Abstract
Purkinje cell cytoplasmic antibody type 1 (PCA-1), or anti-Yo, is the most frequently detected autoantibody in paraneoplastic cerebellar degeneration (PCD). The vast majority of cases of anti-Yo PCD, however, occur in females over 60 years old and are associated with gynecologic tumors. Only 10 cases have been reported in males, and only 2 were associated with cancer of the lung. Here we describe the youngest known case of PCA-1 positive PCD in a male, whose lung tumor was undetectable even on FDG-PET.
Highlights
Paraneoplastic neurological syndromes (PNS) are rare disorders of the central and/or peripheral nervous system due to the remote effects of, rather than directly caused by, an underlying malignancy or its metastases
Paraneoplastic cerebellar degeneration (PCD), known as subacute cerebellar ataxia, is the most common paraneoplastic disease of the brain [6]. It is characterized by severe pancerebellar dysfunction, typically beginning with gait ataxia and progressing, over weeks to months, to severe, symmetrical truncal and limb ataxia, often with dysarthria and nystagmus [7, 8]
It is characterized by acute to subacute onset of gait ataxia progressing to, over the course of weeks to months, truncal and limb ataxia, with dysarthria and often nystagmus and diplopia [7, 25, 26]
Summary
Paraneoplastic neurological syndromes (PNS) are rare disorders of the central and/or peripheral nervous system due to the remote effects of, rather than directly caused by, an underlying malignancy or its metastases They occur in less than 1% of patients with cancer [1], though paraneoplastic peripheral neuropathy can occur in 50% of patients with osteosclerotic myeloma [2], myasthenia gravis in 10–15% of patients with thymoma [3], and Lambert-Eaton myasthenic syndrome in up to 3% of patients with neoplasms of the lung [4]. The remaining PNS are so uncommon that their exact incidence has not been established [5] Of these conditions, paraneoplastic cerebellar degeneration (PCD), known as subacute cerebellar ataxia, is the most common paraneoplastic disease of the brain [6]. Large cell adenocarcinoma of the lung was revealed on autopsy, making this the third case of non-small cell lung cancer associated with anti-Yo PCD
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