Subacute motor neuronopathy: a remote effect of lymphoma

Ten patients developed a subacute lower motor neuron syndrome as a remote effect of Hodgkin's disease or other lymphoma. The illness usually followed a benign course independent of the activity of the underlying neoplasm. Seven of the patients improved spontaneously, and 3 became neurologically normal. Two patients died of intercurrent infections related to immunosuppression. Neuropathological examination of these 2 patients and 3 previously reported cases showed prominent neuronal degeneration restricted to the anterior horns of the spinal cord and mild posterior column demyelination. Demyelination was also present in the anterior roots of our autopsied patients and was accompanied by large, hyperchromatic Schwann cells. The cause of the illness is obscure, but both radiation therapy and opportunistic infection may be contributing factors. Attempts at virus isolation have been unsuccessful. The syndrome should be distinguished from the more common direct effects of lymphoma on the nervous system, since its identification spares the patient additional, potentially harmful therapy.