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Phenotypic and neuroimage differences in Corticobasal syndrome from two clinical cases

2023; Medical Association of São Paulo; Linguagem: Inglês

10.5327/1516-3180.141s1.470

1806-9460

Thais Winkeler Beltrão, Luiz Eduardo Duarte Borges Nunes, Simone Cristina Soares Brandão, Breno José Alencar Pires Barbosa,

Neurological disorders and treatments

Corticobasal syndrome (CBS) is a neurodegenerative condition characterized by cognitive and motor symptoms and neurologic-functional progressive deterioration. The phenotypes can be associated with the underlying proteinopathies like Corticobasal Degeneration (CBD), Progressive Supranuclear Palsy or Alzheimer’s Disease (AD). This work aimed to study, in two cases, the correlations between clinical phenotypes, neuroimage markers and the underlying pathology. Clinical and neuroimage data of two patients was revised. The patient 1, 62-year-old, female, incomplete elementary school, presented progressive non fluent aphasia (NFA) for two years and evolved with limb apraxia and extrapyramidal signs of the right upper limb. The Fluorodeoxyglucose Positron Emission Tomography (FDG-PET) revealed moderate hypometabolism of temporal lobes and middle frontal gyrus predominating on the left side. It fulfilled the criteria for CBS and suggested CBD. The patient 2, 57-year-old, female, graduated, presented NFA, heminegligence, cognitive impairment, bradykinesia and myoclonus. PET-FDG revealed asymmetric hypometabolism in the superior and inferior parietal lobes, posterior cingulum gyrus and worse in precuneus. The investigation of cerebrospinal fluid revealed consumed amyloid beta and increased phosphorylated and total TAU. It fulfilled probable CBS and suggested AD. These cases demonstrate the role of the PET-FDG in CBS and reveal its possible metabolic signatures: when caused by AD, the hypometabolism predominates in the posterior temporoparietal areas, and when caused by tau pathology, in the thalamus and brainstem, mainly contralateral to the most affected side. CBS has been widely studied with relatively new methods, like the cerebral FDG-PET. Studies that deepen the phenotypic heterogeneity and biomarkers of CBS would be important to improve its classification, prognostic and treatment.