September 3, 2015: Paraneoplastic Cerebellar Degeneration
September 3, 2015
Paraneoplastic Cerebellar Degeneration
Paraneoplastic cerebellar degeneration is characterized by purkinje cell death mediated by purkinje cell antibody type 1, also known as anti-Yo. It is mostly commonly associated with breast and ovarian adenocarcinomas, and the pathophysiology is primarily via a cytotoxic T-cell mediated process. In up to 90 percent of patients, paraneoplastic anti-Yo causes a cerebellar clinical syndrome of progressive gait ataxia, nystagmus, and dysarthria, but other signs and symptoms such corticospinal tract dysfunction and peripheral neuropathy have also been noted.
The majority of patients with PCA-1 positivity in conjunction with an underlying tumor are found to have local as opposed to metastatic disease. If no tumor is found, it is recommended that positive patients be fully investigated with PET-CT imaging and followed closely. Despite being discovered at an earlier stage of oncological disease, prognosis for neurological recovery appears worse in PCA-1 positive patients as compared to positivity for other antibodies.
- Schubert M, Panja D, Haugen M, Bramham C, Vedeler C. Paraneoplastic CDR2 and CDR2L antibodies affect Purkinje cell calcium homeostasis. Acta Neuropathol 2014; 128: 835-852.
- McKeon A, Tracy J, Pittock S, Parisi J, Klein C, Lennon V. Purkinje cell cytoplasmic autoantibody type 1 accompaniments: the cerebellum and beyond. Arch Neurol 2011; 68: 1282-1289.
Submitted by Sarah Wesley MD, Department of Neurology, Mount Sinai Beth Israel Medical Center.
Disclosures: Dr. Wesley is a member of the Residents & Fellows Section of Neurology