HEREDITARY NON-PROGRESSIVE MUSCULAR DYSTROPHY INDUCING ARTHROGRYPOSIS SYNDROME12 BY

CLASSICAL arthrogryposis multiplex congenita is a striking clinical condition that has been recognized for over one hundred years. Otto in 1841 originally described an infant born with rigidity of the limbs which were fixed in certain bizarre positions. Since then several hundred cases have been described in numerous articles and the clinical features have been clearly outlined (Brandt, 1947; Banker, Victor and Adams, 1957; Mead et ah, 1958). Many authors have reviewed the historical aspects of this disorder and most of these will not be referred to again. Various concepts as to the fundamental nature of the disease have been entertained and until recently only limited pathological verification for one or another theory was available. Originally the popular notion prevailed that there was a primary disorder of the joints or periarticular tissues. This prompted Rosenkranz in 1905 to report on congenital contractures of the extremities and to first use the term arthrogryposis, meaning curved joints. Rocher in 1913 suggested the title of "multiple congenital articular rigidities " and Stern in 1923 consolidated this into the clumsy but most lasting term "arthrogryposis multiplex congenita." Because of the very nature of the disease the great majority of cases have been reported in the orthopaedic literature, the largest of which is a description of the features of 40 cases by Mead, Lithgow and Sweeney in 1958. It has been shown that fusion of the joints in an ankylosing process does not occur in arthrogryposis, for at autopsy when the periarticular tissues and tendons are sectioned the joints swing free. Originally th