Spontaneous onset of Complex Regional Pain Syndrome

Department of Neurology, Leiden University Medical Center, Leiden, The Netherlands.
European journal of pain (London, England) (Impact Factor: 2.93). 09/2009; 14(5):510-3. DOI: 10.1016/j.ejpain.2009.08.007
Source: PubMed

ABSTRACT Complex Regional Pain Syndrome (CRPS) usually develops after a noxious event, but spontaneous onsets have been described in 3-11% of the cases. The existence of spontaneous-onset CRPS is highly debated and the aim of the present study was therefore to compare the phenotypic characteristics of CRPS patients with a spontaneous onset, with those of patients with a trauma-induced onset. Data of 537 CRPS patients followed up at four departments of anesthesiology were analyzed and comprised 498 (93%) patients with and 39 (7%) patients without a known eliciting event. There where no significant differences between the two groups in gender, or in onset in upper or lower limb or left or right side of the body. Compared to CRPS patients with a trauma-induced onset, spontaneous-onset cases were on average 9 years younger at disease onset and had a 1.4 years longer median disease duration. No significant differences in frequency were found for any of the 34 compared signs and symptoms when the effect of multiple testing was controlled. In conclusion, CRPS may develop both with and without a precipitating noxious event, with both groups exhibiting a largely similar clinical presentation. Spontaneous-onset CRPS patients generally develop the syndrome at a younger age, possibly indicating a susceptibility to develop the condition. The longer disease duration in spontaneous-onset cases may reflect a more gradual disease onset, poorer prognosis, or a delay in diagnosis, possibly as a result of reluctance to make this diagnosis in the absence of a clear initiating event.

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Available from: Johan Marinus, Sep 27, 2015
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    • "Health professionals have often described physical trauma as antecedent events for CRPS, since the condition was first described (Mitchell 1872). However, the role for psychological stressors as an initiator of CRPS, rather than a consequence of the disorder, has not yet been substantiated (de Rooij et al. 2010). Individuals with CPRS have often been viewed as having a pre-existing susceptibility for exaggerated pain and movement disorders. "
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    ABSTRACT: Complex Regional Pain Syndrome (CRPS) is associated with non-dermatomal patterns of pain, unusual movement disorders, and somatovisceral dysfunctions. These symptoms are viewed by some neurologists and psychiatrists as being psychogenic in origin. Recent evidence, however, suggests that an autoimmune attack on self-antigens found in the peripheral and central nervous system may underlie a number of CRPS symptoms. From both animal and human studies, evidence is accumulating that neuroinflammation can spread, either anterograde or retrograde, via axonal projections in the CNS, thereby establishing neuroinflammatory tracks and secondary neuroinflammatory foci within the neuraxis. These findings suggest that neuroinflammatory lesions, as well as their associated functional consequences, should be evaluated during the differential diagnosis of non-dermatomal pain presentations, atypical movement disorders, as well as other "medically unexplained symptoms", which are often attributed to psychogenic illness.
    Journal of Neuroimmune Pharmacology 08/2012; 8(3). DOI:10.1007/s11481-012-9392-x · 4.11 Impact Factor
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    ABSTRACT: Complex regional pain syndrome type 1 (CRPS-1) is a chronic pain disorder that in some patients is associated with fixed dystonia. The pathogenesis of CRPS and its relation to dystonia remain poorly understood. Several genes (so-called DYT genes) identified in other causes of dystonia play a role in mechanisms that have been implicated in CRPS. Because different mutations in the same gene can result in diverse phenotypes, we sequenced all coding exons of the DYT1, DYT5a, DYT5b, DYT6, DYT11, DYT12, and DYT16 genes in 44 CRPS patients with fixed dystonia to investigate whether high-penetrant causal mutations play a role in CRPS. No such mutations were identified, indicating that these genes do not seem to play a major role in CRPS.
    Journal of Neurology 05/2010; 257(5):820-4. DOI:10.1007/s00415-009-5426-6 · 3.38 Impact Factor
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