Cyclic vomiting syndrome (CVS) shares many features with migraine headache, including auras, photophobia, and antimigrainous treatment response being traditionally viewed as a migraine variant. Aims. ...To determine whether CVS is associated with the same disorders as migraine headache, and compare these associations to those in healthy control subjects. Methods. Cross-sectional study of patients utilizing the ODYSA instrument, evaluating the probability of 12 functional/autonomic diagnoses, CVS, migraine, orthostatic intolerance (OI), reflex syncope, interstitial cystitis, Raynaud's syndrome, complex regional pain syndrome (CRPS), irritable bowel syndrome, functional dyspepsia, functional abdominal pain, fibromyalgia, and chronic fatigue syndrome. Control subjects were age-matched gender-matched friends. Patients had to fulfill criteria for CVS or migraine, while control subjects could not. Results. 103 subjects were studied, 21 with CVS, 46 with migraine and 36 healthy controls. CVS and migraine did not differ in the relative frequencies of fibromyalgia, OI, syncope, and functional dyspepsia. However, CVS patients did demonstrate a significantly elevated frequency of CRPS. Conclusions. Although CVS and migraine clearly share many of the same comorbidities, they do differ in one important association, suggesting that they may not be identical in pathophysiology. Since OI is common in CVS, treatment strategies could also target this abnormality.
Chronic regional pain syndrome (CRPS) is a complex disorder, the optimal treatment of which requires an interdisciplinary approach encompassing medical, interventional, psychological, and ...rehabilitation services that emphasize the role of physical and occupational therapies. The central focus of treatment is the restoration of function, utilizing a systematic, coordinated, and progressive set of therapeutic strategies. The poorly delineated pathophysiology and variable course of CRPS suggest that individualized strategies are required for optimal management, but also mean that carefully controlled trials of physiotherapy are difficult to conduct. This article presents a brief review of the nature and pathophysiology of CRPS, the medical and psychological approaches that have been found to be effective, and a review of the current trends in rehabilitation.
Background: The pathophysiology of functional abdominal pain (FAP) is unknown. The upright portion of a tilt table test triggers typical symptoms in certain children. Aim: To compare the ...pathophysiology and treatment response of children with FAP whose gastrointestinal symptoms (GI) were replicated (RGI) by tilt table testing (TTT) to those in whom TTT did not have this effect (NRGI). Methods: An IRB-approved retrospective review of the autonomic laboratory database identified all children tested for GI complaints. We compared results of TTT, Valsalva maneuver, deep breathing and the axon reflex sweat test. Overall treatment response and that specific to fludrocortisone was ranked from 1 to 5, with 1 “much worse,” 3 “neutral,” and 5 “much better.” Results: 32/76 identified children had reproducible symptoms on TTT (RGI) and 44 did not (NRGI). The RGI group was younger, had a shorter duration of symptoms, more postural tachycardia syndrome (POTS) and benefited more from fludrocortisone (73% in RGI vs. 25% in NRGI). Conclusion: Dividing patients with FAP according to the effect of TTT on their symptoms appears to delineate 2 fundamentally different groups, with potentially different pathophysiologies and treatment responses. A prospective study is needed.
Functional gastrointestinal disorders (FGIDs) are a common problem in pediatric patients and can affect quality of life. However, the extent of these disorders may vary in different subpopulations of ...children. This study investigated the prevalence of FGIDs in an inner-city primary care practice. Healthy patients between the ages of 9 and 17 were administered a validated questionnaire that assessed for FGIDs and other somatic complaints. Eleven of 145 patients (7.5%) met criteria for FGIDs based on Rome III Diagnostic Criteria. Raynaud-like symptoms tended to occur more often in patients meeting criteria for FGIDs, although this association was not statistically significant (P = .07). The lower prevalence of FGIDs in this population compared with earlier studies may suggest a link between socioeconomic status and the prevalence of FGIDs. Larger population-based studies consisting of a heterogeneous cohort from a variety of socioeconomic backgrounds are necessary to further elucidate the true connection between FGIDs and socioeconomic status.
Enhanced sympathetic activity causes an exaggerated heart rate response to standing in the postural tachycardia syndrome (POTS). All patients describe symptoms of orthostatic intolerance such as ...dizziness, blurred vision, shortness of breath, palpitations, tremulousness, chest discomfort, headache, lightheadedness and nausea, but only one third suffer loss of consciousness. We report four patients with POTS, who had long ventricular pauses (i.e. asystole) and syncope during head-up tilt test. This suggests that a subset of patients with POTS can have a surge in parasympathetic outflow that precedes vasovagal syncope.
Disorders of the autonomic nervous system, or dysautonomias, affect a large segment of the population, especially women, and represent a diagnostic challenge. Identification of biomarkers for ...autonomic disorders, and the subsequent development of screening methods, would benefit diagnosis and symptom management. We studied the effect of sera from fifteen well-characterized dysautonomia patients (mean age 49
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16 years, 10 females, 5 males) and ten control subjects (mean age 31
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14 years, 5 females, 5 males) on the proliferation of cultured Schwann cells and activity of mitogen-activated protein kinases (MAPKs) in these cells. We correlated characteristics of patients with the effects on cell proliferation and signaling. Overall, we observed a significant increase in proliferation when Schwann cells were incubated with sera from female dysautonomia patients when compared to control subjects and male patients. Interestingly, removal of IgGs significantly reduced the proliferative effect of patient sera. We also observed significant activation of p38 MAPK following incubation with both male and female patient sera. These results suggest that patient sera contain factors that contribute to aberrant Schwann cell proliferation and signaling and may ultimately lead to autonomic nerve dysfunction. Our observations represent a promising first step in the identification of dysautonomia biomarkers.
Despite the extensive impact of autonomic function on the gastrointestinal system, there is little understanding of the mechanisms by which specific autonomic abnormalities translate into particular ...gastrointestinal complaints. Three logical alternatives include: (1) the underlying disorder affects the autonomic and gastrointestinal systems independently; (2) autonomic dysfunction alters gastrointestinal processing directly; (3) gastrointestinal manifestations arise as a delayed, indirect consequence of autonomic dysfunction. The major gastrointestinal manifestations of dysautonomia include esophageal dysmotility such as achalasia, gastroparesis, and small bowel bacterial overgrowth in the upper tract. Lower tract disorders include diarrhea, fecal incontinence, and constipation. Sorting through the varied causes of these disorders requires a careful history and examination in each patient. Supportive diagnostic studies may include radionuclide imaging, motility examination, and electrogastrography. Autonomic studies can (1) distinguish a purely enteric from a more generalized dysautonomia; (2) provide surrogate information about motility; (3) differentiate primary (e.g., multiple system atrophy) from secondary (e.g., irritable bowel syndrome) dysautonomias as the etiology of gastrointestinal symptoms. Several new strategies are available for the treatment of gastroparesis, constipation, irritable bowel, and sphincteric incontinence.