Complex Regional Pain Syndrome Secondary to Leprosy

Pain Medicine

Volume 13, Issue 8, pages 1067–1071, August 2012

1. Deepti Ghia MBBS, DDV, MD, 
2. Reshma Gadkari MBBS, DDV, MD, 
3. Chitra Nayak MBBS, DDV, MD, DHA, AFIH

Abstract

Introduction.  Leprosy is a chronic infectious disease caused by Mycobacterium leprae affecting the skin and the nerves. Complex regional pain syndrome (CRPS/Sudeck's dystrophy) is a painful and disabling condition—a triad of autonomic, sensory, and motor symptoms disproportionate to the inciting event (inflammatory, infective, or traumatic nerve damage).

Case.  A 20-year-old male presented with continuous pain, aggravated by cold and emotions, loss of fine touch and temperature sensation, redness, swelling, along lateral aspect of left hand and forearm with weakness in the grip of 6 months' duration. There was a 5-year history of sensory loss only over left index finger that he ignored. Examination revealed abnormal sensory and autonomic functions along left radial and median nerve distribution that were confirmed by nerve conduction studies suggestive of mononeuritis multiplex. Radial cutaneous nerve biopsy was suggestive of leprosy. Magnetic resonance imaging and ultrasonography showed no compressive etiology; however, MRI showed involvement of brachial plexus. Antileprosy, anti-inflammatory drugs, and steroids were given in view of neuritis because of lepra reaction with supportive measures of physiotherapy, transcutaneous electrical nerve stimulation, to no avail. A surgical median nerve decompression also failed to relieve the pain. Temporary stellate ganglion block improved the pain scale. Thus, excluding all other causes, the final diagnosis was CRPS secondary to leprosy. There is only one reported case of CRPS with leprosy.

Conclusion: Leprous neuropathy caused the nerve damage that lead to CRPS type 2. Very rarely leprosy can lead to CRPS. CRPS is a diagnosis of exclusion.

Intravenous immunoglobulin treatment of the complex regional pain syndrome: a randomized trial.

Ann Intern Med. 2010 Feb 2;152(3):152-8.

Goebel A, Baranowski A, Maurer K, Ghiai A, McCabe C, Ambler G.
University of Liverpool, Clinical Sciences Building, University Hospital Aintree, Liverpool L9 7AL, United Kingdom.
BACKGROUND: Treatment of long-standing complex regional pain syndrome (CRPS) is empirical and often of limited efficacy. Preliminary data suggest that the immune system is involved in sustaining this condition and that treatment with low-dose intravenous immunoglobulin (IVIG) may substantially reduce pain in some patients.
OBJECTIVE: To evaluate the efficacy of IVIG in patients with longstanding CRPS under randomized, controlled conditions.
DESIGN: A randomized, double-blind, placebo-controlled crossover trial. (National Research Registry number: N0263177713; International Standard Randomised Controlled Trial Number Registry: 63918259)
SETTING: University College London Hospitals Pain Management Centre.
PATIENTS: Persons who had pain intensity greater than 4 on an 11-point (0 to 10) numerical rating scale and had CRPS for 6 to 30 months that was refractory to standard treatment. I
NTERVENTION: IVIG, 0.5 g/kg, and normal saline in separate treatments, divided by a washout period of at least 28 days.
MEASUREMENTS: The primary outcome was pain intensity 6 to 19 days after the initial treatment and the crossover treatment.
RESULTS: 13 eligible participants were randomly assigned between November 2005 and May 2008; 12 completed the trial. The average pain intensity was 1.55 units lower after IVIG treatment than after saline (95% CI, 1.29 to 1.82; P < 0.001). In 3 patients, pain intensity after IVIG was less than after saline by 50% or more. No serious adverse reactions were reported.
LIMITATION: The trial was small, and recruitment bias and chance variation could have influenced results and their interpretation.
CONCLUSION: IVIG, 0.5 g/kg, can reduce pain in refractory CRPS. Studies are required to determine the best immunoglobulin dose, the duration of effect, and when repeated treatments are needed.
 PRIMARY FUNDING SOURCE: Association of Anaesthetists of Great Britain and Ireland, University College London Hospitals Charity, and CSL-Behring.



PMID: 20124231 [PubMed

Pain exposure physical therapy may be a safe and effective treatment for longstanding complex regional pain syndrome type 1: a case series

Clinical Rehabilitation  2009 Nov 11.

Ek JW, van Gijn JC, Samwel H, van Egmond J, Klomp FP, van Dongen RT.

Department of Rehabilitation Medicine, Bethesda Hospital, Hoogeveen, The Netherlands.

Objective: To determine if treatment of longstanding complex regional pain syndrome type 1, focusing on functional improvement only while neglecting pain, results in clinical improvement of this syndrome.Design: Prospective description of a case series of 106 patients.Setting: Outpatient clinic for rehabilitation.Interventions: Physical therapy of the affected limb directed at a functional improvement only while neglecting the pain, was performed following an extensive explanation. Normal use of the limb between the treatments was encouraged despite pain. A maximum of five of these sessions were performed in three months.Measures: Radboud Skills Test was used to monitor functional improvement of the arms. Speed and walking distance was used as the measure of outcome for the legs.Results: The function of the affected arm or leg improved in 95 patients. Full functional recovery was experienced in 49 (46%) of them. A reduction in pain presented in 75 patients. In 23 patients functional recovery was reached despite an increase in pain. Four patients stopped early due to pain increase.Conclusions: Our results suggest that 'pain exposure physical therapy' is effective and safe for patients who are unresponsive to accepted standard therapies. Avoiding the use of a limb due to pain will result in loss of function. Forced usage of limbs restores the function, reverses these adaptive processes and leads to regain of control by practice with a reduction of pain in most cases.

PMID: 19906762 [PubMed - as supplied by publisher]