Case Presentation: 59-year-old female with no significant past medical history presented to the hospital with worsening dizziness, vertigo, and diplopia in the right eye. Vital signs were unrevealing. On physical exam, she appeared nontoxic but uncomfortable with eyes closed resting on the bed. The neurologic exam revealed severe nystagmus in the right eye, intact CN 1-12, normal bilateral upper and lower extremities sensation with bilateral lower extremities 3/5 strength, normal deep tendon reflexes, mildly delayed finger-to-nose, and gait deferred due to severe dizziness, no other signs of focal deficit observed. Cardiac and respiratory exams were unremarkable. Initial blood work showed mild leukocytosis with an elevated neutrophil count, otherwise no significant anemia, thrombocytopenia, or electrolyte imbalance. Computed tomography (CT) scan of the head and magnetic resonance imaging (MRI) of the brain were unremarkable. CT scan of the chest/abdomen/pelvis showed significant retroperitoneal lymphadenopathy suspicious for nodal metastatic disease, however MRI of the pelvis was unremarkable. Lymph node biopsy was consistent with metastatic adenocarcinoma of the gynecological tract. Lumbar puncture with cerebrospinal fluid (CSF) studies revealed PCA-1 antibodies, which are seen with paraneoplastic cerebellar degeneration and ovarian cancers. The patient was started on a course of high dose steroids with subsequent intravenous immune globulin (IVIG) by Neurology and then underwent a robotic total hysterectomy with bilateral salpingo-oophorectomy by Gynecology Oncology, which demonstrated an invasive high-grade serous carcinoma arising from the left fallopian tube with benign omentum and no signs of peritoneal disease. The patient was discharged to initiate chemotherapy with carboplatin and paclitaxel as an outpatient.
Discussion: Neoplasms associated with paraneoplastic cerebellar degeneration (PCD) are rare with only a couple of cases reported in the literature. PCD has been observed in approximately 25% of paraneoplastic neurologic syndromes. Often, they are adult onset and more prevalent in females in their sixth decade. Most common presenting symptoms include dizziness, nausea followed by vertigo, dysarthria, dysphagia, and blurry vision. Unfortunately, patients are severely disabled in days to weeks. In average, 60%-70% of patients with PCD have an occult malignancy. Early diagnosis of PCD can lead to early diagnosis and treatment of the occult malignancy. There are multiple antibodies that predominate with PCD and associated malignancies. This case revealed anti-Purkinje cell (PCA-1) antibodies in association with a newly diagnosed ovarian adenocarcinoma. Although the role of PCA-1 antibodies has not been established in living animals, in vitro studies suggest they cause Purkinje cell death in the absence of T lymphocytes.
Conclusions: Vertigo is a frequent problem encountered by hospitalists. Paraneoplastic syndromes with an associated occult malignancy should always be included in the differential diagnosis for a patient being admitted with worsening vertigo and diplopia. In most cases, the paraneoplastic syndrome presents itself before the malignancy is found and diagnosed.