Lower motor neuron disease associated with myelofibrosis

We present a patient who has signs pointing to the involvement of lower mot or neurons and myelofibrosis. To our knowledge, unlike lymphoproliferative disorders, co-occurrence of myelofibrosis and lower motor neuron disease (M ND) has not been reported so far. A 64-year-old male patient was admitted t o our hospital with the complaint of painful cramps in his neck and forearm s. On physical examination marked hepatomegaly and splenomegaly were found. On neurologic examination nasal quality of the voice and slight palatal we akness were detected. There were generalised slight weakness and atrophy in both proximal and distal muscle groups. Fasciculations were observed espec ially in forearm muscles and it was observed that he had been avoiding head movements because of painful muscle cramps. Deep tendon reflexes were hypo active. Nerve conduction studies were normal. By needle electromyography, g iant motor unit action potentials (amplitudes up to 8 mV), fibrillation pot entials, positive sharp waves and fasciculation potentials were detected in all muscles which were investigated. A hypercellular bone marrow (100%) wa s determined by bone marrow biopsy. In addition to increased production of the myeloid and megakaryocytic lines, abnormal aggregation and grouping of megakaryocytes were seen. Reticular fibers were increased. He had some bene fit of dyphenilhydantoin treatment given for the painful clamps in his neck and forearm muscles. Hydroxyurea treatment was started for myelofibrosis. Six months later, his general condition was better, and the painful cramps were completely resolved. No marked deterioration has been detected in neur ologic examination and electromyography for 1 year. (C) 2000 Elsevier Scien ce B.V. All rights reserved.