Von Recklinghausen's Neurofibromatosis

Abstract

FRIEDRICH Daniel von Recklinghausen (1833 to 1910) had a long career as professor of pathology at the University of Strassburg. 1 Among the many disease processes that occupied his interest was neurofibromatosis, which he discussed at length in an 1882 Festschrift dedicated to his teacher, Rudolf Virchow. Von Recklinghausen reviewed the previously reported cases and added the descriptions of two additional patients. He and his predecessors noted the multiple tumors of the skin and the peripheral and autonomic nerves, as well as the cafe au lait spots. Subsequently, it has been discovered that patients with this disease may also have a variety of neoplasms and malformations involving the brain, spinal cord, meninges, autonomic ganglia, and the roots of the cranial and spinal nerves, as well as anomalies in other tissues. 6,7 The disorder is typically familial, being transmitted as an autosomal dominant trait. 8,9 Although he was not the