New Clinical Guidelines: Distinguishing Variations of Progressive Supranuclear Palsy

The original NINDS-SPSP criteria generally led to a diagnosis 3 to 4 years after onset, based on the appearance of primary symptoms such as supranuclear gaze palsy and frequent falls corresponding to the core domains of ocular motor dysfunction and postural instability. The MDS guidelines added the 2 domains of akinesia and cognitive function and increased the sensitivity of the criteria to allow for diagnosis much sooner in the disease course. “The new diagnostic criteria aim to optimize an early recognition of PSP patients, before massive functional deficits are established, capturing the entire clinical spectrum of the disease,” Dr Höglinger said.

He pointed to a number of symptomatic treatments for PSP that are “moderately effective,” such as levodopa and amantadine for akinesia, amitriptyline and zolpidem for ocular motor symptoms, zolpidem for sleep disturbances, coenzyme Q10 to improve motor and neuropsychological symptoms, and botulinum toxin A for focal dystonia.31 More important, he noted, the new criteria allow for the initiation of treatment in the earliest clinical phase with novel disease-modifying therapies now in development, such as tau antibodies, and can be used in the design of future clinical trials.

Despite the added complexity of the new guidelines, Dr Höglinger suggested that application in clinical settings should only take about 15 minutes for an experienced neurologist, particularly when using a schematic grid such as the one provided in the guidelines article published in Movement Disorders.1

“The major novelty is just the awareness that combinations of clinical features can be associated with a neuropathological diagnosis of PSP, which we did not have on the radar previously,” he explained. The investigative group is already at work on a web-based tool that will enable widespread implementation of the guidelines, which they expect to have available within a year.


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