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Pure autonomic failure

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https://www.readbyqxmd.com/read/30250046/skin-%C3%AE-synuclein-deposits-differ-in-clinical-variants-of-synucleinopathy-an-in-vivo-study
#1
V Donadio, A Incensi, O El-Agnaf, G Rizzo, N Vaikath, F Del Sorbo, C Scaglione, S Capellari, A Elia, M Stanzani Maserati, R Pantieri, R Liguori
We aimed to characterize in vivo α-synuclein (α-syn) aggregates in skin nerves to ascertain: 1) the optimal marker to identify them; 2) possible differences between synucleinopathies that may justify the clinical variability. We studied multiple skin nerve α-syn deposits in 44 patients with synucleinopathy: 15 idiopathic Parkinson's disease (IPD), 12 dementia with Lewy Bodies (DLB), 5 pure autonomic failure (PAF) and 12 multiple system atrophy (MSA). Ten healthy subjects were used as controls. Antibodies against native α-syn, C-terminal α-syn epitopes such as phosphorylation at serine 129 (p-syn) and to conformation-specific for α-syn mature amyloid fibrils (syn-F1) were used...
September 24, 2018: Scientific Reports
https://www.readbyqxmd.com/read/30232253/supine-plasma-ne-predicts-the-pressor-response-to-droxidopa-in-neurogenic-orthostatic-hypotension
#2
Jose-Alberto Palma, Lucy Norcliffe-Kaufmann, Jose Martinez, Horacio Kaufmann
OBJECTIVE: To test whether the plasma levels of norepinephrine (NE) in patients with neurogenic orthostatic hypotension (nOH) predict their pressor response to droxidopa. METHODS: This was an observational study, which included patients with nOH. All patients had standardized autonomic function testing including determination of venous plasma catecholamine levels drawn through an indwelling catheter while resting supine. This was followed by a droxidopa titration with 100 mg increments in successive days until relief of symptoms, side effects, or the maximum dose of 600 mg was reached...
October 16, 2018: Neurology
https://www.readbyqxmd.com/read/30144800/characterization-of-the-symptoms-of-neurogenic-orthostatic-hypotension-and-their-impact-from-a-survey-of-patients-and-caregivers
#3
Daniel O Claassen, Charles H Adler, L Arthur Hewitt, Christopher Gibbons
BACKGROUND: Neurogenic orthostatic hypotension (nOH) results from impaired vasoconstriction due to dysfunction of the autonomic nervous system and is commonly associated with Parkinson disease (PD), multiple system atrophy (MSA), and pure autonomic failure. nOH can increase the risk of falls due to symptoms that include postural lightheadedness or dizziness, presyncope, and syncope. The purpose of this study was to obtain information from patients and caregivers regarding the symptoms and burden of nOH to expand on limited knowledge regarding the impact of nOH on quality of life...
August 25, 2018: BMC Neurology
https://www.readbyqxmd.com/read/30062642/roles-of-cardiac-sympathetic-neuroimaging-in-autonomic-medicine
#4
REVIEW
David S Goldstein, William P Cheshire
Sympathetic neuroimaging is based on the injection of compounds that either radiolabel sites of the cell membrane norepinephrine transporter (NET) or that are taken up into sympathetic nerves via the NET and radiolabel intra-neuronal catecholamine storage sites. Detection of the radioactivity is by planar or tomographic radionuclide imaging. The heart stands out among body organs in terms of the intensity of radiolabeling of sympathetic nerves, and virtually all of sympathetic neuroimaging focuses on the left ventricular myocardium...
August 2018: Clinical Autonomic Research: Official Journal of the Clinical Autonomic Research Society
https://www.readbyqxmd.com/read/29883833/rapid-eye-movement-sleep-behavior-disorder-and-the-link-to-alpha-synucleinopathies
#5
REVIEW
Daniel A Barone, Claire Henchcliffe
Rapid eye movement (REM) sleep behavior disorder (RBD) involves REM sleep without atonia in conjunction with a recurrent nocturnal dream enactment behavior, with vocalizations such as shouting and screaming, and motor behaviors such as punching and kicking. Secondary RBD is well described in association with neurological disorders including Parkinson's disease (PD), multiple system atrophy (MSA), and other conditions involving brainstem structures such as tumors. However, RBD alone is now considered to be a potential harbinger of later development of neurodegenerative disorders, in particular PD, MSA, dementia with Lewy bodies (DLB), and pure autonomic failure...
August 2018: Clinical Neurophysiology: Official Journal of the International Federation of Clinical Neurophysiology
https://www.readbyqxmd.com/read/29508456/autonomic-dysfunction-in-genetic-forms-of-synucleinopathies
#6
REVIEW
Viorica Chelban, Ekawat Vichayanrat, Lucia Schottlaende, Valeria Iodice, Henry Houlden
The discovery of genetic links between alpha-synuclein and PD has opened unprecedented opportunities for research into a new group of diseases, now collectively known as synucleinopathies. Autonomic dysfunction, including cardiac sympathetic denervation, has been reported in familial forms of synucleinopathies that have Lewy bodies at the core of their pathogenesis. SNCA mutations and multiplications, LRRK2 disease with Lewy bodies as well as other common, sporadic forms of idiopathic PD, MSA, pure autonomic failure, and dementia with Lewy bodies have all been associated with dysautonomia...
March 2018: Movement Disorders: Official Journal of the Movement Disorder Society
https://www.readbyqxmd.com/read/29508455/treatment-of-autonomic-dysfunction-in-parkinson-disease-and-other-synucleinopathies
#7
REVIEW
Jose-Alberto Palma, Horacio Kaufmann
Dysfunction of the autonomic nervous system afflicts most patients with Parkinson disease and other synucleinopathies such as dementia with Lewy bodies, multiple system atrophy, and pure autonomic failure, reducing quality of life and increasing mortality. For example, gastrointestinal dysfunction can lead to impaired drug pharmacodynamics causing a worsening in motor symptoms, and neurogenic orthostatic hypotension can cause syncope, falls, and fractures. When recognized, autonomic problems can be treated, sometimes successfully...
March 2018: Movement Disorders: Official Journal of the Movement Disorder Society
https://www.readbyqxmd.com/read/29405350/orthostatic-heart-rate-changes-in-patients-with-autonomic-failure-caused-by-neurodegenerative-synucleinopathies
#8
Lucy Norcliffe-Kaufmann, Horacio Kaufmann, Jose-Alberto Palma, Cyndya A Shibao, Italo Biaggioni, Amanda C Peltier, Wolfgang Singer, Phillip A Low, David S Goldstein, Christopher H Gibbons, Roy Freeman, David Robertson
OBJECTIVE: Blunted tachycardia during hypotension is a characteristic feature of patients with autonomic failure, but the range has not been defined. This study reports the range of orthostatic heart rate (HR) changes in patients with autonomic failure caused by neurodegenerative synucleinopathies. METHODS: Patients evaluated at sites of the U.S. Autonomic Consortium (NCT01799915) underwent standardized autonomic function tests and full neurological evaluation. RESULTS: We identified 402 patients with orthostatic hypotension (OH) who had normal sinus rhythm...
March 2018: Annals of Neurology
https://www.readbyqxmd.com/read/29396794/spectrum-of-abnormalities-of-sympathetic-tyrosine-hydroxylase-and-alpha-synuclein-in-chronic-autonomic-failure
#9
Risa Isonaka, Patti Sullivan, Yunden Jinsmaa, Abraham Corrales, David S Goldstein
OBJECTIVE: Lewy body forms of primary chronic autonomic failure (CAF) such as incidental Lewy body disease (ILBD), Parkinson's disease (PD), and pure autonomic failure evolving into dementia with Lewy bodies (PAF+DLB) feature cardiac sympathetic denervation, whereas multiple system atrophy (MSA) in most cases does not. What links Lewy bodies with cardiac sympathetic denervation in CAF? In familial PD, abnormalities of the alpha-synuclein (AS) gene cause CAF and cardiac sympathetic denervation; and in sporadic PD, brainstem Lewy bodies contain AS co-localized with tyrosine hydroxylase (TH), a marker of catecholaminergic neurons...
April 2018: Clinical Autonomic Research: Official Journal of the Clinical Autonomic Research Society
https://www.readbyqxmd.com/read/29297596/neuropathology-of-autonomic-dysfunction-in-synucleinopathies
#10
REVIEW
Elizabeth A Coon, Jeremy K Cutsforth-Gregory, Eduardo E Benarroch
The synucleinopathies-Parkinson's disease, dementia with Lewy bodies, multiple system atrophy, and pure autonomic failure-result from distinct patterns of abnormal α-synuclein aggregation throughout the nervous system. Autonomic dysfunction in these disorders results from variable involvement of the central and peripheral autonomic networks. The major pathologic hallmark of Parkinson's disease and dementia with Lewy bodies is Lewy bodies and Lewy neurites; of multiple system atrophy, oligodendroglial cytoplasmic inclusions; and of pure autonomic failure, peripheral neuronal cytoplasmic inclusions...
March 2018: Movement Disorders: Official Journal of the Movement Disorder Society
https://www.readbyqxmd.com/read/29268978/electrophysiological-changes-of-autonomic-cells-in-left-ventricular-outflow-tract-in-guinea-pigs-with-iron-deficiency-anemia-complicated-with-chronic-heart-failure
#11
Ling Fan, Li-Feng Chen, Jing Fan
OBJECTIVE: To investigate the electrophysiological changes of autonomic cells in left ventricular outflow tract in guinea pigs with iron deficiency anemia complicated with chronic heart failure. METHODS: Guinea pigs model of iron deficiency anemia complicated with chronic heart failure in 10 guinea pigs of the experimental group was made by feeding a low iron diet, pure water and subcutaneous injection of isoproterenol. The control group consisting of 11 guinea pigs was given normal food, normal water and injected with normal saline...
December 2017: Asian Pacific Journal of Tropical Medicine
https://www.readbyqxmd.com/read/29162745/does-sympathetic-dysfunction-occur-before-denervation-in-pure-autonomic-failure
#12
Ling Guo, Murray D Esler, Carolina Sari, Sarah Phillips, Elisabeth A Lambert, Nora E Straznicky, Gavin W Lambert, Susan J Corcoran
Pure autonomic failure (PAF) is a rare sporadic disorder characterized by autonomic failure in the absence of a movement disorder or dementia and is associated with very low plasma norepinephrine (NE) levels-suggesting widespread sympathetic denervation, however due to its rarity the pathology remains poorly elucidated. We sought to correlate clinical and neurochemical findings with sympathetic nerve protein abundances, accessed by way of a forearm vein biopsy, in patients with PAF and in healthy controls and patients with multiple systems atrophy (MSA) in whom sympathetic nerves are considered intact...
January 16, 2018: Clinical Science (1979-)
https://www.readbyqxmd.com/read/29111419/diagnosis-of-multiple-system-atrophy
#13
REVIEW
Jose-Alberto Palma, Lucy Norcliffe-Kaufmann, Horacio Kaufmann
Multiple system atrophy (MSA) may be difficult to distinguish clinically from other disorders, particularly in the early stages of the disease. An autonomic-only presentation can be indistinguishable from pure autonomic failure. Patients presenting with parkinsonism may be misdiagnosed as having Parkinson disease. Patients presenting with the cerebellar phenotype of MSA can mimic other adult-onset ataxias due to alcohol, chemotherapeutic agents, lead, lithium, and toluene, or vitamin E deficiency, as well as paraneoplastic, autoimmune, or genetic ataxias...
May 2018: Autonomic Neuroscience: Basic & Clinical
https://www.readbyqxmd.com/read/29030055/the-heart-of-pd-lewy-body-diseases-as-neurocardiologic-disorders
#14
David S Goldstein, Yehonatan Sharabi
This review provides an update about cardiac sympathetic denervation in Lewy body diseases. The family of Lewy body diseases includes Parkinson's disease (PD), pure autonomic failure (PAF), and dementia with Lewy bodies (DLB). All three feature intra-neuronal cytoplasmic deposits of the protein, alpha-synuclein. Multiple system atrophy (MSA), the parkinsonian form of which can be difficult to distinguish from PD with orthostatic hypotension, involves glial cytoplasmic inclusions that contain alpha-synuclein...
October 10, 2017: Brain Research
https://www.readbyqxmd.com/read/28911152/hypothalamic-glucose-transport-in-humans-during-experimentally-induced-hypoglycemia-associated-autonomic-failure
#15
Elizabeth R Seaquist, Amir Moheet, Anjali Kumar, Dinesh K Deelchand, Melissa Terpstra, Kristine Kubisiak, Lynn E Eberly, Pierre-Gilles Henry, James M Joers, Gülin Öz
Context: Upregulated brain glucose transport in response to recurrent hypoglycemia may contribute to the development of hypoglycemia-associated autonomic failure (HAAF) and impaired awareness of hypoglycemia. Whether recurrent hypoglycemia alters glucose transport in the hypothalamus is unknown. Objective: To test the hypothesis that hypothalamic glucose transport will increase in healthy volunteers preconditioned with recurrent hypoglycemia to induce HAAF. Setting: University medical center...
September 1, 2017: Journal of Clinical Endocrinology and Metabolism
https://www.readbyqxmd.com/read/28889358/erratum-to-managing-neurogenic-orthostatic-hypotension-in-a-patient-presenting-with-pure-autonomic-failure-who-later-developed-parkinson-disease
#16
Fiona Gupta, Daniel Kremens, Steven Vernino, Beverly Karabin
No abstract text is available yet for this article.
December 2017: Clinical Autonomic Research: Official Journal of the Clinical Autonomic Research Society
https://www.readbyqxmd.com/read/28717053/pure-autonomic-failure
#17
REVIEW
Thomas P Brown
Pure autonomic failure is a degenerative disorder of the peripheral autonomic nervous system. Patients experience symptomatic hypotension that requires them to sit, squat or lie down to prevent syncope. It is associated with characteristic histopathological findings, resulting in neuronal cytoplasmic inclusions in the peripheral autonomic nerves. These lesions are responsible for defects in the synthesis and release of norepinephrine from sympathetic nerve terminals, resulting in significant hypotension. Patients with autonomic failure also have exaggerated blood pressure responses to common stimuli such as food or fluid intake, heat, exercise and medications...
October 2017: Practical Neurology
https://www.readbyqxmd.com/read/28713844/epidemiology-diagnosis-and-management-of-neurogenic-orthostatic-hypotension
#18
Jose-Alberto Palma, Horacio Kaufmann
BACKGROUND: Orthostatic hypotension (OH) is a sustained fall in blood pressure on standing which can cause symptoms of organ hypoperfusion. OH is associated with increased morbidity and mortality and leads to a significant number of hospital admissions particularly in the elderly (233 per 100,000 patients over 75 years of age in the US). OH can be due to volume depletion, blood loss, large varicose veins, medications, or due to defective activation of sympathetic nerves and reduced norepinephrine release upon standing (i...
May 2017: Movement Disorders Clinical Practice
https://www.readbyqxmd.com/read/28670690/possible-involvement-of-hypotension-in-postprandial-headache-a-case-series
#19
Tsubasa Takizawa, Mamoru Shibata, Takahiro Hiraide, Morinobu Seki, Shinichi Takahashi, Norihiro Suzuki
BACKGROUND: It is commonly known that headaches are induced by intake of specific food, drink, and/or additive. In addition, some patients experience postprandial headache independent of ingestion of specific items. Currently, information on the pathophysiology underlying this particular type of headache is scarce. CASE REPORTS: We report two cases in which headaches were observed after each meal. Postprandial hypotension was demonstrated in both cases. Tonometry-based continuous blood pressure measurement during head-up tilt revealed sympathetic dysfunction...
October 2017: Headache
https://www.readbyqxmd.com/read/28637099/aggregation-and-phosphorylation-of-%C3%AE-synuclein-with-proteinase-k-resistance-in-focal-%C3%AE-synucleinopathy-predominantly-localized-to-the-cardiac-sympathetic-nervous-system
#20
LETTER
N Fukasawa, T Fukuda, M Nagaoka, T Harada, H Takahashi, M Ikegami
No abstract text is available yet for this article.
April 2018: Neuropathology and Applied Neurobiology
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