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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.
Glossary
- 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
Footnotes
↵* These authors contributed equally to this work.
Go to Neurology.org/N for full disclosures. Funding information and disclosures deemed relevant by the authors, if any, are provided at the end of the article.
- Received January 31, 2018.
- Accepted in final form June 28, 2018.
- © 2018 American Academy of Neurology
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