Motor neuron disease and its association with non-Hodgkins lymphoma.

M otor neuron disease (MND) is a neurodegenerative disorder characterized by muscle atrophy, brisk reflexes, spasticity, fasciculation, and atrophy. The onset of the disease is generally insidious. The pathological process may involve the motor cortex, the spinal cord, and the brainstem. 1 Only upper or only lower motor neurons may be affected. The disease may or may not be seen with neoplasms, however, there have been cases of amyotrophic lateral sclerosis associated with paraneoplastic diseases. This clinical picture occurs with Hodgkin's disease (HD), non-Hodgkin's lymphoma (NHL), and leukemia. The clinical findings frequently start at the lower limbs and become generalized. In this report, we present a patient with NHL and MND to draw attention to this association and to emphasize that these patients should be observed for this condition. A 70-year-old man was admitted to the clinic with weakness in the right foot. He did not have a family history. Results of a physical examination were normal. The results of a neurologic examination revealed 4/5 monoparesis and atrophy of the right foot (4/5), and fasciculation, prominent in the upper and lower proximal muscle groups. Results of standard laboratory analyses for total blood count, routine biochemical tests, protein electrophoresis, Bence Jones protein, and CSF protein level were normal. Results of abdomen-pelvic ultrasonography and spinal MRI did not show any pathological findings. Electromyography of 4 limbs demonstrated motor unit action potentials of long durations with fasciculation. Fibrillation was observed in the rectus femoris, tibialis anterior, and gastrocnemius of the right lower limb. The patient was diagnosed as having MND. Riluzole (100 mg/day) and vitamin E (600 mg/day) were begun. One year later, the patient presented with fever, night sweating, and fatigue. A physical examination revealed a 2 × 2 cm lymph adenomegaly in the right axilla. On neurologic examination, dysarthria, diminished gag reflex, atrophy, and fasciculation of the tongue muscle, and tetraparesis were detected. Deep tendon reflexes were hypoactive, the response to Babinski's sign was negative bilaterally, and the response to Hoffmann's sign was positive bilaterally. Atrophy was present in the thenar, hypothenar, and interosseal muscles of the hand, and the intrinsic foot muscles. Foot drop was present on the right side. The patient required assistance to walk. Results of an abdominal-pelvic ultrasonography were normal. Thoracic CT revealed diffuse lymph adenomegaly in the right axilla and mediastinum. The lymph adenomegaly in the right axilla was excised. Immunohistochemical analyses demonstrated CD20 tumor cells, …