Diagnostic Considerations

The key limiting factors for the diagnostic considerations include the lack of a reliable patient-level in vivobiomarker for corticobasal degeneration (CBD) and the significant heterogeneity of clinical CBD presentations (detailed in the Overview and Presentation sections). A careful historical review (including family history) aiming to determine the tempo of symptom progression is crucial (for example, patients with corticobasal syndrome (CBS) often look and sound like they have had a stroke, but there is no history of acute stroke and no imaging evidence of same), followed by a thorough examination targeting the wide range of motor and non-motor findings present in the phenotypes associated with CBD. Features that suggest a synucleinopathy (Parkinson disease, dementia with Lewy bodies, or multiple system atrophy) rather than a tauopathy like CBD include rapid eye movement sleep behavior disorder (confirmed with polysomnography) and loss of smell (which can be formally evaluated using tests such as the University of Pennsylvania Smell Identification Test). Rapid progression of symptoms in the course of < 2 years should trigger additional workup for rapidly progressive dementia, as patients with Creutzfeldt-Jakob disease can present with CBS. It is important to keep in mind that CBS can represent an atypical presentation of Alzheimer disease (AD), and that AD pathology is responsible for 20–30% of CBS cases.

Differential Diagnoses

Alzheimer Disease
Anterior Circulation Stroke
Apraxia and Related Syndromes
Cardioembolic Stroke
CNS Whipple Disease
Creutzfeldt-Jakob Disease
Lewy Body Dementia
Epilepsia Partialis Continua
Frontal Lobe Syndromes (Primary Progressive Aphasia and Primary Progressive Apraxia)
Frontotemporal Dementia and Frontotemporal Lobar Degeneration
Glioblastoma Multiforme
Huntington Disease
Hydrocephalus
Marchiafava-Bignami Disease
Multiple System Atrophy
Neuroacanthocytosis Syndromes
Neurosyphilis
Niemann-Pick disease type C
Olivopontocerebellar Atrophy
Pantothenate Kinase-Associated Neurodegeneration (PKAN)

and other NBIAs
Parkinson Disease
Parkinson-Plus Syndromes
Pick Disease
Progressive Supranuclear Palsy
Striatonigral Degeneration
Subdural Hematoma
Thalamic Stroke
Vitamin B-12 Associated Neurological Diseases
Vitamin E-Associated Neurological Diseases
Wilson Disease

Workup

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Author

Alexander Pantelyat, MD Assistant Professor, Department of Neurology, Johns Hopkins University School of Medicine; Director, Johns Hopkins Atypical Parkinsonism Center; Co-Director, Johns Hopkins Center for Music and Medicine; Co-Director, Johns Hopkins Movement Disorders Fellowship Program

Alexander Pantelyat, MD is a member of the following medical societies: American Academy of Neurology, American Neurological Association, International Association for Music and Medicine, International Parkinson and Movement Disorder Society, Maryland State Medical Society

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: MedRhythms, Inc.; Biosensics, Inc.<br/>Received research grant from: NIH.

Coauthor(s)

Maitreyi Murthy, MBBS, MPhil Clinical and Research Fellow, Department of Neurology, Movement Disorders Division, Johns Hopkins University School of Medicine

Maitreyi Murthy, MBBS, MPhil is a member of the following medical societies: American Academy of Neurology, Movement Disorder Society

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Nestor Galvez-Jimenez, MD, MSc, MHA The Pauline M Braathen Endowed Chair in Neurology, Chairman, Department of Neurology, Program Director, Movement Disorders, Department of Neurology, Division of Medicine, Cleveland Clinic Florida

Nestor Galvez-Jimenez, MD, MSc, MHA is a member of the following medical societies: American Academy of Neurology, American College of Physicians, International Parkinson and Movement Disorder Society

Disclosure: Nothing to disclose.

Chief Editor

Selim R Benbadis, MD Professor, Director of Comprehensive Epilepsy Program, Departments of Neurology and Neurosurgery, Tampa General Hospital, University of South Florida Morsani College of Medicine

Selim R Benbadis, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Sleep Medicine, American Clinical Neurophysiology Society, American Epilepsy Society, American Medical Association

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: Aquestive, Bioserenity, Ceribell, Eisai, Jazz, LivaNova, Neurelis, Neuropace, Nexus, SK life science, Stratus, Sunovion, UCB<br/>Serve(d) as a speaker or a member of a speakers bureau for: Aquestive, Bioserenity, Ceribell, Eisai, Jazz, LivaNova, Neurelis, Neuropace, Nexus, SK life science, Stratus, Sunovion, UCB<br/>Received research grant from: Cerevel, LivaNova, Greenwich (Jazz), SK biopharmaceuticals, Takeda, Xenon.

Additional Contributors

A M Barrett, MD, FAAN, FANA, FASNR Executive Director, Center for Visual and Neurocognitive Rehabilitation, Atlanta VA Health Care System; Program Director, Division of Neurorehabilitation, Department of Neurology, Emory University School of Medicine

A M Barrett, MD, FAAN, FANA, FASNR is a member of the following medical societies: American Academy of Neurology, American Society of Neurorehabilitation, International Neuropsychological Society

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: Kessler Foundation<br/>Received research grant from: SPR Therapeutics; DART Neuroscience; Wallerstein Foundation for Geriatric Improvement; NJ Commission on Brain Injury Research; NIDILRR; NIH.

Stephen T Gancher, MD Adjunct Associate Professor, Department of Neurology, Oregon Health Sciences University

Stephen T Gancher, MD is a member of the following medical societies: American Academy of Neurology, American Neurological Association, International Parkinson and Movement Disorder Society

Disclosure: Nothing to disclose.

Acknowledgements

Thanks are owed to the family of the man who provided medical records for the author's review, to confirm that pathologically confirmed corticobasal syndrome has occurred with onset before age 45 years.

Sections Corticobasal Syndrome and Corticobasal Degeneration
Overview
Presentation
DDx
Workup
Treatment
Medication
Follow-up
References

Corticobasal Syndrome and Corticobasal Degeneration Differential Diagnoses

Diagnostic Considerations

Differential Diagnoses

References

Tables

Contributor Information and Disclosures

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