Anti-Yo antibody-positive paraneoplastic cerebellar degeneration in a patient with possible cholangiocarcinoma: A case report and review of the literature

Abstract

BACKGROUND Paraneoplastic cerebellar degeneration (PCD), which is rare in clinical practice, is closely related to autoimmunity. Cases positive for anti-Yo antibodies (anti-Purkinje cytoplasmic antibody 1) are the main subtype of PCD. PCD is subacute cerebellar degeneration, and while it progresses over weeks to months, its resultant deficits last much longer. Cancer patients with anti-Yo antibody-positive PCD are very rare. Most of them are breast cancer or ovarian cancer patients but also occasionally lung cancer patients. CASE SUMMARY A 61-year-old woman presented with sudden vertigo, nausea, and vomiting for approximately 10 d. The patient's neurological examination showed torsion with downbeat nystagmus and ataxia of the right limb and trunk. Laboratory examination found that the patient's cerebrospinal fluid and serum were anti-Yo antibody-positive, positron emission tomography computed tomography showed an increased metabolic rate in the retroperitoneal lymph nodes, and the pathology of lymph node punctures in the retroperitoneum and neck suggested adenocarcinoma of the pancreaticobiliary duct, which strengthens the hypothesis of paraneoplastic origin. Intravenous immunoglobulin (IVIg) 0.4 g/kg/d for 5 d and methylprednisolone 160 mg for 3 d were initiated, which was reduced to 80 mg for 3 d and then to 40 mg for 7 d. After treatment with IVIg and a steroid, the patient's vertigo and ataxia alleviated. CONCLUSION The patient's vertigo and ataxia alleviated after treatment, suggesting that early immunotherapeutic intervention may have certain value in stopping neurological loss.

Pontocerebellar Progressive Multifocal Leukoencephalopathy. Radiological, Clinical, Histological and Immunohistochemical Findings in a Hematological Patient

“…Astrocytomas are excluded because of the presence of reactive glial cells with cytopathic changes and of numerous histiocytes. Subacute cerebellar degeneration, a paraneoplastic disorder, is discarded by the integrity of Purkinje neurons [22]. The absence of Negri bodies excludes rabies [23].…”

Update on Paraneoplastic Cerebellar Degeneration

“…CSF analysis typically shows pleocytosis, elevated proteins, and intrathecal synthesis of IgG [ 8 , 20 ]. Absence of inflammatory signs, however, has been reported in case reports and frequency might decrease with age and depends on the associated antibody [ 110 , 111 , 112 ]. In a study on 155 patients with antibody-associated CNS-syndromes (not specific for PCD) aged 60 years and older, 22.6% did not show signs of inflammation in CSF analysis [ 112 ].…”