title: Focal inhibitory seizure with prolonged deficit in adult Sturge-Weber syndrome.
abstract: 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.
authors.affiliation: Centre Hospitalier Universitaire de Bordeaux, Pôle de Neurosciences Cliniques, Institut des Maladies Neurodégénératives, CNRS UMR 5293.
authors.forename: Jerome
authors.initials: J
authors.lastname: Aupy
authors.affiliation: Centre Hospitalier Universitaire de Bordeaux, Pôle de Neurosciences Cliniques.
authors.forename: Charlotte
authors.initials: C
authors.lastname: Bonnet
authors.affiliation: Centre Hospitalier Universitaire de Bordeaux, Service de Médecine Nucléaire, Université de Bordeaux, INCIA, CNRS UMR 5287, Bordeaux, France.
authors.forename: Jean-Simon
authors.initials: JS
authors.lastname: Arnould
authors.affiliation: Centre Hospitalier Universitaire de Bordeaux, Service de Médecine Nucléaire, Université de Bordeaux, INCIA, CNRS UMR 5287, Bordeaux, France.
authors.forename: Philippe
authors.initials: P
authors.lastname: Fernandez
authors.affiliation: Centre Hospitalier Universitaire de Bordeaux, Pôle de Neurosciences Cliniques.
authors.forename: Cecile
authors.initials: C
authors.lastname: Marchal
authors.affiliation: Centre Hospitalier Universitaire de Bordeaux, Service de Médecine Nucléaire, Université de Bordeaux, INCIA, CNRS UMR 5287, Bordeaux, France.
authors.forename: Paolo
authors.initials: P
authors.lastname: Zanotti-Fregonara
mesh_terms: D000328:Adult; D004569:Electroencephalography; D006801:Humans; D008297:Male; D009433:Neural Inhibition / Q000502:physiology; D010291:Paresis / Q000175:diagnosis* / Q000209:etiology; D012640:Seizures / Q000150:complications / Q000175:diagnosis* / Q000209:etiology; D013341:Sturge-Weber Syndrome / Q000150:complications / Q000175:diagnosis*; D015899:Tomography, Emission-Computed, Single-Photon
keywords: HMPAO SPECT; MRI; Sturge-Weber syndrome; inhibitory seizure