Abstract

Complex regional pain syndrome (CRPS) is a long-term, progressive disease characterized by severe pain, swelling, and changes in the skin. It usually affects the arms or legs and can spread to another part of the body and is associated with dysregulation of the autonomic nervous system resulting in loss of function, impairment, and disability. 

Introduction 

CRPS was coined by Silas Weir Mitchell, during the Civil War. Also known as reflex sympathetic dystrophy, it is a chronic, progressive disease characterized by severe pain, swelling, and changes in the skin. It usually affects the arms or legs and can spread to another part of the body and is associated with dysregulation of the autonomic nervous system resulting in loss of function, impairment, and disability. It is commonly seen in adolescent girls but has been described in children. ^{2, 3}. Vitamin C may have a therapeutic role related to its antioxidant properties. Here, we report a case of scurvy and CRPS in the same patient.

Case

A 5 year old girl came with inability to walk, pain in both lower limb. She was bedbound since last 6 months. She had extreme pain to light touch and swelling of both knees & ankles. Her diet was sufficient in proteins and calories. 

On examination patient is conscious, irritable vitals were stable. Patient was pallor, spongy gums with bleeding along with petechiae and hyperkeratosis on lower limbs. There was no lymphadenopathy or hepatosplenomegaly. Central nervous system examination revealed normal tone, 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.

The girl was diagnosed with scurvy and CRPS type 1. Dose of oral vitamin C 100 mg was given daily. Hyperesthesia started improving and child was able to walk with support within 15 days of treatment. After a duration of 1 month, she was walking independently & was perfectly normal.

Discussion

The International Association for the Study of Pain has proposed dividing CRPS into two types based on the presence of nerve lesion following the injury.

• Type I, formerly known as reflex sympathetic dystrophy (RSD), Sudeck′s atrophy, reflex neurovascular dystrophy (RND), or algoneurodystrophy, 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 at diagnosis is 42. CRPS has been diagnosed in children as young as 2 years old. It affects both men and women; however, CRPS is 3 times more frequent in females than males. The number of reported CRPS cases among adolescents and young adults is increasing.

There may be bilateral involvement. The pathophysiology of CRPS remains uncertain. Itmay be due to sympathethic dysfunction, central dysfunction or an inflammatory process. However recent research has suggested that oxidative damage (e.g. by free radicals) may play a role.

The International Association for the Study of Pain (IASP) lists the diagnostic criteria for complex regional pain syndrome I (CRPS I) (RSDS) as follows:

1. The presence of an initiating noxious event or a cause of immobilization
2. Continuing pain, allodynia (perception of pain from a nonpainful stimulus), or hyperalgesia (an exaggerated sense of pain) disproportionate to the inciting event.
3. Evidence at some time of edema, changes in skin blood flow, or abnormal sudomotor activity in the area of pain
4. 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 98–100% and a specificity ranging from 36–55%. Per the IASP guidelines, interobserver reliability for CRPS I diagnosis is poor. Two other criteria used for CRPS I diagnosis are Bruehls criteria and Veldmans criteria which have moderate to good interobserver reliability. In the absence of clear evidence supporting 1 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 the literature including treatment trials. 

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.

In teen-agers and younger patients with CRPS, the diagnosis is outstanding. Maximum of the patients recover evidently without invasive therapy, 75% of children have full recovery. Long term sequalae include shortening of limbs or foot because of prolonged immobilization and osteoporosis.

Our patient had showed response to vitamin C administration, so there might be some association between scurvy & CPRS.

Suggested Reading

1. Mitchell, S.W. (1872). Injuries of Nerves and their Consequences. Philadelphia: JB Lippincott.
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 (reflexsympathetic dystrophy) in a patient with essential mixed cryoglobulinemia and chronic hepatitis C. J Pak Med Assoc 2007; 57: 96-98.
4. Güler-Uysal F, Başaran S, Geertzen JH, Göncü K (2003). "A 2½-year-old girl with reflex sympathetic dystrophy syndrome (CRPS type I): case report". Clin Rehabil17 (2): 224–7. doi:10.1191/0269215503cr589oa
5. "RSDSA :: Reflex Sympathetic Dystrophy Syndrome Association". Rsds.org. 2010-01-21. Retrieved 2010-04-10.
6. Quisel A, Gill JM, Witherell P (2005). "Complex regional pain syndrome underdiagnosed". J Fam Pract54 (6): 524–32. PMID 15939004
7. Sandroni P , Low PA , Ferrer T, Opfer-Gehrking TL, Wilson PR . Complex regional pain syndrome: prospective study and laboratory evaluation. Clin J Pain1998; 14: 282-289.
8. Intenzo C, Kim S, Millin J, Park C. Scintigraphic patterns of reflex sympathetic dystrophy syndrome in lower extremities. ClinNucl Med 1989; 14:657-661.
9. 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: 520-523.
10. Eisenberg E, Geller R, Brill S. Pharmacotherapy options for complex regional pain syndrome. Expert Rev Neurother 2007; 7: 521-531.
11. Low AK, Ward K, Wines AP. Pediatric complex regional pain syndrome. J PediatrOrthop 2007; 27:567-572.
12. ZollingerPE ,Tuinebreijer WE, Breederveld RS ,INDIAN PEDIATRICS 531 Kreis RW. Can vitamin C prevent complex regional pain syndrome in patients with wrist fractures? J Bone Joint Surg Am 2007; 89: 1424-1431.