Why is Charcot marie tooth disease called that?

Jean-Martin Charcot, answered from the texts and cited to the page.

Jean-Martin Charcot answers

The name is a matter of simultaneous discovery — the kind that occurs when a problem is ripe and several observers are working it at once. In 1886, Pierre Marie, who was among my most able students at the Salpêtrière, and Howard Henry Tooth in Cambridge each described, independently and at nearly the same moment, a form of progressive muscular atrophy beginning in the lower extremities and ascending, distinct from the classic Duchenne-Aran type.

My own name attaches because Marie worked under my direction and because I had myself been pressing, in these very years, for a more rigorous sorting of the progressive amyotrophies — separating the spinal from the myopathic, the protopathic from the deuteropathic. The clinical picture is precisely what I had been teaching: a young patient whose atrophy commences in the legs, produces that characteristic high-stepping gait as the dorsal flexors weaken, and then ascends to the upper extremities in turn.1

I showed such a case — a young woman of twenty-four, named Dall —, in whom the disease began at fourteen in the lower extremities and reached the hands by twenty, with flattening of the thenar and hypothenar eminences.2 The gait she exhibited, the foot dropping as the leg swings forward, obliging an exaggerated flexion of the knee, is precisely analogous to what one sees in alcoholic paralysis of the peroneal muscles.3

What Marie and Tooth established was that this form belongs not among the spinal amyotrophies — not among the Duchenne-Aran group — but among the primitive myopathies of non-spinal origin.4 The nosological work of disentangling it from the too-extensive Duchenne-Aran class was already underway in my lectures; their papers gave it a definitive clinical boundary and, in the way of science, attached three names to the result.

Sources

  1. The disease commenced at the age of fourteen, in the lower extremities; then the upper extremities were attacked in their turn at the age of twenty.
    Lectures on Diseases of the Nervous System, Series III
  2. Here is a young woman of 24 years of age, named Dall —, who had been attacked with amyotrophy of the lower extremities, or more correctly of the legs... the patient is scarcely able to walk without support.
    Lectures on Diseases of the Nervous System, Series III
  3. as a consequence of the feebleness of the muscles of the leg, the point of the foot falls when the patient, in walking, raises the leg so as to carry it forward... imitating the movements of a high-stepping horse. It is analogous to what is observed whenever the muscles which produce dorsal flexion of the foot are atrophied, as in alcoholic paralysis for example.
    Lectures on Diseases of the Nervous System, Series III
  4. it is highly probable that, like this, it can be classed amongst the primary progressive myopathies of non-spinal origin.
    Lectures on Diseases of the Nervous System, Series III