Post-polio Syndrome: More Than Just a Lower Motor Neuron Disease

Shing, Stacey Li Hi; Chipika, Rangariroyashe H.; Finegan, Eoin; Murray, Deirdre; Hardiman, Orla; Bede, Peter

doi:10.3389/fneur.2019.00773

Cited by 66 publications

(40 citation statements)

References 191 publications

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“…Being aware of getting pain after physical activity also meant that they avoided activities in general. Pain has previously been reported to be common among people with LEoP affecting physical and psychological functioning (15,18,20,27), which was in line with the findings in our study.…”

Section: Discussionsupporting

confidence: 93%

“…Impairments that the participants were least bothered by were breathing difficulties at rest, concentration and memory difficulties, which are impairments that are commonly reported and indirectly associated with LEoP (8). Deductive analysis showed that general fatigue [38], muscle weakness [32] and muscle fatigue [27] had the highest representation with regard to the number of statements [in brackets]. The participants described that increased general fatigue, muscle weakness and muscle fatigue contributed to making daily life more difficult; for example, carrying out daily chores and limited their participation in social activities.…”

Section: Discussionmentioning

confidence: 99%

“…The highest representation with regard to the number of statements [in brackets] was general fatigue [38], muscle weakness [32] and muscle fatigue [27]. During the interviews, none of the participants reported being bothered by sensory disturbances (item 5) or memory difficulties (item 12).…”

Section: Deductive Analysismentioning

confidence: 99%

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Self-reported impairments among people with late effects of polio: a Mixed-methods study

Hammarlund¹,

Lexell²,

Brogårdh³

2020

J Rehabil Med

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The aim of this study was to explore how much people with late effects of polio are bothered by various impairments and their influence on everyday life. Fourteen participants (7 men), mean age 70 years, participated. Face-to-face interviews and the Self-reported Impairments in Persons with late effects of Polio (SIPP) scale were used. In the SIPP, the participants rated how much they were bothered by their impairments, from 1 (not at all) to 4 (extremely). The interview data were coded with regard to the meaning of living with late effects of polio-related impairments. Each quotation was conceptually integrated with the SIPP ratings. Muscle and/ or joint pain during physical activity, muscle weakness and general fatigue were perceived as most bothersome. The impairments, including the less-affected side, negatively influenced daily life, which caused worry and distress. The interviews complemented the SIPP ratings and improved our understanding of how late effects of polio-related impairments influence everyday life. Objective: To determine how much people with late effects of polio are bothered by various impairments and their influence on everyday life. Design: A mixed-methods design. Subjects/patients: Seven women and 7 men (mean age 70 years) with late effects of poli. Methods: Self-reported Impairments in Persons with late effects of Polio (SIPP) scale and face-to-face interviews. In SIPP, the participants rated, from 1 (not at all) to 4 (extremely), how much they had been bothered by late effects of polio-related impairments. Qualitative data were analysed using systematic text condensation. Each quotation was deductively analysed based on its conceptual representation regarding perceived influence on everyday life. Results: Participants were most bothered by muscle and/or joint pain during physical activity, muscle weakness and general fatigue, which corresponded with the number of interview quotations. The impairments negatively influenced daily life, such as household chores, walking, riding a bicycle and social participation. Increased impairments and reduced functioning on the less-affected side also caused worry and distress. Conclusion: Common late effects of polio-related impairments greatly affected participants' activity and participation. By using both the SIPP scale and face-to-face interviews, an increased understanding of how late effects of polio-related impairments influence everyday life was achieved.

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Section: Discussionsupporting

confidence: 93%

Section: Discussionmentioning

confidence: 99%

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Self-reported impairments among people with late effects of polio: a Mixed-methods study

Hammarlund¹,

Lexell²,

Brogårdh³

2020

J Rehabil Med

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“…In clinical practice however, the main differential diagnosis of KD is ALS, which can be readily distinguished from KD due to the more rapid functional decline, the presence of upper motor neuron signs, the absence of sensory neuropathy and endocrine disorders (Table 2). Less likely differential diagnoses include conditions with lower motor neuron presentations, such as Hirayama disease [124], multifocal motor neuropathy, spinal muscular atrophy (SMA) [125], post-polio syndrome (PPS) [126], CIDP, progressive bulbar palsy (PBP) [41], progressive muscular atrophy (PMA), spinal cord ischaemia [92], myelopathies [127] and Charcot-Marie-Tooth disease, but most of these conditions have specific radiological, molecular or electrophysiological features which help to distinguish them from KD. The other differential diagnoses depend on the initial constellation of clinical signs or symptoms which are often extra-neurological (Table 3).…”

Section: Differential Diagnosismentioning

confidence: 99%

The French national protocol for Kennedy’s disease (SBMA): consensus diagnostic and management recommendations

Pradat¹,

Bernard

Corcia

et al. 2020

Orphanet J Rare Dis

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Background Kennedy’s disease (KD), also known as spinal and bulbar muscular atrophy (SBMA), is a rare, adult-onset, X-linked recessive neuromuscular disease caused by CAG expansions in exon 1 of the androgen receptor gene (AR). The objective of the French national diagnostic and management protocol is to provide evidence-based best practice recommendations and outline an optimised care pathway for patients with KD, based on a systematic literature review and consensus multidisciplinary observations. Results The initial evaluation, confirmation of the diagnosis, and management should ideally take place in a tertiary referral centre for motor neuron diseases, and involve an experienced multidisciplinary team of neurologists, endocrinologists, cardiologists and allied healthcare professionals. The diagnosis should be suspected in an adult male presenting with slowly progressive lower motor neuron symptoms, typically affecting the lower limbs at onset. Bulbar involvement (dysarthria and dysphagia) is often a later manifestation of the disease. Gynecomastia is not a constant feature, but is suggestive of a suspected diagnosis, which is further supported by electromyography showing diffuse motor neuron involvement often with asymptomatic sensory changes. A suspected diagnosis is confirmed by genetic testing. The multidisciplinary assessment should ascertain extra-neurological involvement such as cardiac repolarisation abnormalities (Brugada syndrome), signs of androgen resistance, genitourinary abnormalities, endocrine and metabolic changes (glucose intolerance, hyperlipidemia). In the absence of effective disease modifying therapies, the mainstay of management is symptomatic support using rehabilitation strategies (physiotherapy and speech therapy). Nutritional evaluation by an expert dietician is essential, and enteral nutrition (gastrostomy) may be required. Respiratory management centres on the detection and treatment of bronchial obstructions, as well as screening for aspiration pneumonia (chest physiotherapy, drainage, positioning, breath stacking, mechanical insufflation-exsufflation, cough assist machnie, antibiotics). Non-invasive mechanical ventilation is seldom needed. Symptomatic pharmaceutical therapy includes pain management, endocrine and metabolic interventions. There is no evidence for androgen substitution therapy. Conclusion The French national Kennedy’s disease protocol provides management recommendations for patients with KD. In a low-incidence condition, sharing and integrating regional expertise, multidisciplinary experience and defining consensus best-practice recommendations is particularly important. Well-coordinated collaborative efforts will ultimately pave the way to the development of evidence-based international guidelines.

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“…While the methodological focus of the papers differs depending the expertise profile of the authors, there is a cohesive theme among the papers to appraise biological, molecular, electrophysiological, and radiological markers which may potentially serve as pragmatic clinical indicators confirming the diagnosis, predicting the prognosis, detecting response to therapy or track longitudinal neurodegenerative changes. Beyond the practical relevance of ascertaining and quantifying pathological changes in vivo, biomarkers in MND also provide considerable academic insights such as the exploration of presymptomatic changes (23,24), the description of genotype-associated signatures (25,26), the delineation of natural disease trajectories (11,27), the characterization of low-incidence phenotypes (2,28,29), confirmation of epigenetic and epidemiological factors (30,31), and deciphering the pathological substrate of clinical symptoms [Finegan et al; (32,33)].…”

Section: Introductionmentioning

confidence: 99%