Electrophysiological features of lower motor neuron involvement in progressive supranuclear palsy

Gaweł, Malgorzata; Jamrozik, Zygmunt; Szmidt-Salkowska, Elzbieta; Sławek, Jarosław; Gawel, Damian; Rowińska-Marcińska, K; Kamińska, Anna

doi:10.1016/j.jns.2012.10.023

Cited by 8 publications

(8 citation statements)

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“…The study group of Gawel et al conducted one of the few electrophysiological examinations on 24 PSP patients [17]. The only alterations of the ulnar, peroneal and sural nerves could be detected in the form of reduced motor and sensory amplitudes in the ulnar nerve in 8.3% and 20% of the study population.…”

Section: Discussionmentioning

confidence: 99%

“…A causal link between alpha-synuclein and associated peripheral neuropathy remains unclear; however, alpha-synuclein aggregates are distributed throughout the peripheral nervous system, including sympathetic ganglia [14], skin nerve fibers [15] and Schwann cells [16]. Neurophysiological evidence of peripheral neuropathy in PSP is even more scarce [17]. However, cutaneous denervation involving small and large nerve fiber endings in PSP has recently been demonstrated [18].…”

Section: Introductionmentioning

confidence: 99%

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Prevalence and Characteristics of Polyneuropathy in Atypical Parkinsonian Syndromes: An Explorative Study

et al. 2021

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(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.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Prevalence and Characteristics of Polyneuropathy in Atypical Parkinsonian Syndromes: An Explorative Study

et al. 2021

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“…Whether there is a peripheral nerve involvement also in PSP, contributing to autonomic and sensory symptoms, is still unknown. To date, neurophysiological evidence of neuropathy in PSP is rare [10]. Analogously, cutaneous innervation has been only anecdotally studied in PSP patients, as disease control group, to test the specificity of alpha‐synuclein deposits in synucleinopathies [11, 12] and no systematic description of cutaneous innervation exists in the literature.…”

Section: Introductionmentioning

confidence: 99%

Cutaneous sensory and autonomic denervation in progressive supranuclear palsy

Dubbioso

Provitera

Vitale

et al. 2021

Neuropathology Appl Neurobio

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Aim: Progressive Supranuclear Palsy (PSP) is a progressive neurodegenerative tauopathy characterised by motor, behavioural and cognitive dysfunction. While in the last decade, sensory and autonomic disturbances as well as peripheral nerve involvement are wellrecognised in Parkinson's Disease (PD), little is known in this regard for PSP. Herein, we aim to assess peripheral sensory and autonomic nerve involvement in PSP and to characterise possible differences in morpho-functional pattern compared to PD patients. Methods:We studied 27 PSP and 33 PD patients without electrophysiological signs of neuropathy, and 33 healthy controls (HC). In addition to motor impairment, evaluated by means of UPDRS-III and the PSP rating scale, all patients underwent clinical, functional and morphological assessment of sensory-autonomic nerves through dedicated questionnaires, sympathetic skin response, dynamic sweat test and skin biopsies. The analysis of cutaneous sensory and autonomic innervation was performed using indirect immunofluorescence and confocal microscopy.Results: PSP patients displayed a length-dependent loss of sensory and autonomic nerve fibres associated with functional impairment compared to HC and, overall, a more severe picture than in PD patients. The disease severity correlated with the loss of intraepidermal nerve fibre density in the leg of PSP patients (p < 0.05). Conclusion:We demonstrated a length-dependent small fibre pathology in PSP, more severe compared to PD, and paralleling disease severity. Our findings suggest the morphological and functional study of cutaneous nerves as possible biomarkers to monitor disease progression and response to new treatments.

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“…Central motor conduction was impaired in ~40% PSP patients 112 and surface EMG recordings found abnormalities indicating loss of motor neurons in nearly half of the PSP patients examined. 113 Muscles from PSP patients had evidence of chronic reinnervation without signs of acute denervation, suggesting a very slow disease progression. 113 There is increasing evidence that mitochondrial impairments may contribute to oxidative stress and the pathogene-sis of PSP [S83], including decreased alpha-ketoglutarate dehydrogenase complex and aconitase [S84, S85] activities, in the superior frontal cortex and cerebella of PSP patients compared with age-matched controls.…”

Section: Pathogenesis Of Muscle Health Impairments In Progressive Sup...mentioning

confidence: 99%

Impaired skeletal muscle health in Parkinsonian syndromes: clinical implications, mechanisms and potential treatments

Murphy

Lynch

2023

J cachexia sarcopenia muscle

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There is increasing evidence that neurodegenerative disorders including the Parkinsonian syndromes are associated with impaired skeletal muscle health, manifesting as wasting and weakness. Many of the movement problems, lack of muscle strength and reduction in quality of life that are characteristic of these syndromes can be attributed to impairments in skeletal muscle health, but this concept has been grossly understudied and represents an important area of unmet clinical need. This review describes the changes in skeletal muscle health in idiopathic Parkinson's disease and in two atypical Parkinsonian syndromes, the most aggressive synucleinopathy multiple system atrophy, and the tauopathy progressive supranuclear palsy. The pathogenesis of the skeletal muscle changes is described, including the contribution of impairments to the central and peripheral nervous system and intrinsic alterations. Pharmacological interventions targeting the underlying molecular mechanisms with therapeutic potential to improve skeletal muscle health in affected patients are also discussed. Although little is known about the mechanisms underlying these conditions, current evidence implicates multiple pathways and processes, highlighting the likely need for combination therapies to protect muscle health and emphasizing the merit of personalized interventions for patients with different physical capacities at different stages of their disease. As muscle fatigue is often experienced by patients prior to diagnosis, the identification and measurement of this symptom and related biomarkers to identify early signs of disease require careful interrogation, especially for multiple system atrophy and progressive supranuclear palsy where diagnosis is often made several years after onset of symptoms and only confirmed post‐mortem. We propose a multidisciplinary approach for early diagnosis and implementation of personalized interventions to preserve muscle health and improve quality of life for patients with typical and atypical Parkinsonian syndromes.

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Electrophysiological features of lower motor neuron involvement in progressive supranuclear palsy

Cited by 8 publications

References 24 publications

Prevalence and Characteristics of Polyneuropathy in Atypical Parkinsonian Syndromes: An Explorative Study

Prevalence and Characteristics of Polyneuropathy in Atypical Parkinsonian Syndromes: An Explorative Study

Cutaneous sensory and autonomic denervation in progressive supranuclear palsy

Impaired skeletal muscle health in Parkinsonian syndromes: clinical implications, mechanisms and potential treatments

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