Abstract Details

Title Clinical spectrum of corticobasal degeneration: review of 238 autopsy-confirmed patients

Topic Movement Disorders

Presentation(s) S36 - Movement Disorders: Clinical and Pathologic Characterization of Neurodegenerative Movement Disorders (1:00 PM-1:12 PM)

Poster/Presentation
Number 001

Background Patients with autopsy-confirmed CBD present with various clinical phenotypes. The current diagnostic criteria for CBD (Armstrong et al. 2013) define four clinical phenotypes: Corticobasal syndrome characterized by asymmetric rigidity and apraxia; frontal behavioral-spatial syndrome (FBS); nonfluent/agrammatic variant of primary progressive aphasia (naPPA); and progressive supranuclear palsy syndrome (PSPS). The frequency of these clinical phenotypes in autopsy-confirmed CBD was assessed.

Objective To determine the frequencies of clinical phenotypes of corticobasal degeneration (CBD), including patients meeting multiple criteria for clinical phenotypes.

Design/Methods We reviewed medical records of 238 patients with autopsy-confirmed CBD in the Mayo Clinic brain bank collected between 1998 and 2021. The diagnosis and clinical phenotypes of CBD were assigned based on the Armstrong’s criteria for CBD. The diagnostic criteria for PSP (Höglinger et al. 2017) were also applied where applicable.

Results Of 238 patients, 93 patients (39%) had probable CBD and 114 patients (48%) had possible CBD. Some of the remaining patients met criteria for PSP, including probable Richardson syndrome (N = 5), probable PSP-parkinsonism (N = 3), and possible PSP-predominant ocular motor dysfunction (N = 2). Of the clinical phenotypes, 124 patients (45%) were possible CBS, 19 (7%) were probable CBS, 122 (44%) were PSPS, 86 (31%) were FBS, and 45 (16%) were naPPA. Of note, 114 patients met more than one clinical phenotype; 12 patients met four, 29 patients met three, and 73 patients met two.

Conclusions Although CBS and PSPS were the most common clinical presentations of CBD, more than half of patients met multiple criteria for each clinical phenotype. Some CBD patients also met criteria for PSP; therefore, the specificity of these criteria needs to be further assessed.

Authors/Disclosures
Shunsuke Koga, MD, PhD (Hospital of the University of Pennsylvania) PRESENTER	Dr. Koga has nothing to disclose.
Aya Murakami, MD, PhD (Mayo Clinic)	Dr. Murakami has nothing to disclose.
Ece Bayram, MD, PhD	The institution of Dr. Bayram has received research support from National Institute on Aging (K99AG073453). The institution of Dr. Bayram has received research support from Lewy Body Dementia Association. The institution of Dr. Bayram has received research support from National Institute of Neurological Disorders and Stroke.
Naomi Kouri	No disclosure on file
Irene Litvan, MD, FAAN (UC San Diego Parkinson and Other Movement Disorder Center)	Dr. Litvan has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Lundbeck. Dr. Litvan has received personal compensation in the range of $10,000-$49,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Frontiers in Neurology.
Dennis W. Dickson, MD (Mayo Clinic)	Dr. Dickson has nothing to disclose.