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1.
Continuum (Minneap Minn) ; 26(1): 12-24, 2020 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-31996619

RESUMO

PURPOSE OF THE REVIEW: This article reviews the anatomic, functional, and neurochemical organization of the sympathetic and parasympathetic outputs; the effects on target organs; the central mechanisms controlling autonomic function; and the pathophysiologic basis for core symptoms of autonomic failure. RECENT FINDINGS: Functional neuroimaging studies have elucidated the areas involved in central control of autonomic function in humans. Optogenetic and other novel approaches in animal experiments have provided new insights into the role of these areas in autonomic control across behavioral states, including stress and the sleep-wake cycle. SUMMARY: Control of the function of the sympathetic, parasympathetic, and enteric nervous system functions depends on complex interactions at all levels of the neuraxis. Peripheral sympathetic outputs are critical for maintenance of blood pressure, thermoregulation, and response to stress. Parasympathetic reflexes control lacrimation, salivation, pupil response to light, beat-to-beat control of the heart rate, gastrointestinal motility, micturition, and erectile function. The insular cortex, anterior and midcingulate cortex, and amygdala generate autonomic responses to behaviorally relevant stimuli. Several nuclei of the hypothalamus generate coordinated patterns of autonomic responses to internal or social stressors. Several brainstem nuclei participate in integrated control of autonomic function in relationship to respiration and the sleep-wake cycle. Disorders affecting the central or peripheral autonomic pathways, or both, manifest with autonomic failure (including orthostatic hypotension, anhidrosis, gastrointestinal dysmotility, and neurogenic bladder or erectile dysfunction) or autonomic hyperactivity, primary hypertension, tachycardia, and hyperhidrosis.

2.
Auton Neurosci ; 223: 102550, 2020 01.
Artigo em Inglês | MEDLINE | ID: mdl-31928708

RESUMO

INTRODUCTION: Human papillomavirus (HPV) vaccination has been anecdotally connected to development of dysautonomia, chronic fatigue, complex regional pain syndrome and postural tachycardia syndrome. OBJECTIVES: To critically evaluate a potential connection between HPV vaccination and above noted conditions. METHODS: We reviewed the literature containing the biology of the virus, pathophysiology of infection, epidemiology of associated cancers, indications of HPV vaccination, safety surveillance data and published reports linking HPV vaccination to autonomic disorders. RESULTS: At this time the American Autonomic Society finds that there are no data to support a causal relationship between HPV vaccination and CRPS, chronic fatigue, POTS or other forms of dysautonomia. CONCLUSIONS: Certain conditions are prevalent in the same patient populations that are vaccinated with the HPV vaccine (peri-pubertal males and females). This association, however, is insufficient proof of causality.

3.
Clin Auton Res ; 30(1): 13-18, 2020 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-31475305

RESUMO

INTRODUCTION: Human papillomavirus (HPV) vaccination has been anecdotally connected to the development of dysautonomia, chronic fatigue, complex regional pain syndrome and postural tachycardia syndrome. OBJECTIVES: To critically evaluate a potential connection between HPV vaccination and the above-noted conditions. METHODS: We reviewed the literature containing the biology of the virus, pathophysiology of infection, epidemiology of associated cancers, indications of HPV vaccination, safety surveillance data and published reports linking HPV vaccination to autonomic disorders. RESULTS: At this time, the American Autonomic Society finds that there are no data to support a causal relationship between HPV vaccination and CRPS, chronic fatigue, and postural tachycardia syndrome to other forms of dysautonomia. CONCLUSION: Certain conditions are prevalent in the same populations that are vaccinated with the HPV vaccine (peri-pubertal males and females). This association, however, is an insufficient proof of causality.

4.
Auton Neurosci ; 222: 102589, 2019 12.
Artigo em Inglês | MEDLINE | ID: mdl-31706219

RESUMO

INTRODUCTION: Forearm QSWEAT recordings are occasionally absent in females, likely due to high skin resistance. METHODS: We identified consecutive subjects with no sudomotor abnormalities but absent/markedly reduced QSWEAT forearm volume, and repeated QSWEAT at the same site after gentle abrasion. RESULTS: QSWEAT volumes were absent for 4 subjects and markedly reduced for the other 4 (median 0.01, IQR 0-0.03). After gentle skin abrasion, repeat volumes were significantly higher for all subjects and became normal in 7 of 8 subjects. DISCUSSION: Skin abrasion restores QSWEAT volumes in previously absent/markedly reduced site suggesting that skin preparation using abrasion is more effective.

5.
Neurology ; 93(14): 630-639, 2019 10 01.
Artigo em Inglês | MEDLINE | ID: mdl-31570638

RESUMO

Multiple system atrophy (MSA) is a neurodegenerative disorder characterized by a combination of autonomic failure, cerebellar ataxia, and parkinsonism. Laryngeal stridor is an additional feature for MSA diagnosis, showing a high diagnostic positive predictive value, and its early occurrence might contribute to shorten survival. A consensus definition of stridor in MSA is lacking, and disagreement persists about its diagnosis, prognosis, and treatment. An International Consensus Conference among experts with methodological support was convened in Bologna in 2017 to define stridor in MSA and to reach consensus statements for the diagnosis, prognosis, and treatment. Stridor was defined as a strained, high-pitched, harsh respiratory sound, mainly inspiratory, occurring only during sleep or during both sleep and wakefulness, and caused by laryngeal dysfunction leading to narrowing of the rima glottidis. According to the consensus, stridor may be recognized clinically by the physician if present at the time of examination, with the help of a witness, or by listening to an audio recording. Laryngoscopy is suggested to exclude mechanical lesions or functional vocal cord abnormalities related to different neurologic conditions. If the suspicion of stridor needs confirmation, drug-induced sleep endoscopy or video polysomnography may be useful. The impact of stridor on survival and quality of life remains uncertain. Continuous positive airway pressure and tracheostomy are both suggested as symptomatic treatment of stridor, but whether they improve survival is uncertain. Several research gaps emerged involving diagnosis, prognosis, and treatment. Unmet needs for research were identified.


Assuntos
Conferências de Consenso como Assunto , Atrofia de Múltiplos Sistemas/diagnóstico , Atrofia de Múltiplos Sistemas/fisiopatologia , Sons Respiratórios/fisiopatologia , Humanos , Atrofia de Múltiplos Sistemas/terapia , Prognóstico , Resultado do Tratamento
6.
8.
Ann Neurol ; 86(6): 969-974, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31621939

RESUMO

Accurate antemortem diagnosis of parkinsonism is primarily based on clinical evaluation with limited biomarkers. We evaluated the diagnostic utility of quantitative rapid eye movement (REM) sleep without atonia analysis in the submentalis and anterior tibialis muscles in parkinsonian patients (53 synucleinopathy, 24 tauopathy). Receiver operating characteristic curves determined REM sleep without atonia cutoffs distinguishing synucleinopathies from tauopathies. Elevated submentalis muscle activity was highly sensitive (70-77%) and specific (95-100%) in distinguishing synucleinopathy from tauopathy. In contrast, anterior tibialis synucleinopathy discrimination was poor. Our results suggest that elevated submentalis REM sleep without atonia appears to be a potentially useful biomarker for presumed synucleinopathy etiologies in parkinsonism. ANN NEUROL 2019;86:969-974.

9.
Neurology ; 93(14): e1339-e1347, 2019 10 01.
Artigo em Inglês | MEDLINE | ID: mdl-31484717

RESUMO

OBJECTIVE: To evaluate the pattern and severity of autonomic dysfunction in autopsy-confirmed progressive supranuclear palsy (PSP) compared to α-synuclein pathology. METHODS: Autopsy-confirmed cases of 14 patients with PSP, 18 with multiple system atrophy (MSA), and 24 with Lewy body disease (LBD) with antemortem autonomic testing were reviewed retrospectively. All patients underwent comprehensive clinical evaluations by a movement disorder specialist, formal autonomic testing, and postmortem examinations at Mayo Clinic. RESULTS: The absence of orthostatic hypotension (OH) was the strongest autonomic parameter that distinguished PSP from α-synucleinopathies (0% vs 69%, p < 0.0001). Tests of adrenergic failure, which distinguish neurogenic OH, also differentiated PSP from other groups. These included the pressure recovery time (p = 0.0008), adrenergic impairment score (p = 0.001), and magnitude of change of systolic (p = 0.0002) and diastolic (p = 0.0001) blood pressures (BPs) during upright tilt. In addition, REM sleep behavior disorder was seen less frequently (p = 0.006) in PSP (33%) compared to MSA (87%) and LBD (90%). Antemortem clinical diagnostic accuracy for these phenotypically variable disorders was 57% for PSP and 83% for α-synucleinopathies. CONCLUSION: Our results suggest that the cardiovascular adrenergic system, which sustains BP during standing, is relatively unaffected, if not spared, in PSP. These findings increase our understanding of the clinical signature of PSP and have the potential to improve diagnostic accuracy in atypical parkinsonisms by distinguishing PSP from the α-synucleinopathies.


Assuntos
Hipotensão Ortostática/diagnóstico , Hipotensão Ortostática/fisiopatologia , Paralisia Supranuclear Progressiva/diagnóstico , Paralisia Supranuclear Progressiva/fisiopatologia , Idoso , Idoso de 80 Anos ou mais , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos
10.
Parkinsonism Relat Disord ; 66: 212-215, 2019 09.
Artigo em Inglês | MEDLINE | ID: mdl-31327626

RESUMO

OBJECTIVE: To determine whether smoking or alcohol use impacts the age of onset and disease duration in multiple system atrophy (MSA). METHODS: All patients diagnosed with MSA at Mayo Clinic, Rochester between 1998 and 2012 completed standardized questionnaires surveying smoking and alcohol use at the time of presentation. RESULTS: Of 551 patients with smoking and alcohol use data, 281 were past or present smokers with age of onset of 60.76 years compared to 62.97 years in controls (p = 0.0144). Age of onset in the 87 heavy alcohol users was 56.87 years compared to 62.97 years in controls (p = 0.0133). There was no difference in disease duration for smokers (p = 0.2758) or heavy alcohol users (p = 0.4820) compared to controls. CONCLUSION: Our findings show that smoking history and/or heavy alcohol use is associated with younger age of onset in MSA but do not influence survival.

12.
Auton Neurosci ; 220: 102559, 2019 09.
Artigo em Inglês | MEDLINE | ID: mdl-31331696

RESUMO

Pure autonomic failure (PAF) is a progressive syndrome of neurogenic orthostatic hypotension, widespread anhidrosis, urinary retention, and constipation without other neurologic manifestations. It is generally considered a peripheral ganglionic synucleinopathy. Natural history studies have described risk factors for the conversion of PAF to Parkinson's disease, multiple system atrophy, or dementia with Lewy bodies, yet the early stages of PAF are not well characterized. We present a patient with unilateral anhidrosis, contralateral facial flushing and hyperhidrosis consistent with Harlequin syndrome that, over 6 years, progressed to PAF, suggesting that PAF may present with focal autonomic impairment prior to generalized autonomic failure.

13.
Neurology ; 93(1): e77-e87, 2019 07 02.
Artigo em Inglês | MEDLINE | ID: mdl-31152011

RESUMO

OBJECTIVE: This phase I/II study sought to explore intrathecal administration of mesenchymal stem cells (MSCs) as therapeutic approach to multiple system atrophy (MSA). METHODS: Utilizing a dose-escalation design, we delivered between 10 and 200 million adipose-derived autologous MSCs intrathecally to patients with early MSA. Patients were closely followed with clinical, laboratory, and imaging surveillance. Primary endpoints were frequency and type of adverse events; key secondary endpoint was the rate of disease progression assessed by the Unified MSA Rating Scale (UMSARS). RESULTS: Twenty-four patients received treatment. There were no attributable serious adverse events, and injections were generally well-tolerated. At the highest dose tier, 3 of 4 patients developed low back/posterior leg pain, associated with thickening/enhancement of lumbar nerve roots. Although there were no associated neurologic deficits, we decided that dose-limiting toxicity was reached. A total of 6 of 12 patients in the medium dose tier developed similar, but milder and transient discomfort. Rate of progression (UMSARS total) was markedly lower compared to a matched historical control group (0.40 ± 0.59 vs 1.44 ± 1.42 points/month, p = 0.004) with an apparent dose-dependent effect. CONCLUSIONS: Intrathecal MSC administration in MSA is safe and well-tolerated but can be associated with a painful implantation response at high doses. Compelling dose-dependent efficacy signals are the basis for a planned placebo-controlled trial. CLASSIFICATION OF EVIDENCE: This phase I/II study provides Class IV evidence that for patients with early MSA, intrathecal MSC administration is safe, may result in a painful implantation response at high doses, and is associated with dose-dependent efficacy signals.


Assuntos
Transplante de Células-Tronco Mesenquimais , Atrofia de Múltiplos Sistemas/terapia , Progressão da Doença , Feminino , Seguimentos , Humanos , Injeções Espinhais , Masculino , Transplante de Células-Tronco Mesenquimais/efeitos adversos , Transplante de Células-Tronco Mesenquimais/métodos , Pessoa de Meia-Idade , Atrofia de Múltiplos Sistemas/líquido cefalorraquidiano , Atrofia de Múltiplos Sistemas/diagnóstico por imagem , Resultado do Tratamento
14.
Front Neurol ; 10: 488, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31133978

RESUMO

Background: We compared resting-state functional connectivity (RSFC) among limbic and temporal lobe regions between patients with medial temporal lobe epilepsy (mTLE) and healthy control subjects to identify imaging evidence of functional networks related to seizure frequency, age of seizure onset, and duration of epilepsy. Methods: Twelve patients with drug-resistant, unilateral medial temporal lobe epilepsy and 12 healthy control subjects matched for age, sex, and handedness participated in the imaging experiments. We used network-based statistics to compare functional connectivity graphs in patients with mTLE and healthy controls to investigate the relationship between functional connectivity abnormalities and seizure frequency. Results: Among mTLE patients, we found functional network abnormalities throughout the limbic system, but primarily in the hemisphere ipsilateral to the seizure focus. The RSFCs between ipsilateral hypothalamus and ventral anterior cingulate cortex and between ipsilateral subiculum and contralateral posterior cingulate cortex were highly correlated with seizure frequency. Discussion: These findings suggest that in mTLE, changes in limbic networks ipsilateral to the epileptic focus are common. The pathological changes in connectivity between cingulate cortex, hypothalamus and subiculum ipsilateral to the seizure focus were correlated with increased seizure frequency.

15.
Auton Neurosci ; 219: 49-52, 2019 07.
Artigo em Inglês | MEDLINE | ID: mdl-31122601

RESUMO

To evaluate the influence of sex and gender on clinical characteristics and survival in multiple system atrophy (MSA), we reviewed MSA patients with autonomic testing 1998-2012. Of 685 patients, 52% were male. Median survival overall was 7.3 years for males, 7.6 years for females. Survival from diagnosis was 2.9 years in males, 3.8 years in females. Females were more likely to initially manifest motor symptoms. Males were more likely to have orthostatic intolerance and early catheterization. In conclusion, our data show longer survival from diagnosis in females and slight overall survival benefit which may be related to initial motor manifestations.

19.
Auton Neurosci ; 218: 54-63, 2019 05.
Artigo em Inglês | MEDLINE | ID: mdl-30890349

RESUMO

Sleep and arousal from sleep are associated with profound changes in cardiovascular and respiratory functions. Fluctuations of arterial blood pressure (ABP), heart rate (HR), and respiration occur both during non-rapid eye movement (NREM) and REM sleep and during transitions between sleep and behavioral arousal. These changes reflect complex, state-dependent interactions among several neuronal groups in the hypothalamus and brainstem. These neurons utilize the excitatory amino-acid L-glutamate or the inhibitory amino acid γ-aminobutyric acid (GABA) and are modulated in a state-dependent manner by inputs from cholinergic, monoaminergic, and hypothalamic orexin/hypocretin and melanin-concentrating hormone (MCH) neurons. These different neuronal populations mediate continuous interactions between cortical state and subcortical circuits modulating sympathetic and cardiovagal output, respiratory pattern, and chemosensitivity. Reciprocally, brainstem areas involved in these functions promote behavioral arousal in the setting of hypoxia, hypercapnia, or other stressors. Studies in rodents using optogenetic and other approaches for selective activation or inactivation of specific neuronal groups identified by their unique neurochemical markers, combined with recording of cortical activity, cardiovascular responses, and respiration, have provided new information on the brainstem mechanisms controlling arousal, wake-sleep cycle, cardiovascular and respiratory control (Luppi et al., 2017; Saper and Fuller, 2017; Scammell et al., 2017; Dampney, 2016; Del Negro et al., 2018; Guyenet, 2006; Guyenet and Abbott, 2013; Smith et al., 2013). These findings also provide further insight into the pathophysiology of sleep-related cardiovascular and respiratory disorders including sleep apnea, narcolepsy, congenital central hypoventilation syndrome, sudden infantile death syndrome, and sudden unexpected death in epilepsy.

20.
Neurology ; 92(12): 568-574, 2019 03 19.
Artigo em Inglês | MEDLINE | ID: mdl-30760635

RESUMO

The field of neuro-oncology has recently experienced a renaissance in the understanding of the molecular underpinnings and pathophysiology of glioma. Genetic markers have significant implications regarding treatment responsiveness and prognosis and are now the primary basis for classification. This article gives an updated understanding of the pathogenesis and mechanisms of resistance of glioma via discussion of 4 molecular and genetic markers: MGMT, IDH, 1p/19q, and TERT.


Assuntos
Neoplasias Encefálicas/metabolismo , Glioma/metabolismo , Idoso , Biomarcadores Tumorais/genética , Biomarcadores Tumorais/metabolismo , Neoplasias Encefálicas/diagnóstico , Neoplasias Encefálicas/genética , Neoplasias Encefálicas/terapia , Resistencia a Medicamentos Antineoplásicos/fisiologia , Glioma/diagnóstico , Glioma/genética , Glioma/terapia , Humanos , Masculino
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