Complex regional pain syndromes: new pathophysiological concepts and therapies

Complex regional pain syndromes: new pathophysiological concepts and therapies
European Journal of Neurology
C. Maihöfner, F. Seifert and K. Markovic
Department of Neurology, University Hospital Erlangen, Schwabachanlage ; and Department of Physiology and Experimental Pathophysiology, University of Erlangen – Nuremberg, Universitätsstraße, Erlangen, Germany

Correspondence to Christian Maihöfner, MD, PhD, Department of Neurology/Institute of Physiology and Experimental Pathophysiology, University of Erlangen – Nuremberg, Schwabachanlage 6, D-91054 Erlangen, Germany (tel.: +49 9131 853 3001; fax: +49 9131 852 2497; e-mail: christian.maihoefner@uk-erlangen.de).

Received 7 April 2009 Accepted 22 December 2009
Published Online 18 February 2010


Complex regional pain syndrome (CRPS), formerly known as Sudeck's dystrophy and causalgia, is a disabling and distressing pain syndrome. We here provide a review based on the current literature concerning the epidemiology, etiology, pathophysiology, diagnosis, and therapy of CRPS. CRPS may develop following fractures, limb trauma or lesions of the peripheral or CNS. The clinical picture comprises a characteristic clinical triad of symptoms including autonomic (disturbances of skin temperature, color, presence of sweating abnormalities), sensory (pain and hyperalgesia), and motor (paresis, tremor, dystonia) disturbances. Diagnosis is mainly based on clinical signs. Several pathophysiological concepts have been proposed to explain the complex symptoms of CRPS: (i) facilitated neurogenic inflammation; (ii) pathological sympatho-afferent coupling; and (iii) neuroplastic changes within the CNS. Furthermore, there is accumulating evidence that genetic factors may predispose for CRPS. Therapy is based on a multidisciplinary approach. Non-pharmacological approaches include physiotherapy and occupational therapy. Pharmacotherapy is based on individual symptoms and includes steroids, free radical scavengers, treatment of neuropathic pain, and finally agents interfering with bone metabolism (calcitonin, biphosphonates). Invasive therapeutic concepts include implantation of spinal cord stimulators. This review covers new aspects of pathophysiology and therapy of CRPS.