Lisa M. Benrud-Larson

Complex regional pain syndrome type I: incidence and prevalence in Olmsted county, a population-based study

&NA; The objective of this study is to undertake a population based study on the incidence, prevalence, natural history, and response to treatment of complex regional pain syndrome (CRPS). All Mayo Clinic and Olmsted Medical Group medical records with codes for reflex sympathetic dystrophy (RSD), CRPS, and compatible diagnoses in the period 1989–1999 were reviewed as part of the Rochester Epidemiology Project. We used IASP criteria for CRPS. The study population was in the Olmsted County, Minnesota (1990 population, 106,470). The main outcome measures were CRPS I incidence, prevalence, and outcome. Seventy‐four cases of CRPS I were identified, resulting in an incidence rate of 5.46 per 100,000 person years at risk, and a period prevalence of 20.57 per 100,000. Female:male ratio was 4:1, with a median age of 46 years at onset. Upper limb was affected twice as commonly as lower limb. All cases reported an antecedent event and fracture was the most common trigger (46%). Excellent concordance was found between symptoms and signs and vasomotor symptoms were the most commonly present. Three phase bone scan and autonomic testing diagnosed the condition in >80% of cases. Seventy‐four percent of patients underwent resolution, often spontaneously. CRPS I is of low prevalence, more commonly affects women than men, the upper more than the lower extremity, and three out of four cases undergo resolution. These results suggest that invasive treatment of CRPS may not be warranted in the majority of cases.

The spectrum of autoimmune autonomic neuropathies

We analyzed the clinical characteristics of 18 patients (13 female, 5 male) who had autoimmune autonomic neuropathy (AAN) and ganglionic acetylcholine receptor (AChR) autoantibodies. Mean age was 61.4 years (standard deviation, 12.0 years). Ten patients had subacute symptom onset, six with an antecedent event. Eight patients had chronic AAN, characterized by insidious symptom onset, without antecedent event, and gradual progression. A majority of patients with high antibody values (>1.00 nmol/L) had a combination of sicca complex (marked dry eyes and dry mouth), abnormal pupillary light response, upper gastrointestinal symptoms, and neurogenic bladder. Chronic AAN segregated into two subgroups. One subgroup (N = 4) had low antibody titer (0.09 ± 0.01 nmol/L) and a paucity of cholinergic symptoms. It was indistinguishable from pure autonomic failure. The other subgroup (N = 4) had high antibody titer (11.6 ± 2.08 nmol/L), sicca complex, abnormal pupils, and neurogenic bladder; three had severe upper gastrointestinal dysfunction. Higher antibody titers correlated with greater autonomic dysfunction and more frequent cholinergic dysautonomia. These observations expand the clinical spectrum of AAN to include chronic cases, some being indistinguishable from pure autonomic failure, and support the concept that ganglionic AChR antibodies are important diagnostically and pathophysiologically in acquired dysautonomia.Ann Neurol 2003;53:752–758

Quality of Life in Patients With Postural Tachycardia Syndrome

OBJECTIVES To quantify quality of life and identify demographic and clinical correlates of functioning in a well-characterized sample of patients with postural tachycardia syndrome (POTS). PATIENTS AND METHODS Prospective patients were those seen at the Mayo Clinic Autonomic Disorders Laboratory from September 2000 to June 2001. Neurologists made diagnoses of POTS according to established criteria. Patients completed a questionnaire packet that included measures of quality of life (36-Item Short-Form Health Survey [SF-36]) and symptom severity (Autonomic Symptom Profile). Additional clinical information was abstracted from medical records. RESULTS Ninety-four patients (89% female; mean age, 34.2 years) were enrolled in the study. Patients with POTS reported impairment across multiple domains on the SF-36. Physical functioning, role functioning, bodily pain, general health, vitality, and social functioning were all significantly impaired compared with a healthy population (P<.01 for all) and similar to that reported by patients with other chronic, disabling conditions. Hierarchical regression analyses revealed that symptom severity (beta = -.36, P<.001) and disability status (beta = -36, P<.001) were independent predictors of SF-36 physical component scores, with the full model accounting for 54% of the variance (P<.001). None of the variables examined accounted for a significant amount of the variance in SF-36 mental component scores. CONCLUSIONS Patients with POTS experience clear limitations across multiple domains of quality of life, including physical, social, and role functioning. Treatment should address the multiple and varied impairments experienced by these patients and may require a multidisciplinary approach. Future research must further delineate factors, both disease related and psychosocial, that predict functioning and adjustment in this population.

The impact of pain and symptoms of depression in scleroderma

&NA; Systemic sclerosis (scleroderma) is a rare connective tissue disease that can affect multiple organ systems. Case reports and small treatment studies suggest that pain is significant in scleroderma, but few data speak of the frequency or impact of pain. This study sought to determine the frequency and impact of pain, symptoms of depression, and social network characteristics on physical functioning and social adjustment in patients with scleroderma. One hundred and forty‐two scleroderma patients completed measures of pain, depressive symptoms, social network characteristics, physical functioning, and social adjustment. Sixty‐three percent reported at least mild pain and 50% reported at least mild levels of depressive symptomatology. Hierarchical regression analyses revealed that pain, depressive symptoms, and employment status (disabled/unemployed vs. not) were significant, independent predictors of physical functioning, together accounting for 37% of the total variance. Pain was the single strongest predictor of physical function, accounting for 20% of the variance. Depressive symptoms, physical functioning, diversity of social network, and employment status were significant independent predictors of social adjustment, together accounting for 63% of the variance. Depressive symptoms were the single strongest predictor of social adjustment, accounting for 26% of the variance. The effects of pain and physical function on social adjustment became non‐significant when depressive symptoms were entered into the model, suggesting that symptoms of depression mediate the effect of pain and physical function on social adjustment. These findings indicate that pain is common in scleroderma and that pain and depressive symptoms are significant determinants of physical functioning and social adjustment, two important components of health‐related quality of life. Increased attention to effective management of pain and symptoms of depression in scleroderma will likely lead to improved functioning and quality of life.

Body image dissatisfaction among women with scleroderma: Extent and relationship to psychosocial function

Body image dissatisfaction and its relationship to psychosocial function were investigated in 127 women with scleroderma Results indicated elevated body image dissatisfaction, with participants reporting higher levels than a sample of patients with severe burn injuries. Age, skin tightening above the elbows, and functional disability were related to heightened body image dissatisfaction, suggesting that younger patients with more severe disease may be at greatest risk for developing body image concerns. Path analysis revealed that depression mediated the relationship between body image dissatisfaction and psychosocial function. Results suggest that body image dissatisfaction is a significant concern in women with scleroderma and should be assessed routinely. Early identification and treatment of body image dissatisfaction may help prevent the development of depression and psychosocial impairment in this population.

Evaluation of Orthostatic Hypotension: Relationship of a New Self-report Instrument to Laboratory-Based Measures

OBJECTIVE To compare measured autonomic deficits (composite autonomic severity score [CASS]) with a brief self-report scale we developed to measure severity of symptoms of orthostatic hypotension. PATIENTS AND METHODS Patients were recruited in 2 phases: from August to October 2002 and in April 2004. All patients underwent full evaluation in the autonomic laboratory, from which a CASS of autonomic deficits was derived. Patients also completed the 5-item self-report Orthostatic Grading Scale, which inquires about symptoms of orthostatic intolerance due to orthostatic hypotension (eg, severity, frequency, and interference with daily activities). RESULTS Of 145 patients, 97 (67%) had orthostatic hypotension. The 5-item scale demonstrated strong internal consistency (coefficient alpha=.91). Patients with orthostatic hypotension had significantly higher scores on each questionnaire item and CASS subscores than those without orthostatic hypotension. The scale items correlated significantly with each of the CASS subscores, maximally with the CASS adrenergic subscore. CONCLUSIONS Orthostatic hypotension is not the only cause of reduced orthostatic tolerance, and some patients may have orthostatic hypotension but be asymptomatic. Results of this study indicate that this 5-item questionnaire is a reliable and valid measure of the severity of symptoms of orthostatic hypotension and that it can supplement laboratory-based measures to provide a rapid, more complete clinical assessment. This questionnaire would also be useful as a brief screening device for orthostatic intolerance to aid physicians in identifying patients who may have orthostatic hypotension.

Depressive symptoms and life satisfaction in patients with multiple system atrophy

Multiple system atrophy (MSA) is a progressive neurodegenerative disorder characterized by extrapyramidal signs, prominent autonomic failure, and a poor prognosis. In the absence of restorative treatment, management is aimed at improving quality of life. Little is known about modifiable factors, such as depression, that may affect quality of life in MSA. The present study investigated the rate of depressive symptoms and their relationship to life satisfaction in patients with MSA. Ninety‐nine patients with MSA (54% women; mean age, 67.8 ± 8.8) completed measures of depressive symptoms, life satisfaction, physical function, and disease and demographic factors. Objective autonomic indices were abstracted from the medical chart. Participants reported a high rate of depressive symptoms, with 39% endorsing moderate to severe depressive symptoms on the Beck Depression Inventory (BDI ≥ 7). Reported life satisfaction was low, with a mean of 38.8 on a 100‐point visual analogue scale (0 = Extremely Dissatisfied, 100 = Extremely Satisfied). The SF‐36 Physical Component Scale was approximately 1.5 standard deviations below the mean of a normative sample of healthy adults the same age. Regression analysis revealed that autonomic disease parameters accounted for 22% of the variance in life satisfaction. Physical function did not account for any additional variance; however, depressive symptoms accounted for an additional 15%. Depressive symptoms are common, often severe, and an important determinant of life satisfaction in patients with MSA. Adequate treatment of comorbid depression may improve quality of life in this population, despite the presence of other debilitating deficits.

What is the minimum duration of head-up tilt necessary to detect orthostatic hypotension?

ObjectiveThere is uncertainty as to the minimum duration of head-up tilt (HUT) needed to detect orthostatic hypotension (OH). The orthostatic duration has variably been recommended to be 1, 2, 3, and 5 minutes. The purpose of the current study was 1) to determine the minimum duration of HUT necessary to detect OH and 2) to identify different patterns of orthostatic blood pressure (BP) response in patients with OH.Design/methodsWe evaluated the medical records of 66 consecutive patients (mean age 70.0±10.1 years; 64% male) seen at Mayo Clinic-Rochester from 2000–2001 who fulfilled the criteria for OH (systolic blood pressure [SBP] reduction ≥ 20mm Hg within 3 minutes of HUT) during routine clinical autonomic studies. All patients completed an autonomic reflex screen with continuous monitoring of heart rate and BP during supine rest and 5 minutes of 70 degree HUT. Severity of autonomic deficits was quantified with the Composite Autonomic Severity Score (CASS).ResultsOverall, BP was the lowest at 1 minute with gradual and partial recovery over the following 4 minutes. Eighty-eight percent of patients (N=58) developed OH by 1 minute of HUT, with an additional 11% (N=7) developing OH by 2 minutes and the remaining 1% (N=1) developing OH by 3 minutes. We identified two broad patterns of SBP response to HUT. Forty-eight percent (N=32) of patients demonstrated an initial drop in SBP (≥ 20 mm Hg),which remained stable until tilt-back. Thirty-six percent (N=24) demonstrated an initial drop (≥ 20mm Hg) followed by a progressive decline in SBP until tilt-back. Repeated measures analysis of variance confirmed that the SBP change in response to HUT differed significantly among patients with a stable vs. progressive pattern [F(3,32)=25.1, p<0.001). Patients with the progressive pattern also had more severe adrenergic impairment on the CASS (p=0.03) and were more likely to have their tilt test terminated early (prior to 5minutes) due to presyncope (p<0.0001) than patients with the stable pattern.ConclusionsOne minute of HUT will detect OH in the great majority (88%) of patients and three minutes will detect the balance. Orthostatic stress beyond 2 minutes is necessary to detect the pattern of progressive OH. Since this group has more severe adrenergic deficits than the group with stable OH, we suggest that the progressive pattern is due to greater impairment of compensatory reflexes. Recognition of the group with progressive fall in BP is important since this group may be at greater risk of orthostatic syncope.

Correlates of functional disability in patients with postural tachycardia syndrome: preliminary cross-sectional findings.

The study investigated correlates of functional disability in 94 patients (89.4% women, 10.6% men; mean age=34.2 years) with postural tachycardia syndrome (POTS), a clinical syndrome of orthostatic intolerance characterized by significant functional limitations. Path analysis supported a model in which, controlling for demographic and disease variables, catastrophic cognitions were directly related to the latent variable functional disability, whereas somatic vigilance, anxiety sensitivity, and neuroticism were indirectly associated with functional disability through their relationship with catastrophic cognitions. Results suggest that modifiable psychological factors play a role in the functional limitations experienced by patients with POTS. Longitudinal research is necessary to confirm these relationships.

Factors associated with willingness to try different pain treatments for pain after a spinal cord injury

ObjectiveTo develop and establish the psychometric properties of a pain treatment willingness scale and identify factors associated with willingness to try specific pain treatments for spinal cord injury (SCI)-related pain. DesignAs part of a larger study, a questionnaire was designed to assess willingness to use various pain medications and other types of pain treatments. This questionnaire, which included measures of pain severity, pain interference, mood, hope, and current use of pain treatments, was completed by persons with SCI recruited through the mail or in person. SubjectsOne hundred fifteen persons completed the questionnaire (35% response rate). Seventy-two percent of the participants were men, mean age was 49.1 years, and average time elapsed since injury was 8 years. ResultsFactor analysis indicated two factors—willingness to use opioids and willingness to use nonpharmacological treatment (i.e., physical therapy, relaxation methods and stress management, and alternative medicine). Internal consistency and convergent and divergent validity were established. Persons experiencing SCI-related pain were more willing to use pain treatments than those without current pain, and those who were currently using opioids reported greater willingness to use that treatment. Persons who reported SCI-related pain were more willing to use nonpharmacological treatments than opioid medications. Finally, participants demonstrated different degrees of willingness to use an opioid medication based on its name (i.e., “narcotic,” “codeine,” “morphine,” “methadone”). ConclusionsWillingness to use a specific pain treatment may be a key factor mediating the behavior of using that specific treatment. Assessment of patient attitudes toward various treatments options, particularly regarding opioid medications, is warranted to optimize treatment adherence. Once the factors that determine these attitudes are identified, interventions to increase willingness to use nonpharmacological or opioid treatments can be designed and evaluated.