Scapuloperoneal muscular atrophy: Davidenkow's syndrome. Family report

The nosology of scapuloperoneal syndrome remains controversial. Is it a variant of Charcot-Marie-Tooth's disease, a form of myopathy, or of spinal muscular atrophy is still unknown. A family with a scapuloperoneal syndrome caused by anterior horn cell involvement is described. Data for sensory involvement were also found. In addition one member of the family had motor nerves and corticospinal tract involvement. The distribution of weakness and muscle wasting was unusual--only lower limbs were involved. However, electromyographic data for both upper and lower limbs involvement were evident. The combination of scapuloperoneal syndrome with sensory loss has been described by Davidenkow, but was not proved by neurographic investigation. Later only one case with proved sensory disturbances was reported. Patients with motor nerves involvement in addition were reported, but not received satisfactory explanation. Probably in some patients the disease could be manifested only with anterior horn cell involvement, while in other both motor and sensory nerves could be involved. In conclusion we suppose that the scapuloperoneal muscular atrophy is a form of spinal muscular atrophy which could be manifested by different symptoms.