BACKGROUND: The number of patients with motor neuron disease (MND) and a concomitant hematologic disorder appears to be overrepresented. Angiotropic large cell lymphoma, a rare and aggressive type of lymphoma, has been associated with MND only once (to our knowledge) prior to this report. There are more than 35 cases of MND associated with lymphoma or monoclonal gammopathy reported in the literature. The nature of this association remains disputable. OBJECTIVE: To investigate whether the association between some MNDs and certain hematologic disorders is coincidental or pathogenetically related. CASE PRESENTATION: We describe the clinical and neuropathologic findings in a case involving a 70-year-old man with a rapidly progressive lower MND who at autopsy also exhibited angiotropic large cell lymphoma without ischemic lesions in the nervous system. CONCLUSIONS: This case supports the notion that the association between some MNDs and certain hematologic disorders is not coincidental but pathogenetically related. A 2-hit hypothesis is proposed in which an initial abnormal glycosylation in motoneurons would require the production of an appropriate autoantibody for disease expression.
BACKGROUND: The number of patients with motor neuron disease (MND) and a concomitant hematologic disorder appears to be overrepresented. Angiotropic large cell lymphoma, a rare and aggressive type of lymphoma, has been associated with MND only once (to our knowledge) prior to this report. There are more than 35 cases of MND associated with lymphoma or monoclonal gammopathy reported in the literature. The nature of this association remains disputable. OBJECTIVE: To investigate whether the association between some MNDs and certain hematologic disorders is coincidental or pathogenetically related. CASE PRESENTATION: We describe the clinical and neuropathologic findings in a case involving a 70-year-old man with a rapidly progressive lower MND who at autopsy also exhibited angiotropic large cell lymphoma without ischemic lesions in the nervous system. CONCLUSIONS: This case supports the notion that the association between some MNDs and certain hematologic disorders is not coincidental but pathogenetically related. A 2-hit hypothesis is proposed in which an initial abnormal glycosylation in motoneurons would require the production of an appropriate autoantibody for disease expression.