Complex regional pain syndrome (CRPS) is a poorly understood pain disorder with predominantly neuropathic features. It is often, but not always, associated with damage to the central or peripheral nervous system, and in such cases it may aggravate the predominant disability. We describe a unique case of bilateral CRPS occurring in a patient after traumatic spinal cord injury.
Complex Regional Pain Syndrome (CRPS) manifests with a cluster of symptoms including pain, allodynia, swelling, vasomotor impairment, limited range of motion, and motor dysfunction . It can be divided into two subtypes, namely type 1, where no nerve damage occurs, and type 2, where there is associated underlying nerve damage. Literature on bilateral CRPS in central cord syndrome is sparse and limited to case reports [2,3].
CRPS is easily overlooked by internists treating painful neuromuscular conditions due to a heterogeneous range of clinical differentials. The primary purpose of this case report is to provide a general overview of CRPS and to educate physicians about the complexities of diagnosis and treatment when it presents bilaterally.
Presentation of the case
A 68-year-old man, with no known drug allergies and a history of ischemic heart disease, for which he underwent percutaneous coronary intervention in 2003 and has since been taking aspirin and atorvastatin, hyperlipidemia, and degenerative cervical spondylosis, presented with cord syndrome after falling down a flight of stairs.
He underwent a C5-C6 anterior cervical discectomy and fusion and was transferred to the inpatient rehabilitation unit for spinal care. To standardize his neurological assessment, the International Standards for Neurological Classification of Spinal Cord Injury (ISNCSCI) were applied. . His motor and sensory examination results are presented in the table 1. At the initial evaluation, there were no signs of spasticity.
On the 20th day after the operation, he developed bilateral hand swelling accompanied by pain associated with warmth and redness in both hands, which hampered his therapeutic progress. He was able to ambulate with contact assistance and perform the basic activities of daily living (bADL); however, his fine motor function was affected. He was suspected of having CRPS and started taking prednisolone 30mg once a day. A triphasic bone scan (TPBS) revealed a symmetrical increase in radiotracer activity in both hands (Figure 1), confirming the diagnosis.
He underwent three weeks of hospital rehabilitation. The multidisciplinary team consisted of a rehabilitation physician (physiatrist), a nuclear medicine specialist, occupational therapists (OT), physiotherapists (PT) and a dietitian. The patient received at least five two-hour sessions of physiotherapy and occupational therapy per week. Sessions focused on range of motion, strengthening, endurance, desensitization strategies, and ADL recycling.
His symptoms and functional ability improved after three weeks of steroids and inpatient rehabilitation. For example, his precision grip changed from cylindrical to tripod when working with clothespins and finally to a pincer grip when using the arc ring.
He was referred to outpatient therapy. However, there was still some residual edema noted on his hand, for which he started fluid therapy, acupuncture, pressure bandaging and kinesiology tape. In a subsequent outpatient follow-up, he reported that bandaging did not improve hand swelling, although the other three physical modalities were effective.
Bilateral CRPS type 1 (CRPS-1) is often described, although CRPS type 2 (CRPS-2), in which there is evidence of nerve damage, rarely manifests bilaterally and even then does not has been reported only in the lower limbs. CRPS as a whole may be underreported as the differentials include clinically similar but more common mimics such as heterotopic ossification (HO) and deep vein thrombosis (DVT) [5-7]. There are no studies on the prevalence of bilateral CRPS in patients with central cord syndrome. The diagnosis of CRPS currently follows the criteria of the Budapest Consensus Group [Table 2] .
Clinical diagnosis is confirmed by TPBS, which is a radionucleotide scan, in which increased uptake of radiotracers can be observed during the angiographic, blood and delayed phases of the scan. . In our case, bilateral diagnosis was technically challenging, as asymmetric radiotracer uptake in the affected limb usually aids in visual diagnosis of unilateral CRPS, although this is still feasible based on comparison with other joints. from the body.
The treatment of choice for an acute attack of CRPS is corticosteroid therapy. An acceptable starting dose is prednisolone 60 mg with a dose reduction of 5 mg daily to 20 mg, followed by 5 mg weekly, although a lower starting dose of 40 mg may be considered to reduce potential side effects in elderly and diabetic patients. . We made no dose escalation to account for the bilateral presentation, and his condition improved with 30 mg of prednisolone daily, which we considered an appropriate dose for unilateral conditions.
Delay in diagnosis and initiation of treatment during the acute phase is associated with multiple disabling musculoskeletal complications, including muscle wasting, tendon shortening, contractures, osteoporosis and arthropathies, which will disrupt inevitably ADLs as well as quality of life. . Although our patient experienced good neurological recovery regarding muscle power, the persistent swelling continued to affect his functional recovery in bimanual tasks.
Promising new treatments for subacute CRPS include other pharmacological options such as ketamine, memantine, intravenous immunoglobulin, and epidural or intrathecal drugs such as baclofen and clonidine . The role of sympathetic blockade remains under investigation. Beyond these goals, rehabilitation goals should remain focused on pain relief, functional restoration and psychological stabilization of the patient. Conventional physical and occupational therapy are supplemented with physical modalities such as kinesiology tape, electrical stimulation, and mirror therapy. Fluid therapy is a form of dry heat therapy involving heat transfer through fine particles circulating in a chamber of heated air that can offer both surface heat and tactile stimulation. It has been used to relieve musculoskeletal pain and swelling. The addition of fluid therapy in addition to conventional rehabilitation has also been reported to reduce neuropathic pain and edema in CRPS in a post-stroke population. .
We report a rare case of bilateral CRPS following central cord syndrome and describe the diagnosis and multimodal management of this challenging condition. We look forward to future studies to shed light on new strategies in the assessment and management of these patients. When in doubt, referral to rehabilitation medicine can help with prompt diagnosis and early treatment to address pain and functional impairments.