Abstract
Several neurodegenerative disorders characterized by the presence of intracellular inclusions containing α-synuclein may manifest with prominent autonomic failure (AF). They include multiple system atrophy (MSA), characterized by the presence of synuclein-containing glial cytoplasmic inclusions (GCIs); and disorders associated with accumulation of Lewy bodies and Lewy neurites, including Parkinson disease (PD) and dementia with Lewy bodies (DLB); and the so-called pure autonomic failure (PAF). Early orthostatic hypotension (OH), neurogenic bladder, and sexual dysfunction are distinctive features of MSA, whereas gastrointestinal dysmotility is a prominent manifestation of PD. In general, AF in MSA reflects involvement of brainstem premotor and spinal autonomic nuclei, whereas in PD involvement progresses in a distal-to-proximal fashion from the periphery and autonomic ganglia.In patients with autonomic failure, the presence of laryngeal stridor strongly suggests MSA; therefore all patients with suspected MSA should undergo a polysomnogram
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