General description

Paraneoplastic cerebellar degeneration (PCD) is a paraneoplastic neurological syndrome caused by immune-mediated injury to cerebellar Purkinje cells. It is most commonly associated with breast and pelvic malignancies but has also been reported in patients with Hodgkin lymphoma, small cell lung cancer, gastric cancer, prostate cancer, and other tumors such as testicular cancer, neuroblastoma, thymoma, non-small cell lung cancer, and melanoma.

Clinically, PCD presents acutely or subacutely with progressive symptoms, including limb and truncal ataxia, ataxic dysarthria, and nystagmus, which typically peak within 2–3 months and then stabilize. Additional features may include opsoclonus (irregular, multidirectional, saccadic eye movements), myoclonus, memory impairment, pyramidal signs, and sensory disturbances.

PCD is associated with specific paraneoplastic antibodies, including anti-Yo (linked to breast and gynecologic cancers), anti-Ri (breast cancer), anti-Hu (small cell lung cancer), and anti-Tr (Hodgkin lymphoma). Other antibodies, such as anti-VGKC and anti-CV2, may also be involved. The rapid progression of PCD often results in significant disability, highlighting the importance of early recognition and management.

Radiographic features

MRI reveals cerebellar atrophy, often accompanied by T2WI hyperintensity in the cerebellar cortex and white matter.