The case of a man aged 53 is described, who showed an organic type of dementia accompanied by wasting and fibrillation of the muscles of the upper extremities. The course of the disease was rapidly downhill with increasing mental deterioration and marked muscular wasting, extending to the lower extremities. Vitamin E therapy was used without apparent effect. The duration of the disease was about one year. Pathologically, there were widespread lesions of the neuron cells extending to the cerebral cortex, the basal ganglia and particularly the motor cells of the medulla and spinal cord. The neuroglia was hyperplastic and hypertropic; the microglia had also proliferated and numerous rod-shaped cells were observed. The case is considered an instance of presenile psychosis of the Jakob's type (cortico-striato-spinal degeneration). The few similar cases reported in the literature are summarized and some clinical, pathologic and etiologic aspects of the disease are briefly discussed.Abstract Teaser