Peripheral neuropathy in Ehlers-Danlos syndrome.
Two unrelated male patients with clinical manifestations of Ehlers-Danlos syndrome type III and peripheral neuropathy are presented. At age 13 years, one developed bilateral brachial plexus palsy unrelated to trauma and 2 years later, a right lumbosacral plexopathy. The other presented at age 3 years with a left brachial plexopathy after sustaining a fracture of the lateral condyle of the right humerus. In both patients, nerve conduction velocities demonstrated conduction block across the brachial plexus and recovery was incomplete, indicating that peripheral neuropathy is a serious complication of Ehlers-Danlos syndrome. Its prompt diagnosis facilitates the care of patients with this syndrome. Increased ligament laxity/stretchability and mechanical trauma may play an important role in the pathogenesis of the neuropathy.
Galan
E
E
Department of Pediatrics, University of South Florida, Tampa 33617-3451, USA.
Kousseff
B G
BG
D000293:Adolescent; D001917:Brachial Plexus / Q000503:physiopathology; D002648:Child; D004535:Ehlers-Danlos Syndrome / Q000175:diagnosis / Q000235:genetics* / Q000503:physiopathology; D006801:Humans; D008160:Lumbosacral Plexus / Q000503:physiopathology; D008297:Male; D009460:Neurologic Examination; D010523:Peripheral Nervous System Diseases / Q000175:diagnosis / Q000235:genetics* / Q000503:physiopathology; D009435:Synaptic Transmission / Q000502:physiology