Background: Corticobasal syndrome (CBS) is a rare, clinically heterogeneous neurodegenerative syndrome most commonly associated with corticobasal degeneration (CBD), a 4-repeat tauopathy. CBS presents with asymmetric motor and cortical features, but diagnosis remains challenging, as clinicopathologic concordance is imperfect and other conditions such as Alzheimer's disease (AD), progressive supranuclear palsy (PSP), and frontotemporal lobar degeneration (FTLD) can present with similar phenotypes.
Case presentation: We report the case of a 55-year-old woman with a four-year history of progressive, right-sided spastic hemiparesis and rigidity. Electroneuromyography (ENMG) revealed isolated upper motor neuron (UMN) findings with no lower motor neuron (LMN) involvement. Brain MRI demonstrated cortical atrophy in perirolandic regions, and dopamine transporter (DaT) SPECT imaging revealed a marked, unilateral presynaptic dopaminergic deficit. No sensory, cerebellar, or autonomic features were observed, and levodopa challenge test yielded no benefit. FDG-PET and tau-PET imaging were not performed due to unavailability at our center.
Discussion: The marked clinical asymmetry, dopaminergic deficit strongly support a diagnosis of CBS. While CBD remains the most probable underlying pathology, differential diagnoses include PSP, AD, multiple system atrophy (MSA), idiopathic Parkinson's disease (PD), and, less likely, Mills syndrome or other UMN syndromes. Mills syndrome was considered due to asymmetric UMN findings but was excluded due to parkinsonism, cortical atrophy, and presynaptic dopaminergic loss. This case underscores the diagnostic complexity of CBS, particularly in resource-limited settings where advanced imaging tools are unavailable.
Conclusion: CBS should be considered a leading diagnosis in patients presenting with asymmetric parkinsonism, UMN findings, especially when supported by DaT-SPECT abnormalities. This case highlights the importance of comprehensive clinical evaluation and multimodal imaging in differentiating CBS from other neurodegenerative syndromes.
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