Autonomic dysfunction presenting as postural tachycardia syndrome following traumatic brain injury

Background: Autonomic dysregulation (also called diencephalic epilepsy) has been reported following traumatic brain injuries (TBI). However, until now, postural tachycardia syndrome (POTS) has not been reported as a long-term complication in patients who have suffered a TBI. We report on a series of patients who developed POTS after suffering TBI. Methods: Eight patients who were referred to our center had suffered TBI and developed features of orthostatic intolerance following head trauma. The patients’ neurological, neurosurgical and autonomic data (charts and/or physician letters) were then carefully reviewed for demographic characteristics, comorbid conditions, symptoms of orthostatic intolerance, medications and response to medication. These patients were diagnosed as having POTS, primarily based on their clinical features and findings from the head-up tilt test (HUTT). The data presented is observational and descriptive (percentages or means). Results: Eight patients (seven of them women) aged 21–41 years had suffered from TBI and had developed features of POTS. All had been normal with no symptoms prior to their TBI. All patients experienced orthostatic dizziness, fatigue, palpitations and near syncope. Six patients suffered from frank syncope. Six patients developed significant cognitive dysfunction, and three developed a chronic pain syndrome following trauma. All of the patients reported severe limitations to their daily activities and had been unable to keep their jobs, and two were housebound. Six patients demonstrated a good response to therapy with various combinations of medication. The symptoms of orthostatic intolerance and syncope improved with the initiation of medical therapy, as well as their reported quality of life. Two patients failed to show any improvement with various combinations of medications and tilt training, and continued to experience orthostatic difficulties. Conclusions: Postural tachycardia syndrome may, in some cases, be a late complication of traumatic brain injury. (Cardiol J 2010; 17, 5: 482-487

Postural orthostatic tachycardia syndrome following Lyme disease

Background: A subgroup of patients suffering from Lyme disease (LD) may initially respond to antibiotics only to later develop a syndrome of fatigue, joint pain and cognitive dysfunction referred to as ‘post treatment LD syndrome’. We report on a series of patients who developed autonomic dysfunction in the form of postural orthostatic tachycardia syndrome (POTS). Methods: All of the patients in this report had suffered from LD in the past and were successfully treated with antibiotics. All patients were apparently well, until years later when they presented with fatigue, cognitive dysfunction and orthostatic intolerance. These patients were diagnosed with POTS on the basis of clinical features and results of the tilt table (HUTT) testing. Results: Five patients (all women), aged 22-44 years, were identified for inclusion in this study. These patients developed symptoms of fatigue, cognitive dysfunction, orthostatic palpitations and either near syncope or frank syncope. The debilitating nature of these symptoms had resulted in lost of the employment or inability to attend school. Three patients were also suffering from migraine, two from anxiety and depression and one from hypertension. All patients demonstrated a good response to the employed treatment. Four of the five were able to engage in their activities of daily living and either resumed employment or returned to school. Conclusions: In an appropriate clinical setting, evaluation for POTS in patients suffering from post LD syndrome may lead to early recognition and treatment, with subsequent improvement in symptoms of orthostatic intolerance. (Cardiol J 2011; 18, 1: 63-66

Postpartum Postural Orthostatic Tachycardia Syndrome in a Patient with the Joint Hypermobility Syndrome

Postural orthostatic tachycardia syndrome (POTS) commonly affects women of childbearing age. We report on a 37-year-old woman who developed symptoms of recurrent syncope in the postpartum period. Her head up tilt test and clinical presentation was consistent with POTS

Clinical presentation and management of patients with hyperadrenergic postural orthostatic tachycardia syndrome. A single center experience

Background: We present our single center experience of 27 patients of hyperadrenergic postural orthostatic tachycardia syndrome (POTS). Methods: In a retrospective analysis, we reviewed the charts of 300 POTS patients being followed at our autonomic center from 2003 to 2010, and found 27 patients eligible for inclusion in this study. POTS was defined as symptoms of orthostatic intolerance (of greater than six months’ duration) accompanied by a heart rate increase of at least 30 bpm (or a rate that exceeds 120 bpm) that occurs in the first 10 min of upright posture or head up tilt test (HUTT) occurring in the absence of other chronic debilitating disorders. Patients were diagnosed as having the hyperadrenergic form based on an increase in their systolic blood pressure of ≥ 10 mm Hg during the HUTT (2) with concomitant tachycardia or their serum catecholamine levels (serum norepinephnrine level ≥ 600 pg/mL) upon standing. Results: Twenty seven patients, aged 39 ± 11 years, 24, (89%) of them female and 22 (82%) Caucasian were included in this study. Most of these patients were refractory to most of the first and second line treatments, and all were on multiple combinations of medications. Conclusions: Hyperadrenergic POTS should be identified and differentiated from neuropathic POTS. These patients are usually difficult to treat and there are no standardized treatment protocols known at this time for patients with hyperadrenergic POTS. (Cardiol J 2011; 18, 5: 527–531

Síndromes de disfunción autonómica asociados con intolerancia ortostática.

In recent years increased interest has focused on the nature and pathophysiology of orthostatic intolerance and syndromes associated with autonomic disorders. Understanding the pathophysiology underlying these syndromes has led to the recognition of several distinct clinical entities with overlapping features and the associated need to reclassify many of the previously unrecognized syndromes. Among the clinical manifestations, syncope and near syncope are frequently associated with orthostatic intolerance. In addition, however, a wide spectrum of symptoms have been described ranging from chronic fatigue to recurrent neurally mediated vasodepressor reactions. The present review focuses on the pathophysiology and classification of syndromes of autonomic dysfunction associated with orthostatic intolerance. Primary and secondary causes of dysautonomia as well as therapeutic approach to these frequently unrecognized syndromes is presented.Los síndromes de disfunción autonómica asociados con la intolerancia ortostática son una de las alteraciones cuya fisiopatología fue reconocida hace sólo pocos años, lo que ha llevado a un mejor reconocimiento clínico. Entre las manifestaciones clínicas, el síncope y el presíncope asociados con ortostatismo son muy frecuentes. El espectro de la presentación clínica de estos síndromes varía desde la fatiga crónica hasta el síncope neurocardiogénico recurrente. En este artículo se revisa la fisiopatología y clasificación actual de los síndromes de disfunción autonómica asociados a intolerancia ortostática, así como las causas de disautonomía primaria y secundaria, y su enfoque diagnóstico y terapéutico