Abstract
Background: Early brainstem neurodegeneration is common in Parkinson's disease (PD) and progressive supranuclear palsy (PSP). While previous work showed abnormalities in vestibular evoked myogenic potentials (VEMPs) in patients with either disorder as compared to healthy humans, it remains unclear whether ocular and cervical VEMPs differ between PD and PSP patients.Methods: We prospectively included 12 PD and 11 PSP patients, performed ocular and cervical VEMPs, and calculated specific VEMP scores (0 = normal, 12 = most pathological) based on latencies, amplitude, and absent responses. In addition, we assessed disease duration, presence of imbalance, motor asymmetry, and motor disability using the Movement Disorder Society Unified Parkinson's Disease Rating Scale, part III (MDS-UPDRS III). Moreover, we ascertained various sleep parameters by video-polysomnography.Results: PSP and PD patients had similar oVEMP scores (6 [3–6] vs. 3 [1.3–6], p = 0.06), but PSP patients had higher cVEMP scores (3 [0–6] vs. 0 [0–2.8], p = 0.03) and total VEMP scores (9 [5–12] vs. 4 [2–7.5], p = 0.01). Moreover, total VEMP scores >10 were only observed in PSP patients (45%, p = 0.01). MDS-UPDRS III correlated with cVEMP scores (rho = 0.77, p = 0.01) in PSP, but not in PD. In PD, but not in PSP, polysomnographic markers of disturbed sleep, including decreased rapid eye movement sleep, showed significant correlations with VEMP scores.Conclusions: Our findings suggest that central vestibular pathways are more severely damaged in PSP than in PD, as indicated by higher cervical and total VEMP scores in PSP than PD in a between-groups analysis. Meaningful correlations between VEMPs and motor and non-motor symptoms further encourage its use in neurodegenerative Parkinsonian syndromes.
Highlights
While widespread brainstem degeneration is common to both Parkinson disease (PD) and progressive supranuclear palsy (PSP), differences in the resulting motor and non-motor phenotypes likely reflect differential anatomical damage by pathological alpha-synuclein in PD and tau proteins in PSP
The two groups did not differ with regard to gender distribution, disease duration, and MDS-UPDRS III, but PSP patients were older than PD patients (71.7 ± 7.9 vs. 62.8 ± 7.1 years, p = 0.01)
The mean muscular effort maintained by PD and PSP patients during Cervical vestibular evoked myogenic potentials (VEMPs) (cVEMPs) testing was similar (PD: 86 ± 21 μV; PSP: 87 ± 48 μV)
Summary
While widespread brainstem degeneration is common to both Parkinson disease (PD) and progressive supranuclear palsy (PSP), differences in the resulting motor and non-motor phenotypes likely reflect differential anatomical damage by pathological alpha-synuclein in PD and tau proteins in PSP. Vestibular evoked myogenic potentials (VEMPs), originally designed as a test of peripheral vestibular function, have emerged as a promising neurophysiologic tool to detect brainstem damage in neurodegenerative and other central neurological disorders [1]. Cervical VEMPs (cVEMPs) correspond to the vestibulo-collic reflex and depend on the functional integrity of the medial vestibulospinal tract, linking VIII with ipsilateral XI cranial nerve nuclei [2]. Ocular VEMPs (oVEMPs), on the other hand, assess the functional integrity of the vestibular pathway underlying the vestibulo-ocular reflex, i.e., the medial longitudinal fasciculus linking VIII with contralateral III cranial nerve nuclei [3]. While previous work showed abnormalities in vestibular evoked myogenic potentials (VEMPs) in patients with either disorder as compared to healthy humans, it remains unclear whether ocular and cervical VEMPs differ between PD and PSP patients
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