MOTOR-NEURON DISEASE - A PARANEOPLASTIC PROCESS ASSOCIATED WITH ANTI-HU ANTIBODY AND SMALL-CELL LUNG-CARCINOMA

Although isolated lower motor neuron disease has been reported as a pa raneoplastic complication, it has not been previously described in ass ociation with anti-Hu antibody. We report a 51-year-old man in whom we akness heralded the presence of a small-cell cancer of the lung. His n eurological disorder was characterized by an unremitting progression o f limb, neck, and chest wall weakness and wasting that commenced and r emained predominant in the upper limbs, Electrophysiological studies d emonstrated widespread denervation and examination of a muscle biopsy specimen showed evidence of acute and chronic denervation. High titers of anti-Hu antibody were detected in the serum and cerebrospinal flui d. Neither objective measures of strength nor titers of anti-Hu antibo dy responded to corticosteroids, cyclophosphamide, intravenous immunog lobulins, or plasmapheresis. Death from the complications of motor neu ron disease ensued 23 months after the onset of weakness. Autopsy reve aled tumor in the lung and on pleural and peritoneal surfaces, There w as a loss of anterior horn cells in the spinal cord. Despite the absen ce of symptomatic cerebellar disease, a decrease in the number of Purk inje cells was also detected.