Multiple system atrophy–parkinsonism with slow progression and prolonged survival: A diagnostic catch

Petrović, Igor; Ling, Helen; Asi, Yasmine T.; Ahmed, Zeshan; Kukkle, Prashanth Lingappa; Hazrati, Lili‐Naz; Lang, Anthony E.; Révész, Tamás; Holton, Janice L.; Lees, Andrew J.

doi:10.1002/mds.25115

Cited by 101 publications

(74 citation statements)

References 31 publications

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“…In MSA, OH whose estimated prevalence is 75% [1] may occur early in the disease course or late as in long-surviving patients [7,8]. In PD a recent meta-analysis reported a 30% prevalence at any stage of the disease with a large statistical heterogeneity between studies [9].…”

Section: Introductionmentioning

confidence: 95%

Cardiovascular autonomic testing performed with a new integrated instrumental approach is useful in differentiating MSA-P from PD at an early stage

Baschieri

Calandra-Buonaura

Doria

et al. 2015

Parkinsonism & Related Disorders

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Section: Introductionmentioning

confidence: 95%

Cardiovascular autonomic testing performed with a new integrated instrumental approach is useful in differentiating MSA-P from PD at an early stage

Baschieri

Calandra-Buonaura

Doria

et al. 2015

Parkinsonism & Related Disorders

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“…Traditionally, standard dopaminergic therapy for treatment of Parkinson's disease was thought to provide only minimal improvement in symptoms induced by MSA, as reflected in diagnostic criteria for probable MSA-P [1] . Previous studies have reported survival between 6 and 9 years among MSA patients [3,4,15] , although longer survival (>15 years from symptom onset) has been described in definite MSA-P cases [20] . In the above EMSA study [18] and the US cohort [19] , the overall survival was around 10 years; however, our study indicated an even worse prognosis.…”

Section: Previous Evidencementioning

confidence: 99%

Clinical Features in a Danish Population-Based Cohort of Probable Multiple System Atrophy Patients

Starhof

Korbo²,

Lassen³

et al. 2016

Neuroepidemiology

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Background: Multiple system atrophy (MSA) is a rare, sporadic and progressive neurodegenerative disorder. We aimed to describe the clinical features of Danish probable MSA patients, evaluate their initial response to dopaminergic therapy and examine mortality. Methods: From the Danish National Patient Registry, we identified 782 patients diagnosed with conditions potentially compatible with probable MSA (International Classification of Diseases, version 10 (ICD-10) codes G23.2, G23.8 and G23.9) during 1994-2009. Through medical record review, we narrowed our sample to 115 patients who fulfilled the criteria for probable MSA. We recorded clinical features, examined differences by MSA subtype and used Kaplan-Meier survival analysis to examine mortality. Results: The mean age at onset of patients with probable MSA was 60.2 years (range 36-75 years) and mean time to wheelchair dependency was 4.7 years (range 0-15 years). One-third of patients experienced a transient improvement in motor symptoms with use of levodopa. Median survival from disease onset was 6.9 years (range 1-16 years, 95% CI 6.3-7.5) with no apparent variation according to gender or subtype. Conclusions: Our nationwide approach corroborated that MSA is associated with diverse and grave symptoms, only limited response to levodopa, and poor prognosis.

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“…OH may occur early in the course of the disease, and in these cases is associated with a shorter survival time [10,11]. Similarly, recent studies demonstrated that in long-surviving patients, cardiovascular dysautonomia occurred late in the disease course suggesting that late onset of cardiovascular AF in MSA patients may be a favorable prognostic factor [12,13]. …”

Section: Clinical Observationmentioning

confidence: 99%

Diagnosis and Management of Autonomic Failure in Neurodegenerative Disorders

et al. 2014

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Background: One of the hallmarks of the E-synucleinopathies is the degeneration of the autonomic nervous system. Summary: This review discusses the diagnosis and management of cardiovascular autonomic failure within the context of the E-synucleinopathies. In addition, it outlines the utility of various laboratory assessments including cardiovascular reflex tests for the differential diagnoses of these disorders, as well as general disease management strategies. Key Messages: Laboratory investigations assessing the autonomic control of the cardiovascular system are useful in the differential diagnosis of E-synucleinopathies, especially in early stages of disease. Clinical Implications: The characterization of the different features of AF in patients with E-synucleinopathies is challenging because it might help to improve the accuracy of the differential diagnosis between these diseases at onset. Further cardiovascular AF has been demonstrated to have a negative prognostic role in E-synucleinopathies, therefore an early detection of cardiovascular dysautonomia allows to positively impact the disease course guiding the appropriate therapy. i 2014 S. Karger AG, Basel

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Multiple system atrophy–parkinsonism with slow progression and prolonged survival: A diagnostic catch

Abstract: Late appearance of dysautonomia is a favorable prognostic factor in MSA-P. Greater awareness of this uncommon "benign" subgroup of MSA will improve diagnostic accuracy and help to more accurately inform treatment options.

Cited by 101 publications

References 31 publications

Cardiovascular autonomic testing performed with a new integrated instrumental approach is useful in differentiating MSA-P from PD at an early stage

Cardiovascular autonomic testing performed with a new integrated instrumental approach is useful in differentiating MSA-P from PD at an early stage

Clinical Features in a Danish Population-Based Cohort of Probable Multiple System Atrophy Patients

Diagnosis and Management of Autonomic Failure in Neurodegenerative Disorders

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