Document Detail

Abrupt neurological deterioration in children with Kearns-Sayre syndrome.
MedLine Citation:
PMID:  7213149     Owner:  NLM     Status:  MEDLINE    
The conditions of a young woman and a boy with Kearns-Sayre syndrome (KSS) deteriorated abruptly; they died despite pacemaker control of complete heart block (case 1) and without evidence of arrhythmia or asystole. Extensive spongy vacuolization of the brainstem was shown by serial computerized tomographic scanning (case 2) and at autopsy (case 1). A review of the literature indicated that KSS in childhood is particularly severe and is associated with diffuse, progressive, spongy degeneration of the brain. Children with KSS have clinical, roentgenographic, and neuropathological evidence of spongy degeneration of the brain, which may be related to abrupt deterioration and death despite adequate control of heart block. Periodic brainstem auditory evoked response studies may allow early recognition of this process.
D L Coulter; R J Allen
Related Documents :
9663519 - Reliability of data on smoking habit and coffee drinking collected by personal intervie...
19926119 - Mthfr and the risk for cerebral venous thrombosis--a meta-analysis.
1922649 - A screening and alerting questionnaire for parkinsonism.
24891779 - Preduodenal portal vein in adult with polysplenia syndrome revisited with a case report.
7023269 - A case of status epilepticus. use of thiopentone and ippv to control otherwise refracto...
12705339 - Angiogenin and gestational trophoblastic tumors, a promising prognostic marker.
23508659 - Provisional report on diving-related fatalities in australian waters 2008.
9972169 - Immunohistochemistry of 'tertiary lymphoid follicles' in oral amalgam-associated lichen...
24685599 - Extraoral plasmablastic lymphoma detected using ascitic fluid cytology and flow cytomet...
Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Archives of neurology     Volume:  38     ISSN:  0003-9942     ISO Abbreviation:  Arch. Neurol.     Publication Date:  1981 Apr 
Date Detail:
Created Date:  1981-05-26     Completed Date:  1981-05-26     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  0372436     Medline TA:  Arch Neurol     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  247-50     Citation Subset:  AIM; IM    
Export Citation:
APA/MLA Format     Download EndNote     Download BibTex
MeSH Terms
Brain Stem / physiopathology,  radiography
Heart Block / complications,  pathology,  physiopathology*,  therapy
Nerve Degeneration*
Ophthalmoplegia / pathology,  physiopathology*
Pacemaker, Artificial
Retinitis Pigmentosa / pathology,  physiopathology*
Syncope / etiology,  therapy

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

Previous Document:  Computerized tomography in the diagnosis of central and extrapontine myelinolysis.
Next Document:  Doppler pulse waveform analysis of carotid artery flow in dissecting aortic aneurysm.