Peripheral Demyelinating Diseases: From Biology to Translational Medicine.

Khidhir Kamil,Muhammad Dain Yazid,Srijit Das,Ruszymah Bt Hj Idrus,Jaya Kumar

doi:10.3389/fneur.2019.00087

Abstract

Demyelinating diseases represent a spectrum of disorders that impose significant burden on global economy and society. Generally, the prognosis of these diseases is poor and there is no available cure. In recent decades, research has shed some light on the biology and physiology of Schwann cells and its neuroprotective effects in the peripheral nervous system (PNS). Insults to the PNS by various infectious agents, genetic predisposition and immune-related mechanisms jeopardize Schwann cell functions and cause demyelination. To date, there are no effective and reliable biomarkers for PNS-related diseases. Here, we aim to review the following: pathogenesis of various types of peripheral demyelinating diseases such as Guillain-Barre syndrome, Chronic Inflammatory Demyelinating Polyradiculoneuropathy, Anti-Myelin Associated Glycoprotein Neuropathy, POEMS syndrome, and Charcot-Marie-Tooth disease; emerging novel biomarkers for peripheral demyelinating diseases, and Schwann cell associated markers for demyelination.

Highlights

Peripheral demyelinating diseases (PDD) refer to a spectrum of disorders that involves substantial damage to axons and glial cells, Schwann cells (SC) in the peripheral nervous system (PNS) [1]
Poor prognosis remained a dilemma in the management of the PDDs
Understanding the physiology of myelination and SC biology is vital to help further delineate the mechanisms involved in the pathogenesis of PDD

Summary

Introduction

Peripheral demyelinating diseases (PDD) refer to a spectrum of disorders that involves substantial damage to axons and glial cells, Schwann cells (SC) in the peripheral nervous system (PNS) [1]. The incidence of these diseases is variable [2,3,4]. Schwann cells are principal glial cells in peripheral nerves that originate from the neural crest, which is a multipotent embryonic structure that differentiates into other main glial subtypes of the PNS [10]. These immature SC differentiate into either myelinating or non- myelinating SC that populates the mature nerve trunks and wrap around axons through a process known as myelination [10]

Objectives

Conclusion