Neurology · 2014 · 49 citations · 36 references
MedicineNeurologyNeuroscienceNeuromuscular DisordersNeuropathologyNeurophysiologyAutonomic Nervous System TestingAutonomic NeuropathyMultiple System AtrophyPeripheral Nerve AnatomyPeripheral Nerve BiopsyPeripheral Nerve DisordersCholinergic BiomarkersImmunofluorescence ImagingIndirect Immunofluorescence AssayMorphometric IndicesPostganglionic Sympathetic DenervationSelf-administered QuestionnairesSudomotor DysfunctionSudomotor Function Testing
OBJECTIVE: To evaluate postganglionic autonomic involvement in multiple system atrophy (MSA). METHODS: We quantified sudomotor innervation in skin biopsy of 29 patients with MSA (19 male and 10 female; age 60.0 ± 7.7 years) and 29 age- and sex-matched healthy subjects. Samples were obtained from thigh and leg and, in 20 out of the 29 cases, also from fingertip. Dysautonomic complaints were evaluated by SCOPA-AUT, a self-administered questionnaire. Sudomotor function was evaluated in a subgroup of patients by the silastic imprint test. Skin samples were processed by indirect immunofluorescence using pan-neuronal and selective cholinergic markers. Total length of sudomotor nerves was measured on digital confocal images using a semiautomated morphometric approach. RESULTS: Measurements of sudomotor nerve density (total length of nerve per volume of glandular tissue) favorably correlated to values obtained using a stereologic unbiased method. Sudomotor nerve density was lower in patients compared to controls in all the examined sites (0.9 ± 0.2 vs 1.9 ± 0.4 nm/μm(3), p < 0.001, in fingertip; 0.7 ± 0.2 vs 1.9 ± 0.5 nm/µm(3), p < 0.001, in thigh; 0.6 ± 0.2 vs 1.8 ± 0.4 nm/µm(3), p < 0.001, in leg). CONCLUSIONS: Our data support the hypothesis that postganglionic impairment occurs in MSA and may contribute with the coexisting degeneration of central structures to the development of dysautonomic disorders in this condition.
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