Anti-Yo antibody-associated paraneoplastic cerebellar degeneration: a report of 6 patients

Abstract

Objective Paraneoplastic cerebellar degeneration (PCD) is a neurological syndrome characterized by subacute cerebellar ataxia, specific tumour types and associated antineuronal antibodies. We attempted to identify patient-, tumour- and treatment-related characteristics associated with PCD with anti-Yo antibodies. Methods We examined the antineuronal antibodies associated and studied the neurological symptoms and signs, associated tumours, disability of PCD with anti-Yo antibodies. Results Six patients had anti-Yo antibody-associated PCD. All patients are female with mean age of 55 years. All cases presented with subacute cerebellar ataxia progressive over 1 to 9 weeks (mean 6.23 weeks). The majority of patients had both truncal and appendicular ataxia with unstable gait and dizziness. Spontaneous nystagmus presented in all cases including rotary nystagmus in three cases, horizontal nystagmus in two cases, opsoclonus-like nystagmus in one case. Dysarthria, dysphasia, double vision and behavior changes presented in some cases. Modified Rankin Scale scores were 4-5, indicating severe disability. On cerebrospinal fluid examination, mild increases of white blood cell counts were found in four cases and mild elevation of protein concentration in two cases. Cerebrospinal fluid cytology showed lymphocytic inflammation in 3 cases. The most commonly associated tumours were ovary cancer in four cases while cancer of bladder was found in one patient. Only one patient improved neurologically after receiving anti-tumour treatment and immunotherapy with intravenous immunoglobulin in 3 cases. Conclusions Anti-Yo antibodies are well-characterised on coneuronal antibodies associated with PCD related to gynaecological cancer. Diagnosis and treatment of underlying neoplasm is critical for the patient with subacute cerebellar ataxia and positive anti-Yo antibody. Anti-Yo antibody-associated PCD usually results in severe disability with poor prognosis even after anti-tumor and immunotherapy. Key words: Paraneoplastic cerebellar degeneration; Cerebellar ataxia; Autoantibodies