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Area of Science:

  • Neurology
  • Neuroscience
  • Pathology

Background:

  • Corticobasal syndrome (CBS) is a neurodegenerative disorder characterized by asymmetric motor deficits (rigidity, tremor, dystonia, myoclonus), apraxia, sensory loss, and alien limb phenomena.
  • Non-motor symptoms, including cognitive and language impairments, are also common in CBS.
  • CBS is pathologically heterogeneous, associated with corticobasal degeneration, progressive supranuclear palsy, Alzheimer disease, and other tauopathies.

Purpose of the Study:

  • To review recent advances in understanding corticobasal syndrome (CBS).
  • To highlight challenges in antemortem diagnosis of underlying pathologies in CBS.
  • To discuss the spectrum of tauopathies associated with CBS.

Main Methods:

  • Review of recent literature on clinical features, histopathology, and diagnostic modalities for CBS.
  • Analysis of imaging and cerebrospinal fluid (CSF) biomarkers relevant to CBS diagnosis.
  • Examination of genetic factors and the concept of tauopathy spectrum in CBS.

Main Results:

  • Clinical and pathological features of CBS have been further elucidated.
  • Imaging and CSF biomarkers show promise for improving antemortem diagnosis.
  • Genetic factors and the spectrum of tauopathies offer new insights into CBS pathogenesis.

Conclusions:

  • Accurate diagnosis of underlying pathology in CBS remains challenging but is improving.
  • Advances in biomarkers and understanding tauopathy spectrum are crucial for future management.
  • Further research is needed to refine diagnostic criteria and therapeutic strategies for CBS.