The aim of the study was to identify the clinical and neurophysiological pattern of deficits in spinal arteriovenous malformations (AVM) to allow an early diagnosis which is the prerequisite for effective treatment by early surgery or embolization. Among 26 patients with myelographic signs of a spinal AVM, selective spinal angiography disclosed 20 cases with a dural AV-fistula (dAVF) and 6 patients with an intradural AV-malformation (iAVM). Although the main pathogenetic factor in both disorders may be different, clinical and neurophysiological findings proved to be of limited value in differential diagnosis. Clinical symptoms presented by the patients were a variable combination of lower motor neuron lesion, sphincter disturbance, sensory transverse lesion and partly additional signs of upper motor neuron involvement. Electromyography invariably showed an increased rate of polyphasia and frequently pathological spontaneous activity usually in several myotomes. Normal sensory conduction velocity of the sural nerve contrasted with almost regularly pathological SEP's after tibial nerve stimulation. The distribution if clinical and neurophysiological findings suggests rather widespread lesions of the lower cord and/or cauda equina, frequently at a lower level than the angiographically localized shunt. This suggests a vascular myelopathy on the basis of insufficient venous drainage at least for the frequent dural AV-anomaly.