The Cerebellar Cognitive Affective Syndrome in Essential Tremor Plus

Louis ED, McCreary M. How common is essential tremor? Update on the worldwide prevalence of essential tremor. Tremor Other Hyperkinet Mov (N Y). 2021;11:28. https://doi.org/10.5334/tohm.632.

Article  PubMed  Google Scholar 

Bhatia KP, Bain P, Bajaj N, Elble RJ, Hallett M, Louis ED, et al. Consensus statement on the classification of tremors. From the task force on tremor of the International Parkinson and Movement Disorder Society. Mov Disord. 2018;33:75–87.

Article  PubMed  Google Scholar 

Louis ED, Bares M, Benito-Leon J, Fahn S, Frucht SJ, Jankovic J, et al. Essential tremor-plus: a controversial new concept. Lancet Neurol. 2020;19:266–70.

Article  PubMed  Google Scholar 

Louis ED, Huey ED, Cosentino S. Features of ET plus correlate with age and tremor duration: ET plus May be a disease stage rather than a subtype of essential tremor. Parkinsonism Relat Disord. 2021;91:42–7.

Article  PubMed  PubMed Central  Google Scholar 

Angelini L, Paparella G, De Biase A, Maraone A, Panfili M, Berardelli I, et al. Longitudinal study of clinical and neurophysiological features in essential tremor. Eur J Neurol. 2023;30(3):631–40.

Article  PubMed  Google Scholar 

Erro R, Pilotto A, Esposito M, Olivola E, Nicoletti A, Lazzeri G, et al. The italian tremor network (TITAN): rationale, design and preliminary findings. Neurol Sci. 2022;43:5369–76.

Article  PubMed  PubMed Central  Google Scholar 

Peng J, Li N, Li J, Duan L, Chen C, Zeng Y, Xi J, Jiang Y, Peng R. Reclassification of patients with tremor syndrome and comparisons of essential tremor and essential tremor-plus patients. J Neurol. 2022. https://doi.org/10.1007/s00415-022-10985-4.

Article  PubMed  PubMed Central  Google Scholar 

Erro R, Lazzeri G, Terranova C, Paparella G, Gigante AF, De Micco R, Magistrelli L, Di Biasio F, Valentino F, Moschella V, Pilotto A, Esposito M, Olivola E, Malaguti MC, Ceravolo R, Dallocchio C, Spagnolo F, Nicoletti A, De Rosa A, Di Giacopo R, Sorrentino C, Padovani A, Altavista MC, Pacchetti C, Marchese R, Contaldi E, Tessitore A, Misceo S, Bologna M, Rizzo V, Franco G, Barone P, TITAN study group. Comparing essential tremor with and without soft dystonic signs and tremor combined with dystonia: the TITAN study. Mov Disord Clin Pract. 2024;11(6):645–54.

Article  PubMed  PubMed Central  Google Scholar 

Cosentino S, Shih LC. Does essential tremor increase risk of cognitive impairment and dementia? Yes. Int Rev Neurobiol. 2022;163:195–231.

Article  PubMed  CAS  Google Scholar 

Erro R, Fasano A, Barone P, Bhatia KP. Milestones in tremor research: 10 years later. Mov Disord Clin Pract. 2022;9(4):429–35. https://doi.org/10.1002/mdc3.13418.

Article  PubMed  PubMed Central  Google Scholar 

Cartella SM, Bombaci A, Gallo G, Ledda C, Pengo M, Pignolo A, Pozzi FE, Spina E, Trinchillo A, Palermo G, Terranova C. On behaf of EduNet SIgN. Essential tremor and cognitive impairment: who, how, and why. Neurol Sci. 2022;43(7):4133–43.

Article  PubMed  Google Scholar 

Schmahmann JD, Sherman JC. The cerebellar cognitive affective syndrome. Brain. 1998;121:561–79.

Article  PubMed  Google Scholar 

Hoche F, Guell X, Vangel MG, Sherman JC, Schmahmann JD. The cerebellar cognitive affective/Schmahmann syndrome scale. Brain. 2018;141(1):248–70.

Article  PubMed  Google Scholar 

Destrebecq V, Naeije G. Cognitive impairment in essential tremor assessed by the cerebellar cognitive affective syndrome scale. Front Neurol. 2023;14:1224478.

Article  PubMed  PubMed Central  Google Scholar 

Rao AK, Louis ED, Y). Ataxic gait in essential tremor: A Disease-Associated feature? Tremor other hyperkinet Mov (N. 2019;9. https://doi.org/10.7916/d8-28jq-8t52

Curran PJ, West SG, Finch JF. The robustness of test statistics to nonnormality and specification error in confirmatory factor analysis. Psychol Methods. 1996;1(1):16–29.

Article  Google Scholar 

Huang H, Yang X, Zhao Q, Ning P, Shen Q, Wang H, Xie D, Lu H, Xu Y. Clinical characteristics of patients with essential tremor or essential tremor plus. Acta Neurol Scand. 2020;141(4):335–41.

Article  PubMed  Google Scholar 

Campbell JM, Ballard J, Duff K, Zorn M, Moretti P, Alexander MD, Rolston JD. Balance and cognitive impairments are prevalent and correlated with age in presurgical patients with essential tremor. Clin Park Relat Disord. 2022;6:100134.

PubMed  PubMed Central  Google Scholar 

Tsuboi T, Uematsu T, Sawada K, Higuchi M, Hashida M, Muto M, Ito Y, Ishizaki T, Kato S, Nakatsubo D, Tsugawa T, Maesawa S, Saito Y, Fukushima T, Tamakoshi D, Hiraga K, Suzuki M, Saito R, Ramirez-Zamora A, Okun MS, Katsuno M. Determinants of clinical and neurophysiological features in essential tremor and essential tremor plus. J Neural Transm (Vienna). 2025;132(7):1041–50.

Article  PubMed  CAS  Google Scholar 

Xue A, Kong R, Yang Q, Eldaief MC, Angeli PA, DiNicola LM, Braga RM, Buckner RL, Yeo BTT. The detailed organization of the human cerebellum estimated by intrinsic functional connectivity within the individual. J Neurophysiol. 2021;125(2):358–84.

Article  PubMed  Google Scholar 

O’Reilly JX, Beckmann CF, Tomassini V, Ramnani N, Johansen-Berg H. Distinct and overlapping functional zones in the cerebellum defined by resting state functional connectivity. Cereb Cortex. 2010;20(4):953–65.

Article  PubMed  Google Scholar 

Guell X, Schmahmann JD, de Gabrieli J, Ghosh SS. Functional gradients of the cerebellum. Elife. 2018. https://doi.org/10.7554/eLife.36652.

Article  PubMed  PubMed Central  Google Scholar 

Seitzman BA, Gratton C, Marek S, v Raut R, Dosenbach NUF, Schlaggar BL, et al. A set of functionally-defined brain regions with improved representation of the subcortex and cerebellum. Neuroimage. 2020;206:116290.

Article  PubMed  Google Scholar 

Boonstra JT. The cerebellar connectome. Behav Brain Res. 2025;482:115457. https://doi.org/10.1016/j.bbr.2025.115457.

Article  PubMed  Google Scholar 

Stoodley CJ, Schmahmann JD. Evidence for topographic organization in the cerebellum of motor control versus cognitive and affective processing. Cortex. 2010;46(7):831–44.

Article  PubMed  PubMed Central  Google Scholar 

Ahmadian N, van Baarsen K, van Zandvoort M, Robe PA. The cerebellar cognitive affective syndrome-a meta-analysis. Cerebellum. 2019;18(5):941–50.

Article  PubMed  PubMed Central  Google Scholar 

Widner J, Faust PL, Louis ED, Fujita H. Axonal pathology differentially affects human purkinje cell subpopulations in the essential tremor cerebellum. Proc Natl Acad Sci U S A. 2025;122(27):e2502024122.

Article  PubMed  PubMed Central  CAS  Google Scholar 

Paparella G, Angelini L, Margiotta R, Passaretti M, Birreci D, Costa D, Cannavacciuolo A, De Riggi M, Alunni Fegatelli D, Bologna M. Insight into the relationship between motor and cognitive symptoms in essential tremor. Cerebellum. 2024;23(5):2050–9.

Article  PubMed  PubMed Central  Google Scholar 

Ebner TJ, Hewitt AL, Popa LS. What features of limb movements are encoded in the discharge of cerebellar neurons? Cerebellum. 2011;10:683–93.

Article  PubMed  PubMed Central  Google Scholar 

Hewitt L, Popa S, Pasalar S, Hendrix M, Ebner J. Representation of limb kinematics in Purkinje cell simple spike discharge is conserved across multiple tasks. J Neurophysiol. 2011;106:2232–47.

Bologna M, Paparella G, Fasano A, Hallett M, Berardelli A. Evolving concepts on bradykinesia. Brain. 2020;143:727–50.

Shirinbayan I, Dreyer M, Rieger W. Cortical and subcortical areas involved in the regulation of reach movement speed in the human brain: an fMRI study. Hum Brain Mapp. 2019;40(1):151–62.

Colella D, Passaretti M, Frantellizzi V, Silvia De Feo M, Cannavacciuolo A, Angelini L, et al. Subtle changes in central dopaminergic tone underlie bradykinesia in essential tremor. Neuroimage Clin. 2023;40:103526.

Article  PubMed  PubMed Central  Google Scholar 

Bostan AC, Strick PL. The basal ganglia and the cerebellum: nodes in an integrated network. Nat Rev Neurosci. 2018;19(6):338–50.

Article  PubMed  PubMed Central  CAS  Google Scholar 

Erro R, Picillo M, Pellecchia MT, Barone P. Diagnosis versus classification of essential tremor: a research perspective. J Mov Disord. 2023;16(2):152–7. https://doi.org/10.14802/jmd.23020.

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