Elsevier

World Neurosurgery

Volume 112, April 2018, Pages 117-122
World Neurosurgery

Case Report
Anti-Yo-Associated Paraneoplastic Cerebellar Degeneration Manifesting as Acute Cerebellitis with Posterior Cranial Fossa Hypertension

https://doi.org/10.1016/j.wneu.2018.01.105Get rights and content

Highlights

  • Paraneoplastic neurologic syndromes are rare non-metastatic neurological complications that occur in less than 0.01% of patients with cancer.

  • Anti-Yo antibodies are associated with gynecologic or breast malignancies and may play a role in paraneoplastic cerebellar degeneration due to their targeting of Purkinje cells.

  • Early diagnosis and treatment of acute paraneoplastic cerebellar degeneration should be pursued to improve long-term outcomes.

Background

Paraneoplastic cerebellar degeneration (PCD) is a rare complication of some malignant cancers. It is most commonly described in women with gynecologic or breast malignancies; however, there have been reports in other types of cancers. Symptoms include ataxia, dysarthria, and tremors, which could be the first manifestations of an underlying malignancy.

Case Description

A 50-year-old woman had an acute PCD with anti-Yo antibodies from an underlying breast invasive ductal carcinoma. She presented with intracranial hypertension in the posterior cranial fossa that required an emergent decompressive craniectomy.

Conclusions

PCD is an uncommon disease that may manifest initially as posterior cranial fossa hypertension and subsequent 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. Early diagnosis and treatment should be pursued to improve long-term outcomes.

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.

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