Document Detail


Septo-optic dysplasia with infantile spasms.
MedLine Citation:
PMID:  3233109     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
A 21-month-old boy with septo-optic dysplasia and infantile spasms is reported. Eighteen hours after birth he had generalized convulsions, dyspnea, and hypoglycemia which were followed by recurrent clonic seizures despite administration of phenobarbital and valproic acid. At 16 months of age he had hypoglycemia and apnea attacks during varicella infection. At 19 months of age left hemiconvulsions and left hemiparesis occurred; his mental and motor development, which had been delayed but progressive, deteriorated. Tonic spasms appeared at 21 months of age and electroencephalography revealed multifocal spikes. At 27 months of age electroencephalography disclosed hypsarrhythmia. Cranial computed tomography depicted brain atrophy, right microphthalmia, and intact septum pellucidum. Magnetic resonance imaging demonstrated hypoplasia of the corpus callosum and a small pituitary gland. Coloboma of the right optic disc was detected. Physical examination revealed short stature, left hemiparesis, micropenis, and cryptorchidism. Endocrinologic loading tests revealed hypofunction of the hypophysial anterior lobe.
Authors:
M Kuriyama; Y Shigematsu; K Konishi; Y Konishi; M Sudo; S Haruki; H Ito
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Pediatric neurology     Volume:  4     ISSN:  0887-8994     ISO Abbreviation:  Pediatr. Neurol.     Publication Date:    1988 Jan-Feb
Date Detail:
Created Date:  1989-05-10     Completed Date:  1989-05-10     Revised Date:  2006-05-23    
Medline Journal Info:
Nlm Unique ID:  8508183     Medline TA:  Pediatr Neurol     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  62-5     Citation Subset:  IM    
Affiliation:
Department of Pediatrics, Fukui Medical School, Japan.
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MeSH Terms
Descriptor/Qualifier:
Endocrine Glands / metabolism
Humans
Infant, Newborn
Male
Optic Nerve / abnormalities*
Septum Pellucidum / abnormalities*
Spasms, Infantile / complications*

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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