Clinical Phenotypes in Corticobasal Syndrome with or without Amyloidosis Biomarkers

Article type: Research Article

Authors: Benvenutto, Agnès^{a; *} | Guedj, Eric^{b; c; d} | Felician, Olivier^{a; e} | Eusebio, Alexandre^{f; g} | Azulay, Jean-Philippe^{f; g} | Ceccaldi, Mathieu^{a; e} | Koric, Lejla^{a; d}

Affiliations: [a] Department of Neurology and Neuropsychology, and CMMR PACA Ouest, CHU Timone, Assistance Publique-Hôpitaux de Marseille, Marseille, France | [b] Department of Nuclear Medecine, CHU Timone, Assistance Publique-Hôpitaux de Marseille, Marseille, France | [c] CERIMED, Aix-Marseille Univ, Marseille, France | [d] Aix Marseille Univ, UMR 7249, CNRS, Centrale Marseille, Institut Fresnel, Marseille, France | [e] Aix-Marseille Univ, INSERM UMR 1106, Institut de Neurosciences des Systèmes, Marseille, France | [f] Department of Neurology and Movement Disorders Department, CHU Timone, Assistance Publique-Hôpitaux de Marseille, Marseille, France | [g] Aix-Marseille Univ, CNRS, INT, Institut Neurosciences Timone, Marseille, France

Correspondence: [*] Correspondence to: Agnès Benvenutto, Service de neurologie et neuropsychologie, CMRR PACA Ouest, Hôpital de la Timone, 264 Rue Saint Pierre, 13385 Marseille Cedex05, France. Tel.: +33 491385928; E-mail: [email protected].

Abstract: Corticobasal syndrome (CBS) is a neuropathologically heterogeneous entity. The use of cerebrospinal fluid and amyloid biomarkers enables detection of underlying Alzheimer’s disease (AD) pathology. We thus compared clinical, eye movement, and 18FDG-PET imaging characteristics in CBS in two groups of patients divided according to their amyloid biomarkers profile. Fourteen patients presenting with CBS and amyloidosis (CBS-A+) were compared with 16 CBS patients without amyloidosis (CBS-A-). The two groups showed similar motor abnormalities (parkinsonism, dystonia) and global cognitive functions. Unlike CBS-A+ patients who displayed more posterior cortical abnormalities, CBS-A- patients demonstrated more anterior cortical and brain stem dysfunctions on the basis of neuropsychological testing, study of saccade velocities and brain hypometabolism areas on 18FDG-PET. Interestingly, Dopamine Transporter SPECT imaging showed similar levels of dopaminergic degeneration in both groups. These findings confirm common and distinct brain abnormalities between the different neurodegenerative diseases that result in CBS. We demonstrate the importance of a multidisciplinary approach to improve diagnosis in vivo in particular on oculomotor examination.

Keywords: Alzheimer’s disease, corticobasal syndrome, DaTSCAN^© , eye movements, ¹⁸FDG-PET, video-oculography

DOI: 10.3233/JAD-190961

Journal: Journal of Alzheimer's Disease, vol. 74, no. 1, pp. 331-343, 2020