What Features Improve the Accuracy of the Clinical Diagnosis of Progressive Supranuclear Palsy-parkinsonism (PSP-P)?
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
[Show abstract] [Hide abstract] ABSTRACT: Background: Autonomic urinary dysfunction affects patients with progressive supranuclear palsy (PSP); however, the severity and prevalence of urinary dysfunctions in these patients compared with those observed in patients with Parkinson's disease (PD) and multiple system atrophy (MSA) are unknown. Objective: We compared urinary dysfunction characteristics in patients with PSP, PD, and MSA. Patients and methods: Forty-seven patients who satisfied the probable or possible criteria of the National Institute for Neurological Diseases and Stroke and Society for PSP were assessed using the urinary symptoms questionnaire and the urodynamic study at Chiba and Toho Universities (n = 26 and 21, respectively). The results were compared with those of patients with PD and MSA (n = 218 and 193, respectively). Results: The mean disease duration of PSP and the mean age were 2.97 ± 0.26 and 71.4 ± 0.88 years, respectively. The mini-mental state examination and frontal assessment battery scores were 22.6 ± 0.70 and 10.7 ± 0.49, respectively. Urinary storage and voiding symptoms were observed in 57% and 56% of patients with PSP, respectively. Detrusor overactivity in the urodynamic study was detected in 81% of patients with PSP, which was slightly more than that found in patients with PD (69%) and MSA (67%); however, this was not statistically significant. Postvoid residual volume in patients with PSP was significantly more than that in patients with PD (P < 0.01), but was equivalent to that in patients with MSA. Conclusions: The present study demonstrated that patients with PSP experienced various urinary dysfunctions. Urinary storage dysfunction in patients with PSP was not different from that in patients with PD or MSA, whereas urinary voiding dysfunction in patients with PSP was milder than that in patients with MSA and more severe than that in patients with PD. These features should be taken into account for the differentiation of PSP from PD and MSA.0Comments 0Citations
- "Although it is plausible that urinary storage symptoms might be caused by dysfunction of the frontal cortex which inhibit the voiding reflex during the storage phase, frontal cortical dysfunction is unlikely the sole cause of urinary voiding dysfunction. Neurodegeneration in the brain stem, a commonly observed pathology in patients with PSP [1, 2] , might also be contributing to urinary voiding dysfunction in these patients. These potential mechanisms need to be further investigated in future studies. "
[Show abstract] [Hide abstract] ABSTRACT: Multiple system atrophy (MSA) is a relentless progressive disorder without effective treatment. Its accurate diagnosis is important for the management of individual patients and for the development of new therapeutic strategies. However, there are many disorders which can mimic MSA (so-called 'MSA look-alikes'), and the true rate for over- or under-diagnoses of MSA is not known, especially during the early course of disease when the disease is not fully developed yet. Herein, the authors review the neurodegenerative, genetic, and immunologic conditions that can mimic MSA and thus be part of the differential diagnosis of MSA. Clinicians should be aware of these conditions and be able to differentiate them by clinical features and laboratory findings.0Comments 1Citation
- "Especially, patients with a parkinsonian variant of PSP (PSP-P) may present with normal eye movements initially , complicating the correct diagnosis when symptoms of autonomic failure are present. Although it has been reported that true autonomic dysfunction is uncommon in PSP-P  , urinary incontinence due to reduced mobility can be present . PSP with prominent cerebellar ataxia ('PSP-C') may be misdiagnosed as MSA-C [5,16]. "
[Show abstract] [Hide abstract] 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.0Comments 4Citations
- "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 . We could not find any significant difference in the DAT reduction patterns of patients with PD and APS. "