Materiały źródłowe

• #1 Multiple system atrophy

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10314081/

  Many terms have previously been used to describe this condition. Graham and Oppenheimer introduced MSA in 1969 to encompass multiple neurological entities such as olivopontocerebellar atrophy, striatonigral degeneration and Shy-Drager syndrome. […] Occurs worldwide. Incidence: 0.63 per 100000 people per year. Prevalence: 1.94.9 per 100000 people. Sexes equally affected. Onset typically in sixth decade. Mean age of onset 569 years. Relative frequency of motor subtypes differs by geographical and ethnic regions: MSA-cerebellar more in Japanese, Korean and Mestizo populations (70%80%). MSA-parkinsonian more in European and North American populations (67%84%). […] The average survival from onset for an MSA patient is around 79 years. However, a pathologically confirmed case series of a benign MSA variant found survival of 15 years and the longest reported survival of a neuropathologically confirmed MSA-P case is 23 years from onset. Conversely, there is a report of an aggressive MSA phenotype with a very short disease duration of 3 years.

• #2 Multiple system atrophy | Practical Neurology

  https://pn.bmj.com/content/23/3/208

  Multiple system atrophy (MSA) is a sporadic, progressive, adult-onset (>30 years), degenerative disease that presents with a combination of parkinsonism, cerebellar and autonomic dysfunction. Its diagnosis is challenging and postmortem studies show accuracy rates of only 62%–79%. Delayed diagnosis is common, with an average of 3.8 years from symptom onset to diagnosis. As average survival is only 7–9 years, early diagnosis is crucial for patient quality of life and effective management. […] […] Worldwide epidemiology of multiple system atrophy (MSA) occurs worldwide. Incidence: 0.6–3 per 100,000 people per year. Prevalence: 1.9–4.9 per 100,000 people. Sexes equally affected. Onset typically in sixth decade. Mean age of onset 56±9 years. Relative frequency of motor subtypes differs by geographical and ethnic regions: MSA-cerebellar more in Japanese, Korean and Mestizo populations (70%–80%). MSA-parkinsonian more in European and North American populations (67%–84%).

• #3 Multiple System Atrophy: Practice Essentials, Background, Etiology and Pathophysiology

  https://emedicine.medscape.com/article/1154583-overview

  The prevalence of MSA is reported to be between 3.4-4.9 cases per 100,000 population. The estimated mean incidence is 0.6-0.7 cases per 100,000 person-years. MSA meets orphan disease status. […] Many patients do not receive the correct diagnosis during their lifetime because of the difficulty in differentiating MSA from other disorders (eg, Parkinson disease, pure autonomic failure [PAF], other rare movement disorders). About 29-33% of patients with isolated late-onset cerebellar ataxia and 8-10% of patients with parkinsonism will develop MSA. Therefore, a higher prevalence than that estimated can be assumed. […] In the European Union (EU), the prevalence rates show 4-5 cases per 100,000 persons. The incidence rate is about 0.6 cases per 100,000 persons per year. […] In the United Kingdom, the crude prevalence of MSA, including all probable and possible cases, is 3.3 per 100,000 population.

• #4 Multiple system atrophy | Practical Neurology

  https://pn.bmj.com/content/23/3/208

  Multiple system atrophy (MSA) is a sporadic, progressive, adult-onset (>30 years), degenerative disease that presents with a combination of parkinsonism, cerebellar and autonomic dysfunction. Its diagnosis is challenging and postmortem studies show accuracy rates of only 62%–79%. Delayed diagnosis is common, with an average of 3.8 years from symptom onset to diagnosis. As average survival is only 7–9 years, early diagnosis is crucial for patient quality of life and effective management. […] […] Worldwide epidemiology of multiple system atrophy (MSA) occurs worldwide. Incidence: 0.6–3 per 100,000 people per year. Prevalence: 1.9–4.9 per 100,000 people. Sexes equally affected. Onset typically in sixth decade. Mean age of onset 56±9 years. Relative frequency of motor subtypes differs by geographical and ethnic regions: MSA-cerebellar more in Japanese, Korean and Mestizo populations (70%–80%). MSA-parkinsonian more in European and North American populations (67%–84%).

• #5 Estimating the prevalence and incidence of multiple system atrophy in the USA: Insights from a national claims database – PubMed

  https://pubmed.ncbi.nlm.nih.gov/37951144/

  Published literature on the prevalence and incidence of multiple system atrophy (MSA) in the US are scarce or based on relatively older studies. The objective of this study was to estimate the prevalence and cumulative incidence of MSA in the US. […] The crude prevalence of MSA was 7.2 per 100,000 persons in 2021 and increased with age. After standardization to the US population, the age-adjusted prevalence was 12.4 per 100,000 translating to 41,133 persons in the 2021 US population. […] The crude cumulative incidence of MSA for individuals aged 30 years and older was 9.8 per 100,000 persons in 2021. The estimated cumulative incidence of MSA in individuals 30 years or older, age-adjusted to the 2021 U S. population, was 14.2 per 100,000. […] This study provides real-world evidence on the prevalence and cumulative incidence of MSA in the US to better understand the medical care needs and treatment.

• #6 Multiple system atrophy

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10314081/

  Many terms have previously been used to describe this condition. Graham and Oppenheimer introduced MSA in 1969 to encompass multiple neurological entities such as olivopontocerebellar atrophy, striatonigral degeneration and Shy-Drager syndrome. […] Occurs worldwide. Incidence: 0.63 per 100000 people per year. Prevalence: 1.94.9 per 100000 people. Sexes equally affected. Onset typically in sixth decade. Mean age of onset 569 years. Relative frequency of motor subtypes differs by geographical and ethnic regions: MSA-cerebellar more in Japanese, Korean and Mestizo populations (70%80%). MSA-parkinsonian more in European and North American populations (67%84%). […] The average survival from onset for an MSA patient is around 79 years. However, a pathologically confirmed case series of a benign MSA variant found survival of 15 years and the longest reported survival of a neuropathologically confirmed MSA-P case is 23 years from onset. Conversely, there is a report of an aggressive MSA phenotype with a very short disease duration of 3 years.

• #7 Multiple System Atrophy: Practice Essentials, Background, Etiology and Pathophysiology

  https://emedicine.medscape.com/article/1154583-overview

  The prevalence of MSA is reported to be between 3.4-4.9 cases per 100,000 population. The estimated mean incidence is 0.6-0.7 cases per 100,000 person-years. MSA meets orphan disease status. […] Many patients do not receive the correct diagnosis during their lifetime because of the difficulty in differentiating MSA from other disorders (eg, Parkinson disease, pure autonomic failure [PAF], other rare movement disorders). About 29-33% of patients with isolated late-onset cerebellar ataxia and 8-10% of patients with parkinsonism will develop MSA. Therefore, a higher prevalence than that estimated can be assumed. […] In the European Union (EU), the prevalence rates show 4-5 cases per 100,000 persons. The incidence rate is about 0.6 cases per 100,000 persons per year. […] In the United Kingdom, the crude prevalence of MSA, including all probable and possible cases, is 3.3 per 100,000 population.

• #8 Multiple System Atrophy: Practice Essentials, Background, Etiology and Pathophysiology

  https://emedicine.medscape.com/article/1154583-overview

  The prevalence of MSA is reported to be between 3.4-4.9 cases per 100,000 population. The estimated mean incidence is 0.6-0.7 cases per 100,000 person-years. MSA meets orphan disease status. […] Many patients do not receive the correct diagnosis during their lifetime because of the difficulty in differentiating MSA from other disorders (eg, Parkinson disease, pure autonomic failure [PAF], other rare movement disorders). About 29-33% of patients with isolated late-onset cerebellar ataxia and 8-10% of patients with parkinsonism will develop MSA. Therefore, a higher prevalence than that estimated can be assumed. […] In the European Union (EU), the prevalence rates show 4-5 cases per 100,000 persons. The incidence rate is about 0.6 cases per 100,000 persons per year. […] In the United Kingdom, the crude prevalence of MSA, including all probable and possible cases, is 3.3 per 100,000 population.

• #9 Multiple System Atrophy: Practice Essentials, Background, Etiology and Pathophysiology

  https://emedicine.medscape.com/article/1154583-overview

  In Iceland, the incidence is 0.6 per 100,000 and prevalence is 3.1 per 100,000. […] In Japan, the prevalence is 13.1 per 100,000 individuals. The mean annual incidence is 0.68. […] MSA has been encountered in Caucasian, African, and Asian populations. In Western countries, MSA-P predominates, occurring in 66-82% of patients. In Eastern countries (e.g., Japan), MSA-C is common, occurring in 67% of patients. […] The disease more often affects men than women. The female-to-male ratio is around 1:2. (A ratio of 1:3-9 has also been reported.) However, the early and easy diagnosis of impotence may have led to the male statistical predominance of MSA. The mean age at onset in MSA is 52.5-55 years. The disease progresses over intervals of 1-18 years.

• #10 Multiple System Atrophy: Practice Essentials, Background, Etiology and Pathophysiology

  https://emedicine.medscape.com/article/1154583-overview

  In Iceland, the incidence is 0.6 per 100,000 and prevalence is 3.1 per 100,000. […] In Japan, the prevalence is 13.1 per 100,000 individuals. The mean annual incidence is 0.68. […] MSA has been encountered in Caucasian, African, and Asian populations. In Western countries, MSA-P predominates, occurring in 66-82% of patients. In Eastern countries (e.g., Japan), MSA-C is common, occurring in 67% of patients. […] The disease more often affects men than women. The female-to-male ratio is around 1:2. (A ratio of 1:3-9 has also been reported.) However, the early and easy diagnosis of impotence may have led to the male statistical predominance of MSA. The mean age at onset in MSA is 52.5-55 years. The disease progresses over intervals of 1-18 years.

• #11

  https://link.springer.com/article/10.1007/s00415-024-12269-5

  Multiple system atrophy is a rare disease that could potentially affect the people of all racial groups without gender preference. According to a 10-year nationwide epidemiological study in Iceland, the incidence of MSA was estimated to be approximately 0.7 per 100,000 people. According to a 15-year study conducted in Olmsted County, Minnesota, there were no one who developed MSA before age 50; however, the average annual incidence rate of MSA was 3.0 per 100,000 people. In addition, studies from northern Sweden over a 4-year period and from Russia over a 2-year period found distinct frequencies of 2.1 and 0.1 per 100,000 people, respectively. This suggests that the incidence of MSA is influenced to some degree by region and age. Other studies estimate that the crude prevalence rates range from 1.9 in Gironde, 3.4 in Iceland, and 4.4 in London per 100,000 population.

• #12

  https://link.springer.com/article/10.1007/s00415-024-12269-5

  Multiple system atrophy is a rare disease that could potentially affect the people of all racial groups without gender preference. According to a 10-year nationwide epidemiological study in Iceland, the incidence of MSA was estimated to be approximately 0.7 per 100,000 people. According to a 15-year study conducted in Olmsted County, Minnesota, there were no one who developed MSA before age 50; however, the average annual incidence rate of MSA was 3.0 per 100,000 people. In addition, studies from northern Sweden over a 4-year period and from Russia over a 2-year period found distinct frequencies of 2.1 and 0.1 per 100,000 people, respectively. This suggests that the incidence of MSA is influenced to some degree by region and age. Other studies estimate that the crude prevalence rates range from 1.9 in Gironde, 3.4 in Iceland, and 4.4 in London per 100,000 population.

• #13

  https://link.springer.com/article/10.1007/s00415-024-12269-5

  Multiple system atrophy is a rare disease that could potentially affect the people of all racial groups without gender preference. According to a 10-year nationwide epidemiological study in Iceland, the incidence of MSA was estimated to be approximately 0.7 per 100,000 people. According to a 15-year study conducted in Olmsted County, Minnesota, there were no one who developed MSA before age 50; however, the average annual incidence rate of MSA was 3.0 per 100,000 people. In addition, studies from northern Sweden over a 4-year period and from Russia over a 2-year period found distinct frequencies of 2.1 and 0.1 per 100,000 people, respectively. This suggests that the incidence of MSA is influenced to some degree by region and age. Other studies estimate that the crude prevalence rates range from 1.9 in Gironde, 3.4 in Iceland, and 4.4 in London per 100,000 population.

• #14

  https://link.springer.com/article/10.1007/s00415-024-12561-4

  On 31 December 2021, 16 patients were alive giving a crude point prevalence of 2.43 cases per 100,000 inhabitants: 1.2 in women and 3.69 in men. […] Total incident cases were 32 and the crude average annual incidence rate (IR) was 0.49 per 100,000 person-years in the period 20122021. […] The highest incidence occurred in the age group of 6069 years, with an IR of 2.0. […] Joinpoint analysis for global incidence and prevalence experienced stable annual rates during the whole period, showing an upward trend for prevalence without a statistically significant slop. […] The median duration from onset of symptom to diagnosis was 36 months. […] The median of survival from clinical symptom onset was 84 months and from clinical diagnosis was 42 months. […] Our study provides a detailed description of age, sex-specific and age-adjusted prevalence, and incidence of MSA in Navarre and represents the first population-based epidemiological study on MSA performed in Spain.

• #15 Multiple system atrophy

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10314081/

  Many terms have previously been used to describe this condition. Graham and Oppenheimer introduced MSA in 1969 to encompass multiple neurological entities such as olivopontocerebellar atrophy, striatonigral degeneration and Shy-Drager syndrome. […] Occurs worldwide. Incidence: 0.63 per 100000 people per year. Prevalence: 1.94.9 per 100000 people. Sexes equally affected. Onset typically in sixth decade. Mean age of onset 569 years. Relative frequency of motor subtypes differs by geographical and ethnic regions: MSA-cerebellar more in Japanese, Korean and Mestizo populations (70%80%). MSA-parkinsonian more in European and North American populations (67%84%). […] The average survival from onset for an MSA patient is around 79 years. However, a pathologically confirmed case series of a benign MSA variant found survival of 15 years and the longest reported survival of a neuropathologically confirmed MSA-P case is 23 years from onset. Conversely, there is a report of an aggressive MSA phenotype with a very short disease duration of 3 years.

• #16 Multiple system atrophy | Practical Neurology

  https://pn.bmj.com/content/23/3/208

  Multiple system atrophy (MSA) is a sporadic, progressive, adult-onset (>30 years), degenerative disease that presents with a combination of parkinsonism, cerebellar and autonomic dysfunction. Its diagnosis is challenging and postmortem studies show accuracy rates of only 62%–79%. Delayed diagnosis is common, with an average of 3.8 years from symptom onset to diagnosis. As average survival is only 7–9 years, early diagnosis is crucial for patient quality of life and effective management. […] […] Worldwide epidemiology of multiple system atrophy (MSA) occurs worldwide. Incidence: 0.6–3 per 100,000 people per year. Prevalence: 1.9–4.9 per 100,000 people. Sexes equally affected. Onset typically in sixth decade. Mean age of onset 56±9 years. Relative frequency of motor subtypes differs by geographical and ethnic regions: MSA-cerebellar more in Japanese, Korean and Mestizo populations (70%–80%). MSA-parkinsonian more in European and North American populations (67%–84%).

• #17

  https://link.springer.com/article/10.1007/s00415-024-12561-4

  On 31 December 2021, 16 patients were alive giving a crude point prevalence of 2.43 cases per 100,000 inhabitants: 1.2 in women and 3.69 in men. […] Total incident cases were 32 and the crude average annual incidence rate (IR) was 0.49 per 100,000 person-years in the period 20122021. […] The highest incidence occurred in the age group of 6069 years, with an IR of 2.0. […] Joinpoint analysis for global incidence and prevalence experienced stable annual rates during the whole period, showing an upward trend for prevalence without a statistically significant slop. […] The median duration from onset of symptom to diagnosis was 36 months. […] The median of survival from clinical symptom onset was 84 months and from clinical diagnosis was 42 months. […] Our study provides a detailed description of age, sex-specific and age-adjusted prevalence, and incidence of MSA in Navarre and represents the first population-based epidemiological study on MSA performed in Spain.

• #18 Multiple system atrophy

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10314081/

  Many terms have previously been used to describe this condition. Graham and Oppenheimer introduced MSA in 1969 to encompass multiple neurological entities such as olivopontocerebellar atrophy, striatonigral degeneration and Shy-Drager syndrome. […] Occurs worldwide. Incidence: 0.63 per 100000 people per year. Prevalence: 1.94.9 per 100000 people. Sexes equally affected. Onset typically in sixth decade. Mean age of onset 569 years. Relative frequency of motor subtypes differs by geographical and ethnic regions: MSA-cerebellar more in Japanese, Korean and Mestizo populations (70%80%). MSA-parkinsonian more in European and North American populations (67%84%). […] The average survival from onset for an MSA patient is around 79 years. However, a pathologically confirmed case series of a benign MSA variant found survival of 15 years and the longest reported survival of a neuropathologically confirmed MSA-P case is 23 years from onset. Conversely, there is a report of an aggressive MSA phenotype with a very short disease duration of 3 years.

• #19 Orphanet: Multiple system atrophy

  https://www.orpha.net/en/disease/detail/102

  Multiple system atrophy (MSA) is a neurodegenerative disorder characterized by autonomic failure (cardiovascular and/or urinary), parkinsonism, cerebellar impairment and corticospinal signs with a median survival of 6-9 years. […] Prevalence ranges from 1/50,000-1/20,000. MSA-parkinsonian type (MSA-p) predominates in the Western Hemisphere and MSA-cerebellar type (MSA-c) predominates in the Eastern Hemisphere. Genders are equally distributed. […] MSA is rapidly progressive and is associated with wheelchair dependence, unintelligible speech, intermittent urinary catheterization, disabling orthostatic hypotension, and cognitive impairment (executive dysfunction). Disease progression is assessed using the unified MSA rating scale (UMSARS), which rates activities of daily life, autonomic and motor impairment, as well as overall disability. Prognosis is poor with a median survival of 6-9 years.

• #20 Multiple System Atrophy: Essential Facts for Patients

  https://www.movementdisorders.org/MDS/Resources/Patient-Education/Multiple-System-Atrophy.htm

  MSA is considered rare and affects around three to four people in every 100,000. […] MSA affects men and women equally. MSA usually begins between the age of 50 and 60.

• #21 Multiple System Atrophy: Practice Essentials, Background, Etiology and Pathophysiology

  https://emedicine.medscape.com/article/1154583-overview

  In Iceland, the incidence is 0.6 per 100,000 and prevalence is 3.1 per 100,000. […] In Japan, the prevalence is 13.1 per 100,000 individuals. The mean annual incidence is 0.68. […] MSA has been encountered in Caucasian, African, and Asian populations. In Western countries, MSA-P predominates, occurring in 66-82% of patients. In Eastern countries (e.g., Japan), MSA-C is common, occurring in 67% of patients. […] The disease more often affects men than women. The female-to-male ratio is around 1:2. (A ratio of 1:3-9 has also been reported.) However, the early and easy diagnosis of impotence may have led to the male statistical predominance of MSA. The mean age at onset in MSA is 52.5-55 years. The disease progresses over intervals of 1-18 years.

• #22

  https://link.springer.com/article/10.1007/s00415-024-12561-4

  On 31 December 2021, 16 patients were alive giving a crude point prevalence of 2.43 cases per 100,000 inhabitants: 1.2 in women and 3.69 in men. […] Total incident cases were 32 and the crude average annual incidence rate (IR) was 0.49 per 100,000 person-years in the period 20122021. […] The highest incidence occurred in the age group of 6069 years, with an IR of 2.0. […] Joinpoint analysis for global incidence and prevalence experienced stable annual rates during the whole period, showing an upward trend for prevalence without a statistically significant slop. […] The median duration from onset of symptom to diagnosis was 36 months. […] The median of survival from clinical symptom onset was 84 months and from clinical diagnosis was 42 months. […] Our study provides a detailed description of age, sex-specific and age-adjusted prevalence, and incidence of MSA in Navarre and represents the first population-based epidemiological study on MSA performed in Spain.

• #23 Multiple System Atrophy: Practice Essentials, Background, Etiology and Pathophysiology

  https://emedicine.medscape.com/article/1154583-overview

  In Iceland, the incidence is 0.6 per 100,000 and prevalence is 3.1 per 100,000. […] In Japan, the prevalence is 13.1 per 100,000 individuals. The mean annual incidence is 0.68. […] MSA has been encountered in Caucasian, African, and Asian populations. In Western countries, MSA-P predominates, occurring in 66-82% of patients. In Eastern countries (e.g., Japan), MSA-C is common, occurring in 67% of patients. […] The disease more often affects men than women. The female-to-male ratio is around 1:2. (A ratio of 1:3-9 has also been reported.) However, the early and easy diagnosis of impotence may have led to the male statistical predominance of MSA. The mean age at onset in MSA is 52.5-55 years. The disease progresses over intervals of 1-18 years.

• #24 Multiple system atrophy

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10314081/

  Many terms have previously been used to describe this condition. Graham and Oppenheimer introduced MSA in 1969 to encompass multiple neurological entities such as olivopontocerebellar atrophy, striatonigral degeneration and Shy-Drager syndrome. […] Occurs worldwide. Incidence: 0.63 per 100000 people per year. Prevalence: 1.94.9 per 100000 people. Sexes equally affected. Onset typically in sixth decade. Mean age of onset 569 years. Relative frequency of motor subtypes differs by geographical and ethnic regions: MSA-cerebellar more in Japanese, Korean and Mestizo populations (70%80%). MSA-parkinsonian more in European and North American populations (67%84%). […] The average survival from onset for an MSA patient is around 79 years. However, a pathologically confirmed case series of a benign MSA variant found survival of 15 years and the longest reported survival of a neuropathologically confirmed MSA-P case is 23 years from onset. Conversely, there is a report of an aggressive MSA phenotype with a very short disease duration of 3 years.

• #25 Multiple system atrophy | Practical Neurology

  https://pn.bmj.com/content/23/3/208

  Multiple system atrophy (MSA) is a sporadic, progressive, adult-onset (>30 years), degenerative disease that presents with a combination of parkinsonism, cerebellar and autonomic dysfunction. Its diagnosis is challenging and postmortem studies show accuracy rates of only 62%–79%. Delayed diagnosis is common, with an average of 3.8 years from symptom onset to diagnosis. As average survival is only 7–9 years, early diagnosis is crucial for patient quality of life and effective management. […] […] Worldwide epidemiology of multiple system atrophy (MSA) occurs worldwide. Incidence: 0.6–3 per 100,000 people per year. Prevalence: 1.9–4.9 per 100,000 people. Sexes equally affected. Onset typically in sixth decade. Mean age of onset 56±9 years. Relative frequency of motor subtypes differs by geographical and ethnic regions: MSA-cerebellar more in Japanese, Korean and Mestizo populations (70%–80%). MSA-parkinsonian more in European and North American populations (67%–84%).

• #26 Multiple system atrophy

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10314081/

  Many terms have previously been used to describe this condition. Graham and Oppenheimer introduced MSA in 1969 to encompass multiple neurological entities such as olivopontocerebellar atrophy, striatonigral degeneration and Shy-Drager syndrome. […] Occurs worldwide. Incidence: 0.63 per 100000 people per year. Prevalence: 1.94.9 per 100000 people. Sexes equally affected. Onset typically in sixth decade. Mean age of onset 569 years. Relative frequency of motor subtypes differs by geographical and ethnic regions: MSA-cerebellar more in Japanese, Korean and Mestizo populations (70%80%). MSA-parkinsonian more in European and North American populations (67%84%). […] The average survival from onset for an MSA patient is around 79 years. However, a pathologically confirmed case series of a benign MSA variant found survival of 15 years and the longest reported survival of a neuropathologically confirmed MSA-P case is 23 years from onset. Conversely, there is a report of an aggressive MSA phenotype with a very short disease duration of 3 years.

• #27 Multiple system atrophy | Practical Neurology

  https://pn.bmj.com/content/23/3/208

  Multiple system atrophy (MSA) is a sporadic, progressive, adult-onset (>30 years), degenerative disease that presents with a combination of parkinsonism, cerebellar and autonomic dysfunction. Its diagnosis is challenging and postmortem studies show accuracy rates of only 62%–79%. Delayed diagnosis is common, with an average of 3.8 years from symptom onset to diagnosis. As average survival is only 7–9 years, early diagnosis is crucial for patient quality of life and effective management. […] […] Worldwide epidemiology of multiple system atrophy (MSA) occurs worldwide. Incidence: 0.6–3 per 100,000 people per year. Prevalence: 1.9–4.9 per 100,000 people. Sexes equally affected. Onset typically in sixth decade. Mean age of onset 56±9 years. Relative frequency of motor subtypes differs by geographical and ethnic regions: MSA-cerebellar more in Japanese, Korean and Mestizo populations (70%–80%). MSA-parkinsonian more in European and North American populations (67%–84%).

• #28 Multiple System Atrophy: Practice Essentials, Background, Etiology and Pathophysiology

  https://emedicine.medscape.com/article/1154583-overview

  In Iceland, the incidence is 0.6 per 100,000 and prevalence is 3.1 per 100,000. […] In Japan, the prevalence is 13.1 per 100,000 individuals. The mean annual incidence is 0.68. […] MSA has been encountered in Caucasian, African, and Asian populations. In Western countries, MSA-P predominates, occurring in 66-82% of patients. In Eastern countries (e.g., Japan), MSA-C is common, occurring in 67% of patients. […] The disease more often affects men than women. The female-to-male ratio is around 1:2. (A ratio of 1:3-9 has also been reported.) However, the early and easy diagnosis of impotence may have led to the male statistical predominance of MSA. The mean age at onset in MSA is 52.5-55 years. The disease progresses over intervals of 1-18 years.

• #29 Multiple System Atrophy: Practice Essentials, Background, Etiology and Pathophysiology

  https://emedicine.medscape.com/article/1154583-overview

  The prevalence of MSA is reported to be between 3.4-4.9 cases per 100,000 population. The estimated mean incidence is 0.6-0.7 cases per 100,000 person-years. MSA meets orphan disease status. […] Many patients do not receive the correct diagnosis during their lifetime because of the difficulty in differentiating MSA from other disorders (eg, Parkinson disease, pure autonomic failure [PAF], other rare movement disorders). About 29-33% of patients with isolated late-onset cerebellar ataxia and 8-10% of patients with parkinsonism will develop MSA. Therefore, a higher prevalence than that estimated can be assumed. […] In the European Union (EU), the prevalence rates show 4-5 cases per 100,000 persons. The incidence rate is about 0.6 cases per 100,000 persons per year. […] In the United Kingdom, the crude prevalence of MSA, including all probable and possible cases, is 3.3 per 100,000 population.

• #30 Multiple system atrophy | Practical Neurology

  https://pn.bmj.com/content/23/3/208

  Multiple system atrophy (MSA) is a sporadic, progressive, adult-onset (>30 years), degenerative disease that presents with a combination of parkinsonism, cerebellar and autonomic dysfunction. Its diagnosis is challenging and postmortem studies show accuracy rates of only 62%–79%. Delayed diagnosis is common, with an average of 3.8 years from symptom onset to diagnosis. As average survival is only 7–9 years, early diagnosis is crucial for patient quality of life and effective management. […] […] Worldwide epidemiology of multiple system atrophy (MSA) occurs worldwide. Incidence: 0.6–3 per 100,000 people per year. Prevalence: 1.9–4.9 per 100,000 people. Sexes equally affected. Onset typically in sixth decade. Mean age of onset 56±9 years. Relative frequency of motor subtypes differs by geographical and ethnic regions: MSA-cerebellar more in Japanese, Korean and Mestizo populations (70%–80%). MSA-parkinsonian more in European and North American populations (67%–84%).

• #31

  https://link.springer.com/article/10.1007/s00415-024-12561-4

  On 31 December 2021, 16 patients were alive giving a crude point prevalence of 2.43 cases per 100,000 inhabitants: 1.2 in women and 3.69 in men. […] Total incident cases were 32 and the crude average annual incidence rate (IR) was 0.49 per 100,000 person-years in the period 20122021. […] The highest incidence occurred in the age group of 6069 years, with an IR of 2.0. […] Joinpoint analysis for global incidence and prevalence experienced stable annual rates during the whole period, showing an upward trend for prevalence without a statistically significant slop. […] The median duration from onset of symptom to diagnosis was 36 months. […] The median of survival from clinical symptom onset was 84 months and from clinical diagnosis was 42 months. […] Our study provides a detailed description of age, sex-specific and age-adjusted prevalence, and incidence of MSA in Navarre and represents the first population-based epidemiological study on MSA performed in Spain.

• #32 Multiple System Atrophy: Practice Essentials, Background, Etiology and Pathophysiology

  https://emedicine.medscape.com/article/1154583-overview

  The prevalence of MSA is reported to be between 3.4-4.9 cases per 100,000 population. The estimated mean incidence is 0.6-0.7 cases per 100,000 person-years. MSA meets orphan disease status. […] Many patients do not receive the correct diagnosis during their lifetime because of the difficulty in differentiating MSA from other disorders (eg, Parkinson disease, pure autonomic failure [PAF], other rare movement disorders). About 29-33% of patients with isolated late-onset cerebellar ataxia and 8-10% of patients with parkinsonism will develop MSA. Therefore, a higher prevalence than that estimated can be assumed. […] In the European Union (EU), the prevalence rates show 4-5 cases per 100,000 persons. The incidence rate is about 0.6 cases per 100,000 persons per year. […] In the United Kingdom, the crude prevalence of MSA, including all probable and possible cases, is 3.3 per 100,000 population.

• #33 Multiple system atrophy – Wikipedia

  https://en.wikipedia.org/wiki/Multiple_system_atrophy

  Multiple system atrophy is estimated to affect approximately 5 per 100,000 people. […] At autopsy, many patients diagnosed during life with Parkinson’s disease are found actually to have MSA, suggesting that the actual incidence of MSA is higher than that estimate. […] While some suggest that MSA affects slightly more men than women (1.3:1), others suggest that the two sexes are equally likely to be affected. […] The condition most commonly presents in persons aged 50-60.

• #34 Multiple system atrophy | Practical Neurology

  https://pn.bmj.com/content/23/3/208

  Multiple system atrophy (MSA) is a sporadic, progressive, adult-onset (>30 years), degenerative disease that presents with a combination of parkinsonism, cerebellar and autonomic dysfunction. Its diagnosis is challenging and postmortem studies show accuracy rates of only 62%–79%. Delayed diagnosis is common, with an average of 3.8 years from symptom onset to diagnosis. As average survival is only 7–9 years, early diagnosis is crucial for patient quality of life and effective management. […] […] Worldwide epidemiology of multiple system atrophy (MSA) occurs worldwide. Incidence: 0.6–3 per 100,000 people per year. Prevalence: 1.9–4.9 per 100,000 people. Sexes equally affected. Onset typically in sixth decade. Mean age of onset 56±9 years. Relative frequency of motor subtypes differs by geographical and ethnic regions: MSA-cerebellar more in Japanese, Korean and Mestizo populations (70%–80%). MSA-parkinsonian more in European and North American populations (67%–84%).

• #35 Multiple system atrophy

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10314081/

  Many terms have previously been used to describe this condition. Graham and Oppenheimer introduced MSA in 1969 to encompass multiple neurological entities such as olivopontocerebellar atrophy, striatonigral degeneration and Shy-Drager syndrome. […] Occurs worldwide. Incidence: 0.63 per 100000 people per year. Prevalence: 1.94.9 per 100000 people. Sexes equally affected. Onset typically in sixth decade. Mean age of onset 569 years. Relative frequency of motor subtypes differs by geographical and ethnic regions: MSA-cerebellar more in Japanese, Korean and Mestizo populations (70%80%). MSA-parkinsonian more in European and North American populations (67%84%). […] The average survival from onset for an MSA patient is around 79 years. However, a pathologically confirmed case series of a benign MSA variant found survival of 15 years and the longest reported survival of a neuropathologically confirmed MSA-P case is 23 years from onset. Conversely, there is a report of an aggressive MSA phenotype with a very short disease duration of 3 years.

• #36 Orphanet: Multiple system atrophy

  https://www.orpha.net/en/disease/detail/102

  Multiple system atrophy (MSA) is a neurodegenerative disorder characterized by autonomic failure (cardiovascular and/or urinary), parkinsonism, cerebellar impairment and corticospinal signs with a median survival of 6-9 years. […] Prevalence ranges from 1/50,000-1/20,000. MSA-parkinsonian type (MSA-p) predominates in the Western Hemisphere and MSA-cerebellar type (MSA-c) predominates in the Eastern Hemisphere. Genders are equally distributed. […] MSA is rapidly progressive and is associated with wheelchair dependence, unintelligible speech, intermittent urinary catheterization, disabling orthostatic hypotension, and cognitive impairment (executive dysfunction). Disease progression is assessed using the unified MSA rating scale (UMSARS), which rates activities of daily life, autonomic and motor impairment, as well as overall disability. Prognosis is poor with a median survival of 6-9 years.

• #37 Multiple system atrophy

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10314081/

  Many terms have previously been used to describe this condition. Graham and Oppenheimer introduced MSA in 1969 to encompass multiple neurological entities such as olivopontocerebellar atrophy, striatonigral degeneration and Shy-Drager syndrome. […] Occurs worldwide. Incidence: 0.63 per 100000 people per year. Prevalence: 1.94.9 per 100000 people. Sexes equally affected. Onset typically in sixth decade. Mean age of onset 569 years. Relative frequency of motor subtypes differs by geographical and ethnic regions: MSA-cerebellar more in Japanese, Korean and Mestizo populations (70%80%). MSA-parkinsonian more in European and North American populations (67%84%). […] The average survival from onset for an MSA patient is around 79 years. However, a pathologically confirmed case series of a benign MSA variant found survival of 15 years and the longest reported survival of a neuropathologically confirmed MSA-P case is 23 years from onset. Conversely, there is a report of an aggressive MSA phenotype with a very short disease duration of 3 years.

• #38 Multiple system atrophy

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10314081/

  Many terms have previously been used to describe this condition. Graham and Oppenheimer introduced MSA in 1969 to encompass multiple neurological entities such as olivopontocerebellar atrophy, striatonigral degeneration and Shy-Drager syndrome. […] Occurs worldwide. Incidence: 0.63 per 100000 people per year. Prevalence: 1.94.9 per 100000 people. Sexes equally affected. Onset typically in sixth decade. Mean age of onset 569 years. Relative frequency of motor subtypes differs by geographical and ethnic regions: MSA-cerebellar more in Japanese, Korean and Mestizo populations (70%80%). MSA-parkinsonian more in European and North American populations (67%84%). […] The average survival from onset for an MSA patient is around 79 years. However, a pathologically confirmed case series of a benign MSA variant found survival of 15 years and the longest reported survival of a neuropathologically confirmed MSA-P case is 23 years from onset. Conversely, there is a report of an aggressive MSA phenotype with a very short disease duration of 3 years.

• #39

  https://link.springer.com/article/10.1007/s00415-024-12561-4

  On 31 December 2021, 16 patients were alive giving a crude point prevalence of 2.43 cases per 100,000 inhabitants: 1.2 in women and 3.69 in men. […] Total incident cases were 32 and the crude average annual incidence rate (IR) was 0.49 per 100,000 person-years in the period 20122021. […] The highest incidence occurred in the age group of 6069 years, with an IR of 2.0. […] Joinpoint analysis for global incidence and prevalence experienced stable annual rates during the whole period, showing an upward trend for prevalence without a statistically significant slop. […] The median duration from onset of symptom to diagnosis was 36 months. […] The median of survival from clinical symptom onset was 84 months and from clinical diagnosis was 42 months. […] Our study provides a detailed description of age, sex-specific and age-adjusted prevalence, and incidence of MSA in Navarre and represents the first population-based epidemiological study on MSA performed in Spain.

• #40 Multiple system atrophy

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10314081/

  After the onset of motor symptoms, ~30% of patients require walking aids within 3 years, and up to 60% are wheelchair-bound by 5 years and bedbound by 8 years. At 6 years, ~10% of patients require gastrostomy and have unintelligible speech. MSA-P patients generally have more functional impairment than those with MSA-C, but the overall rate of progression and survival is no different. […] No disease-modifying treatment has proven so far to alter MSA progression, and thus symptom management remains the mainstay of care. Local charities (eg, the MSA Trust (UK) and the MSA Coalition (USA)) are useful sources of information for patients about the disease as well as local services available to patients for example, specialist nursing, social welfare or financial support specific to their needs. […] Patients with suspected MSA should have a postvoiding residual urine scan, a lying and standing blood pressure, and an MR scan of brain with additional susceptibility-weighted and diffusivity images.

• #41 Multiple system atrophy

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10314081/

  After the onset of motor symptoms, ~30% of patients require walking aids within 3 years, and up to 60% are wheelchair-bound by 5 years and bedbound by 8 years. At 6 years, ~10% of patients require gastrostomy and have unintelligible speech. MSA-P patients generally have more functional impairment than those with MSA-C, but the overall rate of progression and survival is no different. […] No disease-modifying treatment has proven so far to alter MSA progression, and thus symptom management remains the mainstay of care. Local charities (eg, the MSA Trust (UK) and the MSA Coalition (USA)) are useful sources of information for patients about the disease as well as local services available to patients for example, specialist nursing, social welfare or financial support specific to their needs. […] Patients with suspected MSA should have a postvoiding residual urine scan, a lying and standing blood pressure, and an MR scan of brain with additional susceptibility-weighted and diffusivity images.

• #42 Multiple system atrophy

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10314081/

  After the onset of motor symptoms, ~30% of patients require walking aids within 3 years, and up to 60% are wheelchair-bound by 5 years and bedbound by 8 years. At 6 years, ~10% of patients require gastrostomy and have unintelligible speech. MSA-P patients generally have more functional impairment than those with MSA-C, but the overall rate of progression and survival is no different. […] No disease-modifying treatment has proven so far to alter MSA progression, and thus symptom management remains the mainstay of care. Local charities (eg, the MSA Trust (UK) and the MSA Coalition (USA)) are useful sources of information for patients about the disease as well as local services available to patients for example, specialist nursing, social welfare or financial support specific to their needs. […] Patients with suspected MSA should have a postvoiding residual urine scan, a lying and standing blood pressure, and an MR scan of brain with additional susceptibility-weighted and diffusivity images.

• #43 Multiple system atrophy

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10314081/

  After the onset of motor symptoms, ~30% of patients require walking aids within 3 years, and up to 60% are wheelchair-bound by 5 years and bedbound by 8 years. At 6 years, ~10% of patients require gastrostomy and have unintelligible speech. MSA-P patients generally have more functional impairment than those with MSA-C, but the overall rate of progression and survival is no different. […] No disease-modifying treatment has proven so far to alter MSA progression, and thus symptom management remains the mainstay of care. Local charities (eg, the MSA Trust (UK) and the MSA Coalition (USA)) are useful sources of information for patients about the disease as well as local services available to patients for example, specialist nursing, social welfare or financial support specific to their needs. […] Patients with suspected MSA should have a postvoiding residual urine scan, a lying and standing blood pressure, and an MR scan of brain with additional susceptibility-weighted and diffusivity images.

• #44

  https://link.springer.com/article/10.1007/s00415-024-12561-4

  Numbers of average annual IR (0.49/100,000 person-years) and age-adjusted prevalence (2.43/100,000) are similar to previous studies with similar methodology conducted in European populations. […] We found no differences in survival between men and women, neither differences in the time from diagnosis to death. […] Time to diagnosis in our series is long, median 36 months, with no differences according to subtypes, in line with other European studies. […] We consider that our study has many strengths: recruitment of cases was performed with searching through extensive sources of health information and covers a long period.

• #45 Systematic Review of Incidence and Prevalence Studies of Multiple System Atrophy – MDS Abstracts

  https://www.mdsabstracts.org/abstract/systematic-review-of-incidence-and-prevalence-studies-of-multiple-system-atrophy/

  Systematic Review of Incidence and Prevalence Studies of Multiple System Atrophy […] This study sought to systematically review the methods and results of previous incidence and prevalence studies of MSA. […] High-quality incidence and prevalence studies are important for health-care planning and epidemiological research. To our knowledge, there are no existing systematic reviews summarising the current evidence on both the incidence and prevalence of multiple system atrophy (MSA). […] Fifteen incidence studies (including three age-restricted studies) and twenty prevalence (including nine age-restricted studies) studies were identified. Studies were conducted in Europe (n=22), Asia (n=6), the United States of America (n=3), Africa (n=2), South America (n=1) and Russia (n=1). Less than half (45.7%) of studies used the Gilman consensus criteria (1998 or 2008 iteration according to study year) for diagnosis and case definition.

• #46 Multiple system atrophy registry

  https://www.jstage.jst.go.jp/article/jsnt/40/1/40_23/_article/-char/en

  Multiple system atrophy (MSA) is a neurodegenerative disease characterized by autonomic failure with various combination of cerebellar ataxia, Parkinsonism and pyramidal signs with the average age at onset in late 50s. The prevalence of MSA in Japan has been estimated to be 12,000. […] To facilitate the research on MSA, we established the MSA registry in 2016. In this registry, detailed clinical information including Unified Multiple System Atrophy Rating Scale parts 1 and 2 has been collected in a prospective manner. […] To date, 530 patients with MSA have been registered in this registry. The detailed natural history of MSA established based on the MSA registry is expected to contribute to design future clinical trials.

• #47 Multiple System Atrophy | Penn State Health

  https://www.pennstatehealth.org/services-treatments/multiple-system-atrophy

  Currently, the exact causes of MSA are unknown. […] Along with researchers around the world, the clinicians and scientists at Penn State College of Medicines Translational Brain Research Center are devoted to identifying the causes of MSA and developing better diagnostic tools through partnership with the National Institute of Neurological Disorders and Stroke (NINDS) Parkinsons Disease Biomarkers Program (PDBP) and clinical trials. […] We have an active clinical science research program and frequently seek volunteers to participate in clinical trials. These studies help our scientists improve diagnostic techniques, develop better treatments, and collaborate with other researchers.

• #48 Multiple System Atrophy: Advances in Diagnosis and Therapy

  https://www.e-jmd.org/journal/view.php?doi=10.14802/jmd.22082

  This review summarizes improvements in understanding the pathophysiology and early clinical symptoms of multiple system atrophy (MSA) and advancements in diagnostic methods and disease-modifying therapies for the condition. […] In 2022, the Movement Disorder Society proposed new diagnostic criteria to develop disease-modifying therapies and promote clinical trials of MSA since the second consensus was proposed in 2008. […] In 2022, the Movement Disorder Society (MDS) proposed new diagnostic criteria to develop disease-modifying therapies and promote clinical trials of MSA, which was the first time that it had been revised since the second consensus was proposed in 2008. […] The proposed new category of possible prodromal MSA with very low specificity is expected to undergo continuous refinement with emerging data, particularly from prospective investigations and biomarker studies.

• #49 Multiple System Atrophy: Advances in Diagnosis and Therapy

  https://www.e-jmd.org/journal/view.php?doi=10.14802/jmd.22082

  This review summarizes improvements in understanding the pathophysiology and early clinical symptoms of multiple system atrophy (MSA) and advancements in diagnostic methods and disease-modifying therapies for the condition. […] In 2022, the Movement Disorder Society proposed new diagnostic criteria to develop disease-modifying therapies and promote clinical trials of MSA since the second consensus was proposed in 2008. […] In 2022, the Movement Disorder Society (MDS) proposed new diagnostic criteria to develop disease-modifying therapies and promote clinical trials of MSA, which was the first time that it had been revised since the second consensus was proposed in 2008. […] The proposed new category of possible prodromal MSA with very low specificity is expected to undergo continuous refinement with emerging data, particularly from prospective investigations and biomarker studies.

• #50 Multiple System Atrophy-D Maybe Something Altogether–Different

  https://practicalneurology.com/diseases-diagnoses/movement-disorders/multiple-system-atrophy-d-maybe-something-altogetherdifferent/30257/

  The incidence of MSA is 3:100,000 in adults aged over 50 years, and the course of illness lasts 5 to 10 years. There are 2 known groups: MSA-P (parkinsonian) and MSA-C (cerebellar) that have clinical presentations associating them closely with more common movement disorders (eg, Parkinsons disease [PD] and ataxia). […] Leaders at the conference discussed the possibility of revising the criteria in order to identify patients with MSA earlier in the disease course for the purpose of improving recruitment of subjects for epidemiologic studies and clinical trials.

• #51 Multiple System Atrophy: Advances in Diagnosis and Therapy

  https://www.e-jmd.org/journal/view.php?doi=10.14802/jmd.22082

  More recently, novel biomarkers for MSA diagnosis have been provided but were excluded from the new MDS criteria given the limited availability, suboptimal diagnostic accuracy, or lack of diagnostic validation. […] The clinical diagnosis of MSA requires the presence of various combinations of AF and/or parkinsonism or cerebellar ataxia. […] Therefore, in addition to the novel MDS diagnostic criteria, a biomarker that can diagnose this stage of monosystem atrophy with high accuracy is desirable. […] The severity and definition of orthostatic hypotension differed from those in the second consensus criteria. […] New diagnostic methods focusing on MSA-type -synuclein are being developed. […] Diagnostic methods for MSA include the development of techniques to detect MSA-type -synuclein and neurofilament light chain release with the loss of neurons and oligodendroglia, monitoring of characteristic laryngeal findings, and the development of imaging methods combining advanced statistical methods such as artificial intelligence, machine learning, and deep learning. […] The development of MDS diagnostic criteria, early diagnostic methods, and cutting-edge disease-modifying treatments based on advances in understanding the pathophysiology of MSA is expected.

• #52 Multiple System Atrophy: Advances in Diagnosis and Therapy

  https://www.e-jmd.org/journal/view.php?doi=10.14802/jmd.22082

  More recently, novel biomarkers for MSA diagnosis have been provided but were excluded from the new MDS criteria given the limited availability, suboptimal diagnostic accuracy, or lack of diagnostic validation. […] The clinical diagnosis of MSA requires the presence of various combinations of AF and/or parkinsonism or cerebellar ataxia. […] Therefore, in addition to the novel MDS diagnostic criteria, a biomarker that can diagnose this stage of monosystem atrophy with high accuracy is desirable. […] The severity and definition of orthostatic hypotension differed from those in the second consensus criteria. […] New diagnostic methods focusing on MSA-type -synuclein are being developed. […] Diagnostic methods for MSA include the development of techniques to detect MSA-type -synuclein and neurofilament light chain release with the loss of neurons and oligodendroglia, monitoring of characteristic laryngeal findings, and the development of imaging methods combining advanced statistical methods such as artificial intelligence, machine learning, and deep learning. […] The development of MDS diagnostic criteria, early diagnostic methods, and cutting-edge disease-modifying treatments based on advances in understanding the pathophysiology of MSA is expected.

• #53 Multiple System Atrophy: Advances in Diagnosis and Therapy

  https://www.e-jmd.org/journal/view.php?doi=10.14802/jmd.22082

  More recently, novel biomarkers for MSA diagnosis have been provided but were excluded from the new MDS criteria given the limited availability, suboptimal diagnostic accuracy, or lack of diagnostic validation. […] The clinical diagnosis of MSA requires the presence of various combinations of AF and/or parkinsonism or cerebellar ataxia. […] Therefore, in addition to the novel MDS diagnostic criteria, a biomarker that can diagnose this stage of monosystem atrophy with high accuracy is desirable. […] The severity and definition of orthostatic hypotension differed from those in the second consensus criteria. […] New diagnostic methods focusing on MSA-type -synuclein are being developed. […] Diagnostic methods for MSA include the development of techniques to detect MSA-type -synuclein and neurofilament light chain release with the loss of neurons and oligodendroglia, monitoring of characteristic laryngeal findings, and the development of imaging methods combining advanced statistical methods such as artificial intelligence, machine learning, and deep learning. […] The development of MDS diagnostic criteria, early diagnostic methods, and cutting-edge disease-modifying treatments based on advances in understanding the pathophysiology of MSA is expected.

• #54 Multiple System Atrophy: Advances in Diagnosis and Therapy

  https://www.e-jmd.org/journal/view.php?doi=10.14802/jmd.22082

  More recently, novel biomarkers for MSA diagnosis have been provided but were excluded from the new MDS criteria given the limited availability, suboptimal diagnostic accuracy, or lack of diagnostic validation. […] The clinical diagnosis of MSA requires the presence of various combinations of AF and/or parkinsonism or cerebellar ataxia. […] Therefore, in addition to the novel MDS diagnostic criteria, a biomarker that can diagnose this stage of monosystem atrophy with high accuracy is desirable. […] The severity and definition of orthostatic hypotension differed from those in the second consensus criteria. […] New diagnostic methods focusing on MSA-type -synuclein are being developed. […] Diagnostic methods for MSA include the development of techniques to detect MSA-type -synuclein and neurofilament light chain release with the loss of neurons and oligodendroglia, monitoring of characteristic laryngeal findings, and the development of imaging methods combining advanced statistical methods such as artificial intelligence, machine learning, and deep learning. […] The development of MDS diagnostic criteria, early diagnostic methods, and cutting-edge disease-modifying treatments based on advances in understanding the pathophysiology of MSA is expected.

• #55 Multiple System Atrophy: Advances in Diagnosis and Therapy

  https://www.e-jmd.org/journal/view.php?doi=10.14802/jmd.22082

  More recently, novel biomarkers for MSA diagnosis have been provided but were excluded from the new MDS criteria given the limited availability, suboptimal diagnostic accuracy, or lack of diagnostic validation. […] The clinical diagnosis of MSA requires the presence of various combinations of AF and/or parkinsonism or cerebellar ataxia. […] Therefore, in addition to the novel MDS diagnostic criteria, a biomarker that can diagnose this stage of monosystem atrophy with high accuracy is desirable. […] The severity and definition of orthostatic hypotension differed from those in the second consensus criteria. […] New diagnostic methods focusing on MSA-type -synuclein are being developed. […] Diagnostic methods for MSA include the development of techniques to detect MSA-type -synuclein and neurofilament light chain release with the loss of neurons and oligodendroglia, monitoring of characteristic laryngeal findings, and the development of imaging methods combining advanced statistical methods such as artificial intelligence, machine learning, and deep learning. […] The development of MDS diagnostic criteria, early diagnostic methods, and cutting-edge disease-modifying treatments based on advances in understanding the pathophysiology of MSA is expected.

• #56 Tremor in Multiple System Atrophy – a review | Tremor and Other Hyperkinetic Movements

  https://tremorjournal.org/articles/10.5334/tohm.166

  Multiple system atrophy (MSA) is a rare neurodegenerative movement disorder characterized by a rapidly progressive course. The clinical presentation can include autonomic failure, parkinsonism, and cerebellar signs. […] Tremor is a common feature among MSA patients. Up to 80% of MSA patients show tremor, and patients with the parkinsonian variant of MSA are more commonly affected. […] The presence of either rest or postural tremor in association with parkinsonism can cause diagnostic confusion. However, there is evidence that tremor presents differently in MSA versus PD: atypical or jerky components have been reported, and the classic parkinsonian (pill-rolling) rest tremor is only present in a minority of patients (10%). […] To date, little has been written about tremor rates and characteristics in MSA, and few attempts have been made to formulate an MSA tremor classification.

• #57 Tremor in Multiple System Atrophy – a review | Tremor and Other Hyperkinetic Movements

  https://tremorjournal.org/articles/10.5334/tohm.166

  Several clinical and clinicopathological studies in MSA have demonstrated that tremor is not rare in this condition, reporting an overall occurrence of up to 80%. […] Comparisons of tremor occurrence in the motor subtypes of MSA have clearly shown that tremor is more common in MSA-P compared with MSA-C. […] In summary, rest tremor is present in about one-third of MSA patients, particularly in those with the parkinsonian subtype. […] Taken together, postural tremor appears to be the most common tremor subtype in MSA, especially in MSA-P. […] Intention tremor is supportive of a diagnosis of MSA and in a differential diagnosis of PD, intention tremor is indicative of MSA. […] This review of the published data on tremor in MSA, including case reports and clinical, clinicopathological, neurophysiological, and imaging studies, demonstrates that postural tremor is the most commonly reported tremor type in MSA.

• #58 Tremor in Multiple System Atrophy – a review | Tremor and Other Hyperkinetic Movements

  https://tremorjournal.org/articles/10.5334/tohm.166

  Several clinical and clinicopathological studies in MSA have demonstrated that tremor is not rare in this condition, reporting an overall occurrence of up to 80%. […] Comparisons of tremor occurrence in the motor subtypes of MSA have clearly shown that tremor is more common in MSA-P compared with MSA-C. […] In summary, rest tremor is present in about one-third of MSA patients, particularly in those with the parkinsonian subtype. […] Taken together, postural tremor appears to be the most common tremor subtype in MSA, especially in MSA-P. […] Intention tremor is supportive of a diagnosis of MSA and in a differential diagnosis of PD, intention tremor is indicative of MSA. […] This review of the published data on tremor in MSA, including case reports and clinical, clinicopathological, neurophysiological, and imaging studies, demonstrates that postural tremor is the most commonly reported tremor type in MSA.

• #59 The Pathobiology of Behavioral Changes in Multiple System Atrophy: An Update

  https://www.mdpi.com/1422-0067/25/13/7464

  Multiple system atrophy (MSA) is a rare disease with an estimated incidence of 0.6–0.7/100,000 person years and a prevalence of 1.9–4.9/100,000. […] According to available data, up to 70% of MSA patients show various forms and degrees of behavioral changes in one or several domains. Frequent forms are anxiety, the prevalence of which ranges from 37% to 71.7%. […] The prevalence of clinical RBD in MSA patients ranges from 68.8% to 90.2%. […] Living with either MSA and/or severe behavioral symptoms, often mixed with depression and cognitive impairment, is difficult and impairs the quality of life of patients and caregivers, since they are fundamental for the progression of the disease, and should be considered as part of its diagnosis and treatment. […] This article, based on a systematic literature research of PubMed, Google Scholar and Cochrane Library until May 2024, aims to explain the relations between MSA and behavioral disturbances, their epidemiology, basic clinical features, neuroimaging findings, pathogenic factors, and current treatment options.

• #60 Multiple system atrophy

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10314081/

  After the onset of motor symptoms, ~30% of patients require walking aids within 3 years, and up to 60% are wheelchair-bound by 5 years and bedbound by 8 years. At 6 years, ~10% of patients require gastrostomy and have unintelligible speech. MSA-P patients generally have more functional impairment than those with MSA-C, but the overall rate of progression and survival is no different. […] No disease-modifying treatment has proven so far to alter MSA progression, and thus symptom management remains the mainstay of care. Local charities (eg, the MSA Trust (UK) and the MSA Coalition (USA)) are useful sources of information for patients about the disease as well as local services available to patients for example, specialist nursing, social welfare or financial support specific to their needs. […] Patients with suspected MSA should have a postvoiding residual urine scan, a lying and standing blood pressure, and an MR scan of brain with additional susceptibility-weighted and diffusivity images.

• #61 Multiple system atrophy

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10314081/

  After the onset of motor symptoms, ~30% of patients require walking aids within 3 years, and up to 60% are wheelchair-bound by 5 years and bedbound by 8 years. At 6 years, ~10% of patients require gastrostomy and have unintelligible speech. MSA-P patients generally have more functional impairment than those with MSA-C, but the overall rate of progression and survival is no different. […] No disease-modifying treatment has proven so far to alter MSA progression, and thus symptom management remains the mainstay of care. Local charities (eg, the MSA Trust (UK) and the MSA Coalition (USA)) are useful sources of information for patients about the disease as well as local services available to patients for example, specialist nursing, social welfare or financial support specific to their needs. […] Patients with suspected MSA should have a postvoiding residual urine scan, a lying and standing blood pressure, and an MR scan of brain with additional susceptibility-weighted and diffusivity images.

• #62 Multiple system atrophy

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10314081/

  After the onset of motor symptoms, ~30% of patients require walking aids within 3 years, and up to 60% are wheelchair-bound by 5 years and bedbound by 8 years. At 6 years, ~10% of patients require gastrostomy and have unintelligible speech. MSA-P patients generally have more functional impairment than those with MSA-C, but the overall rate of progression and survival is no different. […] No disease-modifying treatment has proven so far to alter MSA progression, and thus symptom management remains the mainstay of care. Local charities (eg, the MSA Trust (UK) and the MSA Coalition (USA)) are useful sources of information for patients about the disease as well as local services available to patients for example, specialist nursing, social welfare or financial support specific to their needs. […] Patients with suspected MSA should have a postvoiding residual urine scan, a lying and standing blood pressure, and an MR scan of brain with additional susceptibility-weighted and diffusivity images.

• #63 Estimating the prevalence and incidence of multiple system atrophy in the USA: Insights from a national claims database – PubMed

  https://pubmed.ncbi.nlm.nih.gov/37951144/

  Published literature on the prevalence and incidence of multiple system atrophy (MSA) in the US are scarce or based on relatively older studies. The objective of this study was to estimate the prevalence and cumulative incidence of MSA in the US. […] The crude prevalence of MSA was 7.2 per 100,000 persons in 2021 and increased with age. After standardization to the US population, the age-adjusted prevalence was 12.4 per 100,000 translating to 41,133 persons in the 2021 US population. […] The crude cumulative incidence of MSA for individuals aged 30 years and older was 9.8 per 100,000 persons in 2021. The estimated cumulative incidence of MSA in individuals 30 years or older, age-adjusted to the 2021 U S. population, was 14.2 per 100,000. […] This study provides real-world evidence on the prevalence and cumulative incidence of MSA in the US to better understand the medical care needs and treatment.

• #64 Multiple system atrophy | Practical Neurology

  https://pn.bmj.com/content/23/3/208

  Multiple system atrophy (MSA) is a sporadic, progressive, adult-onset (>30 years), degenerative disease that presents with a combination of parkinsonism, cerebellar and autonomic dysfunction. Its diagnosis is challenging and postmortem studies show accuracy rates of only 62%–79%. Delayed diagnosis is common, with an average of 3.8 years from symptom onset to diagnosis. As average survival is only 7–9 years, early diagnosis is crucial for patient quality of life and effective management. […] […] Worldwide epidemiology of multiple system atrophy (MSA) occurs worldwide. Incidence: 0.6–3 per 100,000 people per year. Prevalence: 1.9–4.9 per 100,000 people. Sexes equally affected. Onset typically in sixth decade. Mean age of onset 56±9 years. Relative frequency of motor subtypes differs by geographical and ethnic regions: MSA-cerebellar more in Japanese, Korean and Mestizo populations (70%–80%). MSA-parkinsonian more in European and North American populations (67%–84%).

• #65 Multiple system atrophy | Practical Neurology

  https://pn.bmj.com/content/23/3/208

  Multiple system atrophy (MSA) is a sporadic, progressive, adult-onset (>30 years), degenerative disease that presents with a combination of parkinsonism, cerebellar and autonomic dysfunction. Its diagnosis is challenging and postmortem studies show accuracy rates of only 62%–79%. Delayed diagnosis is common, with an average of 3.8 years from symptom onset to diagnosis. As average survival is only 7–9 years, early diagnosis is crucial for patient quality of life and effective management. […] […] Worldwide epidemiology of multiple system atrophy (MSA) occurs worldwide. Incidence: 0.6–3 per 100,000 people per year. Prevalence: 1.9–4.9 per 100,000 people. Sexes equally affected. Onset typically in sixth decade. Mean age of onset 56±9 years. Relative frequency of motor subtypes differs by geographical and ethnic regions: MSA-cerebellar more in Japanese, Korean and Mestizo populations (70%–80%). MSA-parkinsonian more in European and North American populations (67%–84%).

• #66 Multiple system atrophy

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10314081/

  After the onset of motor symptoms, ~30% of patients require walking aids within 3 years, and up to 60% are wheelchair-bound by 5 years and bedbound by 8 years. At 6 years, ~10% of patients require gastrostomy and have unintelligible speech. MSA-P patients generally have more functional impairment than those with MSA-C, but the overall rate of progression and survival is no different. […] No disease-modifying treatment has proven so far to alter MSA progression, and thus symptom management remains the mainstay of care. Local charities (eg, the MSA Trust (UK) and the MSA Coalition (USA)) are useful sources of information for patients about the disease as well as local services available to patients for example, specialist nursing, social welfare or financial support specific to their needs. […] Patients with suspected MSA should have a postvoiding residual urine scan, a lying and standing blood pressure, and an MR scan of brain with additional susceptibility-weighted and diffusivity images.

• #67 Multiple System Atrophy | Baylor Medicine

  https://www.bcm.edu/healthcare/specialties/neurology/parkinsons-disease-and-movement-disorders/multiple-system-atrophy

  Multiple system atrophy (MSA) is considered a rare disease affecting potentially 15,000 to 50,000 people in the United States. […] In North America, the parkinsonian subtype seems to predominate whereas in Japan, the cerebellar pattern is more common. […] Because MSA is a rare disease, these characteristic changes are sometimes not recognized by radiologists.

• #68 Multiple system atrophy

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10314081/

  After the onset of motor symptoms, ~30% of patients require walking aids within 3 years, and up to 60% are wheelchair-bound by 5 years and bedbound by 8 years. At 6 years, ~10% of patients require gastrostomy and have unintelligible speech. MSA-P patients generally have more functional impairment than those with MSA-C, but the overall rate of progression and survival is no different. […] No disease-modifying treatment has proven so far to alter MSA progression, and thus symptom management remains the mainstay of care. Local charities (eg, the MSA Trust (UK) and the MSA Coalition (USA)) are useful sources of information for patients about the disease as well as local services available to patients for example, specialist nursing, social welfare or financial support specific to their needs. […] Patients with suspected MSA should have a postvoiding residual urine scan, a lying and standing blood pressure, and an MR scan of brain with additional susceptibility-weighted and diffusivity images.

• #69 Multiple System Atrophy (MSA) Market is expected to reach

  https://www.globenewswire.com/news-release/2024/10/14/2962687/0/en/Multiple-System-Atrophy-MSA-Market-is-expected-to-reach-US-202-3-Million-by-2031-Report-by-CoherentMI.html

  The prevalence of Multiple System Atrophy (MSA) is increasing worldwide which is a major market driver. As per recent studies, the prevalence of MSA is estimated to be around 4 to 5 cases per 100,000 people. […] The growth of the MSA market is attributed to the increasing research and developmental activities to develop disease modifying therapies for MSA. […] The increased prevalence of MSA-Parkinsonism subtype is contributing to the high market share of this segment. […] Imaging-Based Diagnosis segment is expected to witness fastest growth over the forecast period owing to the advancements in imaging modalities such as MRI and PET imaging which help in early and accurate diagnosis of MSA.

• #70 Multiple System Atrophy (MSA) Market is expected to reach

  https://www.globenewswire.com/news-release/2024/10/14/2962687/0/en/Multiple-System-Atrophy-MSA-Market-is-expected-to-reach-US-202-3-Million-by-2031-Report-by-CoherentMI.html

  The prevalence of Multiple System Atrophy (MSA) is increasing worldwide which is a major market driver. As per recent studies, the prevalence of MSA is estimated to be around 4 to 5 cases per 100,000 people. […] The growth of the MSA market is attributed to the increasing research and developmental activities to develop disease modifying therapies for MSA. […] The increased prevalence of MSA-Parkinsonism subtype is contributing to the high market share of this segment. […] Imaging-Based Diagnosis segment is expected to witness fastest growth over the forecast period owing to the advancements in imaging modalities such as MRI and PET imaging which help in early and accurate diagnosis of MSA.

• #71 Multiple System Atrophy | University of Michigan Health

  https://www.uofmhealth.org/conditions-treatments/multiple-system-atrophy

  Multiple system atrophy (MSA) is a rare but serious neurological disorder that can affect coordination, balance, speech, blood pressure and bladder and bowel function. MSA affects approximately 3 out of every 100,000 people over the age of 50. […] Since MSA symptoms overlap with that of Parkinson disease or cerebellar ataxia, our neurologists assess patients closely and run all necessary tests to ensure the correct diagnosis is made. […] The University of Michigan Atypical Parkinsonian Clinic was created in the summer of 2020 to promote up-to-date and comprehensive clinical care and access to research for those patients and families with diseases such as MSA.

• #72 Multiple system atrophy

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10314081/

  After the onset of motor symptoms, ~30% of patients require walking aids within 3 years, and up to 60% are wheelchair-bound by 5 years and bedbound by 8 years. At 6 years, ~10% of patients require gastrostomy and have unintelligible speech. MSA-P patients generally have more functional impairment than those with MSA-C, but the overall rate of progression and survival is no different. […] No disease-modifying treatment has proven so far to alter MSA progression, and thus symptom management remains the mainstay of care. Local charities (eg, the MSA Trust (UK) and the MSA Coalition (USA)) are useful sources of information for patients about the disease as well as local services available to patients for example, specialist nursing, social welfare or financial support specific to their needs. […] Patients with suspected MSA should have a postvoiding residual urine scan, a lying and standing blood pressure, and an MR scan of brain with additional susceptibility-weighted and diffusivity images.

• #73 Multiple System Atrophy

  https://neurosciences.ucsd.edu/centers-programs/movement-disorders/community/disease-overview/msa.html

  Although the cause of MSA is currently unknown, there is evidence that the primary defect occurs in glial cells, a type of cells in the nervous system that support and protect neurons in the brain, and help maintain physiological balance. […] MSA is a disabling disease in its advanced stage. Social support through local, regional, and national associations can play an integral role in improving the lives of patients, family members, and caregivers.

• #74 Multiple System Atrophy (MSA) – Brain, Spinal Cord, and Nerve Disorders – Merck Manual Consumer Version

  https://www.merckmanuals.com/home/brain-spinal-cord-and-nerve-disorders/autonomic-nervous-system-disorders/multiple-system-atrophy-msa

  Multiple system atrophy usually begins when people are in their 50s. It affects men and women equally. […] The diagnosis of multiple system atrophy is based on a doctor’s evaluation and results of certain tests. For example, doctors may suspect multiple system atrophy if parkinsonian symptoms are rapidly worsening and levodopa (used to treat Parkinson disease) has little or no effect on symptoms. […] Tests to evaluate the autonomic nervous system are done. They include the thermoregulatory sweat test and measurement of blood pressure while the person is sitting and after the person stands to check for orthostatic hypotension. […] Many people are confined to a wheelchair or are otherwise severely disabled within 5 years after symptoms begin. The disorder results in death usually about 9 to 10 years after symptoms begin. […] Because the disorder is progressive and ultimately fatal, people should prepare advance directives soon after the disorder is diagnosed. These directives should indicate what kind of medical care people want at the end of life.

• #75 Systematic Review of Incidence and Prevalence Studies of Multiple System Atrophy – MDS Abstracts

  https://www.mdsabstracts.org/abstract/systematic-review-of-incidence-and-prevalence-studies-of-multiple-system-atrophy/

  Crude incidence rates ranged from 0.1 to 2.1 per 100,000 person-years and crude prevalence rates ranged from 1.0 to 16.6 cases per 100,000 in studies unrestricted by age. Few studies reported age- or sex- stratified rates. Based on crude rates, there is little evidence to suggest clear sex differences in the incidence or prevalence of MSA but some evidence to suggest that both the incidence and prevalence of MSA increases with advancing age. […] Due to differences in study methodology, only three incidence studies were sufficiently similar to merit comparison, giving a pooled incidence rate of 0.9 per 100,000 person-years (95% CI 0.5, 1.3) while two similar prevalence studies gave a pooled prevalence rate of 3.6 per 100,000 (95% CI 2.2, 5.0). […] There are few incidence and prevalence studies in MSA. Existing studies are methodologically and statistically heterogeneous, which restricts pooled analyses. Further high-quality studies are required using standardised methodology.

• #76 Systematic Review of Incidence and Prevalence Studies of Multiple System Atrophy – MDS Abstracts

  https://www.mdsabstracts.org/abstract/systematic-review-of-incidence-and-prevalence-studies-of-multiple-system-atrophy/

  Crude incidence rates ranged from 0.1 to 2.1 per 100,000 person-years and crude prevalence rates ranged from 1.0 to 16.6 cases per 100,000 in studies unrestricted by age. Few studies reported age- or sex- stratified rates. Based on crude rates, there is little evidence to suggest clear sex differences in the incidence or prevalence of MSA but some evidence to suggest that both the incidence and prevalence of MSA increases with advancing age. […] Due to differences in study methodology, only three incidence studies were sufficiently similar to merit comparison, giving a pooled incidence rate of 0.9 per 100,000 person-years (95% CI 0.5, 1.3) while two similar prevalence studies gave a pooled prevalence rate of 3.6 per 100,000 (95% CI 2.2, 5.0). […] There are few incidence and prevalence studies in MSA. Existing studies are methodologically and statistically heterogeneous, which restricts pooled analyses. Further high-quality studies are required using standardised methodology.

• #77 Clinical and Imaging Features of Multiple System Atrophy: Challenges for an Early and Clinically Definitive Diagnosis

  https://www.e-jmd.org/journal/view.php?doi=10.14802/jmd.18020

  Thus, it is crucial to establish well-validated biomarkers for a very early and clinically definitive diagnosis. […] However, there are no known biomarkers that fulfill the criteria of objectivity, semi or quantitative, and uncomplicated and worldwide availability. Currently, several biomarkers, such as DWI, automated and observer-independent volumetric MRI, skin biopsy, -syn PET imaging, and blood and CSF biomarkers that have the potential for overcoming such limitations, are under development.

• #78

  https://link.springer.com/article/10.1007/s00415-024-12561-4

  Numbers of average annual IR (0.49/100,000 person-years) and age-adjusted prevalence (2.43/100,000) are similar to previous studies with similar methodology conducted in European populations. […] We found no differences in survival between men and women, neither differences in the time from diagnosis to death. […] Time to diagnosis in our series is long, median 36 months, with no differences according to subtypes, in line with other European studies. […] We consider that our study has many strengths: recruitment of cases was performed with searching through extensive sources of health information and covers a long period.

• #79

  https://link.springer.com/article/10.1007/s00415-024-12561-4

  Numbers of average annual IR (0.49/100,000 person-years) and age-adjusted prevalence (2.43/100,000) are similar to previous studies with similar methodology conducted in European populations. […] We found no differences in survival between men and women, neither differences in the time from diagnosis to death. […] Time to diagnosis in our series is long, median 36 months, with no differences according to subtypes, in line with other European studies. […] We consider that our study has many strengths: recruitment of cases was performed with searching through extensive sources of health information and covers a long period.

• #80 Clinical and Imaging Features of Multiple System Atrophy: Challenges for an Early and Clinically Definitive Diagnosis

  https://www.e-jmd.org/journal/view.php?doi=10.14802/jmd.18020

  The median time from initial symptom presentation to combined motor and autonomic dysfunction in MSA (probable MSA) is 2 years but ranges from 1 to 10 years. […] Since the median time from onset to death is 9 years but that from receiving a probable MSA diagnosis to death is 6 years, patients with probable MSA may be at severely advanced stages at diagnosis. […] Thus, it is important to focus on the clinical characteristics of the isolated autonomic failure or motor impairment (mono system atrophy) stage for early diagnosis. […] It is urgent to establish a strategy for the early diagnosis of MSA during the isolated autonomic failure phase. […] For the successful development of DMT or symptomatic interventions for MSA, we need early diagnostic criteria and clinically definitive categorization.

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