Abstract
The discovery of focal or multifocal cortical lesions using magnetic resonance imaging
(MRI) and positron emission tomography (PET) scanning in the majority of infants with
West syndrome has led to a surgical approach in the treatment of some patients with
intractable infantile spasms. The locations of these lesions should be concordant
with localization of focal ictal and/or interictal electroencephalographic (EEG) abnormalities
prior to proceeding with cortical resection. When a single lesion is present on the
MRI or PET, and there is good correlation with EEG localization, surgical treatment
is generally quite favorable in terms of both seizure control and cognitive development.
Interictal glucose metabolism PET scans in children with intractable cryptogenic infantile
spasms show unifocal cortical hypometabolism in about 20% of cases. In the majority,
however, multifocal asymmetric hypometabolism is suggestive of multifocal underlying
lesions, possibly multifocal cortical dysplasia. When the pattern of glucose hypometabolism
is symmetric, a lesional etiology is less likely, thus neurometabolic or neurogenetic
disorders should be considered. Therefore, the pattern of glucose hypometabolism on
PET in infants with intractable cryptogenic spasms is a useful guide to decide whether
a medical or surgical approach should be undertaken. In order to achieve the best
cognitive outcome with surgery, it is important to resect the entire ‘nociferous’
area rather than just the seizure focus. Our research with new PET imaging probes
has attempted to provide a comprehensive evaluation of the epileptogenic zone including
the ‘nociferous’ cortex. We have used [11C]flumazenil (FMZ), which labels gamma aminobutyric acidA (GABAA) receptors, and have found this to be particularly useful in showing: (i) decreased
receptor binding with medial temporal involvement thus indicating resection of medial
temporal structures, (ii) the peri-lesional epileptogenic zone surrounding MRI lesions,
(iii) the seizure onset zone in MRI-negative cases, and (iv) potential secondary epileptic
foci. Another recently developed PET probe, alpha[11C]methyl-l-tryptophan (AMT) which is a precursor for the serotonin and the kynurenine metabolism
pathways, is capable of differentiating between epileptogenic and non-epileptogenic
tubers in patients with tuberous sclerosis complex and intractable epilepsy (including
infantile spasms). Subsequently, we have applied AMT PET in patients with multifocal
cortical dysplasia to determine the predominant seizure focus, and the results have
been promising with regard to seizure control but not cognitive development. Thus,
the introduction of newer more specific PET probes for epilepsy has led to improved
and more accurate localization of seizure foci that should ultimately improve outcome
of epilepsy surgery in West syndrome.
Keywords
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Article info
Publication history
Accepted:
June 21,
2001
Received in revised form:
June 18,
2001
Received:
June 4,
2001
Identification
Copyright
© 2001 Elsevier Science B.V. Published by Elsevier Inc. All rights reserved.
