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The reliability of the Progressive Supranuclear Palsy Rating Scale (PSPRS) using teleneurology has not been assessed.To test whether removing items inadequately assessed by video would impact measurement of PSP severity and progression.We performed secondary analyses of two data sets: the phase 2/3 trial of Davunetide in PSP and a large single-center cohort. We examined two modifications of the PSPRS: (1) removing neck rigidity, limb rigidity, and postural stability (25 items; mPSPRS-25) and (2) also removing three ocular motor items and limb dystonia (21 items; mPSPRS-21). Proportional agreement relative to the possible total scores was measured using the intraclass correlation coefficient, compared to the original PSPRS baseline values and change over 6 and 12 months. We examined the ability of both scales to predict survival in the single-center cohort using proportional hazards models.The mPSPRS-25 showed excellent agreement (0.99; P 0.001) with the original PSPRS at baseline, 0.98 (P 0.001) agreement in measuring change over 6 months, and 0.98 (P 0.001) over 12 months. The mPSPRS-21 showed agreement of 0.94 (P 0.001) with the original PSPRS at baseline, 0.92 (P 0.001) at 6 months, and 0.95 (P 0.001) at 12 months. Baseline and 6-month change in both modified scales were highly predictive of survival in the single-center cohort.Modified versions of the PSPRS which can be administered remotely show excellent agreement with the original scale and predict survival in PSP. The mPSPRS-21 should facilitate clinical care and research in PSP via teleneurology. © 2022 International Parkinson and Movement Disorder Society.

Given the past two decades of over 40 failed trials of amyloid-lowering therapies in Alzheimer's Disease (AD), many of which succeeded in lowering amyloid as designed, we present an ethical argument for emptying the drug pipeline of tests of amyloid-lowering agents so as to end the historical dominance of the amyloid-reducing therapeutic approach in AD.

BACKGROUND: The reliability of the Progressive Supranuclear Palsy Rating Scale (PSPRS) using teleneurology has not been assessed. OBJECTIVES: To test whether removing items inadequately assessed by video would impact measurement of PSP severity and progression. METHODS: We performed secondary analyses of two data sets: the phase 2/3 trial of Davunetide in PSP and a large single-center cohort. We examined two modifications of the PSPRS: (1) removing neck rigidity, limb rigidity, and postural stability (25 items; mPSPRS-25) and (2) also removing three ocular motor items and limb dystonia (21 items; mPSPRS-21). Proportional agreement relative to the possible total scores was measured using the intraclass correlation coefficient, compared to the original PSPRS baseline values and change over 6 and 12 months. We examined the ability of both scales to predict survival in the single-center cohort using proportional hazards models. RESULTS: The mPSPRS-25 showed excellent agreement (0.99; P < 0.001) with the original PSPRS at baseline, 0.98 (P < 0.001) agreement in measuring change over 6 months, and 0.98 (P < 0.001) over 12 months. The mPSPRS-21 showed agreement of 0.94 (P < 0.001) with the original PSPRS at baseline, 0.92 (P < 0.001) at 6 months, and 0.95 (P < 0.001) at 12 months. Baseline and 6-month change in both modified scales were highly predictive of survival in the single-center cohort. CONCLUSIONS: Modified versions of the PSPRS which can be administered remotely show excellent agreement with the original scale and predict survival in PSP. The mPSPRS-21 should facilitate clinical care and research in PSP via teleneurology.

Clinical case studies are fundamental in cementing theoretical training, especially for neurological disorders where diagnosis can be difficult. This book describes a variety of clinical scenarios associated with either the misdiagnosis or incorrect management of cognitive and behavioral neurological syndromes, identifying common pitfalls, which are discussed in detail. Each case emphasizes the importance of information derived from the patient's history and physical examination in forming a correct diagnosis. Focusing on disorders and presentations that are a frequent source of confusion, key diagnostic principles are illustrated clearly. Questions to the reader move the narrative along logically, whilst highlighting specific aspects of clinical presentation that lead to the correct diagnosis. Videos of patients connect readers to the cases and demonstrate how to avoid diagnostic pitfalls. An online version of the book can be accessed on Cambridge Core, via the code printed on the inside of the cover.

BackgroundThe Parkinson's Progression Markers Initiative (PPMI) is an ongoing observational, longitudinal cohort study of participants with Parkinson's disease, healthy controls, and carriers of the most common Parkinson's disease-related genetic mutations, which aims to define biomarkers of Parkinson's disease diagnosis and progression. All participants are assessed annually with a battery of motor and non-motor scales, 123-I Ioflupane dopamine transporter (DAT) imaging, and biological variables. We aimed to examine whether non-manifesting carriers of LRRK2 and GBA mutations have prodromal features of Parkinson's disease that correlate with reduced DAT binding.MethodsThis cross-sectional analysis is based on assessments done at enrolment in the subset of non-manifesting carriers of LRRK2 and GBA mutations enrolled into the PPMI study from 33 participating sites worldwide. The primary objective was to examine baseline clinical and DAT imaging characteristics in non-manifesting carriers with GBA and LRRK2 mutations compared with healthy controls. DAT deficit was defined as less than 65% of putamen striatal binding ratio expected for the individual's age. We used t tests, χ2 tests, and Fisher's exact tests to compare baseline demographics across groups. An inverse probability weighting method was applied to control for potential confounders such as age and sex. To account for multiple comparisons, we applied a family-wise error rate to each set of analyses. This study is registered with ClinicalTrials.gov, number NCT01141023.FindingsBetween Jan 1, 2014, and Jan 1, 2019, the study enrolled 208 LRRK2 (93% G2019S) and 184 GBA (96% N370S) non-manifesting carriers. Both groups were similar with respect to mean age, and about 60% were female. Of the 286 (73%) non-manifesting carriers that had DAT imaging results, 18 (11%) LRRK2 and four (3%) GBA non-manifesting carriers had a DAT deficit. Compared with healthy controls, both LRRK2 and GBA non-manifesting carriers had significantly increased mean scores on the Movement Disorders Society Unified Parkinson's Disease Rating Scale (total score 4·6 [SD 4·4] healthy controls vs 8·4 [7·3] LRRK2 vs 9·5 [9·2] GBA, p

The diagnosis of psychogenic movement disorders (PMDs) may be challenging, given the lack of accurate and reliable tests.1 Two sets of clinical criteria, the Fahn-Williams (FW)2 and Shill-Gerber (SG)3 criteria, have been developed to assist in the diagnostic process and avoid the diagnosis-by-exclusion approach to PMD. Reliability and applicability of these criteria are essential because misdiagnosis may delay the initiation of appropriate treatment, leading to poor outcomes.4 We measured interobserver diagnostic agreement and clinical certainty judgment (according to FW and SG criteria) of PMD among movement disorder specialists (MDS) and general neurologists (non-MDS). We also examined whether diagnostic agreement based exclusively on motor phenomenology was satisfactory.

This is the visual CC BY 4.0 interpretation and simplificationby Marina Noordegraaf ofthe article byMalmberg, M., Malm, T., Gustafsson, O., Sturchio, A., Graff, C., Espay, A.J., Wright, A.P., El Andaloussi, S., Lindén, A., Ezzat, K. (2020) Disentangling the amyloid pathways: A mechanistic approach to etiology, Frontiers in Neuroscience, vol.14, p 256. https://doi.org/10.3389/fnins.2020.00256 Apart from the poster/infographic, the 7 images which constitute the poster can be downloaded on its own as .png. The poster and/or images can be cited as indicated.&nbsp