Spinocerebellar Degeneration Associated with a Selective Defect of Vitamin E Absorption

Abstract

A NUMBER of recent reports have suggested that some patients with chronic fat malabsorption have spinocerebellar degeneration secondary to a severe and prolonged deficiency of vitamin E. The clinical features of this syndrome comprise progressive ataxia of gait, incoordination of the limbs, areflexia, and distal loss of proprioception and vibration sense. Dysarthria, ophthalmoplegia, pigmentary retinopathy, generalized muscle weakness, extensor plantar responses, and superficial sensory loss occur in some cases.¹ A relation between neurologic dysfunction and vitamin E deficiency was first suggested by Binder and colleagues in a report on patients with chronic steatorrhea,² and subsequently confirmed in patients with abetalipoproteinemia, . . .