Objective: To evaluate clinical features of corticobasal syndrome in a tertiary care setting.

Background: None.

Methods: A retrospective chart review was done. All patients diagnosed with corticobasal syndrome (CBS) by a fellowship trained movement disorders neurologist as per the clinical criteria, were included.

Results: Nineteen patients met the inclusion criteria; 13 were male (68%). Mean age was 75± 7.03 years. Duration of symptoms at the time of presentation was 5.5 ± 3.6 (CI 3.7-7.3). Cognitive impairment was common with the majority, 13 (76%) in the dementia range. MOCA score on initial appointment was available for 5/19 patients with a score of 14.8 ± 4.9 (CI 8.7-20.9). Asymmetrical akinetic-rigidity and ideomotor apraxia was present in all patients. Limb dystonia was present in almost all 17/19 (90%) patients. Agraphesthesia was present in 2 out of 8 (25%) who were tested. Myoclonus was present in 5 (26%) and alien limb phenomena in 5 (26%) patients. Speech was unintelligible in 3 (16%) subjects; commonest problem was getting words out. Symptoms of REM sleep behavior disorder in 4 (21%), hallucinations in 2 (10%). Eye movement abnormalities were present in the majority, 13 (68%); impairment in saccade generation 12/13 (92%), vertical gaze palsy in 2/13 (15%). History of falls was present in 8(42%) with a fall frequency of 1.5 ± 1.9 falls per month. Beck Depression Inventory (BDI) score was available for 10/19 patients with a mean score of 11.7 ± 7.3 (6.4-16.8). 14/19 patients had motor Unified Parkinson’s Disease Rating Scale (UPDRS) performed with a mean score of 45.2± 13.4 (CI 37.4-52.9). Complete lack of subjective response to levodopa was present in 75%. PDQ 39 was available for about half 10/19 of the subjects. Total disability score was 80 ± 27.4 (CI 58.8- 101) with subgroup scores of; communication 5.6 ± 2.5, bodily discomfort 5.3 ± 3.7. Cognition 7.7 ± 4.1, social support 1.4 ± 1.5, stigma 5.5 ± 4.5, emotional well-being 8.8 ± 7.6, ADLs 16.8 ± 6.7, mobility 30.6 ± 11.1. Majority of the CBS, 12 (63%), had been incorrectly diagnosed as Parkinson’s disease by the general neurologist.

Conclusions: For the diagnosis of CBS, signs which were most helpful were asymmetrical akinetic rigid Parkinsonism, ideomotor apraxia and limb dystonia. Myoclonus and cortical sensory loss were less helpful. Brain MRI was not helpful.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/corticobasal-syndrome-a-single-center-case-series/