Evolutional changes of cortical hypometabolism in West's syndrome

Summary The pathophysiological basis of the epileptic encephalopathy West's syndrome remains unknown. We have done serial positron emission tomography (PET) with fluorine-18-labelled 2-deoxy-3-fluoro-D-glucose (FDG) in twelve patients with newly diagnosed West's syndrome. Throughout follow...

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Veröffentlicht in:The Lancet (British edition) 1994-06, Vol.343 (8913), p.1620-1623
Hauptverfasser: Maeda, N., Watanabe, K., Negoro, T., Aso, K., Ohki, T., lto, K., Kato, T.
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container_end_page 1623
container_issue 8913
container_start_page 1620
container_title The Lancet (British edition)
container_volume 343
creator Maeda, N.
Watanabe, K.
Negoro, T.
Aso, K.
Ohki, T.
lto, K.
Kato, T.
description Summary The pathophysiological basis of the epileptic encephalopathy West's syndrome remains unknown. We have done serial positron emission tomography (PET) with fluorine-18-labelled 2-deoxy-3-fluoro-D-glucose (FDG) in twelve patients with newly diagnosed West's syndrome. Throughout follow-up, PET revealed diffuse or focal cortical hypometabolism in eleven patients, whereas magnetic resonance imaging (MRI) showed morphological abnormalities in only five. At disease onset, PET showed cortical hypometabolism in eight patients (diffuse in three, focal in five). The second PET showed normal metabolism in six of these patients but focal abnormalities in three of the four with normal results on first PET. In all seven patients with normal findings on the second PET, tonic spasms ceased after initial treatment and no epileptic seizure occurred thereafter. In the five patients with cortical hypometabolism on the second PET, tonic spasms persisted or recurred, or partial seizures appeared. However, in two patients PET abnormalities disappeared in accordance with the later resolution of epileptic seizures. All patients with normal MRI and second PET results had normal psychomotor development. Diffuse or focal cortical hypometabolism that cannot be detected by MRI or computed tomography is common in patients with West's syndrome. However, this anomaly is not permanent and changes with clinical symptoms. These functional abnormalities in the cerebral cortex may be associated with the development of West's syndrome.
doi_str_mv 10.1016/S0140-6736(94)93065-1
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We have done serial positron emission tomography (PET) with fluorine-18-labelled 2-deoxy-3-fluoro-D-glucose (FDG) in twelve patients with newly diagnosed West's syndrome. Throughout follow-up, PET revealed diffuse or focal cortical hypometabolism in eleven patients, whereas magnetic resonance imaging (MRI) showed morphological abnormalities in only five. At disease onset, PET showed cortical hypometabolism in eight patients (diffuse in three, focal in five). The second PET showed normal metabolism in six of these patients but focal abnormalities in three of the four with normal results on first PET. In all seven patients with normal findings on the second PET, tonic spasms ceased after initial treatment and no epileptic seizure occurred thereafter. In the five patients with cortical hypometabolism on the second PET, tonic spasms persisted or recurred, or partial seizures appeared. However, in two patients PET abnormalities disappeared in accordance with the later resolution of epileptic seizures. All patients with normal MRI and second PET results had normal psychomotor development. Diffuse or focal cortical hypometabolism that cannot be detected by MRI or computed tomography is common in patients with West's syndrome. However, this anomaly is not permanent and changes with clinical symptoms. These functional abnormalities in the cerebral cortex may be associated with the development of West's syndrome.</description><identifier>ISSN: 0140-6736</identifier><identifier>EISSN: 1474-547X</identifier><identifier>DOI: 10.1016/S0140-6736(94)93065-1</identifier><identifier>PMID: 7911927</identifier><identifier>CODEN: LANCAO</identifier><language>eng</language><publisher>London: Elsevier Ltd</publisher><subject>Biological and medical sciences ; Cerebral Cortex - diagnostic imaging ; Cerebral Cortex - metabolism ; Cerebral Cortex - pathology ; Computed tomography ; Deoxyglucose - analogs &amp; derivatives ; Emissions ; Encephalitis ; Female ; Fluorine ; Fluorodeoxyglucose F18 ; Headache. Facial pains. Syncopes. Epilepsia. Intracranial hypertension. Brain oedema. 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We have done serial positron emission tomography (PET) with fluorine-18-labelled 2-deoxy-3-fluoro-D-glucose (FDG) in twelve patients with newly diagnosed West's syndrome. Throughout follow-up, PET revealed diffuse or focal cortical hypometabolism in eleven patients, whereas magnetic resonance imaging (MRI) showed morphological abnormalities in only five. At disease onset, PET showed cortical hypometabolism in eight patients (diffuse in three, focal in five). The second PET showed normal metabolism in six of these patients but focal abnormalities in three of the four with normal results on first PET. In all seven patients with normal findings on the second PET, tonic spasms ceased after initial treatment and no epileptic seizure occurred thereafter. In the five patients with cortical hypometabolism on the second PET, tonic spasms persisted or recurred, or partial seizures appeared. 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We have done serial positron emission tomography (PET) with fluorine-18-labelled 2-deoxy-3-fluoro-D-glucose (FDG) in twelve patients with newly diagnosed West's syndrome. Throughout follow-up, PET revealed diffuse or focal cortical hypometabolism in eleven patients, whereas magnetic resonance imaging (MRI) showed morphological abnormalities in only five. At disease onset, PET showed cortical hypometabolism in eight patients (diffuse in three, focal in five). The second PET showed normal metabolism in six of these patients but focal abnormalities in three of the four with normal results on first PET. In all seven patients with normal findings on the second PET, tonic spasms ceased after initial treatment and no epileptic seizure occurred thereafter. In the five patients with cortical hypometabolism on the second PET, tonic spasms persisted or recurred, or partial seizures appeared. 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subjects Biological and medical sciences
Cerebral Cortex - diagnostic imaging
Cerebral Cortex - metabolism
Cerebral Cortex - pathology
Computed tomography
Deoxyglucose - analogs & derivatives
Emissions
Encephalitis
Female
Fluorine
Fluorodeoxyglucose F18
Headache. Facial pains. Syncopes. Epilepsia. Intracranial hypertension. Brain oedema. Cerebral palsy
Humans
Infant
Magnetic Resonance Imaging
Male
Medical research
Medical sciences
Nervous system (semeiology, syndromes)
Neurology
Spasms, Infantile - diagnosis
Spasms, Infantile - diagnostic imaging
Spasms, Infantile - metabolism
Tomography
Tomography, Emission-Computed
title Evolutional changes of cortical hypometabolism in West's syndrome
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