Paraneoplastic cerebellar degeneration in olfactory neuroepithelioma
- 1Division of Neurology, Department of Medicine, Shiga University of Medical Science, Otsu, Shiga, Japan 520–2192
- 2Department of Clinical Laboratory Medicine, Shiga University of Medical Science, Otsu, Shiga, Japan 520–2192
- 3Department of Neurology, Brain Research Institute, Niigata University, Niigata, Japan
- Correspondence to: Kengo Maeda Division of Neurology, Department of Medicine, Shiga University of Medical Science, Otsu, Shiga, Japan 520–2192;
Anti-Hu antibody was first discovered in patients with paraneoplastic encephalomyelitis associated with small cell lung cancer (SCLC). This antibody recognises proteins comprised in the Hu family expressed by neuronal cells as well as SCLC. After the first report, anti-Hu antibody was found in other neoplasms including prostate and breast cancer, adrenal carcinoma, chondromyxosarcoma, neuroblastoma, and neuroendocrine neoplasms at other sites.1 Olfactory neuroepithelioma (9523/3)2 is thought to differ from classic neuroblastoma (9500/3) in its expression pattern of tyrosine hydroxylase, MYCN amplification, and fusion of the Ewing sarcoma gene and the Friend leukaemia virus integration 1 gene or the ETS related gene.3
Anti-Hu antibody in association with olfactory neuroepithelioma has not been reported previously. We report a patient with cerebellar ataxia that paralleled the recurrence of the tumour. Serum and cerebrospinal fluid (CSF) from the patient contained anti-Hu antibody, and the olfactory neuroepithelioma resected from the patient expressed Hu antigen.
Seven years before admission, a 65 year old man presented with olfactory neuroepithelioma that had invaded the orbit and frontal lobe. The tumour was dissected surgically, and dura mater graft was not used in the surgery. The patient underwent irradiation (total dose of 50 Gy). …