Document Detail


Fitzsimmons syndrome: spastic paraplegia, brachydactyly and cognitive impairment.
MedLine Citation:
PMID:  19760657     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Fitzsimmons syndrome is an infrequently described entity comprising slowly progressive spastic paraplegia, brachydactyly, and cone-shaped epiphyses, dysarthria, and low-normal intelligence. Five patients with this syndrome have been reported. The cause remains unknown. Here we describe a 16-year-old boy with Fitzsimmons syndrome. He was noted to toe-walk at age 18 months and spasticity progressed slowly into a spastic gait with contractures. He has mild dysarthria and hypernasal speech. Brachydactyly is notable but cannot be classified into one of the recognized types. A cone-shaped epiphysis was apparent on the only available childhood radiograph. He has moderate cognitive handicap and pervasive developmental delay. A detailed comparison of this patient with the earlier described cases is presented to further delineate the condition and increase awareness of this syndrome.
Authors:
Christine M Armour; Peter Humphreys; Raoul C M Hennekam; Kym M Boycott
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  American journal of medical genetics. Part A     Volume:  149A     ISSN:  1552-4833     ISO Abbreviation:  Am. J. Med. Genet. A     Publication Date:  2009 Oct 
Date Detail:
Created Date:  2009-10-01     Completed Date:  2009-12-17     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  101235741     Medline TA:  Am J Med Genet A     Country:  United States    
Other Details:
Languages:  eng     Pagination:  2254-7     Citation Subset:  IM    
Affiliation:
Department of Genetics, Children's Hospital of Eastern Ontario, Ottawa, Ontario, Canada.
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MeSH Terms
Descriptor/Qualifier:
Abnormalities, Multiple / diagnosis
Adolescent
Cognition Disorders / complications*
Foot Deformities, Congenital / complications*
Humans
Male
Paraplegia / complications*
Syndrome

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


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