Second consensus statement on the diagnosis of multiple system atrophy

Gilman, Sid; Wenning, Gregor K.; Low, Phillip A.; Brooks, David J.; Mathias, C. J.; Trojanowski, John Q.; Wood, Nicholas W.; Colosimo, Carlo; Dürr, Alexandra; Fowler, Clare J.; Kaufmann, Horacio; Klockgether, Thomas; Lees, Andrew J.; Poewe, Werner; Quinn, Niall; Révész, Tamás; Robertson, David L.; Sandroni, Paola; Seppi, Klaus; Vidailhet, Marie

doi:10.1212/01.wnl.0000324625.00404.15

Cited by 2,689 publications

(2,579 citation statements)

References 53 publications

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“…A diagnosis of PD was made based on the UK PD Society Brain Bank clinical diagnostic criteria (Hughes, Daniel, Kilford, & Lees, 1992). MSA and PSP were diagnosed according to the second consensus statement on the diagnosis of MSA (Gilman et al., 2008) and the diagnostic criteria of the National Institute of Neurological Disorders and Stroke and the Society for PSP (Litvan et al., 1996), respectively. Disease severity was rated using Hoehn and Yahr (HY) staging (Hoehn & Yahr, 1967).…”

Section: Methodsmentioning

confidence: 99%

Serum uric acid levels in Parkinson's disease and related disorders

et al. 2016

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ObjectiveSerum uric acid (UA) levels are reported to be decreased in patients with Parkinson's disease (PD) and multiple system atrophy (MSA). However, clinical correlates of serum UA levels are still unclear in PD‐related disorders. We conducted a cross‐sectional study to evaluate the associations between serum UA levels and disease duration, disease severity, and motor function among PD, MSA, and progressive supranuclear palsy (PSP) patients.MethodsA total of 100 patients with PD, 42 patients with MSA, 30 patients with PSP, and 100 controls were included in this study. Serum UA levels were determined, and associations among serum UA levels and disease duration, disease severity, and motor function in PD, PSP, and MSA patients were evaluated.ResultsSerum UA levels were significantly lower in male PD, MSA, and PSP patients compared with the controls, but not in female patients. Serum UA levels were negatively correlated with disease duration and severity in MSA and PSP patients, but no correlations were observed in PD patients. The serum UA levels were significantly decreased in the tauopathy group (PSP patients) compared with the synucleinopathy group (PD and MSA patients) after adjusting for age, gender, and body mass index.ConclusionWe found decreased serum UA levels in male patients with PD‐related disorders (PD, MSA, and PSP) compared with male controls, and significant correlations between serum UA levels and disease severity in MSA and PSP patients.

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Section: Methodsmentioning

confidence: 99%

Serum uric acid levels in Parkinson's disease and related disorders

et al. 2016

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“…Twenty-one consecutive patients with a clinical diagnosis of PD, defined according to the United Kingdom PD Brain criteria 11 , 11 consecutive patients with MSA-c, eight patients with MSA-p, according to MSA consensus 3 , and 20 patients with clinical criteria for PSP 2 were examined. All patients were clinically evaluated by a neurologist and submitted to brain MRI.…”

Section: Methodsmentioning

confidence: 99%

“…Other characteristics include fast rate of disease progression and poor response to levodopa 1,2 . Multiple system atrophy (MSA) is usually associated with fast rate of disease progression, autonomic dysfunction, cerebellar signs and upper motor neuron signs 3 . In MSA cases, patients have been classified as MSA-c or MSA-p depending on the predominance of cerebellar ataxia or parkinsonian features, with dysautonomia being a constant feature in both subtypes 4 .…”

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confidence: 99%

Morphometry MRI in the differential diagnosis of parkinsonian syndromes

Gama

Távora

Bomfim

et al. 2010

Arq. Neuro-Psiquiatr.

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This study evaluates the diagnostic value of morphometric magnetic resonance imaging (MRI) in the differential diagnosis among Parkinson's disease (PD), progressive supranuclear palsy (PSP) and multiple system atrophy (MSA). We studied 21 PD cases, 11 MSA-c, 8 MSA-p and 20 PSP cases. Midbrain area (Ams), pons area (Apn), middle cerebellar peduncle (MCP) and superior cerebellar peduncle (SCP) were measured using MRI. Comparisons were made between PD, MSA-p, MSA-c and PSP. Apn , MCP and SCP morphometry dimensions presented differences among groups. Ams below 105 mm 2 and SCP smaller than 3 mm were the most predictive measures of PSP (sensitivity 95.0 and 80.0%, respectively). For the group of MSA-c patients, Apn area below 315 mm 2 showed good specificity and positive predictive value (93.8% and 72.7%, respectively). In conclusion, dimensions and cut off values obtained from routine MRI can differentiate between PD, PSP and MSA-c with good sensitivity, specificity and accuracy. Key words: Parkinson, multiple system atrophy, progressive supranuclear palsy, magnetic resonance imaging, pons, midbrain. Morfometria por ressonância magnética no diagnóstico diferencial das síndromes parkinsonianas rEsUMOMorfometria pela ressonância magnética (RM) no diagnóstico diferencial entre doença de Parkinson (DP), paralisia supranuclear progressiva (PSP) e atrofia de múltiplos sistemas (AMS). Este estudo avaliou a RM no diagnóstico diferencial de 21 casos com DP, 11 AMS-c, 8 AMS-p e 20 com PSP. A área sagital do mesencéfalo (Ams), área sagital da ponte (Apn), largura do pedúnculo cerebelar médio (PCM) e pedúnculo cerebelar superior (PCS) foram medidas pela RM e realizadas comparações entre destes pacientes. A Ams <105 mm 2 e a largura média do PCS <3 mm foram preditivas para PSP (sensibilidade de 95,0 e 80,0%, respectivamente). Nos casos de AMS-c a área pontina <315 mm 2 apresentou boa especificidade e valor preditivo positivo para o diagnóstico (93,8% e 72,7%). Em conclusão, as dimensões e valores de cortes obtidos a partir da RM podem diferenciar PD, PSP e

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“…The vast majority of patients have been evaluated at least once by trained movement disorders specialists and the following data has been obtained: gender, age at disease onset, disease duration at the time of SPECT, body side predominance and final clinical diagnosis. Regarding degenerative parkinsonian syndromes, we used the UK Parkinson's Disease Study Brain Bank criteria for PD [15], the second consensus statement for the diagnosis of MSA [16], the National Institute of Neurological Disorders Society (NINDS) clinical research criteria for PSP [17] and the criteria for the diagnosis of CBD proposed by Armstrong et al [18]. Patients were classified as having a non-degenerative parkinsonian syndrome or tremor, whenever they fulfilled the criteria for essential tremor [19], dystonic tremor [20], psychogenic parkinsonism [21] or drug-induced parkinsonism as described in Ref.…”

Section: Patientsmentioning

confidence: 99%

Scan without evidence of dopaminergic deficit: A 10-year retrospective study

Nicastro

Garibotto

Badoud

et al. 2016

Parkinsonism & Related Disorders

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Introduction: 123 I-ioflupane SPECT is a powerful method to assess nigrostriatal dopamine system integrity. Several independent studies have shown that 1e15% of patients with suspected degenerative parkinsonism, mainly PD, have scans without evidence of dopaminergic deficit (SWEDD). It has been proposed that most SWEDD patients either present with a non-degenerative condition mimicking PD, such as atypical tremor or dystonia, or demonstrate an abnormal scan when repeated later. We here hypothesized that scan interpretation methods may also play a crucial yet underestimated role in this issue. Methods: We previously established age-dependent reference values of striatal uptake by analyzing scans from a cohort of patients with non-degenerative conditions. We then studied a large population with well-established degenerative parkinsonism (N ¼ 410, 80% with PD), using identical imaging protocol, to evaluate the prevalence of patients with normal scans based on routine visual assessment. Each scan was eventually reassessed using the same automated method as for controls and a detailed 3D analysis. Results: Ten potential SWEDD cases (2.4%) were identified. However, both reassessment methods independently showed that these scans were all outside reference limits and/or visually abnormal when reexamined carefully, except for one case (0.2%) with corticobasal syndrome. Conclusion: SPECT misinterpretation emerges as an important contributor to the SWEDD population, suggesting that suspected SWEDD cases should prompt not only a serious diagnosis challenge but, equally important, a detailed scan reassessment. True SWEDD cases seem extremely rare in degenerative parkinsonism. We propose that the very concept of SWEDD is more confusing than helpful and should be definitely abandoned.

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Second consensus statement on the diagnosis of multiple system atrophy

Abstract: These new criteria have simplified the previous criteria, have incorporated current knowledge, and are expected to enhance future assessments of the disease.

Cited by 2,689 publications

References 53 publications

Serum uric acid levels in Parkinson's disease and related disorders

Serum uric acid levels in Parkinson's disease and related disorders

Morphometry MRI in the differential diagnosis of parkinsonian syndromes

Scan without evidence of dopaminergic deficit: A 10-year retrospective study

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