Best Practices in the Clinical Management of Progressive Supranuclear Palsy and Corticobasal Syndrome: A Consensus Statement of the CurePSP Centers of Care.

Progressive supranuclear palsy (PSP) and corticobasal syndrome (CBS; the most common phenotype of corticobasal degeneration) are tauopathies with a relentless course, usually starting in the mid-60s and leading to death after an average of 7 years. There is as yet no specific or disease-modifying treatment. Clinical deficits in PSP are numerous, involve the entire neuraxis, and present as several discrete phenotypes. They center on rigidity, bradykinesia, postural instability, gait freezing, supranuclear ocular motor impairment, dysarthria, dysphagia, incontinence, sleep disorders, frontal cognitive dysfunction, and a variety of behavioral changes. CBS presents with prominent and usually asymmetric dystonia, apraxia, myoclonus, pyramidal signs, and cortical sensory loss. The symptoms and deficits of PSP and CBS are amenable to a variety of treatment strategies but most physicians, including many neurologists, are reluctant to care for patients with these conditions because of unfamiliarity with their multiplicity of interacting symptoms and deficits. CurePSP, the organization devoted to support, research, and education for PSP and CBS, created its CurePSP Centers of Care network in North America in 2017 to improve patient access to clinical expertise and develop collaborations. The directors of the 25 centers have created this consensus document outlining best practices in the management of PSP and CBS. They formed a writing committee for each of 12 sub-topics. A 4-member Steering Committee collated and edited the contributions. The result was returned to the entire cohort of authors for further comments, which were considered for incorporation by the Steering Committee. The authors hope that this publication will serve as a convenient guide for all clinicians caring for patients with PSP and CBS and that it will improve care for patients with these devastating but manageable disorders.

Minimal clinically important difference of the functional gait assessment in older adults.

BACKGROUND: The Functional Gait Assessment (FGA) is commonly used to measure walking balance. The minimal clinically important difference (MCID) has yet to be determined for the FGA. OBJECTIVE: The purposes of this study were to determine: (1) the MCID in the FGA for older community-dwelling adults relative to patients' and physical therapists' estimates of change and (2) the extent of agreement between patients' and physical therapists' estimates of change. DESIGN: This study was a prospective case series. METHODS: Patients and physical therapists rated the amount of change in balance while walking after an episode of physical therapy for balance retraining on a 15-point global rating of change (GROC) scale. Weighted kappa statistics were calculated to express agreement between patients' and physical therapists' GROC ratings. Functional Gait Assessment change scores were plotted on receiver operating characteristic curves. A cutoff of +3 on the GROC was the criterion used for important change. The optimal FGA change cutoff score for MCID was determined, and sensitivity (SN), specificity (SP), and likelihood ratios (LRs) were calculated. RESULTS: One hundred thirty-five community-dwelling older adults (average age=78.8 years) and 14 physical therapists participated. There was poor agreement between the patients' and therapists' ratings of change (weighted kappa=.163). The estimated MCID value for the FGA using physical therapists' ratings of change as an anchor was 4 points (SN=0.66, SP=0.84, LR+=4.07, LR-=0.40). No accurate value for the FGA MCID could be determined based on the patients' ratings of change. LIMITATIONS: The small sample size was a limitation. CONCLUSION: Poor agreement between therapists' and patients' ratings indicate the need for further communication relative to patient goals. The 4-point MCID value for the FGA can be used for goal setting, tracking patient progress, and program evaluation.