Lewy body disease presenting with pure autonomic failure and immune-mediated autonomic neuropathies

Abstract

Autonomic dysfunctions can result from various diseases, including neurodegenerative diseases and immune-mediated neuropathies. Pure autonomic failure (PAF) is a kind of Lewy body-related alpha-synucleinopathy manifesting only peripheral autonomic dysfunctions, such as orthostatic hypotension, without motor and sensory impairments. By contrast, autonomic neuropathies can occur primarily or secondarily to various underlying diseases. Primary autonomic neuropathies are divided into autoimmune autonomic ganglionopathy, acute autonomic and sensory neuropathy, and acute autonomic sensory and motor neuropathy based on the concomitance or absence of sensory or motor dysfunctions. The monophasic clinical course and frequent presence of a history of antecedent infections suggests that immune mechanisms participate in these neuropathies. The discovery of the anti-ganglionic acetylcholine receptor antibody significantly expanded the spectrum of autonomic neuropathies, especially autoimmune autonomic ganglionopathy, to include cases with chronic progression mimicking PAF. Some immunological diseases, such as Sjögren’s syndrome and paraneoplastic neurological syndrome may also cause diverse autonomic neuropathies. Differentiating these diseases is important from the viewpoint of appropriate management. .