Corticobasal Degeneration

Excerpt

Corticobasal ganglionic degeneration (CBD) is a rare and progressive neurodegenerative disorder with diverse clinical and pathological features. The most common clinical presentation, corticobasal syndrome, includes asymmetric limb rigidity, Parkinsonism, dystonia, and cortical dysfunction, such as apraxia or sensory deficits. Pathologically, CBD is associated with 4-repeat (4R; this refers to the presence of 4 microtubule-binding domains in the tau protein isoform) hyperphosphorylated tau protein inclusions in neurons and glial cells, forming astrocytic plaques. Despite advancements in understanding the pathology, the mechanisms underlying tau-induced neurodegeneration remain unclear, and disease-modifying treatments are unavailable. There is a need to delineate the disease's intricate pathophysiologic processes further. Accurate early diagnosis is essential, as it can guide patient care and facilitate the development of targeted therapies and disease-specific biomarkers.

Interprofessional care plays a critical role in managing CBD, particularly given its progressive nature and the lack of curative treatments. Collaborative care teams—neurologists, physical therapists, occupational therapists, and palliative care specialists—can optimize symptom management and address functional impairments. Early discussions of end-of-life care and support for patients and families are essential in improving the quality of life for those with this debilitating condition.