Objective: To highlight the prodromal and syndromic features of Anti-Yo/PCA-1 syndrome.

Background: A 50-year-old female initially presented with a 1-month history of headaches and vertigo. A mild head tremor was incidentally noted. The remainder of her neurological exam was normal. Her head tremor worsened over the next several weeks. The patient also noticed difficulties with her balance. Her subsequent exam was notable for a multi-directional head tremor, voice tremor with ataxic dysarthria, limitation of down gaze, dysmetria with finger to nose, and a kinetic arm tremor bilaterally. She had significant truncal ataxia and was unable to stand without assistance. MRI brain with and without contrast was normal. CSF studies showed a protein of 49, 29 nucleated cells with a lymphocyte predominance, and normal glucose. Anti-Yo/PCA-1 antibodies were positive in the CSF with a titer of 1:1024. Subsequent imaging showed bilateral complex adnexal masses and peritoneal carcinomatosis. Biopsy noted an ovarian high-grade serous carcinoma. The patient received 5 doses of intravenous steroids without improvement. IVIG was deferred as she had developed a pulmonary embolism while hospitalized. There was no improvement in her symptoms, and the patient, unfortunately, passed away from hemorrhagic shock due to a retroperitoneal hemorrhage.

Method: N/A

Results: N/A

Conclusion: Anti-Yo/PCA-1 syndrome is a paraneoplastic cerebellar disorder that involves a subacute onset of cerebellar dysfunction. This syndrome is most common with middle-aged women with a gynecologic cancer. A prodrome of vertigo can precede cerebellar ataxia in around 66% of patients by a couple weeks.  The most common symptoms in sequential order of frequency include ataxia with a truncal predominance with nystagmus, dysarthria, vertigo, and diplopia¹. This patient presented with an isolated head tremor and was initially diagnosed with essential tremor (ET). Exclusion criteria for ET and ET plus include isolated focal tremors such as the head. Such a patient should instead be evaluated for cerebellar ataxia or cervical dystonia based on clinical context². Oncologic treatment can result in some improvement or disease stability. However, most outcomes are still, unfortunately, poor. Immunotherapy is usually provided in tandem but has largely poor results as well.

References: 1. Mendes NT, Ronchi NR, Silva GD. A Systematic Review on Anti-Yo/PCA-1 Antibody: Beyond Cerebellar Ataxia in Middle-Aged Women with Gynecologic Cancer. Cerebellum. 2023 Dec;22(6):1287-1292. doi: 10.1007/s12311-022-01492-3. Epub 2022 Nov 5. PMID: 36334195.

2. Bhatia KP, Bain P, Bajaj N, Elble RJ, Hallett M, Louis ED, Raethjen J, Stamelou M, Testa CM, Deuschl G; Tremor Task Force of the International Parkinson and Movement Disorder Society. Consensus Statement on the classification of tremors. from the task force on tremor of the International Parkinson and Movement Disorder Society. Mov Disord. 2018 Jan;33(1):75-87. doi: 10.1002/mds.27121. Epub 2017 Nov 30. PMID: 29193359; PMCID: PMC6530552.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/anti-yo-syndrome-presenting-as-an-isolated-head-tremor-with-vertigo/