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Paraneoplastic cerebellar degeneration is a neurological disorder of unknown cause occurring in patients with an identified or occult cancer. An autoimmune etiology is likely since autoantibodies directed against the Purkinje cells of the cerebellum have been found in the serum and cerebrospinal fluid of some patients. Two Purkinje cell-specific antigens are recognized by these autoantibodies, a major antigen of 62 kDa (CDR 62, cerebellar degeneration-related 62-kDa protein) and a minor antigen of 34 kDa (CDR 34). Our previous studies have described the isolation and characterization of a human cerebellar cDNA that encodes an epitope recognized by sera from patients with paraneoplastic cerebellar degeneration. We have now established by two independent methods that this gene is uniquely expressed in Purkinje cells of the cerebellum and corresponds to the minor antigen CDR 34. This antigen is also expressed in tumor tissue from a patient with paraneoplastic cerebellar degeneration.

Thrombotic microangiopathic hemolytic anemia has been associated with several chemotherapeutic agents. The authors describe a patient who developed this syndrome while receiving carboplatin, an analog of cisplatin. The clinical course was marked by encephalopathy and multifocal neurologic deficits. Progressive brainstem dysfunction culminated in coma and respiratory arrest. Pathologic examination revealed widespread microvascular thrombosis, particularly severe in the heart, kidney, and brain. Although the pathogenesis of chemotherapy-related thrombotic microangiopathy remains unclear, an elevated von Willebrand factor antigen and pathologic evidence of endothelial hyperplasia in this patient suggest that an abnormality of the endothelium is related to the development of the clinical syndrome.