Abstract

Arthrogryposis multiplex congenita is believed to be a specific clinical entity which is aetiologically unrelated to the "arthrogryposis-like" deformities of known neurological diseases such as myelomeningocele and myelodysplasia. The observation that the condition appeared to be three times as common in Melbourne, Australia, as in four centres in the United Kingdom (Wynne-Davies and Lloyd-Roberts 1976), prompted this survey of 132 patients: 73 from the United Kingdom, 34 from Australia and 25 from Wilmington, Delaware, USA. The survey aimed to established the same criteria for diagnosis in the three countries and to search for prenatal and genetic aetiological factors. It was shown that all centers treated more newborn children with this disorder during the 1960s than either before or after that period. All cases were sporadic and there was no family association with talipes equinovarus, congenital dislocation of the hip or hereditary neuromuscular disease. "Environmental" findings from all three centers were similar and it was concluded that arthrogryposis multiplex cogenita is a non-genetic disease of early pregnancy, associated with a variety of unfavourable intra-uterine factors. In addition, an unknown but possibly viral environmental agent may have been present to a significant extent only during recent decades and is now declining.