Date: Monday, June 5, 2017
Session Title: Parkinsonism, MSA, PSP (Secondary and Parkinsonism-Plus)
Session Time: 1:45pm-3:15pm
Location: Exhibit Hall C
Objective: This clinical case intends to highlight the continuum between frontotemporal dementia and corticobasal syndrome.
Background: A substantive overlap between corticobasal degeneration and frontotemporal dementia has been demonstrated, although perfectly mimicking cases are sparsely described in literature.
Methods: We obtained retrospective data from clinical history, neurological examination, resonance imaging, nuclear medicine, and genetic testing.
Results: We report a case of a 66-year old male presenting to the emergency department with suicidal ideation for the past two months. The patient was referred to the Psychiatry outpatient clinic and started on antidepressants. A few weeks later he is observed in the ER for loss of consciousness and neurology observation was then requested. Examination showed signs of frontal dysfunction, ideomotor apraxia on the left side, visual and tactile extinction, alien hand, supranuclear vertical gaze palsy, axial and left bradykinesia (mUPDRSIII 42) as well as absence of postural reflexes. Neuropsychological examination disclosed significant frontal function impairment and severe construction deficit (MMSE 20, MoCA 9). Brain MRI showed mild right frontotemporal atrophy, PET(11C)beta-CIT was normal; in the 18F-FDG a right-left perfusion asymmetry was clear with a normal 11C-PIB-PET. Genetic studies (c9orf72, progranulin, MAPT) were negative. Final diagnosis: Frontotemporal dementia.
Conclusions: The presented case highlights an continuum between two clinical entities whose boundaries are further diluted as we realize that, like in the presented case, they can be clinically indistinguishable. Overlapping cases settle the clinical ground for unifying definition in need.
References: 1. Stamelou M, Quinn NP, Bhatia KP. “Atypical” atypical parkinsonism: new genetic conditions presenting with features of progressive supranuclear palsy, corticobasal degeneration, or multiple system atrophy-a diagnostic guide. Movement disorders : official journal of the Movement Disorder Society. 2013;28(9):1184-99.
2. Boeve BF. The multiple phenotypes of corticobasal syndrome and corticobasal degeneration: implications for further study. Journal of molecular neuroscience : MN. 2011;45(3):350-3.
To cite this abstract in AMA style:R. Varela, C. Duque, J. Carvalho, A. Moreira, A. Morgadinho, C. Januário. Corticobasal degeneration and frontotemporal dementia – an overlapping continuum [abstract]. Mov Disord. 2017; 32 (suppl 2). https://www.mdsabstracts.org/abstract/corticobasal-degeneration-and-frontotemporal-dementia-an-overlapping-continuum/. Accessed December 5, 2023.
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MDS Abstracts - https://www.mdsabstracts.org/abstract/corticobasal-degeneration-and-frontotemporal-dementia-an-overlapping-continuum/