The Non-Motor Symptom Profile of Progressive Supranuclear Palsy

Chaithra, Sudhakar Pushpa; Prasad, Shweta; Holla, Vikram V.; Stezin, Albert; Kamble, Nitish; Yadav, Ravi; Pal, Pramod Kumar

doi:10.14802/jmd.19066

Cited by 20 publications

(26 citation statements)

References 29 publications

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“…Thus, PNP symptoms in APS may also be attributable to a central origin. Non-motor symptoms, including autonomic dysfunction and pain dominate the clinical picture of APS [6,41]. The facilitation of nociceptive processing at the spinal level, as demonstrated in advanced PSP patients, as well as the marked involvement of non-dopaminergic central sensory pathways could enhance subjective neuropathic symptom perception in APS [42].…”

Section: Discussionmentioning

confidence: 99%

“…Growing evidence on non-motor symptoms imply the complex multisystemic nature of APS. Non-motor symptoms, including pain, mood, cognition, sleep, gastrointestinal and urogenital disturbances are frequently prevalent and burdensome not only in PD, but also in APS [5,6]. While peripheral nerve involvement has been increasingly recognized in PD [7][8][9][10], only few studies have addressed peripheral nerve alterations in APS thus far.…”

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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“…Fatigue is a disabling symptom in various neurological disorders including Parkinson's disease (PD), and studies showed that fatigue signi cantly affects the patients' quality of life (QoL) [3,4]. Several studies have investigated NMS including fatigue in patients with PSP and reported that sleep/fatigue problems are common in these patients, with a prevalence of 82.9%-100% [2,[5][6][7][8]. However, none of the previous studies used the specialized scale to assess fatigue, and their results were based on the non-motor symptoms scale for PD (NMSS).…”

Section: Introductionmentioning

confidence: 99%

“…8]. Chaithra et al investigated the NMS in patients with PSP using NMSS and reported that sleep/fatigue was the most common NMS in these patients, with a prevalence of 82.9%[7]. Ou et al also investigated the NMS in patients with PSP using NMSS and reported that 100% of these patients (27/27) had sleep/fatigue symptoms[5].…”

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Understanding Fatigue in Progressive Supranuclear Palsy: Prevalence and Associated Factors

Ahn¹,

Song²,

Lee³

et al. 2021

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Background: Fatigue is a common and disabling non-motor symptom (NMS) of Parkinson’s disease (PD); however, it has been poorly understood in patients with progressive supranuclear palsy (PSP). We investigated the association between fatigue, clinical features, and other NMS in patients with probable PSP.Methods: In 72 probable PSP patients, fatigue was investigated using the PD fatigue scale (PFS). Further, all patients were evaluated using the PSP rating scale (PSPRS), Beck Depression Inventory (BDI), Mini-Mental State Examination (MMSE), Frontal Assessment Battery (FAB), PD Sleep Scale (PDSS), NMS scale (NMSS), PD Questionnaire-39 summary index (PDQ-39 SI), and Scale for Outcomes in PD-Autonomic (SCOPA-AUT). Results: The prevalence of fatigue was 38.9% (28/72) in patients with PSP. Logistic regression analysis showed that depression (BDI) was the factor associated with fatigue. We divided the patients into primary (fatigue without depression, n =16), secondary (fatigue with depression, n = 12), and non-fatigue groups. There were no differences in age, sex, disease duration, and PSPRS, PDSS, MMSE, and FAB scores among the three groups. The primary fatigue group had higher scores in PDQ-39 SI compared to the non-fatigue group. The secondary fatigue group showed higher scores in NMSS, PDQ-39 SI, and SCOPA-AUT compared to the non-fatigue group. PFS was positively correlated with NMSS and PDQ-39 SI and SCOPA-AUT.Conclusions: Fatigue is common in patients with PSP and is associated with the NMS and the quality of life in these patients. The present study provides meaningful insight into fatigue in patients with PSP.

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Autonomic dysfunction in progressive supranuclear palsy

et al. 2022

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Background The degree of involvement of the autonomic nervous system in progressive supranuclear palsy (PSP) has been investigated in several studies, often providing conflicting results. There is a need for a better characterization of autonomic dysfunction in PSP, to enhance our understanding of this highly disabling neurodegenerative disease including patients’ needs and possibly be of value for clinicians in the differential diagnosis among Parkinsonian syndromes. Methods We applied a systematic methodology to review existing literature on Pubmed regarding autonomic nervous system involvement in PSP. Results PSP reported quite frequently symptoms suggestive of autonomic dysfunction in all domains. Cardiovascular autonomic testing showed in some cases a certain degree of impairment (never severe). There was some evidence suggesting bladder dysfunction particularly in the storage phase. Dysphagia and constipation were the most common gastrointestinal symptoms. Instrumental tests seemed to confirm sudomotor and pupillomotor disturbances. Conclusions PSP patients frequently reported visceral symptoms, however objective testing showed that not always these reflected actual autonomic impairment. Further studies are needed to better delineate autonomic profile and its prognostic role in PSP.

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The Non-Motor Symptom Profile of Progressive Supranuclear Palsy

Cited by 20 publications

References 29 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

Understanding Fatigue in Progressive Supranuclear Palsy: Prevalence and Associated Factors

Autonomic dysfunction in progressive supranuclear palsy

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