Abstract
Ross syndrome (RS) is a rare peripheral autonomic system disorder characterized by tonic pupil, hyporeflexia, and segmental anhidrosis. Neuropathological studies show that RS results from the selective cholinergic nerve degeneration. However, the cause and underlying mechanisms are largely unknown. Here, we show α-synuclein accumulation in the autonomic nerve terminals in the lesser curvature of stomach of patients with RS. In addition, immunohistochemical findings demonstrate that a dominant degeneration of cholinergic fibers is exhibited in patients with RS, while main degeneration of adrenergic fibers is demonstrated in patients with pure autonomic failure in their gastrointestinal and urinary system. Our study suggests that RS belongs to α-synucleinopathies. Moreover, our findings indicate that adrenergic nerves and cholinergic nerves are not equally damaged in different types of pure autonomic dysfunctions.
Highlights
Ross syndrome (RS) is a rare disorder characterized by segmental anhidrosis, tonic pupil, and hyporeflexia first reported by Ross (1958). Sommer et al (2002) found a selective loss of cholinergic sudomotor fibers in Ross patients
Ross Syndrome and Synucleinopathy findings show that lesions in the peripheral autonomic nervous system of Pure autonomic failure (PAF) are associated with α-Syn-positive deposits (Thaisetthawatkul, 2016)
Twenty-three subjects with RS, PAF, Parkinson’s disease (PD), or multiple system atrophy (MSA) participated in the study
Summary
Ross syndrome (RS) is a rare disorder characterized by segmental anhidrosis, tonic pupil, and hyporeflexia first reported by Ross (1958). Sommer et al (2002) found a selective loss of cholinergic sudomotor fibers in Ross patients. Sommer et al (2002) found a selective loss of cholinergic sudomotor fibers in Ross patients. Α-Synucleinopathies are a group of neurodegenerative diseases characterized by the abnormal accumulation of α-synuclein (α-Syn) aggregates in neurons, glial cells, or nerve fibers, including Parkinson’s disease (PD), Lewy body dementia (LBD), and multiple system atrophy (MSA). Ross Syndrome and Synucleinopathy findings show that lesions in the peripheral autonomic nervous system of PAF are associated with α-Syn-positive deposits (Thaisetthawatkul, 2016). We investigated neuropathological changes or innervation of skin, stomach, and bladder from patients with RS, PAF, PD, or MSA. We found that α-Syn aggregates deposit in autonomic nerve terminals of skin and lesser curvature of stomach in patients with RS. Immunohistochemical studies showed that there is a dominant loss of cholinergic fibers in patients with RS, while the mainly damaged fibers are adrenergic in patients with PAF
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