Abstract:
:Infantile spasms are generalized seizures specific to early infancy, and are believed to result from complex cortical-subcortical interactions during a critical period of development. We used positron emission tomography (PET) to determine local cerebral metabolic rates for glucose (1CMRG1c) in 44 infants with spasms, in an attempt to define the neuroanatomical substrates that mediate these seizures. All infants were studied in the awake state during continuous electroencephalographic monitoring. The most consistent abnormality on PET, seen in 32 infants, was the symmetrical increase in 1CMRG1c in the lenticular nuclei, compared to age-matched normal infants (p less than 0.05). In 21 infants, even though the brain stem appeared to be visually more prominent compared to normal infants, statistically significant differences could not be demonstrated. Relative hypermetabolism of the lenticular nuclei (1) occurred irrespective of whether the spasms were cryptogenic or symptomatic, (2) was associated with focal cortical hypometabolism in 22 and focal cortical hypermetabolism in 5 of the 44 infants, and (3) was not characterized by any specific electroencephalographic abnormality during PET. These findings suggest that the lenticular nuclei may contribute to the pathophysiological state that predisposes to infantile spasms, and is consistent with the observation that spasms are clinically symmetrical even when focal cortical lesions are present. A scheme describing the neuronal circuitry likely to be involved in the generation of infantile spasms is proposed.
journal_name
Ann Neuroljournal_title
Annals of neurologyauthors
Chugani HT,Shewmon DA,Sankar R,Chen BC,Phelps MEdoi
10.1002/ana.410310212keywords:
subject
Has Abstractpub_date
1992-02-01 00:00:00pages
212-9issue
2eissn
0364-5134issn
1531-8249journal_volume
31pub_type
杂志文章abstract::Spinal muscular atrophy (SMA) is a motor neuron disease caused by dysfunction of the survival motor neuron (SMN) gene. Human SMN gene is present in duplicated copies: SMN1 and SMN2. More than 95% of patients with SMA lack a functional SMN1 but retain at least one copy of SMN2. Unlike SMN1, SMN2 is primarily transcribe...
journal_title:Annals of neurology
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journal_title:Annals of neurology
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journal_title:Annals of neurology
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journal_title:Annals of neurology
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pub_type: 临床试验,杂志文章
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journal_title:Annals of neurology
pub_type: 杂志文章
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更新日期:1993-03-01 00:00:00
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journal_title:Annals of neurology
pub_type: 临床试验,杂志文章
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journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410290513
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pub_type: 杂志文章
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更新日期:1992-11-01 00:00:00
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journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410230213
更新日期:1988-02-01 00:00:00
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doi:10.1002/ana.22102
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pub_type: 杂志文章,评审
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journal_title:Annals of neurology
pub_type: 临床试验,杂志文章
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更新日期:1999-07-01 00:00:00
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更新日期:1995-10-01 00:00:00