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Autonomic Nervous System Disorders
Published in Philip B. Gorelick, Fernando D. Testai, Graeme J. Hankey, Joanna M. Wardlaw, Hankey's Clinical Neurology, 2020
Pure autonomic failure (PAF):3Degeneration of predominantly peripheral autonomic fibers.Deposition of synuclein in peripheral autonomic structures.Age of onset: 40–60 years.Postural hypotension (dizziness and weakness on standing or walking).Erectile failure.Bowel and bladder disturbances.Loss of sweating.Initially normal neurologic examination (which largely separates PAF from more widespread neurodegenerative disorders; however, rapid eye movement [REM] sleep behavior may predict other neurodegenerative disorders).Slowly progressive course.
Clinical spectrum of Lewy body disease
Published in John O'Brien, Ian McKeith, David Ames, Edmond Chiu, Dementia with Lewy Bodies and Parkinson's Disease Dementia, 2005
Maria J Martí, Jaume Campdelacreu, Eduardo Tolosa
The paradigm of autonomic dysfunction within the LB disorders is pure autonomic failure (PAF). It is an uncommon progressive, adult-onset, sporadic disorder characterized by orthostatic hypotension as the cardinal symptom, usually with evidence of more widespread autonomic failure (Consensus, 1996). Postural related symptoms as dizziness, syncope, visual disturbances and suboccipital/paracervical 'coat-hanger' neck pain are common in these patients (Mathias et al, 1999). Patients may also present with decreased sweating and heat intolerance, urinary dysfunction (frequency, urgency and incontinence), constipation or diarrhoea and erectile dysfunction (Hague et al, 1997; Arai et al, 2000). No other neurological symptoms are present. However, some patients who initially present with features of PAF develop years later symptomatology that prompt reclassification of the diagnosis within the Lewy body disorders spectrum (Larner et al, 2000; Kaufmann et al, 2004).
Shy-Drager Syndrome and Multiple System Atrophy
Published in David Robertson, Italo Biaggioni, Disorders of the Autonomic Nervous System, 2019
Concomitant with the progress in clinical investigation there has been an attempt to unify the terminology applied to disorders of the autonomic nervous system. A multitude of terms has been utilized, often interchangeably, in publications dealing with MSA. This indiscriminate, imprecise use of nomenclature hampers comparison among reports in the medical literature. Critical review of the clinical details is required to maintain the proper perspective. Nosology is particularly relevant to the separation between pure autonomic failure (PAF) and MSA since these distinct disorders are characterized by clear differences in clinical features, pathophysiology and prognosis. Pure autonomic failure is a syndrome of chronic autonomic dysfunction occurring in the absence of any identifiable etiology, peripheral neuropathy or central neurological signs (Bradbury and Eggleston, 1925). In contrast, MSA includes several degenerative neurological disorders that may be attended by autonomic failure (Graham and Oppenheimer, 1969). Implicit in the descriptive term multiple system atrophy is the neuronal loss affecting a variety of central nervous system regions. The primary involvement of several autonomic centers justifies inclusion of this involuntary movement disorder within the scope of a textbook focused on autonomic dysfunction. This chapter will review many of the accomplishments achieved in elucidating various aspects of this complex disorder. Since treatment of autonomic dysfunction is discussed in a separate section of this book, only those therapeutic aspects specifically relevant to MSA will be mentioned at the end of this chapter.
Spinal cord involvement in Lewy body-related α-synucleinopathies
Published in The Journal of Spinal Cord Medicine, 2020
Raffaele Nardone, Yvonne Höller, Francesco Brigo, Viviana Versace, Luca Sebastianelli, Cristina Florea, Kerstin Schwenker, Stefan Golaszewski, Leopold Saltuari, Eugen Trinka
Fibers originating in the rostroventral nucleus of the medulla that traveling in the Th/IML provide the efferent innervation. Of great importance is also the capacitance of the splanchnic-mesenteric bed. This region is supplied by the splanchnic nerve with cell bodies at the thoracic level and synapses at the celiac ganglion.91 Orthostatic hypotension is a chief complaint in the pure autonomic failure, in LBD and in MSA. Some cases starting as isolated autonomic failure and subsequently developing PD or LBD features were described.71,92,93 Moreover, dysfunctions of cardiac sympathetic and parasympathetic neurons were observed in the initial stages of PD.93 Even is also the genesis of orthostatic hypotension is likely to be multifactorial, the pathology of the dorsal motor nucleus of the vagus may explain the early occurrence of orthostatic hypotension.
State-of-the-art pharmacotherapy for autonomic dysfunction in Parkinson’s disease
Published in Expert Opinion on Pharmacotherapy, 2020
Cecilia Quarracino, Matilde Otero-Losada, Francisco Capani, Santiago Pérez-Lloret
Its efficacy in PD patients was demonstrated in post hoc analysis or the integrated analysis of the 4-phase III trials [38–40]. Biaggioni, et al [38] studied the response of patients with PD, multiple system atrophy, and others, all with neurogenic OH, to droxidopa withdrawal in a multinational, randomized, placebo-controlled double-blind trial. Subjective worsening of symptoms was higher in 43 patients in the placebo group compared with 44 patients treated with droxidopa. Almost 60% of treated patients reported mild adverse effects. Kauffman et al. [40] also studied patients with neurogenic OH (with pure autonomic failure, multiple system atrophy, or PD) responsive to droxidopa treatment in a randomized placebo-controlled multicenter trial. After a 7-day wash-out period, the patients were assigned to droxidopa (n = 82), or placebo (n = 80) for 7 days. Subjective improvement in the mean score of the Orthostatic Hypotension Questionnaire and the actual improvement in standing systolic blood pressure were statistically significant in the droxidopa arm. Post-hoc analysis of the results in the PD subgroup did not reach statistical significance likely because of insufficient data (31 and 35 patients in the placebo and droxidopa arms respectively). Adverse events were mild, headache the most frequent (7% of droxidopa treated patients).
Autoimmune autonomic ganglionopathy: an update on diagnosis and treatment
Published in Expert Review of Neurotherapeutics, 2018
Shunya Nakane, Akihiro Mukaino, Osamu Higuchi, Mari Watari, Yasuhiro Maeda, Makoto Yamakawa, Keiichi Nakahara, Koutaro Takamatsu, Hidenori Matsuo, Yukio Ando
Goldstein et al. report tried to differentiate AAG from pure autonomic failure (PAF), because some part of AAG and PAF are same chronic forms of autonomic failure [75]. AAG often has a chronic progressive course that may resemble degenerative forms of autonomic failure such as PAF. Both disorders feature low plasma levels of catecholamines during supine rest, but plasma levels of the other endogenous catechols, dihydroxyphenylalanine, dihydroxyphenylacetic acid, and dihydroxyphenylglyco seem to be lower in PAF than in AAG. In this article, they report results of QSART, Valsalva maneuver, head-up tilt test, and 6-[18F] fluorodopamine PET scanning besides the measurement of plasma catechols, in patients with AAG, PAF, and two other forms of chronic autonomic failure manifesting with OH – multiple system atrophy (MSA) and Parkinson’s disease with neurogenic OH (PD with NOH) [75]. Baroreflex – cardiovagal gain, calculated from the slope of the line of best fit for the relationship between interbeat interval and systolic blood pressure during Phase II of the Valsalva maneuver, was also low in all four groups. QSART responses were remarkably subnormal in AAG patients. Conversely, QSART responses were often within the normal range three patient groups in the three patient groups. 6-[18F] fluorodopamine-derived radioactivity in the interventricular septum was normal in the AAG and MSA groups and low in the PAF and PD with NOH groups.