Restricted disease propagation in multiple system atrophy with prolonged survival

McCann, Heather; McGeachie, Andrew B.; Silberstein, Paul; Lewis, Simon J.G.; Halliday, Glenda M.

doi:10.1111/nan.12195

Cited by 5 publications

(3 citation statements)

References 20 publications

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“…The average survival from onset for an MSA patient is around 7–9 years 4. However, a pathologically confirmed case series of a ‘benign’ MSA variant found survival of >15 years17 and the longest reported survival of a neuropathologically confirmed MSA-P case is 23 years from onset 18. Conversely, there is a report of an aggressive MSA phenotype with a very short disease duration of <3 years 19.…”

Section: Diagnosing Msamentioning

confidence: 99%

“… 4 However, a pathologically confirmed case series of a ‘benign’ MSA variant found survival of >15 years 17 and the longest reported survival of a neuropathologically confirmed MSA-P case is 23 years from onset. 18 Conversely, there is a report of an aggressive MSA phenotype with a very short disease duration of <3 years. 19 Respiratory and urinary tract infections are common causes of death, together with sudden death (probably from central apnoea, epiglottal airway obstruction and/or cardiac dysautonomia).…”

Section: Diagnosing Msamentioning

confidence: 99%

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Multiple system atrophy

et al. 2023

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This is a practical guide to diagnosing and managing multiple system atrophy (MSA). We explain the newly published Movement Disorders Society Consensus Diagnostic Criteria, which include new ‘Clinically Established MSA’ and ‘Possible Prodromal MSA’ categories, hopefully reducing time to diagnosis. We then highlight the key clinical features of MSA to aid diagnosis. We include a list of MSA mimics with suggested methods of differentiation from MSA. Lastly, we discuss practical symptom management in people living with MSA, including balancing side effects, with the ultimate aim of improving quality of life.

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Section: Diagnosing Msamentioning

confidence: 99%

Section: Diagnosing Msamentioning

confidence: 99%

Multiple system atrophy

et al. 2023

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“…Two or more of six red flags (warning signs) had a specificity of 98.3% and a sensitivity of 84.2% 14 . Several subtypes, e.g., "minimal-change" aggressive and prolonged "benign" forms do not fit into the current classification 10,[15][16][17][18][19] . Due to overlapping clinical presentation, the distinction between MSA, early disease PD or atypical parkinsonian disorders (DLB, progressive supranuclear palsy/PSP) may be difficult [20][21][22] .…”

Section: Introductionmentioning

confidence: 96%

Recent advances in multiple system atrophy

Jellinger¹

2016

J Neurol Neuromedicine

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Multiple system atrophy (MSA) is a fatal orphan neurodegenerative disorder that manifests with autonomic, parkinsonian, cerebellar, and pyramidal features. It is characterized by the accumulation of misfolded α-synuclein (αSyn) in oligodendroglia and neurons, affecting multiple parts of the central, autonomic and perípheral system. Both the etiology and pathogenesis of MSA are unknown, although a genetic component has been proposed. Accumulation of aberrant αSyn in oligodendrocytes, preceded by relocation of p25α protein from myelin into oligodendroglia, results in the formation of insoluble glial cytoplasmic inclusions (GCIs). These changes are associated with proteasomal, mitochondrial and lipid transport dysfunction, oxidative stress, reduced trophic transport, neuroinflammation and other noxious factors. Their interaction induces dysfunction of the oligodendroglial-myelin-axon-neuron complex, resulting in the system-specific pattern of neurodegeneration characterizing MSA as a synucleinopathy with oligodendroglio-neuronopathy. Propagation of modified toxic αSyn species from neurons to oligodendroglia by "prion-like" transfer and its spreading to associate neuronal pathways result in multi-system involvement. No reliable biomarkers are currently available for the clinical diagnosis and prognosis of MSA and neither effective neuroprotective nor disease-modifying therapies of MSA are available, although novel treatment strategies targeting αSyn are under discussion. Multidisciplinary research to elucidate the genetic and molecular background of the deleterious cycle of noxious processes to develop reliable diagnostic biomarkers and to deliver targets for effective treatment of this hitherto incurable disorder is urgently needed.

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Multiple system atrophy: pathogenic mechanisms and biomarkers

Jellinger¹,

Wenning

2016

J Neural Transm

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Multiple system atrophy (MSA) is a unique proteinopathy that differs from other α-synucleinopathies since the pathological process resulting from accumulation of aberrant α-synuclein (αSyn) involves the oligodendroglia rather than neurons, although both pathologies affect multiple parts of the brain, spinal cord, autonomic and peripheral nervous system. Both the etiology and pathogenesis of MSA are unknown, although animal models have provided insight into the basic molecular changes of this disorder. Accumulation of aberrant αSyn in oligodendroglial cells and preceded by relocation of p25α protein from myelin to oligodendroglia results in the formation of insoluble glial cytoplasmic inclusions that cause cell dysfunction and demise. These changes are associated with proteasomal, mitochondrial and lipid transport dysfunction, oxidative stress, reduced trophic transport, neuroinflammation and other noxious factors. Their complex interaction induces dysfunction of the oligodendroglial-myelin-axon-neuron complex, resulting in the system-specific pattern of neurodegeneration characterizing MSA as a synucleinopathy with oligodendroglio-neuronopathy. Propagation of modified toxic αSyn species from neurons to oligodendroglia by "prion-like" transfer and its spreading associated with neuronal pathways result in a multi-system involvement. No reliable biomarkers are currently available for the clinical diagnosis and prognosis of MSA. Multidisciplinary research to elucidate the genetic and molecular background of the deleterious cycle of noxious processes, to develop reliable diagnostic biomarkers and to deliver targets for effective treatment of this hitherto incurable disorder is urgently needed.

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Restricted disease propagation in multiple system atrophy with prolonged survival

Cited by 5 publications

References 20 publications

Multiple system atrophy

Multiple system atrophy

Recent advances in multiple system atrophy

Multiple system atrophy: pathogenic mechanisms and biomarkers

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