What causes complex regional pain syndrome (CRPS) in hemiplegia?

Updated: Feb 08, 2019
  • Author: Robert Gould, DO; Chief Editor: Stephen Kishner, MD, MHA  more...
  • Print


Complex regional pain syndrome (CRPS) is also known as shoulder-hand syndrome, RSD, causalgia, sympathetically maintained pain, Sudeck atrophy, and minor dystrophy. The International Association for the Study of Pain (IASP) has advocated using the terms CRPS type 1 (RSD) and CRPS type 2 (causalgia). The IASP categorization states that RSD develops secondary to noxious stimuli that are not limited to the distribution of a single peripheral nerve, while causalgia starts after a nerve injury.

The incidence of CRPS varies in the literature. Davis and coauthors reported that CRPS occurs in 12.5% of patients who have had a stroke, [27] while Chalsen and colleagues reported the incidence to be 61%. [28]

Onset of CRPS is within 3 months poststroke and rarely after 5 months poststroke. In a study, Davis and coworkers demonstrated that of those patients who developed CRPS, 65% had done so by 3 months poststroke, and 98% had done so by 5 months poststroke. [27]

CRPS is most commonly precipitated by bone or soft-tissue injuries, but in up to 30% of the cases, the patient does not remember experiencing an injury. Snider reported that about 5-8% of patients have an incomplete nerve injury. [29] Other precipitating factors may include the following:

  • Upper extremity immobilization

  • Myocardial infarction

  • Stroke

  • Rotator cuff tear

  • Shoulder spasticity

  • Glenohumeral joint subluxation

CRPS more commonly affects the upper extremity. Tepperman and colleagues reported metacarpophalangeal (MCP) joint tenderness to be the best diagnostic indicator, having a sensitivity and specificity of 85.7% and 100%, respectively. [30] Intuitively, however, it is questionable whether any one physical examination maneuver could have such high sensitivity and specificity for a syndrome as complex as RSD.

Using electromyography (EMG), Cheng and Hong found a significant correlation between the presence of spontaneous activity and the development of clinical RSD in 65% of subjects, whereas only 4% of those without spontaneous activity developed RSD. [31] However, this is not consistent with the definition of RSD set forth by the IASP, which dictates that patients with identifiable nerve lesions may have causalgia but not RSD.

Did this answer your question?
Additional feedback? (Optional)
Thank you for your feedback!