Objective: To describe the frequency, phenomenology, and clinical and neuropsychological correlates of stereotypies in PSP.

Background: Stereotypies are repetitive, patterned and predictable purposeless movements. Unlike tics or compulsions, they are not done to relieve obsessive thoughts, and are not related to underlying urge(1). Frontotemporal dementia is the most commonly reported neurodegenerative disease to cause stereotypies(1). Very few studies have analyzed the frequency of stereotypies in PSP, and no detailed description of the phenomenon, its clinical correlates, and potential usefulness for PSP diagnosis has been published(2).

Methods: Prospective recruitment of PSP patients diagnosed at a Movement Disorders Unit according to current PSP-MDS criteria(5). Comprehensive neurological examination searching for motor and verbal stereotypies, environmental dependency syndrome, and oculomotor disturbances was performed. Stereotypies were also assessed using the Stereotypy Rating Inventory (SRI). We assessed disease severity by PSPRS, neuropsychiatry by Frontal Behavioral Inventory, and cognition by a comprehensive neuropsychological battery.

Results: Thirty PSP patients (age 74±9 y.; 65% women; disease duration 3.8±2 y.; 65% RS, 20% PGF, 13% SL, 7% PSP-P) were recruited. Motor stereotypies (MS) were found in 66.6% (20/30) of patients, showing a similar and distinctive pattern between them. In all cases, MS developed within the first 3 years from disease onset. The most commonly observed MS were: rubbing or touching repeatedly their own face (cheeks, forehead, nose), thighs, or hands fingers, or touching repeatedly nearby objects (furniture borders, clothes). Verbal stereotypies were found in 16%, and a positive applause sign in only 33.3% of patients. The presence of “greed for food” (50%) was the only alteration in eating behavior. Patients with MS did not differ in age or disease duration. No difference in MS frequency or phenomenology was observed between PSP phenotypes. MS correlated with greater PSPRS-postural instability score (0.007), higher “greed for food” frequency (0.003), and better Buschke immediate (0.02) and delayed (0.02) recall scores.

Conclusions: 1. We describe for the first time the clinical phenotype of motor stereotypies in PSP. 2. Motor stereotypies are frequent and present from the early stages of the disease, suggesting that their screening could be useful for early differential diagnosis of parkinsonian syndromes.

References: 1. Mateen FJ, Josephs KA. The clinical spectrum of stereotypies in frontotemporal lobar degeneration. Mov Disord. 2009; 24: 1237–40. 2. Yatabe Y, Hashimoto M, Kaneda K, Honda K, Ogawa Y, Yuuki S, et al. Neuropsychiatric symptoms of progressive supranuclear palsy in a dementia clinic. Psychogeriatrics. 2011;11:54-9.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/the-clinical-spectrum-of-motor-stereotypies-in-progressive-supranuclear-palsy-an-early-frequent-and-underestimated-symptom/