Letters to the Editor
I am a friend and on-call caregiver of a woman with complex regional pain syndrome [CRPS]. This month she marked the 21st "anniversary" of living with CRPS. In the past 12 months, I have watched the acceleration of CRPS degrade her ability to walk, speak, and lift her 1-year-old child, and irreversibly damage her immune, motor, and vascular systems. Most painful of all to observe are the limbic system dysfunctions.
I am a former medical school professor. I have used my research skills to read every article, case report, textbook, conference paper, poster, newsletter, and blog about CRPS in search of an elusive treatment that might work . . . or that ever worked for anyone. Internet chat boards for patients document the futility and despair far better than do the Cochrane reviews. (Links to online patient boards can be found on Web sites such as those of American RSD Hope [www.rsdhope.org], For Grace [www.forgrace.org], and the Reflex Sympathetic Dystrophy Syndrome Association [www.rsds.org]. Yahoo! Health:Groups also lists 70 online Reflex Sympathetic Dystrophy-CRPS support groups.)
I applaud Applied Neurology for summarizing the controversy and experience of CRPS and for giving an overview of some of the science behind CRPS treatment [in the article, "Understanding and Treating Complex Regional Pain Syndrome," Applied Neurology, October 2006]. The article also does a good job of capturing the unusual dedication of the few doctors in the United States who choose to take on this tricky, malevolent, and intractable disease.
I must object, however, to the misleadingly hopeful conclusion of the article's author Devon Schuyler. The final paragraph concludes: "Oaklander [Anne Louise Oaklander, MD, PhD, associate professor of neurology at Harvard Medical School, Boston] pointed out that she never sees geriatric patients with CRPS; the average age of most patients is about 40, and the prevalence decreases as patients age. 'These kind of epidemiologic data are consistent with a disease that does not last forever,' she said. 'It's one of the things that keeps me optimistic.'"
Whether Dr Oaklander "sees geriatric patients with CRPS" or not is not "epidemiologic data," it is merely clinical experience, not evidence of any sort. Even if there were valid population-based data demonstrating a decline in prevalence of CRPS from age 40 onward, although that data may be consistent with a hypothesis that CRPS is a "disease that does not last forever," the most plausible hypothesis regarding the age-adjusted prevalence of CRPS is that the decline is caused by suicide. These affected persons presumably might be those "30% for whom it doesn't matter what you do; they're miserable," according to Ricardo A. Cruciani, MD, PhD [vice chair of the Department of Pain Medicine and Palliative Care at Beth Israel Medical Center, New York].
-Molly McNulty, JD
Rochester, New York
Editor's Note: Molly McNulty is a former professor of health law and policy at the University of Rochester School of Medicine and Dentistry.
Dr Oaklander replies:
Ms McNulty objects to my statement that patients with complex regional pain syndrome [CRPS] eventually recover, but that is more than opinion; it is based on peer-reviewed and published scientific data. The epidemiologic study by Sandroni and colleagues1 that appeared in the journal Pain in 2003 provides the highest quality data. In this comprehensive study, the medical records of people living in Olmsted County, Minnesota (1990 population, 106,470) who were seen for medical care between 1989 and 1999 were reviewed. The median age of CRPS onset was 46 years. Disease resolved in 74% of patients, often without specific care.
Every other published study of CRPS has reported a mean age at CRPS onset of about 40. I draw attention to a study by Veldman and colleagues2 of 829 patients with CRPS that was published in the Lancet in 1993. Patients' average age was 42 years, and the most common duration of CRPS was 2 to 6 months (in 242 patients). Only 28% of patients had CRPS lasting longer than 1 year.
There is absolutely no evidence either from clinical experience or research to support Ms McNulty's suggestion that older patients with CRPS commit suicide. In the article by Smith and colleagues3 that looked at suicide in patients with nonfatal pain conditions, only 5% had a suicide plan or a previous suicide attempt. The number who complete suicide is thankfully much lower.
Ms McNulty's letter makes me wonder whether her friend does indeed have CRPS. She describes several features (in addition to a 21-year history) that are not typical of CRPS. They include weakness affecting both the arms and legs and loss of the ability to speak. CRPS begins in association with a trauma to either an arm or leg, and symptoms are worse in the affected limb.
Because CRPS is so poorly understood, incorrect diagnoses are common. It is most commonly confused with a generalized problem-usually a small-fiber polyneuropathy. I would be delighted to speak with Ms McNulty's friend to find out more about her condition. Accurate diagnosis is the foundation for effective treatment.
REFERENCES1. Sandroni P, Benrud-Larson LM, McClelland RL, Low PA. Complex regional pain syndrome type I: incidence and prevalence in Olmsted county, a population-based study. Pain. 2003;103:199-207.
2. Veldman PH, Reynen HM, Arntz IE, Goris RJ. Signs and symptoms of reflex sympathetic dystrophy: prospective study of 829 patients. Lancet. 1993; 342:1012-1016.
3. Smith MT, Edwards RR, Robinson RC, Dworkin RH. Suicidal ideation, plans, and attempts in chronic pain patients: factors associated with increased risk. Pain. 2004;111:201-208.
I am a 48-year-old family physician from Minnesota. I have always prided myself on practicing evidence-based medicine. Four years ago, I received a hands-on lesson about the paucity of clinical experience and evidence regarding the diagnosis and optimal treatment of late neurologic Lyme disease. I saw multiple specialists over a period of 30 months in an effort to obtain a diagnosis. When I finally found a neuromuscular specialist who properly diagnosed my illness, I had findings consistent with upper and lower motor neuron disease; autonomic, sensory, and cranial neuropathies; and cognitive deficits.
An Infectious Diseases Society of America member offered me 30 days of ceftriaxone therapy. This seemed inadequate; instead I elected to be treated by a physician member of the International Lyme and Associated Diseases Society. It took 13 weeks of continuous antibiotic therapy before I had any improvement. At 13 months, I was able to walk without my ankle-foot orthoses, which I had worn for the previous 15 months.
The evidence on this topic is far from settled; we do an injustice to patients when we imply otherwise.
-Elizabeth Maloney, MD
Editor's note: The letter is a response to the article, "Two Opposing Camps Offer Insight Into Treatment of Neurologic Lyme Disease," Applied Neurology, September 2006.