Clinical Nuclear Medicine
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Subtraction Ictal SPECT Co-registered to MRI (SISCOM) in Sturge–Weber Syndrome
Namer, I J. MD, PhD‡; Battaglia, F MD†; Hirsch, E MD†; Constantinesco, A MD, PhD*; Marescaux, C MD†
From the *Service de Biophysique et de Médecine Nucléaire and †Clinique Neurologique, Hôpitaux Universitaires de Strasbourg; and ‡UMR 7004 ULP/CNRS/IFR37, Faculté de Médecine, Strasbourg, France.
Received for publication September 12, 2003; revision accepted April 6, 2004.
Reprints: I. J. Namer, MD, PhD, Service de Biophysique et de Médecine Nucléaire, Hôpitaux Universitaires de Strasbourg Hôpital de Hautepierre, 1, avenue Molière, 67098 Strasbourg Cedex 09, France. E-mail: [email protected]
Abstract
A 33-year-old woman with Sturge–Weber syndrome and pharmacoresistant partial seizures since age 5 was studied using Tc-99m ethyl cysteinate dimer (ECD) SPECT. Mental retardation has been present with significant diminution of IQ since the beginning of the follow-up in our unit for the last 20 years. (There was a loss of more than 20 points in all scales.) SPECT imaging showed a massive steal phenomenon in affected areas during seizures, leading to a critical ischemic condition in remote brain regions. The progressive worsening course of the syndrome, particularly with mental retardation, could be explained by repeated seizures with severe ischemia of healthy brain parenchyma.