[Emery-Dreifuss disease. A discussion of 2 new cases]. / A propos de la maladie d'Emery-Dreifuss. Discussion de deux nouvelles observations.

Two new cases of Emery-Dreifuss dystrophy in two brothers are reported. Both shared common characteristics: X-linked inheritance, early contractures, scapulo-humeroperoneal muscle weakness and wasting, cardiac conduction abnormalities with permanent atrial paralysis. A review of the literature is presented. Genetically, the disease appears as a syndrome rather than an entity because of recently published cases with dominant autosomal inheritance. Finally, contractures and their relationship with myosclerosis, notably rigid spine syndrome, are discussed. Fibrosis could be the common feature of both cardiac and muscular abnormalities. It could explain the ambiguous results of electromyogram and muscle biopsy.

[Familial thenar amyotrophy of carpal origin]. / Amyotrophies thénariennes familiales d'origine carpienne.

Thenar amyotrophy of carpal origin was found in two sisters aged respectively 49 and 59 years and in a 75 year-old woman and her 56 year-old daughter. The literature contains reports on about 15 cases of familial carpal tunnel syndrome. The clinical features were sensory symptoms in most patients but there was also cases with amyotrophy. The coexistence in a same family of several cases of carpal tunnel syndrome is not by itself, evidence of a genetic factor.