Postural orthostatic tachycardia syndrome after mRNA COVID-19 vaccine

Clinical Autonomic Research -     

Postural orthostatic tachycardia syndrome: insights into pathogenesis and treatment

Journal of Neurology -     

Platypnea-orthodeoxia syndrome mimicking postural orthostatic tachycardia syndrome

Clinical Autonomic Research -     

Diagnosis of tachycardia syndromes associated with orthostatic symptoms

The postural tachycardia syndrome (POTS) is characterized by excessive tachycardia only in upright position without evidence of a cardiac or metabolic disease in combination with orthostatic symptoms like dizziness, lightheadedness or syncope but without relevant falls in blood pressure. The cause is unknown. A specific diagnostic marker has not been found so far. Eighteen patients with typical symptoms of POTS were examined. They underwent standard autonomic function tests with continuous measurement of heart rate (HR) and blood pressure. All fulfilled the inclusion criteria of pathologically increased HR activation during passive tiltor standing over 90 seconds. The upper limits of normal were based on data from 137 healthy volunteers between 18 and 85 years of age. Actively standing up induced more POTS-typical HR increases and lead to more consistent results than passive tilt. HR responses during Valsalva manoeuvre and deep breathing were normal in all except one patient each, indicating that assessment of HR during these tests does not contribute to the diagnosis of POTS. Frequency of symptoms reducing overall well-being and the degree of impairment of life quality by symptoms typical of POTS were measured with a self-assessment scale. The majority of patients reported a permanent reduction of overall wellbeing and a relevant impairment of life quality due to dizziness, tachycardia, and syncopes. This underlines the importance of considering POTS as a differential diagnosis of orthostatic syndromes and the necessity of treating it adequately.     

Postural tachycardia syndrome with asystole on head-up tilt

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. Disclosures: The authors have nothing to disclose.     

Case report Goodpasture's syndrome associated with multiple sclerosis

Multiple sclerosis (MS) has been reported to occur in association with other autoimmune diseases. Here we report the case of a woman with primary progressive MS who developed and died of Goodpasture's syndrome associated with anti-glomerular basement membrane antibodies. Of interest she had the human leukocyte antigen haplotype (DRB1*1501-DQA1*0102-DQB1*0602) most frequently associated with these two diseases; however, it is likely that other genes, particularly those involved with immunoregulation, also contributed to her susceptibility to these diseases. The association of MS and autoimmune renal disease may be more common than currently recognized.     

Cerebrovascular and cardiovascular responses associated with orthostatic intolerance and tachycardia

Idiopathic orthostatic intolerance syndrome is characterized by postural symptoms of cerebral hypoperfusion without arterial hypotension. Abnormal baroreceptor responses with deranged cerebral autoregulation leading to cerebral vasoconstriction have been proposed as a causative mechanism. The authors report the cerebrovascular and cardiovascular responses in a patient who recovered from orthostatic intolerance and tachycardia. Changes in the orthostatic responses of mean arterial pressure (MAP), heart rate (HR), cardiac output (CO), and transcranial Doppler middle cerebral artery (MCA) mean blood flow velocity (V_mean) were assessed at admission and again 6 months after recovery. Normal cardiovascular responses to forced breathing and to standing indicated intact overall baroreflex integrity with normal baroreflex sensitivity (10.2 msec·mm Hg⁻¹). After the patient stood for 8 minutes, presyncopal symptoms developed, with unchanged MAP but increased HR (+41 beats/min) and reduced stroke volume (SV) (−69%), CO (−50%), and MCA V_mean (−46%; 57 to 31 cm·s⁻¹). After a reconditioning program and recovery, the patient was reexamined. The supine MCA V_mean was larger (79 cm·s⁻¹), as were MAP (76 versus 70 mm Hg) and CO (+15%). The orthostatic HR increase was smaller (+5 beats/min), as was the reduction in SV (−44%) and CO (−30%), with an increase in MAP to 93 mm Hg. The orthostatic reduction in MCA V_mean was smaller (−13 versus −26 cm·s⁻¹) and standing cerebrovascular resistance decreased (1.41 versus 2.39 mm Hg·cm·s⁻¹). In this patient who had intact baroreflex control and no postural decrease in blood pressure, the reduction in MCA V_mean, concomitant with a large decrease in CO, seemed reversible. The result suggests that a symptomatic reduction in cerebrovascular conductance during standing is to be interpreted as being an adaptive response to a critically limited systemic blood flow, rather than to derangement of cerebral autoregulation.     

Uveitis associated with multiple sclerosis

Our study aimed to determine the frequency of uveitis among MS patients through the Lyon MS Database and compare the clinical spectrum and time course of MS in patients with or without uveitis. Twenty-eight patients with uveitis were detected. The prevalence of uveitis in our population was 0.65% (28/4,300). Uveitis preceded onset of MS in 46% of the patients; it occurred simultaneously or after MS, in 18% and 36% of the cases, respectively. The topography and timing of uveitis were not associated with any significant difference in MS course and prognosis. There was no difference in the course and prognosis in patients with or without uveitis.     

Leber's hereditary optic neuropathy associated with multiple sclerosis: Harding's syndrome

We describe a 32-year-old woman with sequential, severe, painless visual loss in one eye and then the other, and three temporally distinct episodes of neurological disturbance suggestive of demyelination in the spinal cord. She was positive for the T14484C mutation in the mitochondrial genome, one of three common mutations causing Leber's hereditary optic neuropathy. In addition, MRI identified areas of demyelination within the periventricular white matter of the brain and within the spinal cord. The coexistence of multiple sclerosis and Leber's hereditary optic neuropathy (Harding's syndrome) is known to occur more often than would be expected by chance; therefore, screening for the Leber's mutations in multiple sclerosis patients with severe visual loss should be considered because this has important prognostic and genetic implications.     

Cardiovascular autonomic dysfunction in multiple sclerosis: correlation with orthostatic intolerance

Autonomic dysfunction is frequently observed in patients with multiple sclerosis (MS), but clinical studies disagree on the frequency and type of abnormalities in autonomic function tests. Orthostatic dizziness (OD) has been reported in up to 49% of patients, but the pathophysiological mechanisms are poorly understood. This study investigated cardiovascular reflex tests and their association with OD in patients with MS in order to examine the hypothesis that the sympathetic nervous system is specifically involved in these patients. Forty patients with clinically active relapsing-remitting (n = 27) and secondary progressive MS (n = 13), aged 35.0 ± 8.5 years, were studied by parasympathetic (heart rate responses to the Valsalva maneuver, deep breathing, and active change in posture) and sympathetic function tests (blood pressure responses to active change in posture and sustained handgrip), and by spectral analysis of heart rate variability during rest and during standing. Results were compared to those obtained in 24 healthy volunteers, aged 29.4 ± 7.2 years. A standardized questionnaire was used to evaluate symptoms of orthostatic intolerance. Abnormal responses on at least one cardiovascular reflex test were observed in 40% of MS patients, compared to 17% of the control group, with a statistically significant involvement of the sympathetic vasomotor system. Orthostatic intolerance was reported in 50% of patients (controls: 14%, P < 0.006). Subgroup comparison of patients with and without OD suggests that orthostatic intolerance results from impaired sympathetic vasoconstriction. These results provide further evidence that the sympathetic nervous system is involved in patients with MS. Received: 18 September 1998 Received in revised form: 28 December 1998 Accepted: 3 January 1999