| Radicular dysfunction due to spinal deformities in Marfan syndrome at older age: three case reports. | |
| | |
MedLine Citation:
|
PMID: 19879983 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
|
Marfan syndrome is a inherited connective tissue disorder due to mutations in fibrillin-1. It presents with cardiovascular, ocular, skeletal, pulmonary and dural signs and symptoms. Some of the symptoms of later onset are those associated with scoliosis and dural ectasia. This is the enlargement of the neural canal especially in the lower lumbar and sacral region and occurs in over 90% of Marfan patients. We here report three patients with lumbar and/or sacral radiculopathy due to (kypho)scoliosis and dural ectasia with spinal meningeal cysts. The pain, muscle weakness, muscle atrophy, and sensory disturbances illustrate the severe neurological complications which may occur in Marfan syndrome, especially at later age. Awareness of these complications and development of management protocols is essential since life expectancy of Marfan patients has increased. Marfan syndrome might gradually become recognized as an inherited connective tissue disorder with potentially severe neurological complications during ageing. |
| | |
Authors:
|
N C Voermans; A J Hosman; N van Alfen; R H Bartels; M de Kleuver; J W op den Akker; B G van Engelen |
Related Documents
:
|
11399383 - The carcinoid syndrome: an unusual cause of valvular heart disease. 20613543 - Health-related quality of life in marfan syndrome: a cross-sectional study of short for... 8281273 - Hunter-mcalpine syndrome: report of a third family. |
Publication Detail:
|
Type: Case Reports; Journal Article; Research Support, Non-U.S. Gov't Date: 2009-10-30 |
Journal Detail:
|
Title: European journal of medical genetics Volume: 53 ISSN: 1878-0849 ISO Abbreviation: Eur J Med Genet Publication Date: 2010 Jan-Feb |
Date Detail:
|
Created Date: 2010-02-04 Completed Date: 2010-04-16 Revised Date: - |
Medline Journal Info:
|
Nlm Unique ID: 101247089 Medline TA: Eur J Med Genet Country: Netherlands |
Other Details:
|
Languages: eng Pagination: 35-9 Citation Subset: IM |
Copyright Information:
|
Copyright (c) 2009 Elsevier Masson SAS. All rights reserved. |
Affiliation:
|
Donders Centre for Neuroscience, Department of Neurology, Radboud University Nijmegen Medical Centre, The Netherlands. n.voermans@neuro.umcn.nl |
Export Citation:
|
APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
|
Aging* Dura Mater / physiopathology Eye / physiopathology Female Humans Lumbar Vertebrae* / physiopathology, radiography Male Marfan Syndrome* / genetics, physiopathology Middle Aged Sacrococcygeal Region* / physiopathology, radiography Scoliosis / physiopathology |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
Previous Document: Loading and release of doxycycline hyclate from strontium-substituted calcium phosphate cement.
Next Document: Optimized synthesis of 1,3-dioleoyl-2-palmitoylglycerol rich triacylglycerol via interesterfication ...