PROGRESSIVE MUSCULAR ATROPHY

In 1849 Duchenne described a patient with spreading progressive muscular atrophy that started in the hands and spread slowly to the arms and legs, with no sensory signs, pain, or sphincter disturbance. Characteristically self effacing, he did not publish the case himself, but passed on his observations to François Amilcar Aran, physician to the Hôpital Saint Antoine. Aran published the paper1 and acknowledged: “I owe a thousand thanks to my friend Duchenne de Boulogne who freely put at my disposal all his material …”

Duchenne’s account (translated by G V Poore) followed in 18702: “Muscles … often jerked by little fibrillary or partial contractions … agitated with worm-like movements. Progressive muscular atrophy attacks the upper limbs, and destroys its muscles in an irregular fashion. It begins in such cases by attacking one after another the muscles of the thenar eminence, spreading from the superficial to the deep layer. As soon as the abductor pollicis is wasted, its absence is marked by a depression, and by the attitude, during repose, of the first metacarpal bone, which lies too close to the second … Depressions of the hypothenar eminence and interosseal spaces next announce the atrophy of the muscles of those regions. The loss of the interossei muscles is shown by the claw-like attitude of the fingers … The atrophy may remain localised for many years … The flexors of the elbow and the deltoid are the first to atrophy. The triceps extensor cubiti is the last of the …