Posters
Presenting Author Academic/Professional Position
Hugo Zamarron
Academic Level (Author 1)
Medical Student
Discipline/Specialty (Author 1)
Neurology
Academic Level (Author 2)
Faculty
Discipline/Specialty (Author 2)
Neurology
Academic Level (Author 3)
Faculty
Discipline/Specialty (Author 3)
Neurology
Academic Level (Author 4)
Faculty
Discipline/Specialty (Author 4)
Neurology
Academic Level (Author 5)
Faculty
Discipline/Specialty (Author 5)
Neurology
Discipline Track
Clinical Science
Abstract Type
Case Report
Abstract
Background: Corticobasal syndrome (CBS) is a neurodegenerative disorder characterized by often asymmetric fronto-pariteal and extra-pyramidal features that is traditionally associated with tauopathy, but pathological findings are heterogenous, including other forms of frontotemporal lobar degeneration (FTLD) and mixed pathologies of aging. We present clinical, radiographic, and histopathologic features of asymmetry in a unique patient with CBS and underlying FTLD with TDP-43 pathology (FTLD-TDP), co-occurring with other age-related pathologies.
Case Presentation: A 76-year-old man presented with progressive cognitive and motor dysfunction including asymmetric parkinsonism, left-sided dystonia and rigidity, apraxia, visuospatial impairment, and a subtle social disorder including apathy and social withdrawal. The onset included navigational difficulties, loss of motivation, and word-finding issues. Neurological examination showed asymmetric rigidity, cortical sensory deficits, left limb apraxia, and impaired visuoconstruction clinically consistent with clinical criteria for corticobasal syndrome. MRI demonstrated right frontoparietal atrophy with associated Wallerian degeneration. Neuropathological examination revealed frontotemporal lobar degeneration with TDP-43 pathology (FTLD-TDP), along with moderate Alzheimer’s disease neuropathology (Braak IV, Thal Phase B2), Lewy body pathology limited to limbic regions, and ARTAG. Gross pathology showed marked substantia nigra depigmentation and right-sided frontoparietal atrophy. The patient was negative for GRN mutation.
Conclusions: This case illustrates the clinical and pathological complexity of corticobasal syndrome, emphasizing the importance of recognizing FTLD-TDP as a cause of CBS even in the absence of a GRN mutation. The coexistence of AD and Lewy body pathology complicates clinical-pathological correlation and underscores the need for antemortem biomarkers to better predict underlying disease in atypical parkinsonian syndromes. Clinicians should consider mixed and non-tau pathologies in CBS presentations, especially those with asymmetric motor findings and progressive behavioral dysfunction.
Presentation Type
Poster
Recommended Citation
Zamarron, Hugo; Irwin, David; Phillips, Jeffery; Lee, Edward; Tisdall, Matthew; and McMillan, Corey, "Corticobasal Syndrome with Mixed Pathology in the Absence of GRN Mutation: A Clinico-Pathological Case of FTLD-TDP with Coexisting Alzheimer’s and Lewy Body Pathology" (2025). Research Colloquium. 48.
https://scholarworks.utrgv.edu/colloquium/2025/posters/48
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Corticobasal Syndrome with Mixed Pathology in the Absence of GRN Mutation: A Clinico-Pathological Case of FTLD-TDP with Coexisting Alzheimer’s and Lewy Body Pathology
Background: Corticobasal syndrome (CBS) is a neurodegenerative disorder characterized by often asymmetric fronto-pariteal and extra-pyramidal features that is traditionally associated with tauopathy, but pathological findings are heterogenous, including other forms of frontotemporal lobar degeneration (FTLD) and mixed pathologies of aging. We present clinical, radiographic, and histopathologic features of asymmetry in a unique patient with CBS and underlying FTLD with TDP-43 pathology (FTLD-TDP), co-occurring with other age-related pathologies.
Case Presentation: A 76-year-old man presented with progressive cognitive and motor dysfunction including asymmetric parkinsonism, left-sided dystonia and rigidity, apraxia, visuospatial impairment, and a subtle social disorder including apathy and social withdrawal. The onset included navigational difficulties, loss of motivation, and word-finding issues. Neurological examination showed asymmetric rigidity, cortical sensory deficits, left limb apraxia, and impaired visuoconstruction clinically consistent with clinical criteria for corticobasal syndrome. MRI demonstrated right frontoparietal atrophy with associated Wallerian degeneration. Neuropathological examination revealed frontotemporal lobar degeneration with TDP-43 pathology (FTLD-TDP), along with moderate Alzheimer’s disease neuropathology (Braak IV, Thal Phase B2), Lewy body pathology limited to limbic regions, and ARTAG. Gross pathology showed marked substantia nigra depigmentation and right-sided frontoparietal atrophy. The patient was negative for GRN mutation.
Conclusions: This case illustrates the clinical and pathological complexity of corticobasal syndrome, emphasizing the importance of recognizing FTLD-TDP as a cause of CBS even in the absence of a GRN mutation. The coexistence of AD and Lewy body pathology complicates clinical-pathological correlation and underscores the need for antemortem biomarkers to better predict underlying disease in atypical parkinsonian syndromes. Clinicians should consider mixed and non-tau pathologies in CBS presentations, especially those with asymmetric motor findings and progressive behavioral dysfunction.
