Objective: To evaluate the role of reduced cardiac postganglionic sympathetic innervation as a prognostic factor in Multiple System Atrophy(MSA)

Background: Cardiovascular autonomic dysfunction in MSA clinically manifests with orthostatic hypotension and in most of patients is related to preganglionic degeneration, as supported by cardiac imaging using iodine-123-metaiodobenzylguanidine(123I-MIBG),an index of sympathetic innervation, that is typically normal[1,2]. However, reduced tracer uptake has been reported in one third of patients with MSA. Whether cardiac postganglionic denervation in MSA is associated to a different and/or more severe clinical phenotype is still unclear

Method: Clinical records, plasma cathecolamine values and cardiovascular autonomic tests findings of patients affected by MSA, who underwent cardiac 123I-MIBG scintigraphy were retrospectively revised

Results: Data from sixty patients diagnosed with clinically established or probable MSA according to the MDS 2022 diagnostic criteria[3] were collected. Fourty-six had normal(N) and fourteen had reduced(R) sympathetic innervation on cardiac 123I-MIBG scintigraphy. Mean age of disease onset(57.4-N vs 59.1-R), disease duration (3.6vs4 yrs), LEDD (527.1vs513.5), Unified MSA Rating Scale scores in part I (18.5vs19.4) and part II(20.7vs22.6), Composite Autonomic System Scale total score(35.3vs33) and subscores were similar between groups. Parkinsonian and cerebellar phenotypes were equally represented. Patients with reduced cardiac innervation had lower supine noradrenaline plasmatic concentration, while no difference was found in the orthostatic condition. No difference was found in the incidence of orthostatic hypotension at head-up tilt test and in other indexes of sympathetic dysfunction on autonomic testing, i.e. mean blood pressure(BP) fall in early phase II, mean BP rise in late phase II, pressure recovery time and presence of BP overshoot during Valsalva maneuver. Cardiac vagal indexes were also similar in both groups.

Conclusion: Patients with MSA and postganglionic cardiovascular dysfunction show similar clinical features, disease severity scales scores and cardiovascular autonomic testing results compared to those with preganglionic, while they had lower supine plasma noradrenaline levels.The mechanism underling postganglionic denervation in MSA, as long as its pathophysiological implications, remain unclear and needs further investigations.

References: 1. Eckhardt C, Krismer F, Donnemiller E, Eschlböck S, Fanciulli A, Raccagni C, Bösch S, Mair K, Scherfler C, Djamshidian A, Uprimny C, Metzler B, Seppi K, Poewe W, Kiechl S, Virgolini I, Wenning GK. – Cardiac sympathetic innervation in Parkinson’s disease versus multiple system atrophy – Clin
Auton Res – 2022 – 32(2) – 103-114
2. Brumberg J, Kuzkina A, Lapa C, Mammadova S, Buck A, Volkmann J, Sommer C, Isaias IU, Doppler K – Dermal and cardiac autonomic fiber involvement in Parkinson’s disease and multiple system atrophy – Neurobiol Dis – 2021 – 153-105332
3. Wenning GK, Stankovic I, Vignatelli L, Fanciulli A, Calandra-Buonaura G, Seppi K, Palma JA, Meissner WG, Krismer F, Berg D, Cortelli P, Freeman R, Halliday G, Höglinger G, Lang A, Ling H, Litvan I, Low P, Miki Y, Panicker J, Pellecchia MT, Quinn N, Sakakibara R, Stamelou M, Tolosa E,
Tsuji S, Warner T, Poewe W, Kaufmann H. – The Movement Disorder Society Criteria for the Diagnosis of Multiple System Atrophy. Mov Disord – Mov Disord – 2022 – 37(6) – 1131-1148

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MDS Abstracts - https://www.mdsabstracts.org/abstract/prognostic-role-of-postganglionic-cardiovascular-dysfunction-in-multiple-system-atrophy/