Consensus statement on the diagnosis of multiple system atrophy

@article{Gilman1999ConsensusSO,
  title={Consensus statement on the diagnosis of multiple system atrophy},
  author={Sid Gilman and Phillip A. Low and Niall P. Quinn and Alberto Albanese and Yoav Ben-Shlomo and ClareJ. Fowler and Horacio Kaufmann and Thomas Klockgether and Antony E. Lang and P. L. Lantos and Irene Litvan and Christopher J. Mathias and Eugene Oliver and David Robertson and Irwin J. Schatz and Gregor K. Wenning},
  journal={Journal of the Neurological Sciences},
  year={1999},
  volume={163},
  pages={94-98}
}
The diagnosis of multiple system atrophy
TLDR
The diagnosis of multiple system atrophy in life rests on clinical history and neurological examination, and additional investigations such as autonomic function tests, sphincter electromyography, or neuroimaging may be used to support or to exclude other diseases.
The diagnosis of multiple system atrophy
TLDR
The diagnosis of multiple system atrophy in life rests on clinical history and neurological examination, and additional investigations such as autonomic function tests, sphincter electromyography, or neuroimaging may be used to support or to exclude other diseases.
Can Autonomic Testing and Imaging Contribute to the Early Diagnosis of Multiple System Atrophy? A Systematic Review and Recommendations by the Movement Disorder Society Multiple System Atrophy Study Group
TLDR
There are overlapping phenotypes between MSA‐parkinsonian type and Parkinson's disease, progressive supranuclear palsy, and dementia with Lewy bodies, and between M SA‐cerebellar type and sporadic adult‐onset ataxia resulting in a significant diagnostic delay and misdiagnosis of MSA during life.
Cognitive impairment in multiple system atrophy: Changing concepts
TLDR
Cognitive impairment was found to be a frequent feature in MSA, and clinically-defined dementia is now reported in 14-16% of cases, and comprehensive neuropsychological assessment is reviewed.
Multiple system atrophy
Multiple system atrophy: clinical-radiological correlation. Report of two cases
TLDR
It is thought that brain MRI might increase the accuracy diagnostic of MSA.
How to diagnose multiple system atrophy
  • N. Quinn
  • Medicine, Psychology
    Movement disorders : official journal of the Movement Disorder Society
  • 2005
The diagnosis of multiple system atrophy (MSA) in life remains entirely clinical. Consensus diagnostic criteria have been developed, but their use does not particularly render a diagnosis of MSA more
Pathological description of a non-motor variant of multiple system atrophy
TLDR
A patient with pathologically confirmed MSA who presented with a longstanding history of stridor, RBD and autonomic disturbances but did not develop overt parkinsonism or cerebellar signs is reported, illustrating that MSA may present clinically without its cardinal motor symptoms, and thatstridor and RBD may be clues to recognise the disease in a patient with autonomic failure.
Clinical Diagnostic Criteria
TLDR
The diagnosis of MSA represents a major challenge in movement disorders clinics and features overlapping with the clinical presentation of progressive supranuclear palsy (PSP) or dementia with Lewy bodies (DLB) may be also present in MSA.
Definite multiple system atrophy in a German family
TLDR
Severe atrophy of the putamen and depigmentation of the substantia nigra and pontine and cerebellar atrophy were found upon macroscopic examination of the brain.
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References

SHOWING 1-10 OF 18 REFERENCES
What is the accuracy of the clinical diagnosis of multiple system atrophy? A clinicopathologic study.
TLDR
The low sensitivity for the clinical diagnosis of MSA, particularly among neurologists who followed up these patients in the tertiary centers, suggests that this disorder is underdiagnosed.
Multiple system atrophy presenting as parkinsonism: clinical features and diagnostic criteria.
TLDR
No single item could predict whether patients presenting with just parkinsonian signs went on during the two year follow up period to develop fully symptomatic MSA, and the number of abnormalities offered a predictive value for the clinical prognosis of these parkinsonia patients.
Clinical features and natural history of multiple system atrophy. An analysis of 100 cases.
The clinical features and natural history of 100 patients diagnosed as probable multiple system atrophy (MSA) are described. In all 14 (of 41 deceased) cases who underwent post-mortem examination of
Multiple system atrophy: natural history, MRI morphology, and dopamine receptor imaging with 123IBZM-SPECT.
TLDR
MRI and IBZM-SPECT provide in vivo evidence for combined basal ganglia and pontocerebellar involvement in almost all patients in this series.
The natural history of degenerative ataxia: a retrospective study in 466 patients.
TLDR
In FRDA, the time until confinement to wheelchair was shorter in patients with earlier disease onset, suggesting that patients with long GAA repeats and early disease onset have a poor prognosis, and in MSA, later age of onset increased the risk of rapid progression and death.
Clinical research criteria for the diagnosis of progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome)
TLDR
Criteria that support the diagnosis of progressive supranuclear palsy, and that exclude diseases often confused with PSP, are presented.
The dopaminergic response in multiple system atrophy.
TLDR
The occurrence of levodopa-induced dyskinesias without a concomitant motor response and delayed deterioration several days afterlevodopa withdrawal may be more typical of patients with MSA than Parkinson's disease.
Value of sphincter electromyography in the diagnosis of multiple system atrophy
TLDR
A systematic analysis of motor units recorded from the sphincter looking for changes of chronic reinnervation has been used to distinguish between idiopathic Parkinson's disease (IPD) and multiple system atrophy (MSA).
Response to L-DOPA in multiple system atrophy.
TLDR
Motor response was consistently good in all PD patients, but only four MSA patients had a (moderate) response to L-DOPA, and Pharmacokinetic parameters did not differ between the groups.
Clinical usefulness of magnetic resonance imaging in multiple system atrophy
OBJECTIVES To determine the sensitivity, specificity, and positive predictive values of a selection of abnormal findings in the putamen and infratentorial structures on routine magnetic resonance
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