Consensus statement on the diagnosis of multiple system atrophy

Gilman, Sid; Low, Phillip A.; Quinn, Niall; Albanese, Alberto; Ben‐Shlomo, Yoav; Fowler, Clare J.; Kaufmann, Horacio; Klockgether, Thomas; Lang, Anthony E.; Lantos, Peter L.; Litvan, Irene; Mathias, Christopher J.; Oliver, Eugene; Robertson, David L.; Schatz, Irwin J.; Wenning, Gregor K.

doi:10.1016/s0022-510x(98)00304-9

Cited by 1,078 publications

(496 citation statements)

References 20 publications

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“…In 154 of the 248 cases, the investigators reached consensus and were certain enough to make a final diagnosis from the clinical records alone, according to generally accepted criteria [3, 8,25,26,27,28]. Of the remaining 94 patients, 5 patients had died, 2 could not be traced and 2 lived outside the district.…”

Section: Resultsmentioning

confidence: 99%

“…checked each patient record and filled in a standard form. The investigators established a clinical diagnosis according to generally accepted clinical criteria [3, 8,25,26,27,28]. If the investigators did not reach consensus, or if the patient did not fulfill the accepted criteria for a final diagnosis, a movement disorder specialist neurologist (A.W., W.W.) was asked for advice.…”

Section: Methodsmentioning

confidence: 99%

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Diagnostic Value of 123I-Ioflupane and 123I-Iodobenzamide SPECT Scans in 248 Patients with Parkinsonian Syndromes

et al. 2008

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Background: SPECT is one of the most employed techniques in the diagnostic workup of idiopathic Parkinson’s disease (IPD). Despite its widespread use, the exact diagnostic accuracy of this technique in parkinsonian syndromes remains controversial. Methods: In this study, we investigated the diagnostic accuracy of an initial ¹²³I-ioflupane (FP-CIT) and/or ¹²³I-iodobenzamide (IBZM) SPECT to differentiate between IPD and other parkinsonian disorders. 248 patients underwent a SPECT scan because of an as yet unclassified parkinsonian syndrome in our clinic between 2001 and 2006. Gold standard was the clinical diagnosis derived from the latest available clinical record, or, when this was not possible, a new complete physical and neurological examination by a blinded movement disorder specialist neurologist. Mean follow-up between SPECT and the latest clinical information was 18 months (range 3 months to 5 years). Results: 223 of the 248 patients were clinically definitely diagnosed after follow-up: IPD 127, atypical parkinsonian syndromes (APS) 27, essential tremor (ET) 22, vascular parkinsonism (VP) 16, drug-induced parkinsonism (DIP) 5, doubt between PD and APS 2, other diseases without dopaminergic involvement 24. The mean odds ratio (95% CI) for FP-CIT SPECT’s ability to distinguish between IPD and ET was 82 (11–674); between IPD and VP 61 (8–490); between IPD and DIP 36 (2–697) and between IPD and APS was 1 (0–4). The odds ratio for the IBZM SPECT tracer to differentiate between IPD and APS was 7 (2–17). Conclusions: FP-CIT SPECT is accurate to differentiate patients with IPD from those with ET, and IPD from VP and DIP. The accuracy of both FP-CIT and IBZM SPECT scans to differentiate between IPD and APS is low.

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Section: Resultsmentioning

confidence: 99%

Section: Methodsmentioning

confidence: 99%

Diagnostic Value of 123I-Ioflupane and 123I-Iodobenzamide SPECT Scans in 248 Patients with Parkinsonian Syndromes

et al. 2008

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“…Diagnostic criteria for MSA proposed by a Consensus Conference in 1998 [81] recommend designating patients as having MSA-P if parkinsonian features are predominate or MSA-C if cerebellar features predominate.…”

Section: Multiple System Atrophymentioning

confidence: 99%

“…Although dementia consistent with the Diagnostic and Statistical Manual of Mental Disorders (DSM IV) is an exclusion criterion for the diagnosis of MSA [81], some studies have reported that MSA patients have a cognitive decline when compared with controls [82,83,84,85,86,87,88]. MSA-P is the more common manifestation of MSA in Western populations [89, 90]; therefore, cognitive dysfunction in MSA-P had attracted considerable attention, particularly in comparison with PD and/or progressive supranuclear palsy [82,83,84,86,87,88].…”

Section: Multiple System Atrophymentioning

confidence: 99%

Cognitive Impairment in Spinocerebellar Degeneration

et al. 2009

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It has been reported that patients with spinocerebellar degenerations (SCDs) have cognitive dysfunction as well as limb and truncal ataxia, dysarthria and dysphagia. We review cognitive dysfunction in common types of SCD, including spinocerebellar ataxia types 1, 2, 3, 6, and 17, dentatorubral-pallidoluysian atrophy, Friedreich’s ataxia, and multiple system atrophy. There are few studies that address cognitive function in SCD. Although there are few comparison studies among the various SCDs, cognitive dysfunction may be more common and severe in spinocerebellar ataxia type 17 and dentatorubral-pallidoluysian atrophy. While cognitive dysfunction in SCD appears to represent frontal dysfunction, the mechanisms of cognitive dysfunction have not been directly clarified. Nevertheless, various lesions, including those in the cerebrocerebellar circuitry, cortico-striatal-thalamocortical circuitry, and the frontal lobe, may influence cognitive function to various degrees for each disease.

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“…Briefly, tissue blocks of cingulate cortex from DLB or cerebellum from MSA brains were fixed with 70% ethanol, 150 mM NaCl or neutral buffered formalin and infiltrated with paraffin. The diagnostic assessment of all DLB and MSA cases (both of which are ␣-synucleinopathies characterized by ␣-synuclein inclusions) was performed in accordance with published guidelines (26,37). Whole mouse brains (wild type or parkin null) were fixed with 70% ethanol, 150 mM NaCl and paraffin-embedded.…”

Section: Immunofluorescence and Immunohistochemistrymentioning

confidence: 99%

Novel Monoclonal Antibodies Demonstrate Biochemical Variation of Brain Parkin with Age

Pawlyk

Giasson

Sampathu

et al. 2003

Journal of Biological Chemistry

145

117

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Autosomal recessive juvenile parkinsonism is a movement disorder associated with the degeneration of dopaminergic neurons in substantia nigra pars compacta. The loss of functional parkin caused by parkin gene mutations is the most common single cause of juvenile parkinsonism. Parkin has been shown to aid in protecting cells from endoplasmic reticulum and oxidative stressors presumably due to ubiquitin ligase activity of parkin that targets proteins for proteasomal degradation. However, studies on parkin have been impeded because of limited reagents specific for this protein. Here we report the generation and characterization of a panel of parkin-specific monoclonal antibodies. Biochemical analyses indicate that parkin is present only in the high salt-extractable fraction of mouse brain, whereas it is present in both the high salt-extractable and RIPA-resistant, SDS-extractable fraction in young human brain. Parkin is present at decreased levels in the high salt-extractable fraction and at increased levels in the SDS-extractable fraction from aged human brain. This shift in the extractability of parkin upon aging is seen in humans but not in mice, demonstrating species-specific differences in the biochemical characteristics of murine versus human parkin. Finally, by using these highly specific anti-parkin monoclonal antibodies, it was not possible to detect parkin in ␣-synuclein-containing lesions in ␣-synucleinopathies, thereby challenging prior inferences about the role of parkin in movement disorders other than autosomal recessive juvenile parkinsonism. Autosomal recessive juvenile parkinsonism (AR-JP)1 is an especially insidious form of parkinsonism that can strike as early as the 1st decade of life. A major locus for this disease was mapped to chromosome 6q, and the gene was subsequently identified and termed parkin (1). Human parkin is a 465-amino acid protein with a predicted molecular mass of 52 kDa that contains an N-terminal ubiquitin-like domain, a linker region, and a C-terminal TRIAD domain consisting of two RING fingers on either side of an in-between RING (IBR) finger region (2). Deletions and insertions of one or more exons resulting in premature translation termination are some of the most common mutations in parkin, but numerous missense point mutations in parkin have also been shown to be causal of AR-JP (3). Since the identification of parkin, many studies focused on elucidating the function of this protein, and it has been shown it can function as a ubiquitin-protein isopeptide ligase and that its overexpression in vivo enhances the ubiquitinylation of synphilin-1, Pael-R, and CDCRel-1. Immunoprecipitated parkin has been reported to catalyze the ubiquination of these substrates in vitro as well as O-glycosylated ␣-synuclein p22 and cyclin E (4 -8). Furthermore, mutations linked to AR-JP have been reported to reduce the ubiquination of these substrates. Moreover, emerging data suggest that parkin may protect cells from premature death by targeting misfolded or damaged proteins for degradation ...

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Consensus statement on the diagnosis of multiple system atrophy

Cited by 1,078 publications

References 20 publications

Diagnostic Value of <sup>123</sup>I-Ioflupane and <sup>123</sup>I-Iodobenzamide SPECT Scans in 248 Patients with Parkinsonian Syndromes

Diagnostic Value of <sup>123</sup>I-Ioflupane and <sup>123</sup>I-Iodobenzamide SPECT Scans in 248 Patients with Parkinsonian Syndromes

Cognitive Impairment in Spinocerebellar Degeneration

Novel Monoclonal Antibodies Demonstrate Biochemical Variation of Brain Parkin with Age

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