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Titre du document / Document title

Patients with Ehlers Danlos syndrome and CRPS : A possible association?

Auteur(s) / Author(s)

STOLER Joan M. (1) ; OAKLANDER Anne Louise (2 3 4) ;

Affiliation(s) du ou des auteurs / Author(s) Affiliation(s)

(1) Genetics and Teratology Unit, Department of Pediatrics, Massachusetts General Hospital, Harvard Medical School, Boston, MA, ETATS-UNIS
(2) Nerve Injury Unit, Department of Anesthesia and Critical Care, Massachusetts General Hospital, Harvard Medical School, Boston, MA, ETATS-UNIS
(3) Nerve Injury Unit, Department of Neurology, Massachusetts General Hospital, Harvard Medical School, Boston, MA, ETATS-UNIS
(4) Nerve Injury Unit, Department of Neuropathology, Massachusetts General Hospital, Harvard Medical School, Boston, MA, ETATS-UNIS

Résumé / Abstract

Rare patients are left with chronic pain, vasodysregulation, and other symptoms that define complex regional pain syndrome (CRPS), after limb traumas. The predisposing factors are unknown. Genetic factors undoubtedly contribute, but have not yet been identified. We report four CRPS patients also diagnosed with the classical or hypermobility forms of Ehlers Danlos syndrome (EDS), inherited disorders of connective tissue. These patients had been diagnosed using standard diagnostic criteria for CRPS and for EDS. All had sustained joint injury; in three this had been surgically treated. The association of these two diagnoses leads us to hypothesize that EDS might contribute to the development of CRPS in one or more of the following ways: via stretch injury to nerves traversing hypermobile joints, increased fragility of nerve connective tissue, or nerve trauma from more frequent surgery. We review the clinical presentation of the different Ehlers Danlos syndromes and provide clinical criteria that can be used to screen CRPS patients for EDS for clinical or research purposes.

Revue / Journal Title

Pain    ISSN  0304-3959   CODEN PAINDB 

Source / Source

2006, vol. 123, no1-2, pp. 204-209 [6 page(s) (article)] (25 ref.)

Langue / Language

Anglais

Editeur / Publisher

Elsevier, Philadelphia, PA, ETATS-UNIS  (1975) (Revue)

Mots-clés anglais / English Keywords

Nervous system diseases

;

Diseases of the autonomic nervous system

;

Osteoarticular system

;

Human

;

Reflex sympathetic dystrophy

;

Complex regional pain syndrome

;

Diagnosis

;

Joint

;

Trauma

;

Mots-clés français / French Keywords

Système nerveux pathologie

;

Système nerveux autonome pathologie

;

Système ostéoarticulaire

;

Homme

;

Dystrophie sympathique réflexe

;

Douleur régionale complexe syndrome

;

Diagnostic

;

Articulation

;

Traumatisme

;

Mots-clés espagnols / Spanish Keywords

Sistema nervioso patología

;

Sistema nervioso autónomo patología

;

Sistema osteoarticular

;

Hombre

;

Distrofia simpática refleja

;

Dolor regional complejo síndrome

;

Diagnóstico

;

Articulación

;

Traumatismo

;

Mots-clés d'auteur / Author Keywords

Connective tissue

;

Pain

;

Inherited disorder of collagen

;

Genetics

;

Reflex sympathetic dystrophy

;

Localisation / Location

INIST-CNRS, Cote INIST : 17241, 35400011570695.0250

Nº notice refdoc (ud4) : 17878835



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