Corticobasal syndrome as a phenotype of various neurodegenerative disorders: a case series

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Abstract

Corticobasal syndrome (CBS) is a variant of atypical parkinsonism. The underlying cause may be corticobasal degeneration or other proteinopathies, which can be verified only after studying specific biomarkers. The disease aetiology in CBS needs to be established to determine the disease prognosis. It can also affect the choice of pathogenetic treatment due to the differences in the molecular pathogenesis of proteinopathies that cause neurodegenerative processes. Four clinical cases of CBS are presented: in patients with four-repeat tauopathy, Alzheimer's disease, frontotemporal dementia and Creutzfeldt–Jakob disease. Examples are provided of the clinical, genetic and biochemical biomarkers available for differential diagnosis of CBS.

About the authors

Yuliya A. Shpilyukova

Research Center of Neurology

Author for correspondence.
Email: annaly-nevrologii@neurology.ru
ORCID iD: 0000-0001-7214-583X

Cand. Sci. (Med.), junior researcher, neurologist, 5th Neurology department

Russian Federation, Moscow

Ekaterina Yu. Fedotova

Research Center of Neurology

Email: annaly-nevrologii@neurology.ru
ORCID iD: 0000-0001-8070-7644

D. Sci. (Med.), Head of the 5th Neurology department

Russian Federation, Moscow

Sergey N. Illarioshkin

Research Center of Neurology

Email: annaly-nevrologii@neurology.ru
ORCID iD: 0000-0002-2704-6282

D. Sci. (Med.), Prof., Corr. Member of the Russian Academy of Sciences, Deputy Director, Head, Department for brain research

Russian Federation, Moscow

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2. Fig. 1. Brain MRI of patients with the CBS phenotype, the normal ratio (A) and altered biomarkers (β-amyloid 1–42 and tau protein in the CSF — B). Arrows indicate areas of hypotrophy in the left temporal lobe (contralateral to the clinical symptoms), with preserved hippocampus in both cases.

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3. Fig. 2. Brain MRI of patient with CBS phenotype as part of behavioral variant of frontotemporal dementia with a mutation in the GRN gene

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4. Fig. 3. Brain MRI (DWI mode) of patient with CJD, presenting with CBS, 10 (A) and 12 (B) months after disease onset.

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Copyright (c) 2022 Shpilyukova Y.A., Fedotova E.Y., Illarioshkin S.N.

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This work is licensed under a Creative Commons Attribution 4.0 International License.

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