Transient focal cortical hypometabolism in idiopathic West syndrome

doi:10.1016/0887-8994(93)90021-4

Pediatric Neurology

Volume 9, Issue 6, November–December 1993, Pages 430-434

https://doi.org/10.1016/0887-8994(93)90021-4 Get rights and content

Abstract

Positron emission tomography (PET) using ¹⁸F-labeled 2-deoxy-d-glucose was performed serially in 5 infants with idiopathic West syndrome. While tonic spasms persisted, 2 infants had hypometabolism in the bilateral temporo-parieto-occipital regions, which disappeared after cessation of spasms. In 2 other infants, PET revealed focal hypometabolism in the temporal region a few months after the disappearance of tonic spasms, but subsequent PET studies were normal. PET can detect transient metabolic abnormalities of the cerebral cortex which may be associated with the pathophysiology of West syndrome.

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Cited by (18)

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Besides MRI, positron emission tomography (PET) has also been used to assess alterations in West syndrome. Using FDG, hypo(energy)metabolism was qualitatively assessed in cortical areas (Maeda et al., 1994, 1993; Natsume et al., 1996), which was found to be related to delayed myelination (Natsume et al., 1996). However, FDG is not be a specific tracer for myelin.
Myelin is a vital element of normal brain development and structure. Myelination is most prominent during the first two years of life and proceeds until the age of 30. Abnormal myelination is related to several neurological and neuropsychiatric disorders such as Alzheimer’s disease and multiple sclerosis. Recently, abnormal myelin content has also been reported in children with epilepsy. Furthermore, more and more literature hints at a link between abnormal myelination and epilepsy, hence it is worthwhile to evaluate the benefits of non-invasive myelin imaging. In this literature review, we provide an overview of the current evidence of myelin abnormalities in epilepsy from imaging and histological studies. After preselection, 21 histological and 21 in vivo imaging studies were identified. Primarily, epilepsy is found to be associated with a reduced myelin content. This review shows that the currently available literature does not provide a complete view into the nature of myelin abnormalities in epilepsy. However, the reported literature is indicative of a relation between the pathophysiology of epilepsy and the myelin content. More studies that apply myelin-specific imaging techniques are needed to determine whether the myelin abnormalities are an underlying cause of epilepsy, or a consequence of the excessive activity in epilepsy.
Hypsarrhythmia is associated with widespread, asymmetric cerebral hypermetabolism
2019, Seizure
Citation Excerpt :
Previous studies using FDG-PET have implicated the the brainstem, lentiform nuclei, and temporal cortices in the pathogenesis of infantile spasms [8–10]. Several studies have linked normalization of RCA with cessation of spasms as well as favorable neurodevelopmental outcomes [11–15]. Conversely, the persistence of bitemporal hypometabolism is associated with particularly poor development, severe language impairment, and autism 10].
Hypsarrhythmia is the interictal EEG pattern most often associated with infantile spasms. We set out to evaluate the metabolic impact of hypsarrhythmia among patients with infantile spasms by contrasting regional cerebral metabolic activity among children with and without hypsarrhythmia.
Patients with video-EEG confirmed infantile spasms who underwent simultaneous interictal EEG and FDG-PET as part of a surgical evaluation were retrospectively identified. Pons-normalized relative cerebral metabolic activity (RCA) was ascertained in 18 cortical and 6 subcortical pre-specified regions of interest (ROIs).
We identified 63 patients with infantile spasms who underwent simultaneous EEG/PET, including children with hypsarrhythmia (n = 9), high-voltage EEG background (n = 20), and multifocal independent spike discharges (MISD) (n = 34). Among them, a putative epileptogenic zone was identified within the left-hemisphere only (n = 27), right-hemisphere only (n = 20), or assumed to be bilateral (n = 16). After adjustment for age at PET, the presence of hypsarrhythmia was associated with hypermetabolism in 11 of 18 cortical ROI’s. After adjustment for lateralized epileptogenic zones, the association between hypsarrhythmia and hypermetabolism was generally stronger within the left hemisphere.
Hypsarrhythmia is associated with widespread—and curiously left more than right—elevations in pons-normalized RCA, which is not evident on routine clinical review of individual PET studies. This study suggests that hypsarrhythmia may be a quasi-ictal phenomenon based on widespread and usually bilateral cortical hypermetabolism.
Positron emission tomography findings in children with infantile spasms and autism
2013, Journal of Clinical Neuroscience
Citation Excerpt :
The first PET studies of infantile spasm patients were done to shed light on cortical dysgenesis in patients with cryptogenic spasms.14 Maeda et al,4–6 who repeated MRI scans of patients with infantile spasms, showed hypometabolic cortical areas that did not overlap with cortical malformations and regressed when seizures stopped. In a recent study,15 two sets of PET scans of 15 patients with treatment-resistant focal epilepsy were done at an average interval of 19 months and showed an increase in hypometabolic cortical areas in patients whose seizures had continued or increased in frequency and a decrease in those whose seizures had decreased in frequency.
The purpose of this study was to evaluate positron emission tomography (PET) findings in patients diagnosed with infantile spasms and autism. This study includes 90 patients who were diagnosed with infantile spasms at the Department of Pediatric Neurology in the Istanbul University Medical Faculty between 1995 and 2007. Of the 90 patients, 15 patients who were diagnosed with autism using the Autism Behaviour Checklist and Childhood Autism Rating Scale and a control group of nine patients without autism but with infantile spasms underwent PET examination. Mean patient age (± standard error, SE) varied between 3 years and 16 years (7.8 ± 4 years), while the mean follow-up time (±SE) varied between 2 years and 16 years (average: 7.1 ± 4 years). Autism was present in 11 patients with symptomatic spasms and in four patients with cryptogenic spasms (p = 0.009). On the PET scans of the 15 patients with autism, 13 (86.7%) had significantly decreased metabolic activity in the temporal lobe (p < 0.001), nine (60%) had significantly decreased activity in the frontal lobe (p = 0.004), and seven (46.7%) had significantly decreased activity in the parietal lobe (p = 0.022). In our opinion, hypometabolism in the frontal and parietal lobes, in addition to that previously reported in the temporal lobe, plays a role in the development of autism in patients with infantile spasms.
Regional specificity of localized cortical lesions in West syndrome
2000, Pediatric Neurology
West syndrome, although classified as a generalized epilepsy, is associated with localized cerebral lesions in some cases. However, similar localized cortical abnormalities usually can result in partial epilepsy, instead of West syndrome. We performed this study to determine the additional factors that result in West syndrome instead of partial epilepsy in patients with localized cerebral lesions. We reviewed the pathologic features, topographic localization, and side of unilaterally defined cerebral lesions in relation to the seizure types in 39 epileptic patients, including five patients who presented with West syndrome. The lesions of all five patients with West syndrome involved the temporal or occipital lobes (or both). Among the nine with an occipital lesion, four had West syndrome. In the 19 with a temporal lesion, three had West syndrome. However, in the 16 patients with a frontal lesion, none exhibited epileptic spasms. In four of the five with West syndrome the lesions were on the right side; 23 had lesions on the right, 16 had them on the left. Temporo-occipital lesions and lesions on the right were related to the genesis of West syndrome, which would be in close correlation with normal brain maturation.
Cortical hypoperfusion in symptomatic West syndrome: A SPECT study
1997, European Journal of Radiology
Diffuse and focal changes in glucose utilization and abnormal cerebral cortical perfusion were found in West syndrome by PET and SPECT investigations. In this study^99mTc-HMPAO SPECT was performed on seven patients with symptomatic West syndrome several months after the onset of the spasms. Regions of interest of the cerebral cortex and cerebellum were delineated by an automated computer program and cortical/cerebellar ratios of the HMPAO uptake in the patients were compared to those of controls. The basal ganglia/cerebellar ratios were compared after manual placement of regions of interest. Significantly reduced perfusion was found in the bilateral anterior, mid frontal and perisylvian cortex, and in the left posterior frontal and temporal areas. Well localized, focal changes in the cortical perfusion were not found and the perfusion in the basal ganglia proved to be normal. These abnormalities in the cortical perfusion may reflect a pre-existing brain pathology together with an encephalopathy due to the hypsarrhythmia and infantile spasms.
Recent advances and some problems in the delineation of epileptic syndromes in children
1996, Brain and Development
The current International Classification of Epilepsies and Epileptic Syndromes has classified a number of age-related epileptic syndromes in children, but some of these entities have not been well delineated and many syndromes not included in the International Classification have been proposed. This article is intended to supplement some of the currently listed childhood epileptic syndromes, discuss certain problems with the classification system, and review some of the proposed pediatric syndromes.

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^☆: This study was supported in part by grants from the Japan Epilepsy Research Foundation, and a grant for Nervous and Mental Disorders from the Ministry of Health and Welfare.

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Original articleTransient focal cortical hypometabolism in idiopathic West syndrome☆

Abstract

Long-term follow-up study of 214 children with the syndrome of infantile spasms

Neuropediatrics

Long-term prognosis after infantile spasms: A statistical study of prognostic factor in 200 cases

Dev Med Child Neurol

Long-term prognosis of 150 cases of “West syndrome.”

Epilepsia

Infantile spasms: I. PET identifies focal cortical dysgenesis in cryptogenic cases for surgical treatment

Ann Neurol

Infantile spasms: II. Lenticular nuclei and brain stem activation on positron emission tomography

Ann Neurol

Clinical effects of thyrotropin-releasing hormone for severe epilepsy in childhood: A comparative study with ACTH

Epilepsia

Original article
Transient focal cortical hypometabolism in idiopathic West syndrome☆