Document Detail


Spinal cord atrophy in triple A syndrome associated with a novel compound heterozygous mutation.
MedLine Citation:
PMID:  20588230     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
A 38-year-old male patient was admitted with slowly progressive spastic gait disturbance. Imaging revealed general spinal cord atrophy. Because of adrenal insufficiency, alacrima and achalasia, triple A syndrome was suspected. This is a case report of a triple A syndrome patient with a predominance of neurological features and a new heterozygous compound mutation in triple A syndrome gene.
Authors:
Hagen Kunte; George Trendelenburg; Julia Matzen; Manfred Ventz; Uwe Kornak; Lutz Harms
Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Neuro endocrinology letters     Volume:  31     ISSN:  0172-780X     ISO Abbreviation:  Neuro Endocrinol. Lett.     Publication Date:  2010  
Date Detail:
Created Date:  2010-07-23     Completed Date:  2010-10-08     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  8008373     Medline TA:  Neuro Endocrinol Lett     Country:  Sweden    
Other Details:
Languages:  eng     Pagination:  301-3     Citation Subset:  IM    
Affiliation:
Department of Neurology, Charité-Universitätsmedizin Berlin, Germany. hagen.kunte@charite.de
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MeSH Terms
Descriptor/Qualifier:
Adrenal Insufficiency / complications,  genetics*
Adult
Atrophy / complications
Esophageal Achalasia / complications,  genetics*
Heterozygote
Humans
Lacrimal Apparatus Diseases / complications,  genetics*
Male
Mutation*
Nerve Tissue Proteins / genetics*
Nuclear Pore Complex Proteins / genetics*
Spinal Cord / pathology*
Syndrome
Chemical
Reg. No./Substance:
0/AAAS protein, human; 0/Nerve Tissue Proteins; 0/Nuclear Pore Complex Proteins

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


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