Clinical features of Emery-Dreifuss muscular dystrophy. (A) Clinical characteristics of an 18-year-old male patient with proven mutation in the LMNA gene (see Fig. 2). The phenotype is suggestive of EDMD with limitation of neck flexion and marked elbow and heel contractures. Due to complete atrioventricular block, an implantable defibrillator has been inserted at age 18. Courtesy of J.-A. Urtizberea, Service de Mèdecine Physique et Rèadaptation de l'Enfant, Hôpital Raymond Poincarè, Garches, France. (B) Clinical features of partial lipodystrophy of the Dunnigan type in a 39-year-old woman. Note the accumulation of adipose tissue in the face and neck, constrasting with peripheral lipoatrophy with muscle prominence. This clinical aspect appeared gradually after puberty and the patient subsequently developed insulin-resistant diabetes and hypertriglyceridemia.