Extrapyramidal symptoms in frontotemporal dementia

ANABEL CHADE; MARÍA ROCA; EZEQUIEL GLEICHGERRCHT; TERESA TORRALVA; FACUNDO MANES

Buenos Aires

Congreso; International Congress of Parkinson's Disease and Movement Disorders; 2010

The International Parkinson and Movement Disorder Society (MDS)

Objective: To evaluate the prevalence of parkinsonism in patients with behavioral variant frontotemporal dementia (bvFTD) and primary progressive aphasia (PPA). Background: FTD is pathologically heterogeneous, both macroscopically and on a molecular level, with tau positive, TDP-43 positive, and FUS positive intraneuronal inclusions recognized on immunohistochemical analysis. The prevalence of extrapyramidal signs in patients with behavioral variant frontotemporal dementia (bvFTD) and primary progressive aphasia (PPA) has not yet been systematically studied Methods: We enrolled patients fulfilling diagnosis of FTD. Patients with parkinsonian symptoms were diagnosed according to the diagnostic criteria of the United Kingdom Parkinson´s Disease Society Brain Bank. The study participants were assessed with the motor part of the Unified Parkinson´s Disease Rating Scale (UPDRS) to quantify their motor status and with the Hoehn and Yahr rating scale for severity of motor signs. The results were compared with those of patients with temporal variant of FTD using U Mann Whitney´s test for between-group comparison on each item of the UPDRS. Results: A total of 23 patients with bvFTD and 9 primary progressive aphasia were compared. Mean age was 69.84, SD 8.65, range 49 to 84, 62.5 % were men. A total of 78.26 % with bvFTD presented parkinsonism and 55.5 % of temporal FTD. The mean total UPDRS score for bvFTD was 17.35 SD 12.74 (range 0 to 37) vs temporal FTD 10.72 SD 15.67 (range 0 to 51).The Hoehn & Yahr scale for bvFTD was 2.13 SD 0.92 (range 0 to 4) and for temporal variant FTD was 1.72 SD 0.75 (range 0 to 3). Tremor when present was postural or action type. Left side predominance was 44 % and left side was 22 % of all FTD cases. The two groups differed significantly for speech (p = 0.018), facial expression (p = 0.045), left arm postural tremor (p = 0.035), left arm rigidity (p = 0.031). Conclusion: Rigidity, bradykinesia, postural tremor were the most typical findings more severe in bvFTD than in primary progressive aphasia. Detecting parkisonism in patients with FTD is crucial for good clinical practice given potential therapeutic possibilities targeting Tau.