PT  - JOURNAL ARTICLE
AU  - Giannini, Giulia
AU  - Calandra-Buonaura, Giovanna
AU  - Asioli, Gian Maria
AU  - Cecere, Annagrazia
AU  - Barletta, Giorgio
AU  - Mignani, Francesco
AU  - Ratti, Stefano
AU  - Guaraldi, Pietro
AU  - Provini, Federica
AU  - Cortelli, Pietro
TI  - The natural history of idiopathic autonomic failure
AID  - 10.1212/WNL.0000000000006243
DP  - 2018 Sep 25
TA  - Neurology
PG  - e1245--e1254
VI  - 91
IP  - 13
4099  - http://n.neurology.org/content/91/13/e1245.short
4100  - http://n.neurology.org/content/91/13/e1245.full
SO  - Neurology2018 Sep 25; 91
AB  - 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.BP=blood pressure; DLB=dementia with Lewy bodies; HR=heart rate; HUTT=head-up tilt test; IAF=idiopathic autonomic failure; IAF-Bo=patients with IAF longitudinally evaluated in the Department of Biomedical and Neuromotor Sciences, University of Bologna; IQR=interquartile range; MIBG=123I-metaiodobenzylguanidin; MSA=multiple system atrophy; ncIAF=nonconverters idiopathic autonomic failure; nOH=noncoverter orthostatic hypotension; PAF=pure autonomic failure; PD=Parkinson disease; RBD=REM sleep behavior disorder; SBP=systolic blood pressure; VM=Valsalva maneuver; VPSG=video-polysomnography; VR=Valsalva ratio