Abstract
(1) Background: Peripheral nerve involvement is increasingly recognized in Parkinson’s disease (PD). Although non-motor symptoms and postural instability are early features of atypical parkinsonian syndromes (APS), peripheral neuropathies in APS have not been addressed in detail thus far. Therefore, the aim of this study was to investigate the prevalence and characteristics of polyneuropathies (PNP) in multiple system atrophy (MSA) and progressive supranuclear palsy (PSP), as representative syndromes of APS. (2) Methods: In total, 8 MSA and 6 PSP patients were comprehensively analyzed regarding subjective, clinical (motor and non-motor) and paraclinical PNP features using nerve conduction studies and high resolution nerve ultrasounds (HRUS). (3) Results: A total of 87.5% of MSA and 66.7% of PSP patients complained of at least one neuropathic symptom, with electrophysiological confirmation of PNP in 50.0% of both, MSA and PSP patients. PNP symptom severity in PSP and motor nerve amplitude in MSA were associated with compromised motor function. Morphologic nerve examination by HRUS showed few alterations according to the axonal type of PNP. (4) Conclusions: The overall high PNP symptom burden may be partially credited to the significant prevalence of electrophysiologically diagnosed PNP, and impact motor aspects of APS. The findings of this exploratory study reinforce further investigations on a larger scale, in order to elucidate peripheral nerve involvement and the underlying pathophysiological mechanisms of APS.
Highlights
Atypical parkinsonian syndromes (APS) are neurodegenerative disorders which present with additional features beyond parkinsonism, often associated with an insufficient response to levodopa and a more rapid disease course
Demographic amndodCelriantiec,asleDnsaotraimotor PNP, and one patient fulfilled the criteria for a severe, sensorimotor patients, subdividTewdo ipnatotiegnrtsouwpesrewciatthegaonridzewd iitnhtoouat ma iledle, csternospohryysPioNlPo,gaincadl odnieagpnaotiseinst ofuf lfilled the PNP
While Neuropathy Symptom Score (NSS) in Parkinson’s disease (PD) patients significantly correlated with motor, non-motor symptoms as well as poor quality of life in our study, we found a significant correlation between NSS and UPDRS part III in progressive supranuclear palsy (PSP) patients, including within the subscales of midline function and lower limb bradykinesia
Summary
Atypical parkinsonian syndromes (APS) are neurodegenerative disorders which present with additional features beyond parkinsonism, often associated with an insufficient response to levodopa and a more rapid disease course. Based on the pathogenic protein aggregates, multiple system atrophy (MSA) belongs to the spectrum of synucleinopathies alongside Parkinson’s disease (PD) and dementia with Lewy bodies (DLB), whereas progressive supranuclear palsy (PSP) and corticobasal degeneration (CBD) represent tauopathies. MSA is characterized by a variable combination of parkinsonism, cerebellar ataxia and autonomic failure, involving urinary incontinence, erectile dysfunction and orthostatic hypotension. The accumulation of alpha-synuclein in the cytoplasm of oligodendrocytes (glial cytoplasmic inclusions, GCI) in striatonigral and olivopontocerebellar structures presents as the pathological hallmark [1,2]. PSP manifests with multiple phenotypic variants, of which core symptoms consist of ocular motor dysfunction, postural instability, akinesia and cognitive impairment [3]. The clinical heterogeneity arises from the regional distribution of the 4-repeat tau protein deposition predominantly in the basal ganglia and brain stem [4]
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