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W2336843022 abstract "Objective: To review the spectrum and severity of autonomic dysfunction in Dementia with Lewy Bodies (DLB). Background: DLB is among a group of neurodegenerative disorders characterized by intracellular accumulation of α-synuclein aggregates (alpha-synucleinopathies). Among these disorders, autonomic involvement has been well-described in Parkinson9s disease (PD) and Multi-Systems Atrophy (MSA), occurring independently of motor manifestations, though similar systematic evaluation has not been conducted in DLB. Methods: We present a case series of clinically diagnosed DLB patients and a review of the literature, with the aim of illustrating the clinical significance of autonomic dysfunction in this group. Chart-documented clinical course and quantitative clinical and laboratory characteristics were reviewed, including autonomic symptoms (COMPASS-31) and function testing (CASS). Chart review included: orthostatic intolerance/hypotension (OI/OH), syncope, falls, urinary incontinence/retention, constipation, sudomotor changes, sicca syndrome, erectile dysfunction. Results: Four patients (ages 70-76; 50[percnt] female) with clinically diagnosed DLB (diagnosed as possible/probable DLB by a movement disorder specialist) underwent comprehensive autonomic evaluation.OI, urinary incontinence and constipation were the most prominent symptoms of autonomic involvement. All 4 patients had at least moderate cardiovagal and moderate-severe adrenergic dysfunction and orthostatic hypotension on tilt table testing (mean CASS 5.7; range 4-8).Two had supine hypertension on tilt testing. Mean autonomic symptom (COMPASS-31) score was 36.8 (range 24-55). Most notably, all patients were minimally symptomatic despite marked orthostatic hypotension on head-up tilt. Conclusions: The degree of autonomic dysfunction in this case series of DLB patients was substantial, with multi-domain symptom and function scores congruent with moderate to severe autonomic failure. Autonomic dysfunction is likely under-appreciated, and importantly possibly subclinical in the presence of marked OH. The clinical recognition of this aspect of DLB is important, considering potential fall related morbidity. Further prospective studies are needed to better characterize this component of autonomic dysfunction and limit morbidity associated with these features. Disclosure: Dr. Heiry has nothing to disclose. Dr. Cortez has nothing to disclose." @default.
W2336843022 created "2016-06-24" @default.
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W2336843022 date "2016-04-05" @default.
W2336843022 modified "2023-09-24" @default.
W2336843022 title "Autonomic Dysfunction in Dementia with Lewy Bodies, a Case Series (P6.237)" @default.
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