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A. Gordon Smith, MD

Arch Neurol. 2001;58:1014-1016.

	Since this article does not have an abstract, we have provided the first 150 words of the full text and any section headings.

INTRODUCTION

In 1886, Jean-Martin Charcot and Pierre Marie^{1, 2} and Henry Tooth³ independently described the hereditary motor and sensory neuropathy that now bears their names. Their precise clinical description provided the foundation on which later physiological and genetic understanding of hereditary neuropathies is based. This article will trace the historical development of our understanding of Charcot-Marie-Tooth disease (CMT) from its early descriptions to recent genetic discoveries.

EARLY DESCRIPTIONS

[I]t seems that the principal characteristics of the type of muscular atrophy which we propose to identify and to describe are:

A progressive muscular atrophy, which first of all, involves the feet and legs, but does not affect the upper extremities until several years later (first the hand, then the forearm). Therefore, the progression of the disease is slow.

Relative preservation of the integrity of the proximal muscles, or at least preservation for a much longer time than of the distal muscles.

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Corresponding author and reprints: A. Gordon Smith, MD, Department of Neurology, University of Utah School of Medicine, 50 N Medical Dr, Salt Lake City, UT 84132 (e-mail: Gordon.smith@hsc.utah.edu).