The natural history of idiopathic autonomic failure: The IAF-BO cohort study

Giulia Giannini, Giovanna Calandra-Buonaura, Gian Maria Asioli, Annagrazia Cecere, Giorgio Barletta, Francesco Mignani, Stefano Ratti, Pietro Guaraldi, Federica Provini, Pietro Cortelli

Research output: Contribution to journalArticle

Abstract

OBJECTIVE: To retrospectively describe clinical and instrumental features of a cohort of patients with at least a 5-year history of idiopathic autonomic failure (IAF) longitudinally evaluated at the Autonomic Unit of the University of Bologna (IAF-Bo cohort).

METHODS: We identified patients with at least a 5-year history of IAF who were referred to our department from 1989 to 2016 and evaluated at least once a year during the disease course. Clinical and instrumental data were collected from medical records. Clinical variables were categorized as early if presenting within 3 years from disease onset. Predictors associated with conversion to other synucleinopathies were identified in a Cox regression analysis.

RESULTS: The IAF-Bo cohort included 50 patients (39 male, 19 deceased at the last follow-up). At the last follow-up visit, 34 patients retained IAF phenotype (ncIAF group), while 16 developed a CNS synucleinopathy (converters group). Specific clinical and instrumental features were represented differently in the converters and ncIAF groups. The converters group showed a higher risk of death than the ncIAF group. Early onset of urinary dysfunction, early onset of REM sleep behavior disorder, and a Valsalva ratio ≥1.25 were identified as variables associated with phenoconversion.

CONCLUSIONS: This is one of the largest studies on the natural history of a cohort of patients with at least a 5-year history of IAF, showing a percentage of phenoconversion of 32%. We demonstrated that specific clinical and instrumental features entail an increased probability of phenoconversion. These findings could contribute to a better definition of the nature of IAF and to the identification of early markers of phenoconversion.

Original languageEnglish
Pages (from-to)e1245-e1254
JournalNeurology
Volume91
Issue number13
DOIs
Publication statusPublished - Sep 25 2018

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