Abstract
Disentangling Parkinson's disease (PD) and progressive supranuclear palsy (PSP) may be a diagnostic challenge. Cognitive signs may be useful, but existing screens are often insufficiently sensitive or unsuitable for assessing people with motor disorders. We investigated whether the newly developed ECAS, designed to be used with people with even severe motor disability, was sensitive to the cognitive impairment seen in PD and PSP and able to distinguish between these two disorders. Thirty patients with PD, 11 patients with PSP, and 40 healthy controls were assessed using the ECAS, as well as an extensive neuropsychological assessment. The ECAS detected cognitive impairment in 30% of the PD patients, all of whom fulfilled the diagnostic criteria for mild cognitive impairment. The ECAS was also able to detect cognitive impairment in PSP patients, with 81.8% of patients performing in the impaired range. The ECAS total score distinguished between the patients with PSP and healthy controls with high sensitivity (91.0) and specificity (86.8). Importantly, the ECAS was also able to distinguish between the two syndromes, with the measures of verbal fluency offering high sensitivity (82.0) and specificity (80.0). In sum, the ECAS is a quick, simple, and inexpensive test that can be used to support the differential diagnosis of PSP.
Highlights
It has been over 50 years since progressive supranuclear palsy (PSP) was first described as a progressive neurological disorder with motor, ocular, and cognitive features [1]
PSP patients had significantly lower total scores than Parkinson’s disease (PD) patients and healthy controls, and PD patients had significantly lower total scores than healthy controls. ere was a significant effect of diagnosis on all domains, except visuospatial
PSP patients performed worse than PD patients and healthy controls on fluency, language, executive function, and memory
Summary
It has been over 50 years since progressive supranuclear palsy (PSP) was first described as a progressive neurological disorder with motor, ocular, and cognitive features [1]. In the absence of any disease-specific biomarkers, there is a need for a quick, simple, and inexpensive test that can be used for the differential diagnosis of PSP Both PSP and PD are characterised by extrapyramidal syndromes, each of which can comprise symptoms of bradykinesia, rigidity, and/or postural instability [7]. Both disorders can feature eye movement abnormalities, and the presence of the supranuclear vertical gaze palsy in PSP is diagnostically helpful, it is not universal [8, 9] and may be absent until quite late in the disease [10].
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