EXPLORE!

Complex Regional Pain Syndrome Type 1 Treated with Vitamin C

  972 Views

    19 April 2022

Abstract

Complex regional pain syndrome, formerly reflex sympathetic dystrophy or causalgia, is a chronic progressive disease characterized by severe pain, swelling, and changes in the skin. It often affects an arm or a leg and may spread to another part of the body and is associated with dysregulation of the autonomic nervous system resulting in multiple functional loss, impairment, and disability. It occurs mostly in adolescence. Although treatment is often unsatisfactory, we report a young girl treated satisfactorily with vitamin C.

Introduction

The condition currently known as complex regional pain syndrome (CRPS) was originally described during the American Civil War by Silas Weir Mitchell, who is sometimes also creditedwith inventing the name “causalgia,”1 also known as reflex sympathetic dystrophy (RSD). It is a chronic progressive disease characterized by severe pain, swelling, and changes in the skin. It often affects an arm or a leg and may spread to another part of the body; it is associated with dysregulation of the autonomic nervous system resulting in multiple functional loss, impairment, and disability. It is usually seen in adolescent girls but has been described in children.2,3 Vitamin C may have a therapeutic role related to its antioxidant properties; vitamin C deficiency has not been implicated as cause of CRPS. We are reporting scurvy and CRPS in the same patient.

Case Report

A 3-year-old female child presented with inability to walk, pain in both lower limb, and was bedridden since 6 months. After a minor accident, the patient developed swelling of both knees and ankles; she had excessive pain when touched lightly and there was excessive sweating. Diet was adequate in proteins and calories. Developmental milestones were normal. On examination, the patient was conscious, irritable, and the vitals were stable. Patient had pallor with bleeding spongy gums along with petechiae and hyperkeratosis on lower limbs. There was no lymphadenopathy or hepatosplenomegaly. Central nervous system examination revealed normaltone, power and reflexes in all limbs. There was hyperesthesia in both lower limbs. On local examination, she was not moving her lower limbs; both lower limbs were in flexed attitude.

Temperature of the swollen part was raised. As given in the Gerald Fenichel’s Clinical Pediatric Neurology, we immersed the affected limbs in warm water. Wrinkling of the skin of toes was absent as compared to wrinkling of hands. As wrinkling of fingers and toes requires intact sympathetic innervation, this manner was helpful in diagnosing. Hemoglobin was 6 g/dL, TLC was 11,000/mm3, and platelet count was 6,12,000/mm3. Peripheral smear revealed microcytic, hypochromic anemia; there were no abnormal cells. CPK and VDRL were normal. X-ray of the hip and knee joints revealed pencil-thin cortex, decreased bone density, and white line of Frankel, suggestive of scurvy. A provisional diagnosis of scurvy and CRPS type 1 was made.

Hundred milligram of oral vitamin C was given daily. Hyperesthesia started improving and the child was able to walk with support within 15 days of treatment. After 1 month duration, she started walking independently and was perfectly normal. 

Discussion

CRPS, formerly RSD or causalgia, is a chronic progressive disease characterized by severe pain, swelling, and changes in the skin. It often affects an arm or a leg and may spread to another part of the body; it is associated with dysregulation of the autonomic nervous system resulting in multiple functional loss, impairment, and disability. Although treatment is often unsatisfactory, early multimodal therapy can cause dramatic improvement or remission of the syndrome in some patients.4 The International Association for the Study of Pain has proposed dividing CRPS into two types based on the presence of nerve lesion following an injury.

  • Type I, formerly known as RSD, Sudeck’s atrophy, reflex neurovascular dystrophy, oralgoneurodystrophy, does not have demonstrable nerve lesions.
  • Type II, formerly known as causalgia, has evidence of obvious nerve damage.

CRPS can strike at any age, but the mean age of diagnosis is 42.5 CRPS has been diagnosed in children as young as 2 years old.6 It affects both men and women; however, CRPS is three times more frequent in females than males.5 The number of reported CRPS cases among adolescents and young adults is increasing.7 There may be bilateral involvement.2 The pathophysiology of CRPS remains uncertain. It may be due to sympathetic dysfunction, central dysfunction, or an inflammatory process. However, recent research has suggested that oxidative damage (e.g., by free radicals) may play a role.5 The International Association for the Study of Pain (IASP) lists the diagnostic criteria for CRPS I (RSDS) as follows:

  • The presence of an initiating noxious event or a cause of immobilization.
  • Continuing pain, allodynia (perception of pain from a nonpainful stimulus), or hyperalgesia (an exaggerated sense of pain) disproportionate to the inciting event.
  • Evidence at some time of edema, changes in skin blood flow, or abnormal sudomotor activity in the area of pain.
  • The diagnosis is excluded by the existence of any condition that would otherwise account for the degree of pain and dysfunction.

The IASP criteria for CRPS I diagnosis has shown a sensitivity ranging from 98% to 100% and a specificity ranging from 36% to 55%. As per the IASP guidelines, interobserver reliability for CRPS I diagnosis is poor. Two other criteria used for CRPS I diagnosis are Bruehl’s criteria and Veldman’s criteria that have moderate-togoodinterobserver reliability. In the absence of clear evidence supporting one set of criteria over the others, clinicians may use IASP, Bruehl’s, or Veldman’s clinical criteria for diagnosis. While the IASP criteria are nonspecific and possibly not as reproducible as Bruehl’s or Veldman’s criteria, they are cited more widely in the literature including treatment trials.8 According to Veldman et al,5 diagnosis of CRPS can be made clinically based on the following: 

  • At least four of the five symptoms and signs are present: unexplained diffuse pain, altered skin color, altered skin temperature, edema, and reduced active range of movements.
  • Symptoms aggravated by activity of the extremity.
  • Symptoms present in an area much larger than and distal to the area primary injury.

All these features were seen in our patient. The International Association for Study of Pain criteria are also similar, with electromyography (EMG) and nerve conduction velocity (NCV) required to distinguish between type 1 and 2, although the clinical validation of these criteria are still debated.2 Veldman’s criteria are most widely used. No specific test is available for CRPS and diagnosis is primarily through observations of symptoms. However, thermography, sweat test, X-ray, and sympathetic blocks can be used to build a picture of the disorder.9 EMG/NCV can help differentiate early phases of CRPS type 2. Scintigraphy and bone scan have a sensitivity of 72% and 50%, respectively.10,11 Absence of abnormal tests does not preclude diagnosis of CRPS. Early diagnosis is the mainstay of successful treatment of RSD. Management consists of physiotherapy, sympathetic blocks, epidural blocks, drug treatment (alpha blockers, calcium channel blockers, NSAIDs, calcitonin, corticosteroid and antidepressants) and surgical sympathectomy.12,13 Vitamin C could have some efficacy related to its antioxidant properties. One double-blind study showed that vitamin C given to patients with wrist fractures reduced the incidence of CRPS.14 In two placebo-controlled randomized clinical trials, Zollinger et al14 showed that 500 mg vitamin C daily reduces the chance for the occurrence of CRPS afterwrist fractures.14 In teens and younger patients with CRPS, the prognosis is excellent. Most of the patients improved markedly without invasive therapy; 75% of children had full recovery. Long-term sequalae include shortening of limbs or foot because of prolonged immobilization and osteoporosis.5

Since our patient had showed response to vitamin C administration, there might be some association between scurvy and CRPS.

References

  1. Mitchell SW. Injuries of Nerves and their Consequences.Philadelphia: JB Lippincott 1872.
  2. de Mos M, de Bruijn AG, Huygen FJ, Dieleman JP, Stricker BH, Sturkenboom MC. The incidence of complex regional pain syndrome: a population-based study. Pain 2007;129:12-20.
  3. Bant A, Hurowitz B, Hassan N, Du VT, Nadir A. Complex regional pain syndrome (reflex sympathetic dystrophy) in a patient with essential mixed cryoglobulinemia and chronic hepatitis C. J Pak Med Assoc 2007;57:96-8.
  4. Neuropathic pain. Merck Manual for Healthcare Professionals. Available at:  http://www.merckmanuals.com/professional/
  5. Veldman PH, Reynen HM, Arntz IE, Goris RJ. Signs and symptoms of reflex sympatheticdystrophy: prospective study of 829 patients. Lancet 1993;342(8878):1012-6.
  6. Güler-Uysal F, Başaran S, Geertzen JH, Göncü K. A 2½-year-old girl with reflex sympathetic dystrophy syndrome (CRPS type I): case report. ClinRehabil 2003;17(2):224-7.
  7. RSDSA: Reflex Sympathetic Dystrophy Syndrome Association. Available at: http://www.rsds.org.
  8. Quisel A, Gill JM, Witherell P. Complex regional pain syndrome underdiagnosed. J FamPract 2005;54(6):524-32.
  9. Sandroni P, Low PA, Ferrer T, Opfer-Gehrking TL, Wilson PR. Complex regional pain syndrome: prospective study and laboratory evaluation. Clin J Pain 1998;14:282-9.
  10. Intenzo C, Kim S, Millin J, Park C. Scintigraphic patterns of reflex sympathetic dystrophy syndrome in lower extremities. ClinNucl Med 1989;14:657-61.
  11. Werner R, Davidcoff G, Jackson HD, Cremer S, Ventocilla C, Wolf L. Factors affecting the sensitivity and specificity of the three phase bone scan in the diagnosis of reflex sympathetic dystrophy in the upper extremity. J Hand Surg (Am) 1989;14:520-3.
  12. Eisenberg E, Geller R, Brill S. Pharmacotherapy options for complex regional pain syndrome. Expert Rev Neurother 2007;7:521-31.
  13. Low AK, Ward K, Wines AP. Pediatric complex regional pain syndrome. J PediatrOrthop 2007;27:567-72.
  14. Zollinger PE, Tuinebreijer WE, Breederveld RS, Kreis RW. Can vitamin C prevent complex regional pain syndrome in patients with wrist fractures? J Bone Joint Surg Am 2007;89:1424-31.

To comment on this article,
create a free account.

Sign Up to instantly get access to 10000+ Articles & 1000+ Cases

Already registered?

Login Now

Most Popular Articles

News and Updates

eMediNexus provides latest updates on medical news, medical case studies from India. In-depth medical case studies and research designed for doctors and healthcare professionals.