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Causalgia and complex regional pain syndrome (CRPS) type II with nerve injury can be difficult to treat. Surgical peripheral nerve denervation for causalgia has been largely abandoned by pain clinicians because of a perception that this may aggravate a central component (anesthesia dolorosa).
Methods:
We selectively searched Pubmed, Cochrane, MEDLINE, EMBASE, CINAHL Plus, and Scopus from 1947 for articles, books, and book chapters for evidence of surgical treatments (nerve resection and amputation) and treatment related to autoimmunity and immune deficiency with CRPS.
Results:
Reviews were found for the treatment of causalgia or CRPS type II (n = 6), causalgia relieved by nerve resection (n = 6), and causalgia and CRPS II treated by amputation (n = 8). Twelve reports were found of autoimmunity with CRPS, one paper of these on associated immune deficiency and autoimmunity, and two were chosen for discussion regarding treatment with immunoglobulin and one by plasma exchange. We document a report of a detailed and unique pathological examination of a CRPS type II affected amputated limb and related successful treatment with immunoglobulin.
Conclusions:
Nerve resection, with grafting, and relocation may relieve uncomplicated causalgia and CRPS type II in some patients in the long term. However, an unrecognized and treatable immunological condition may underly some CRPS II cases and can lead to the ultimate failure of surgical treatments.
By
Robert J. Shprintzen, Center for Diagnosis, Treatment and Study of Velo-Cardio-Facial Syndrome Syracuse, New York, USA
Edited by
Kieran C. Murphy, Education and Research Centre, Royal College of Surgeons of Ireland,Peter J. Scambler, Institute of Child Health, University College London
The recognition of velo-cardio-facial syndrome (VCFS) as a specific congenital malformation syndrome is a relatively recent development for so common a disorder. The failure to recognize VCFS earlier is probably related to a number of factors. Therefore, with the larger pattern of anomalies going undetected as a syndromic association, many earlier clinicians and researchers reported on the individual components of the syndrome as the focus of investigation, such as the speech problems, immune deficiency, endocrine disorders, and heart anomalies. As molecular genetics research moved forward, a number of candidate genes for the development of heart anomalies were identified. Molecular analysis showed that two of 100 cases studied had 22q11.2 deletions indicating that among people diagnosed as schizophrenic, there were likely to be individuals with VCFS. It was suggested that the psychiatric illness seen in individuals with VCFS was syndrome-specific and therefore was atypical for both schizophrenia and bipolar disorder.
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