What Features Improve the Accuracy of the Clinical Diagnosis of Progressive Supranuclear Palsy-parkinsonism (PSP-P)?

Movement Disorders (Impact Factor: 5.68). 02/2010; 25(3):357 - 362. DOI: 10.1002/mds.22977


Progressive supranuclear palsy-parkinsonism (PSP-P) is a primary tauopathy characterised by neurofibrillary degeneration, which is frequently mistaken for Parkinson's disease (PD), multiple system atrophy (MSA), and vascular parkinsonism (VP) at presentation. The aim of this study was to identify particular clinical features (green flags) that may be helpful in differentiating PSP-P from these other disorders. We identified 37 patients with PSP-P from 726 patients archived at the Queen Square Brain Bank. Using a retrospective case notes review the clinical features were compared between the PSP-P group and Lewy body associated parkinsonism (PD, n = 444 and dementia with Lewy bodies (DLB), n = 46), MSA (n = 90), and VP (n = 19), using the χ2-test for proportions for a two-by-two contingency table. The sensitivity, specificity, and positive predictive values (PPV) and negative predictive values (NPV) were calculated for individual clinical features. A specificity of >0.85 or a PPV of >0.85 were considered reliable discriminators. No clinical features were predictive of PSP-P, but late drug induced dyskinesias (specificity 0.92, PPV 0.99), late autonomic dysfunction (specificity 0.94, PPV 0.99) and any visual hallucinations (specificity 0.94, PPV 0.99) were better in distinguishing PD and PSP-P than predicted using operational diagnostic criteria for PD. PSP-P shares many clinical features with PD and DLB, MSA and VP, but visual hallucinations, drug induced dyskinesias and autonomic dysfunction are very uncommon and may be helpful exclusion criteria. © 2010 Movement Disorder Society

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    • "In early studies, researchers argued that relatively more severe DAT reduction in the posterolateral putamen of patients with PD was characteristic, unlike in APS. However, the following studies reported that they could not differentiate PD from APS based on this anterior-posterior gradient and therefore denied that it is a characteristic feature in the differential diagnosis of PD from APS [22]. We could not find any significant difference in the DAT reduction patterns of patients with PD and APS. "
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    ABSTRACT: Purpose It is often difficult to differentiate parkinsonism, especially when patients show uncertain parkinsonian features. We investigated the usefulness of dopamine transporter (DAT) imaging for the differential diagnosis of inconclusive parkinsonism using [18F]FP-CIT PET. Methods Twenty-four patients with inconclusive parkinsonian features at initial clinical evaluation and nine healthy controls were studied. Patients consisted of three subgroups: nine patients whose diagnoses were unclear concerning whether they had idiopathic Parkinson’s disease or drug-induced parkinsonism (‘PD/DIP’), nine patients who fulfilled neither the diagnostic criteria of PD nor of essential tremor (‘PD/ET’), and six patients who were alleged to have either PD or atypical parkinsonian syndrome (‘PD/APS’). Brain PET images were obtained 120 min after injection of 185 MBq [18F]FP-CIT. Imaging results were quantified and compared with follow-up clinical diagnoses. Results Overall, 11 of 24 patients demonstrated abnormally decreased DAT availability on the PET scans, whereas 13 were normal. PET results could diagnose PD/DIP and PD/ET patients as having PD in six patients, DIP in seven, and ET in five; however, the diagnoses of all six PD/APS patients remained inconclusive. Among 15 patients who obtained a final follow-up diagnosis, the image-based diagnosis was congruent with the follow-up diagnosis in 11 patients. Four unsolved cases had normal DAT availability, but clinically progressed to PD during the follow-up period. Conclusion [18F]FP-CIT PET imaging is useful in the differential diagnosis of patients with inconclusive parkinsonian features, except in patients who show atypical features or who eventually progress to PD.
    06/2014; 48(2):106-113. DOI:10.1007/s13139-013-0253-1
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    • "A recent retrospective study showed that PD might mimic PSP in some cases, including eye movement abnormalities and subcortical cognitive dysfunction; the authors concluded that this is an advantage for the correct diagnosis of PSP, and in particular in the identification of cases of PSP-P. They also stated that the patients with the clinical syndromes of DLB and underlying Lewy body pathology are unlikely to be confused with PSP-P because of the early cognitive decline and visual hallucinations that are major components in the clinical diagnostic criteria of DLB [18]. We had a different experience. "
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    ABSTRACT: BACKGROUND: Atypical parkinsonian syndromes are currently divided into two groups based on their pathological appearance: synucleinopathies and tauopathies. Based on recent clinico-pathological studies it is increasingly clear, that some pathological characteristics are shared by both groups. STUDY OBJECTIVE: To describe two pathologically proven cases of tauopathy manifesting in vivo in two typical synucleinopathy phenotypes: multiple system atrophy and dementia with Lewy bodies. PATIENTS AND METHODS: There were 67-year-old woman with a phenotype of multiple system atrophy and a 70-year-old man with a phenotype of dementia with Lewy bodies. The clinical diagnosis was based on the commonly used clinical diagnostic criteria. A detailed neuropathological examination of the brain was conducted post-mortem in both cases. RESULTS: The overall pathological picture corresponded with a rare combination of two neurodegenerative entities: 4R tauopathy (meeting the diagnostic criteria for typical progressive supranuclear palsy) and neocortical stage of Alzheimer's disease. CONCLUSION: The interesting feature in both our cases was the presence of dual pathology: diffuse tauopathy and Alzheimer's pathology. We believe, that our two unique cases should serve as an evidence that tauopathies such as CBS and PSP might mimic practically anything from the family of atypical parkinsonian syndromes, particularly when another concomitant neurodegenerative disease is present.
    Journal of the neurological sciences 04/2013; 329(1-2). DOI:10.1016/j.jns.2013.03.008 · 2.47 Impact Factor
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    • "Richardson syndrome is the most common presentation of progressive supranuclear palsy, but there is increasing recognition of clinical heterogeneity of progressive supranuclear palsy, with some patients presenting with pure akinesia with gait freezing, progressive non-fluent aphasia or even Parkinson's disease (Williams et al., 2005, 2007; Ahmed et al., 2008). Moreover, there are other pathological processes that can produce the Richardson syndrome clinical phenotype, including corticobasal degeneration, Lewy body disease, multiple system atrophy and cerebrovascular disease (Hughes et al., 2002; Josephs and Dickson, 2003; Williams and Lees, 2010). Results of the present study suggest that patients with Richardson syndrome who also have severe cognitive impairment may have underlying pathology of corticobasal degeneration, but confirmation requires additional longitudinal studies of Richardson syndrome coupled with post-mortem examination. "
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    ABSTRACT: Patients with corticobasal degeneration can present with several different clinical syndromes, making ante-mortem diagnosis a challenge. Corticobasal syndrome is the clinical phenotype originally described for corticobasal degeneration, characterized by asymmetric rigidity and apraxia, cortical sensory deficits, dystonia and myoclonus. Some patients do not develop these features, but instead have clinical features consistent with the Richardson syndrome presentation of progressive supranuclear palsy, characterized by postural instability, early unexplained falls, vertical supranuclear gaze palsy, symmetric motor disability and dysphagia. The aim of this study was to identify differences in corticobasal degeneration presenting with corticobasal syndrome (n = 11) or Richardson syndrome (n = 15) with respect to demographic, clinical and neuropathological features. Corticobasal degeneration cases were also compared with patients with pathologically proven progressive supranuclear palsy with Richardson syndrome (n = 15). Cases with corticobasal degeneration, regardless of presentation, shared histopathological and tau biochemical characteristics, but they had differing densities of tau pathology in neuroanatomical regions that correlated with their clinical presentation. In particular, those with corticobasal syndrome had greater tau pathology in the primary motor and somatosensory cortices and putamen, while those with Richardson syndrome had greater tau pathology in limbic and hindbrain structures. Compared with progressive supranuclear palsy, patients with corticobasal degeneration and Richardson syndrome had less neuronal loss in the subthalamic nucleus, but more severe neuronal loss in the medial substantia nigra and greater atrophy of the anterior corpus callosum. Clinically, they had more cognitive impairment and frontal behavioural dysfunction. The results suggest that Richardson syndrome can be a clinicopathological presentation of corticobasal degeneration. Atrophy of anterior corpus callosum may be a potential neuroimaging marker to differentiate corticobasal degeneration from progressive supranuclear palsy in patients with Richardson syndrome.
    Brain 09/2011; 134(Pt 11):3264-75. DOI:10.1093/brain/awr234 · 9.20 Impact Factor
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