Document Detail


Nerve conduction studies on SPOAN syndrome.
MedLine Citation:
PMID:  24123118     Owner:  NLM     Status:  Publisher    
Abstract/OtherAbstract:
Introduction: SPOAN syndrome (spastic paraplegia, optic atrophy, and neuropathy), is an autosomal recessive neurodegenerative disorder identified in a large consanguineous Brazilian family. Methods: 27 patients with SPOAN syndrome (20 women) aged 4 to 58 years underwent nerve conduction studies (NCS) of median, ulnar, tibial, and fibular nerves and sensory NCS of median, ulnar, radial, sural, and superficial fibular nerves. Results: Sensory nerve action potentials were absent in the lower limbs and absent in more than 80% of upper limbs. Motor NCS had reduced amplitudes and borderline velocities in the upper limbs and absent compound muscle action potentials (CMAPs) in the lower limbs. Conclusion: The neuropathy in SPOAN syndrome is a severe early-onset sensory-motor axonal polyneuropathy. Normal NCS seem to rule out this condition. © 2013 Wiley Periodicals, Inc.
Authors:
Simone Amorim; Carlos Otto Heise; Silvana Santos; Lúcia Ines Macedo-Souza; Mayana Zatz; Fernando Kok
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2013-10-7
Journal Detail:
Title:  Muscle & nerve     Volume:  -     ISSN:  1097-4598     ISO Abbreviation:  Muscle Nerve     Publication Date:  2013 Oct 
Date Detail:
Created Date:  2013-10-14     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  7803146     Medline TA:  Muscle Nerve     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Copyright Information:
Copyright © 2013 Wiley Periodicals, Inc., a Wiley company.
Affiliation:
Neurology Department, University of São Paulo School of Medicine, São Paulo, Brazil.
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