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Neurophysiology and neurochemistry of corticobasal syndrome

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Abstract

Corticobasal syndrome is a rare neurodegenerative disorder, which presents with a progressive, asymmetrical, akinetic rigid syndrome and early cortical signs. However, clinical, pathological, and electrophysiological heterogeneity makes the understanding of this syndrome challenging. Corticobasal syndrome can have various pathological substrates including corticobasal degeneration, Alzheimer’s disease, Fronto-temporal degeneration with TDP inclusions, Creutzfeldt–Jakob disease, and progressive supranuclear palsy (PSP). Furthermore, tools such as transcranial magnetic stimulation (TMS) and functional neuroimaging techniques like PET and SPECT have not been adequately used to supplement the clinico-pathological heterogeneity. TMS studies in CBS have revealed changes in cortical excitability and transcortical inhibition. Despite the availability of more than 2 decades, its potential in CBS has not been fully utilized in studying the cortical plasticity and effect of Levodopa on central neurophysiology. PET and SPECT studies in CBS have shown abnormalities in regional glucose metabolism, asymmetrical involvement of presynaptic dopaminergic system, and ascending cholinergic connections to the cortex. While most studies have shown normal D2 receptor-binding activity in striatum of CBS cases, the results have not been unanimous. Functional neuroimaging and TMS studies in CBS have shown the involvement of GABAergic, muscarinic, and dopaminergic systems. In this review, we aim to provide the current state of understanding of central neurophysiology and neurochemistry of CBS using TMS and functional neuroimaging techniques. We also highlight the heterogeneous nature of this disorder and the existing knowledge gaps.

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Authors and Affiliations

  1. Department of Clinical Neurological Sciences, London Health Sciences Centre, Western University, 339 Windermere Road, A10-026, London, ON, N6A 5A5, Canada

    Aditya A. Murgai & Mandar S. Jog

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  1. Aditya A. Murgai
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  2. Mandar S. Jog
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AAM Conception, design, and writing the first manuscript. MSJ Design, review, and critique.

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Correspondence to Mandar S. Jog.

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Conflicts of interest

Dr. Murgai reports no disclosures relevant to manuscript. Dr. Jog receives speaker and consultant honoraria from Merz Pharmaceuticals, Allergan, AbbVie. He also receives research grants from CIHR, AMOSO, Allergan, Merz Pharmaceuticals, and Lawson Health Research Institute, and is part of the AGE-WELL Network of Centers of Excellence (NCE) of Canada program. From time to time, he serves on advisory boards of Allergan, Boston Scientific, AbbVie and Merz Pharmaceuticals.

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The authors confirm that the approval of an institutional review board was not required for this work. We also confirm that we have read the Journal’s position on issues involved in ethical publication and affirm that this work is consistent with those guidelines.

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Murgai, A.A., Jog, M.S. Neurophysiology and neurochemistry of corticobasal syndrome. J Neurol 265, 991–998 (2018). https://doi.org/10.1007/s00415-017-8731-5

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