Case ReportAnti-Yo-Associated Paraneoplastic Cerebellar Degeneration Manifesting as Acute Cerebellitis with Posterior Cranial Fossa Hypertension
Introduction
Paraneoplastic neurologic syndromes (PNSs) represent a group of disorders that result in damage to the nervous system in the setting of an underlying cancer and that are not related to metastasis, infection, or metabolic derangements associated with cancer.1, 2, 3, 4 Although PNS may manifest in a patient with diagnosed cancer, most commonly it is the presenting symptom in a previously well individual with a hidden tumor that may be detected only by advanced imaging techniques.1, 5, 6 These rare syndromes are estimated to occur in <0.01% of patients with cancer1, 7, 8; however, accurate data regarding their prevalence are still unavailable. The incidence of PNS seems to be increasing owing to both improvements in the recognition of clinical syndromes and technologic advances in diagnostic testing.
One of the most common characterized PNSs includes paraneoplastic cerebellar degeneration (PCD), which is characterized by cerebellar symptoms including truncal and appendicular ataxia, nystagmus, dysarthria, diplopia, dysarthria, dysphagia, and sometimes oscillopsia and transient opsoclonus that begin abruptly and progress over weeks to months and then stabilize by 6 months.1, 6, 8, 9, 10, 11 We describe an uncommon case of PCD owing to anti-Yo antibodies, which initially manifested as acute cerebellitis and posterior cranial fossa hypertension and required a suboccipital decompressive craniectomy.
Section snippets
Case Description
A previously healthy, 50-year-old woman presented to the emergency department with vertigo, ataxia, intense occipital headache, and projectile vomiting without preceding nausea that occurred when standing or with Valsalva maneuvers. She denied recent flulike symptoms, and her past medical history was unremarkable. Magnetic resonance imaging (MRI) of the brain performed on admission showed a diffuse cerebellar edema with tonsillar descent and leptomeningeal enhancement (Figure 1). The next day
Discussion
In PNSs, onconeural antigens are expressed by both tumoral cells and neuronal tissue and are targeted by the immune response.1, 6 This occurs especially in tumors originating from tissues derived from the ectoderm that have the capacity to produce neuronal proteins.2 The role of onconeural antigens in the pathogenesis of PNSs remains unclear but may relate to humoral versus cellular immunity.1, 2, 3 Certain antibodies may exert direct damage to neural tissue (humoral mechanism) because the
Conclusions
PCD is an uncommon disease that may manifest initially as posterior cranial fossa hypertension and subsequently as acute hydrocephalus owing to diffuse cerebellar swelling. To our knowledge, this is the first described case of an anti-Yo PCD that has manifested as acute posterior cranial fossa hypertension owing to diffuse cerebellar edema. This diagnosis should be considered in women with risk factors for gynecologic or breast cancers. Early diagnosis and treatment should be pursued to improve
Acknowledgments
The authors thank Dr. Helen Reina for her help with copyediting this manuscript.
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Conflict of interest statement: The authors declare that the article content was composed in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.