Case reports and clinical series provide invaluable insights into corticobasal syndrome (CBS), a rare and heterogeneous neurodegenerative disorder. Due to the limited number of patients at any single center, individual case reports and small case series have historically been crucial for characterizing the clinical phenotype, pathological diversity, and treatment responses in CBS. This page summarizes key case reports and clinical series that have shaped our understanding of CBS.
Pathological Case Series
Vascular CBS
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Overview
Mermaid diagram (expand to render)
Case reports and clinical series provide invaluable insights into corticobasal syndrome (CBS), a rare and heterogeneous neurodegenerative disorder. Due to the limited number of patients at any single center, individual case reports and small case series have historically been crucial for characterizing the clinical phenotype, pathological diversity, and treatment responses in CBS. This page summarizes key case reports and clinical series that have shaped our understanding of CBS.
Pathological Case Series
Vascular CBS
Cerebrovascular pathology presenting as corticobasal syndrome: An autopsy case series
PMID: 31621626
Finding: Describes cases of CBS resulting from cerebrovascular pathology rather than corticobasal degeneration
Significance: Demonstrates that CBS is a syndrome with multiple potential underlying pathologies
Clinical implications: Vascular lesions can mimic CBS clinically, affecting diagnosis and treatment approach
CBS with Alzheimer's Disease Pathology
Early stage memory impairment, visual hallucinations, and myoclonus combined with temporal lobe atrophy predict Alzheimer's disease pathology in corticobasal syndrome
PMID: 29987978
Finding: Certain clinical features (myoclonus, visual hallucinations, temporal atrophy) predict underlying AD pathology in CBS patients
Significance: Helps explain the heterogeneity of CBS and guides diagnostic workup
Clinical implications: Presence of these features should raise suspicion for AD pathology
Early-Stage CBD Electrophysiology
Electrophysiological studies of early stage corticobasal degeneration
PMID: 9452339
Finding: Clinical series of patients with probable CBD documenting markedly asymmetric clinical manifestations
Significance: Early electrophysiological abnormalities can aid in diagnosis
Clinical Phenotype Case Reports
Myoclonus-Dominant CBS
Myoclonus-Dominant Corticobasal Degeneration
Characterized by prominent action myoclonus with voluntary movement
Often affects upper limbs, sometimes superimposed with limb dystonia
May have initially normal DaT-SPECT imaging
Often shows refractoriness to standard treatments including levodopa, levetiracetam, and clonazepam
Reference: Caballero et al., Movement Disorders Clinical Practice (2019)
Alien Limb Phenomenon
Case reports of alien limb phenomenon in CBS
Posterior variant (visual) vs anterior (motor) variants
Associated with callosal and supplementary motor area pathology
Differentiates CBS from PSP (rare in PSP)
Management includes botulinum toxin, occupational therapy, safety measures
Key PMIDs: 37252456, 35674763, 23439070
Cortical Sensory Loss
Case reports of cortical sensory loss in CBS
Tactile agnosia, astereognosis, graphesthesia
Prevalence: 50-70% of CBS patients
High diagnostic value for distinguishing CBS from PSP/PD
[Unknown, Cerebrovascular pathology presenting as corticobasal syndrome (2019) (2019)](https://pubmed.ncbi.nlm.nih.gov/31621626/)
[Unknown, Early stage memory impairment, visual hallucinations, and myoclonus predict AD pathology in CBS (2018) (2018)](https://pubmed.ncbi.nlm.nih.gov/29987978/)
[Unknown, Electrophysiological studies of early stage CBD (1998) (1998)](https://pubmed.ncbi.nlm.nih.gov/9452339/)