Paralysis with muscle atrophy

Abstract

The mid-1800s were fruitful for French neurologists, especially at the Hôpital de la Charité in Paris. At this hospital, Pierre Eloy Fouquier, the protégée of renowned physicians Corvisart and Laënnec, worked as the private physician of Charles X and Louis Philippe, kings of France. It was here that Fouquier met François-Amilcar Aran who, in 1850, described a new disease that he referred to as “progressive muscular atrophy”.1Aran FA Recherches sur une maladie non encore décrite du système musculaire (atrophie musculaire progressive).Archives Générales de Médecine. 1850; 4 (172–214 (in French).): 5-35Google Scholar Later, commenting on a presentation at the Royal Medical and Chirurgical Society in 1851, Edward Meryon went on to officially describe fatty degeneration of skeletal muscles. He discussed several cases of young boys who had experienced increasing weakness of the limbs that could not be halted “in spite of all remedial measures”, and in particular noted the condition's predilection for male patients and its familial nature.2Meryon E On fatty degeneration of the voluntary muscles (report of the Royal Medical and Chirurgical Society).Lancet. 1851; 2: 588-589Google Scholar In 1853, Jean Cruveilhier described progressive atrophic muscle paralysis (“paralysie musculaire progressive atrophique”),3Cruveilhier J Sur la paralysie musculaire progressive atrophique.Archives Générales de Médecine. 1853; 5 (in French): 561-603Google Scholar the characteristics of which match those of amyotrophic lateral sclerosis, later known as Charcot disease. At the time, the topic generated controversy, with Cruveilhier defending the disease as a primitative nerve lesion, whereas Duchenne de Boulogne argued for initial muscle involvement. Duchenne is indeed renowned for his later contribution to the specialty of muscular diseases: the eponym Duchenne muscular dystrophy is applied to the most common and most severe dystrophy in childhood.4Duchenne (de Boulogne) G De la paralysie musculaire pseudo-hypertrophique ou paralysie myo-sclérotique.Archives Générales de Médecine. 1868; 6 (179–209, 305–21, 421–43, 552–88 (in French).): 5-25Google Scholar Interestingly, although Aran and Meryon were believed to be the first to describe this motor neuron disease, we had the opportunity to obtain a handwritten unpublished medical course by Fouquier (“Cours complet de médecine”), dated 1817. In this text, there is a chapter dedicated to paralysis that includes descriptions of “paralysis with muscle fatty degeneration and atrophy”. Thus, it seems that Fouquier, rather than Meryon or Aran, was in fact the first to describe this disorder. The mid-1800s were fruitful for French neurologists, especially at the Hôpital de la Charité in Paris. At this hospital, Pierre Eloy Fouquier, the protégée of renowned physicians Corvisart and Laënnec, worked as the private physician of Charles X and Louis Philippe, kings of France. It was here that Fouquier met François-Amilcar Aran who, in 1850, described a new disease that he referred to as “progressive muscular atrophy”.1Aran FA Recherches sur une maladie non encore décrite du système musculaire (atrophie musculaire progressive).Archives Générales de Médecine. 1850; 4 (172–214 (in French).): 5-35Google Scholar Later, commenting on a presentation at the Royal Medical and Chirurgical Society in 1851, Edward Meryon went on to officially describe fatty degeneration of skeletal muscles. He discussed several cases of young boys who had experienced increasing weakness of the limbs that could not be halted “in spite of all remedial measures”, and in particular noted the condition's predilection for male patients and its familial nature.2Meryon E On fatty degeneration of the voluntary muscles (report of the Royal Medical and Chirurgical Society).Lancet. 1851; 2: 588-589Google Scholar In 1853, Jean Cruveilhier described progressive atrophic muscle paralysis (“paralysie musculaire progressive atrophique”),3Cruveilhier J Sur la paralysie musculaire progressive atrophique.Archives Générales de Médecine. 1853; 5 (in French): 561-603Google Scholar the characteristics of which match those of amyotrophic lateral sclerosis, later known as Charcot disease. At the time, the topic generated controversy, with Cruveilhier defending the disease as a primitative nerve lesion, whereas Duchenne de Boulogne argued for initial muscle involvement. Duchenne is indeed renowned for his later contribution to the specialty of muscular diseases: the eponym Duchenne muscular dystrophy is applied to the most common and most severe dystrophy in childhood.4Duchenne (de Boulogne) G De la paralysie musculaire pseudo-hypertrophique ou paralysie myo-sclérotique.Archives Générales de Médecine. 1868; 6 (179–209, 305–21, 421–43, 552–88 (in French).): 5-25Google Scholar Interestingly, although Aran and Meryon were believed to be the first to describe this motor neuron disease, we had the opportunity to obtain a handwritten unpublished medical course by Fouquier (“Cours complet de médecine”), dated 1817. In this text, there is a chapter dedicated to paralysis that includes descriptions of “paralysis with muscle fatty degeneration and atrophy”. Thus, it seems that Fouquier, rather than Meryon or Aran, was in fact the first to describe this disorder.