We present the case of a 64-year-old woman who, in the past 5 years, complained of constipation/diarrhea, hyposudoresis, xerostomia and xerophthalmia, dysuria and orthostatic hypotension. Cardiovascular reflexes analysis revealed sympathetic and parasympathetic failure. Norepinephrine was markedly reduced, both lying and after tilt. Norepinephrine infusion determined a significant rise in blood pressure, allowing the diagnosis of denervation hypersensitivity. The diagnosis of pure autonomic failure was made. Therapy with 9 alpha fludrocortisone and metoclopramide was initiated with marked and sustained symptomatic effect.
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Central Involvement in Pure Autonomic Failure: Insights from Neuromelanin-Sensitive Magnetic Resonance Imaging and F-Fluorodopa-Positron Emission Tomography.
Mov Disord
January 2025
Department of Neurology, Vanderbilt University Medical Center, Nashville, Tennessee, USA.
Background: Central synucleinopathies, including Parkinson's disease (PD), dementia with Lewy bodies (DLB), and multiple system atrophy (MSA), involve alpha-synuclein accumulation and dopaminergic cell loss in the substantia nigra (SN) and locus coeruleus (LC). Pure autonomic failure (PAF), a peripheral synucleinopathy, often precedes central synucleinopathies.
Objectives: To assess early brain involvement in PAF using neuromelanin-sensitive magnetic resonance imaging (NM-MRI) and fluorodopa-positron emission tomography (FDOPA-PET), and to determine whether PAF patients with a high likelihood ratio (LR) for conversion to a central synucleinopathy exhibit reduced NM-MRI contrast in the LC and SN compared with controls and low-LR patients.
Pathophysiological Significance of α-Synuclein in Sympathetic Nerves: In Vivo Observations.
Neurology
February 2025
From the Autonomic Medicine Section, Clinical Neurosciences Program, Division of Intramural Research, National Institute of Neurological Disorders and Stroke, NIH, Bethesda, MD.
Background And Objectives: Lewy body diseases (LBDs) such as Parkinson disease (PD) feature increased deposition of α-synuclein (α-syn) in cutaneous sympathetic noradrenergic nerves. The pathophysiologic significance of sympathetic intraneuronal α-syn is unclear. We reviewed data about immunoreactive α-syn, tyrosine hydroxylase (TH, a marker of catecholaminergic fibers), and the sympathetic neurotransmitter norepinephrine (NE) in skin biopsies from control participants and patients with PD, the related LBD pure autonomic failure (PAF), the non-LBD synucleinopathy multiple system atrophy (MSA), or neurologic postacute sequelae of severe acute respiratory syndrome coronavirus 2 (neuro-PASC).
View Article and Find Full Text PDFClinical Correlates of Efficacy of Pyridostigmine in the Treatment of Orthostatic Hypotension.
Hypertension
December 2024
Vanderbilt Autonomic Dysfunction Center, Division of Clinical Pharmacology, Vanderbilt University Medical Center, Nashville, TN. (L.E.O., A.D., C.A.S., A.G., B.K.B., S.P., I.B.).
Background: The cholinesterase inhibitor pyridostigmine is used to treat orthostatic hypotension by facilitating cholinergic neurotransmission in autonomic ganglia, thereby harnessing residual sympathetic tone to increase blood pressure (BP) preferentially in the upright posture. We hypothesized that less severe autonomic impairment was associated with greater pressor responses to pyridostigmine.
Methods: To identify predictors of pressor response, linear regression analyses between the effect of pyridostigmine on upright BP and markers of autonomic impairment were retrospectively conducted on 38 patients who had a medication trial with pyridostigmine (60 mg single dose).
Decreased urinary excretion of norepinephrine and dopamine in autonomic synucleinopathies.
Clin Auton Res
December 2024
Autonomic Medicine Section (AMS), Clinical Neurosciences Program (CNP), Division of Intramural Research (DIR), National Institute of Neurological Disorders and Stroke (NINDS), National Institutes of Health (NIH), 10 Center Drive MSC-1620, Building 10 Room 8N260, Bethesda, MD, 20892-1620, USA.
Article Synopsis
- Autonomic synucleinopathies like Parkinson's disease (PD), pure autonomic failure (PAF), and multiple system atrophy (MSA) are characterized by autonomic failure and the abnormal buildup of alpha-synuclein protein in the body. !* -
- Research showed that patients with these conditions had significantly lower urinary excretion rates of norepinephrine and dopamine compared to controls, suggesting a potential dysfunction in their renal sympathetic function. !* -
- The study analyzed urine samples from participants at the NIH over nearly three decades and found no significant differences in DOPA and epinephrine levels among the groups, indicating that only norepinephrine and dopamine were impacted by these diseases. !*
Ambulatory autonomic nervous system activity in relation to hearing impairment.
Psychophysiology
December 2024
Department of Biological Psychology, Faculty of Behavioral and Movement Sciences, Vrije Universiteit, Amsterdam, The Netherlands.
Previous research has demonstrated that hearing impairment is associated with heightened subjective experiences of listening effort, fatigue, and stress, impacting daily functioning. This study aimed to evaluate whether hearing impairment alters physiological stress systems and whether different aspects of hearing impairment could vary in predicting dysregulation in these systems. Hallmark measures of parasympathetic and sympathetic nervous system activity were derived from electrocardiography, impedance cardiography, and electrodermal activity recordings taken from 133 individuals, aged 37 to 73, over two 24-hr periods, including sleep.
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