalier Universitaire de Bordeaux, Service de Médecine Nucléaire,
Université de Bordeaux, INCIA, CNRS UMR 5287, Bordeaux, France.

Sturge-Weber syndrome is a sporadic congenital neurocutaneous disorder often
related to varying degrees of motor impairment. The phenomenon of prolonged ictal
paresis is a rare seizure sign that can be due to lesions affecting the
centro-parietal lobe. Focal inhibitory motor seizures can be difficult to
differentiate from other clinical entities such as stroke, migraine or postictal
paresis. We describe the case of a 40-year-old patient suffering from
Sturge-Weber syndrome, admitted due to prolonged right-sided hemiparesis
following a usual seizure. Repeated EEGs during the prolonged deficit showed only
intermittent left fronto-parietal sharp waves. (99m)Tc HMPAO-brain SPECT
performed seven days after the last seizure showed a vast area of
parieto-occipital hyperperfusion in the left hemisphere. Aggressive antiepileptic
therapy dramatically improved the clinical symptoms and scintigraphic images,
which corroborated the diagnosis of ictal paresis. This case highlights the role
of SPECT in the evaluation of Sturge-Weber syndrome, not only to investigate
progressive neurological deterioration, but also exacerbation of seizures or
prolonged neurological deficits. In fact, it may be possible to document ongoing
epileptic activity using SPECT, despite a non-contributory EEG, which may be of
help in adapting a therapeutic strategy.

DOI: 10.1684/epd.2015.0755
PMID: 26235101 [Indexed for MEDLINE]

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