Motor Neuron Diseases

An illness similar to motor neuron disease manifested as spinal cord or anterior horn cell disease has been linked with cancer. Motor neuronopathy with loss of anterior horn cells can occur as part of a paraneoplastic syndrome. In addition, a motor neuronopathy with multifocal conduction blocks can be seen. Patients with predominant motor neuron involvement may have the anti-Hu antibody, and patients with motor neuron disease and cancer may have resolution of their neurological symptoms congruent with successful treatment of their cancer. Patients in whom motor neuron disease is a fragment of a widespread encephalomyelitis syndrome should be examined for anti-Hu antibodies and, if positive, suspected of harboring small cell lung cancer. An association between motor neuron disease and non-Hodgkin's lymphoma, infrequently with a paraproteinemia, has also been observed. y Subacute motor neuronopathy can develop with Hodgkin's disease and is characterized by subacute progressive painless and often asymmetrical lower motor neuron weakness; some patients have sensory symptoms, but there are few or no signs of sensory involvement. Reflexes are decreased or absent, and the bulbar muscles are usually spared. Fasciculations are rare, and the course is usually benign. A pure lower motor neuron syndrome, such as subacute motor neuronopathy, requires a careful evaluation to detect an underlying lymphoma. y Women who have an upper motor neuron syndrome resembling primary lateral sclerosis should undergo mammography to rule out breast cancer. Rarely, a necrotizing myelopathy that mimics an acute transverse myelitis or spinal cord compression has been described. Usually acute and rapidly evolving, the process extends to involve the spinal cord at the thoracic level. The CSF protein level is usually elevated, and MRI can show patchy gadolinium enhancement and T2-weighted signal abnormality occurring within 2 to 4 weeks.y Pathologically, widespread spinal cord necrosis with little inflammatory response or vascular occlusion is seen. y

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