Behavioral Changes as the Earliest Clinical Manifestation of Progressive Supranuclear Palsy

Han, Hyun Jeong; Kim, Hyeyun; Park, Jong Ho; Shin, Hyung Woo; Kim, Go-Eun; Kim, Dong Sun; Lee, Eun Ja; Oh, Hidemasa; Park, Seung Hye; Kim, Yun Jung

doi:10.3988/jcn.2010.6.3.148

Cited by 20 publications

(20 citation statements)

References 10 publications

(12 reference statements)

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“…Therefore, most of these cases were not identified early (or at all) for the purposes of routine clinical care, standardized acquisition of natural history data, or inclusion in therapeutic trials. Our proposed criteria overcome these limitations by providing evidence‐ and consensus‐based guidelines to diagnose PSP‐OM, PSP‐PI, PSP‐P, PSP‐F, PSP‐CBS, PSP‐PGF, and PSP‐SL …”

Section: Discussionmentioning

confidence: 99%

“…Patients with autopsy‐confirmed PSP have been reported with variant PSP clinical presentations, including initial predominance of ocular motor dysfunction (PSP‐OM), postural instability (PSP‐PI), Parkinsonism resembling idiopathic Parkinson's disease (PSP‐P), frontal lobe cognitive or behavioral presentations (PSP‐F), including behavioral variant frontotemporal dementia (bvFTD), progressive gait freezing (PSP‐PGF), corticobasal syndrome (PSP‐CBS), primary lateral sclerosis (PSP‐PLS), cerebellar ataxia (PSP‐C), and speech/language disorders (PSP‐SL), including nonfluent/agrammatic primary progressive aphasia (nfaPPA) and progressive apraxia of speech (AOS) . Patients with presentations other than PSP‐RS occurred in 76% of autopsy‐confirmed PSP cases in a recent series and met the NINDS‐SPSP criteria at significantly lower frequencies and longer latencies from symptom onset …”

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confidence: 99%

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Clinical diagnosis of progressive supranuclear palsy: The movement disorder society criteria

et al. 2017

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Background PSP is a neuropathologically defined disease entity. Clinical diagnostic criteria, published in 1996 by the National Institute of Neurological Disorders and Stroke/Society for PSP, have excellent specificity, but their sensitivity is limited for variant PSP syndromes with presentations other than Richardson’s syndrome. Objective We aimed to provide an evidence- and consensus-based revision of the clinical diagnostic criteria for PSP. Methods We searched the PubMed, Cochrane, Medline, and PSYCInfo databases for articles published in English since 1996, using postmortem diagnosis or highly specific clinical criteria as the diagnostic standard. Second, we generated retrospective standardized clinical data from patients with autopsy-confirmed PSP and control diseases. On this basis, diagnostic criteria were drafted, optimized in two modified Delphi evaluations, submitted to structured discussions with consensus procedures during a 2-day meeting, and refined in three further Delphi rounds. Results Defined clinical, imaging, laboratory, and genetic findings serve as mandatory basic features, mandatory exclusion criteria, or context-dependent exclusion criteria. We identified four functional domains (ocular motor dysfunction, postural instability, akinesia, and cognitive dysfunction) as clinical predictors of PSP. Within each of these domains, we propose three clinical features that contribute different levels of diagnostic certainty. Specific combinations of these features define the diagnostic criteria, stratified by three degrees of diagnostic certainty (probable PSP, possible PSP, and suggestive of PSP). Clinical clues and imaging findings represent supportive features. Conclusions Here, we present new criteria aimed to optimize early, sensitive, and specific clinical diagnosis of PSP on the basis of currently available evidence.

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Section: Discussionmentioning

confidence: 99%

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confidence: 99%

Clinical diagnosis of progressive supranuclear palsy: The movement disorder society criteria

et al. 2017

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“…The literature from the past 20 years emphasizes vPSP syndromes other than PSP-RS: definite PSP patients were reported in small series with initial predominance of ocular motor dysfunction, 4 PI, 4,46 parkinsonism resembling idiopathic PD, 10,33 frontal lobe cognitive or behavioral presentations, 18,23,24 progressive gait freezing, 16,19,34,47 speech/language disorders, including nonfluent/agrammatic variant of primary progressive aphasia (nfaPPA), 15,22,31 and progressive apraxia of speech (AOS), 22,48 corticobasal syndrome, 14,27,49 primary lateral sclerosis, 32 or cerebellar ataxia. 50,51 A broad spectrum of diseases other than PSP have been clinically reported to present similarly to PSP (PSP look-alikes) and need to be considered as differential diagnosis (Supplemental Table 1).…”

Section: Systematic Literature Reviewmentioning

confidence: 99%

Which ante mortem clinical features predict progressive supranuclear palsy pathology?

et al. 2017

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Background Progressive supranuclear palsy (PSP) is a neuropathologically defined disease presenting with a broad spectrum of clinical phenotypes. Objective To identify clinical features and investigations that predict or exclude PSP pathology during life, aiming at an optimization of the clinical diagnostic criteria for PSP. Methods We performed a systematic review of the literature published since 1996 to identify clinical features and investigations that may predict or exclude PSP pathology. We then extracted standardized data from clinical charts of patients with pathologically diagnosed PSP and relevant disease controls, and calculated sensitivity, specificity and positive predictive value of key clinical features for PSP in this cohort. Results Of 4166 articles identified by database inquiry, 269 met predefined standards. The literature review identified clinical features predictive of PSP, including features of the 4 functional domains, i.e., “ocular motor dysfunction”, “postural instability”, “akinesia”, and “cognitive dysfunction”. No biomarker or genetic feature was found reliably validated to predict definite PSP. High-quality original natural history data were available from 206 patients with pathologically diagnosed PSP and from 231 pathologically diagnosed disease controls (54 corticobasal degeneration, 51 multiple system atrophy with predominant parkinsonism, 53 Parkinson's disease, 73 behavioral variant frontotemporal dementia). We identified clinical features that predicted PSP pathology, including phenotypes other than Richardson's syndrome, with varying sensitivity and specificity. Conclusions Our results highlight the clinical variability of PSP and the high prevalence of phenotypes other than Richardson's syndrome. Features of variant phenotypes with high specificity and sensitivity should serve to optimize clinical diagnosis of PSP.

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“…In 1963, J.C. Steele, J.C. Richardson, and J. Olszewski described eight cases of progressive supranuclear palsy ( PSP) with a clinical syndrome, now termed Richardson's syndrome (RS) . Several atypical phenotypes have been described since then . In 2005, a single‐center systematic analysis of 103 definite PSP cases highlighted a second distinct phenotype, PSP with parkinsonism (PSP‐P) .…”

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confidence: 99%

The phenotypic spectrum of progressive supranuclear palsy: A retrospective multicenter study of 100 definite cases

et al. 2014

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The phenotypic variability of progressive supranuclear palsy (PSP) may account for its frequent misdiagnosis, in particular in early stages of the disease. However, large multicenter studies to define the frequency and natural history of PSP phenotypes are missing. In a cohort of 100 autopsy-confirmed patients we studied the phenotypic spectrum of PSP by retrospective chart review. Patients were derived from five brain banks with expertise in neurodegenerative disorders with referrals from multiple academic hospitals. The clinical characteristics of the 100 cases showed remarkable heterogeneity. Most strikingly, only 24% of cases presented as Richardson's Syndrome (RS), and more than half of the cases either showed overlapping features of several predescribed phenotypes, or features not fitting proposed classification criteria for PSP phenotypes. Classification of patients according to predominant clinical features in the first 2 years of the disease course allowed a more comprehensive description of the phenotypic spectrum. These predominance types differed significantly with regard to survival time and frequency of cognitive deficits. In summary, the phenotypic spectrum of PSP may be broader and more variable than previously described in single-center studies. Thus, too strict clinical criteria defining distinct phenotypes may not reflect this variability. A more pragmatic clinical approach using predominance types could potentially be more helpful in the early recognition of and for making prognostic predictions for these patients. Given the limitations arising from the retrospective nature of this analysis, a systematic validation in a prospective cohort study is imperative.

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Behavioral Changes as the Earliest Clinical Manifestation of Progressive Supranuclear Palsy

Cited by 20 publications

References 10 publications

Clinical diagnosis of progressive supranuclear palsy: The movement disorder society criteria

Clinical diagnosis of progressive supranuclear palsy: The movement disorder society criteria

Which ante mortem clinical features predict progressive supranuclear palsy pathology?

The phenotypic spectrum of progressive supranuclear palsy: A retrospective multicenter study of 100 definite cases

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