Hypermobility and Ehlers-Danlos Syndrome Symptoms are Explained by Abnormal Sympathetic Responses to Head-Up Postural Change

Introduction. Ehlers-Danlos Syndrome/Hypermobility (EDSh) patients demonstrate abnormal sympathetic responses to head-up postural change. This has been labeled “Sympathetic Withdrawal” (SW) an alpha-adrenergic dysfunction associated with the range of Orthostatic dysfunctions. Methods. From a suburban cariology and autonomic clinic (Sicklerville, NJ) drawing patients from around the world, a cohort of 243 patients (223 female, 91.8%; average age 34.0 yrs, range 13 to 65 y/o; average BMI 27.3#/in2) previously diagnosed by Rheumatology with EDSh were followed from between November 2018 through May 2020. Baseline and at least two follow-up autonomic tests, with questionnaires, were administered. At follow-up testing autonomic therapy for SW was titrated, including fluids, electrolytes, compression garments, Alpha-Lipoic Acid and oral Vasoactives. Results. From the questionnaires, SW is associated with many of the symptoms of EDSh associated with poor cerebral perfusion, including: lightheadedness, fatigue, brain-fog, memory and cognitive difficulties, headache or migraine, sleep difficulties, and depression-like and anxiety-like symptoms. Additional symptoms of Orthostatic dysfunction due to blood pooling in the lower extremities are also common. Conclusion. SW in EDSh patients is often debilitating and simply treating the symptoms often exacerbates the case. Therapy durations are not short, lasting at least 9 months and perhaps up to 24 months depending on the duration and severity of the disorder.