Key Words: CPRS, Autonomic nervous system, Vit. C

Introduction

The condition currently known as CRPS was originally described during the American Civil War by Silas Weir Mitchell, who is sometimes also credited with inventing the name "causalgia."[1] ; also known as Reflex Sympathetic Dystrophy, 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 and 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

A 3 year old female child presented with inability to walk, pain in both lower limb & she was bedridden from 6 months. After a minor accident, patient developed swelling of both knees & ankles, she had excessive pain to light touch and excessive sweating. Diet was adequate in proteins and calories. Developmental milestones were normal.

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

 As given in 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 innervations this manure is helpful in diagnosing. 

Hemoglobin was 6 g/dL, TLC of 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 hip &knee joints revealed pencil thin cortex, decreased bone density and white line of Frankel, suggestive of scurvy. A provisional diagnosis of scurvy and CRPS type1 was made.

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

Discussion

Complex regional pain syndrome (CRPS), 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. Though 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 the injury.

1. Type I, formerly known as reflex sympathetic dystrophy (RSD), Sudecks atrophy, reflex neurovascular dystrophy (RND), or algoneurodystrophy, does not have demonstrable nerve lesions.
2. 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.[5] CRPS has been diagnosed in children as young as 2 years old. [6]It affects both men and women; however, CRPS is 3 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 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[5].

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.[8]

According to Veldman, et al.[5] diagnosis of CRPS can be made clinically if

1.  at least 4 of the 5symptoms and signs are present: unexplained diffuse pain, altered skin color, altered skin temperature,edema and reduced active range of movements
2. symptoms aggravated by activity of the extremity;and (iii)symptoms are present in an area much larger than and distal to primary injury.

 All these featureswere seen in our patient. International Association for Study of Pain criteria are also similar, with NCVand EMG required to distinguish between type 1 and2, though 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 uppicture 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 consist of physiotherapy, sympathetic blocks, epidural blocks, drug treatment (alpha blocker, calcium channel blocker, NSAID, calcitonin, corticosteroid, antidepressant) and surgical sympathectomy[12.13].

Vitamin C could have some efficacy related to its antioxidant properties. One double blind study showed thatvitamin C given to patients with wrist fractures reduced the incidence of CRPS[14].

In two placebo-controlled randomized clinical trials Zollinger et al. show that 500 mg vitamin C daily, reduces the chance for the occurrence of CRPS after wrist fractures.[15]

In teens and younger patients with CRPS, the prognosis is excellent. Most of the patients improve markedly without invasive therapy, 75% of children have full recovery. Long term sequalae include shortening of limbs or foot because of prolonged immobilization and osteoporosis [5].

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

About the Authors

Dr Shital Bhattad Gondhali, Assistant Professor in MIMSR  Medical, College Dept. Of paediatrics, Latur; Maharashtra.

Dr Suresh H. Bhattad, Professor in MIMSR Medical, College Dept. Of paediatrics, Latur; Maharashtra.

Dr Girish Nanoti, Associate Professor in NKPSIMS & Lata Mangeshkar Medical College, Dept. Of paediatrics, Nagpur

Dr Himanshu Dua, Assistant Professor in NKPSIMS& Lata Mangeshkar Medical College

Dept. Of paediatrics, Nagpur

Dr. Bhupendra Asudani, Assistant  Lecturer in NKPSIMS& Lata Mangeshkar Medical College

Dept. Of paediatrics, Nagpur

REFERENCES

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 (reflex sympathetic dystrophy) in a patient with essential mixed cryoglobulinemia and chronic hepatitis C. J Pak Med Assoc 2007; 57: 96-98.
4. "Neuropathic pain". Merck Manual for Healthcare Professionals.
5. Veldman PH, Reynen HM, Arntz IE, Goris RJ (1993). "Signs and symptoms of reflex sympathetic dystrophy: prospective study of 829 patients". Lancet 342 (8878): 1012–6. doi:10.1016/0140-6736(93)92877-V
6. 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 Rehabil 17 (2): 224–7. doi:10.1191/0269215503cr589oa
7. "RSDSA :: Reflex Sympathetic Dystrophy Syndrome Association". Rsds.org. 2010-01-21. Retrieved 2010-04-10.
8. Quisel A, Gill JM, Witherell P (2005). "Complex regional pain syndrome underdiagnosed". J Fam Pract 54 (6): 524–32. PMID15939004
9. 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.
10. Intenzo C, Kim S, Millin J, Park C. Scintigraphic patterns of reflex sympathetic dystrophy syndrome in lower extremities. Clin Nucl Med 1989; 14:657-661.
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: 520-523.
12. Eisenberg E, Geller R, Brill S. Pharmacotherapy options for complex regional pain syndrome. Expert Rev Neurother 2007; 7: 521-531.
13. Low AK, Ward K, Wines AP. Pediatric complex regional pain syndrome. J Pediatr Orthop 2007; 27:567-572.
14. Zollinger PE , 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.