Keith A. Josephs is a prominent researcher in the field of neurodegenerative diseases, particularly Alzheimer's disease, Parkinson's disease, and related disorders. This page provides comprehensive information about their research contributions, publications, and impact on the field.
Keith A. Josephs has made significant contributions to understanding the mechanisms of neurodegenerative diseases through decades of research. Their work has advanced our understanding of disease pathogenesis, biomarker development, and therapeutic strategies.
Keith A. Josephs, MD, MST, MSc is a neurologist specializing in frontotemporal dementia, progressive supranuclear palsy, and corticobasal syndrome. He is Professor of Neurology at the Mayo Clinic in Rochester, Minnesota, and holds the Ralph B. and Rita J. Levenson Professorship in Neurology. [1]
Dr. Josephs received his MD from the University of Saskatchewan, Canada, where he also completed a Master of Science (MST) degree. He completed his neurology residency at the University of Western Ontario, followed by a prestigious fellowship in cognitive neurology at the Mayo Clinic. He has established one of the largest FTD and PSP clinical research programs in the world. [2]
Throughout his career, Dr. Josephs has been dedicated to understanding the clinical manifestations, underlying pathology, and biomarkers of frontotemporal lobar degenerations and atypical parkinsonian disorders. His work has directly influenced how these conditions are diagnosed and classified worldwide.
Dr. Josephs's research spans multiple interconnected areas:
Dr. Josephs has been instrumental in characterizing the clinical heterogeneity of PSP: [3]
- Richardson syndrome: The classic presentation with vertical gaze palsy, axial rigidity, and postural instability
- PSP-parkinsonism: A variant presenting with asymmetric tremor and bradykinesia
- PSP-pure akinesia with gait freezing (PAGF): Early gait freezing without typical PSP signs
- Corticobasal syndrome: Overlapping features with CBS
- Understanding the clinical and pathological spectrum of CBS [4]
- Differentiating CBS from PSP and other mimics
- Characterizing the asymmetric cortical syndromes
- Detailed clinical phenotyping of FTD subtypes [5]
- Correlation of clinical features with neuroimaging and pathology
- Language variant characterization including semantic and nonfluent variants
¶ Speech and Language Disorders
- Aphasia and apraxia in neurodegenerative disease [6]
- Speech characteristics in PSP and CBS
- Development of speech assessment protocols
Dr. Josephs has made significant contributions to the field: [7]
- Clinical characterization of PSP variants: His work defined the clinical spectrum of PSP presentations
- Development of clinical diagnostic criteria: Contributing to the NINDS-SPSP criteria and subsequent revisions
- Neuroimaging biomarkers: Working with Dr. Jennifer Whitwell to develop MRI biomarkers for predicting tau pathology
- Speech and language abnormalities: Documenting the progressive aphasias and apraxias in FTD spectrum disorders
- Clinicopathological correlations: Linking clinical presentations to underlying tau and TDP-43 pathology
He has authored over 300 peer-reviewed publications, with his work cited extensively in diagnostic guidelines and clinical practice.
Dr. Josephs has authored numerous high-impact papers:
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Josephs KA, et al. "The evolution of diagnostic criteria for progressive supranuclear palsy." Brain. 2023
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Josephs KA, et al. "Clinicopathological correlations in progressive supranuclear palsy." Neurology. 2022
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Whitwell JL, Josephs KA, et al. "Neuroimaging in the diagnosis of PSP." Lancet Neurology. 2021
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Josephs KA, et al. "Phenotypic variability in corticobasal syndrome." Neurology. 2020
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Josephs KA, et al. "Progressive supranuclear palsy: clinicopathological concepts." Mov Disord. 2019
Dr. Josephs leads or participates in several major research initiatives: [8]
- Mayo Clinic Alzheimer's Disease Research Center: Comprehensive studies on FTD and related disorders
- International PSP Working Group: Development of updated diagnostic criteria
- AllFTD Consortium: Characterizing the full spectrum of FTD disorders
- CorticoBasal Degeneration International Consortium: Understanding CBS heterogeneity
¶ Training and Mentorship
Dr. Josephs has trained numerous clinicians and researchers: [9]
- Cognitive neurology fellows
- Behavioral neurology residents
- Graduate students in neuroscience
His mentorship has produced many leaders in neurodegenerative disease research worldwide.
¶ Awards and Recognition
- Ralph B. and Rita J. Levenson Professorship: Endowed chair recognizing his contributions
- Fellow, American Academy of Neurology: Recognition of excellence in neurology
- Mayo Clinic Distinguished Investigator Award: Multiple recognitions for research excellence
Dr. Josephs's ongoing work focuses on: [10]
- Biomarker validation: Refining imaging and fluid biomarkers for PSP diagnosis
- Genotype-phenotype correlations: Understanding how genetic variants influence clinical presentation
- Disease progression modeling: Characterizing the temporal evolution of different PSP variants
- Clinical trial readiness: Developing endpoints and enrichment strategies for therapeutic trials
Recent PubMed-indexed publications (2024-present):
- FDG-PET imaging to identify brain regions associated with Alzheimer's disease-related cognitive decline.. Journal of Alzheimer's disease : JAD. 2026.
- A repeat expansion in GOLGA8A is a major risk factor for atypical frontotemporal lobar degeneration.. Nature genetics. 2026.
- Longitudinal Videofluorographic Dysphagia Measures in Progressive Supranuclear Palsy.. Movement disorders clinical practice. 2026.
- Hippocampal subfield thickness and shape analysis in examining the impact of TDP-43 pathology in Alzheimer's disease.. Alzheimer's & dementia : the journal of the Alzheimer's Association. 2026.
- Uptake of tau-PET and neuropathological and autoradiographic findings from a global longitudinal study of aging.. Alzheimer's & dementia (New York, N. Y.). 2026.
Recent PubMed-indexed publications (2024-present):
- FDG-PET imaging to identify brain regions associated with Alzheimer's disease-related cognitive decline.. Journal of Alzheimer's disease : JAD. 2026.
- A repeat expansion in GOLGA8A is a major risk factor for atypical frontotemporal lobar degeneration.. Nature genetics. 2026.
- Longitudinal Videofluorographic Dysphagia Measures in Progressive Supranuclear Palsy.. Movement disorders clinical practice. 2026.
- Hippocampal subfield thickness and shape analysis in examining the impact of TDP-43 pathology in Alzheimer's disease.. Alzheimer's & dementia : the journal of the Alzheimer's Association. 2026.
- Uptake of tau-PET and neuropathological and autoradiographic findings from a global longitudinal study of aging.. Alzheimer's & dementia (New York, N. Y.). 2026.
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