Lessons From Leprosy: Peripheral Neuropathies and Deformities in Chronic Demyelinating Diseases

J Hand Surg Am. 2019 May;44(5):411-415. doi: 10.1016/j.jhsa.2018.07.007. Epub 2018 Sep 1.

Abstract

In light of the World Health Organization's push to accelerate progress toward a leprosy-free world by 2020, it is fitting to look back on the evolution of progress in treating lepromatous neuropathy and limb deformities. To date, no surgeon has had as great an impact on the understanding and treatment of this disease as Dr Paul Brand. Before Dr Brand's accomplishments, few surgeons participated in the management of the deformed leprous patient. By challenging conventional beliefs, Dr Brand revealed that many of the deformities associated with leprosy were in fact caused by nerve damage and subsequent limb anesthesia. His pioneering work centered on tendon transfers to provide hand and foot mobility to leprous patients, revolutionizing the surgical management of this patient population and restoring functionality to the lives of otherwise stigmatized and functionally handicapped individuals. In the process, he provided us with the surgical principles and techniques that we still apply today. Because of its predilection for the peripheral nervous system, leprosy also provides an excellent opportunity to investigate mechanisms of demyelination and chronic nerve degeneration in nonacute peripheral neuropathies. Processes underlying demyelination of infectious, traumatic, and genetic etiologies overlap and precede the onset of acute neuronal derangement. Glial pathology has been shown to be a common pathological element in leprosy, Charcot-Marie-Tooth type I, multiple sclerosis, and chronic nerve compression injury. The aim of this article is to provide an overview of lepromatous neuropathy with its subsequent deformities as it relates to the pathophysiology, surgical management, and potential therapeutic targets of other modern peripheral neuropathies.

Keywords: Demyelination; Hansen disease; hand deformities; leprosy; peripheral neuropathy.

Publication types

  • Historical Article
  • Review

MeSH terms

  • Charcot-Marie-Tooth Disease / diagnosis
  • Charcot-Marie-Tooth Disease / genetics
  • Charcot-Marie-Tooth Disease / surgery
  • Hand Deformities, Acquired / etiology
  • Hand Deformities, Acquired / surgery
  • History, 19th Century
  • Humans
  • Leprosy / history*
  • Multiple Sclerosis / diagnosis
  • Multiple Sclerosis / surgery
  • Nerve Compression Syndromes / diagnosis
  • Nerve Compression Syndromes / surgery
  • Peripheral Nervous System Diseases / etiology*
  • Peripheral Nervous System Diseases / surgery*