Abstract
Paraneoplastic cerebellar degeneration (PCD) is an uncommon autoimmune disorder targeting antigens within the nervous system and is usually associated with an underlying malignancy. Neurologic symptoms frequently precede the cancer diagnosis, which is most often seen in women with breast or gynecologic tumors. Anti-Yo-related PCD is the most common PCD syndrome, and one of the best understood. Although cerebellar signs are characteristic of anti-Yo PCD, myelopathy is an unusual presentation of anti-Yo PCD based on published case series and reports. Unfortunately, the prognosis for anti-Yo PCD is often poor, and most patients become bedridden. We report a case highlighting a severe presentation of cerebellar degeneration along with an unusual finding of myelopathy in a patient with a newly diagnosed gynecologic cancer.
Highlights
Paraneoplastic cerebellar degeneration (PCD) results from autoantibodies produced by tumors that target specific antigens within the nervous system [1]
We highlight an unusual presentation of PCD with typical cerebellar signs, as well as myelopathy, in a patient that was found to have positive anti-Yo titers and a gynecologic tumor, which is not typical of this disorder according to the limited literature on the topic
90 to 98% of patients who present with cerebellar ataxia and found to have positive anti-Yo antibody titers have an underlying malignancy, often a gynecologic or breast cancer [1]
Summary
PCD results from autoantibodies produced by tumors that target specific antigens within the nervous system [1]. In the case of anti-Yo PCD, the most common form of PCD, the target of the autoantibodies is Purkinje cell cytoplasmic antibody type 1 (PCA1). Anti-Yo PCD accounts for approximately 50% of patients with PCD [2] and is most commonly associated with breast and gynecologic cancers. Given the rarity of PCD, most of the knowledge of PCD is based off of case series and reports. In this case report, we highlight an unusual presentation of PCD with typical cerebellar signs, as well as myelopathy, in a patient that was found to have positive anti-Yo titers and a gynecologic tumor, which is not typical of this disorder according to the limited literature on the topic. The patient showed very mild improvement after receiving treatment and physical therapy
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