Die Myopathien

The Facio-Scapulo-Limb (or the Facioscapulohumeral) Type of Muscular Dystrophy

“…the pelvic girdle and proximal parts of the legs being the main sites of involvement in the lower part of the body [Becker, 1952[Becker, , 1953[Becker, , 1964Walton and Nattrass, 1954;Walton, 1955Walton, , 1960Walton, , 1964Morton et al, 1963;Moser et al, 1966;E mery and Walton, 1967;Zellweger et al, 1972;Beckmann, 1972], Our purpose was to settle in some way the contradictions that have ap peared, and to elucidate the question of the sequence of extension of atro phy of muscle groups in these patients. We feel that the sequence of de velopment and the rate of myodystrophical process should be considered as the most important signs of different types of hereditary muscle affec tions.…”

“…The authors who have taken part in it may be divided into three groups. A first group considers that FSH myodystrophy should be included in 'the autosomal-dominant descending shoulder girdle form" [Becker, 1952[Becker, , 1964E rbsloh, 1958;G oebell, 1962;Moser et al, 1966;Brugsch and Brockmann-Rohne, 1958; Mertens et al, 1966; R icker and Mertens, 1968;Zellweger and McCormick, 1968]. In this case, the LandouzyDejerine facioscapulohumeral type and the scapulo-humeral variety of Erb's juvenile form are classified together.…”

“…Thus, in Southern Baden the incidence of the disease is 1:20,000 inhabit ant [Becker, 1964], while in the state of Wisconsin it is 3.8 per million [Morton and Chung, 1959].…”

“…In this case, the LandouzyDejerine facioscapulohumeral type and the scapulo-humeral variety of Erb's juvenile form are classified together. Though the data on clinical manifestations and genetics were taken into consideration by Becker [1953], nevertheless types of myodystrophies which are different from the genetic point of view are included into one nosologic unit.…”