other cases. In 1903 Dr. F. E. Batten [1] showed three patients before the Neurological Society of London which were undoubtedly cases of amyotonia congenita. He described them as cases of "Myopathy of an Infantile Type.” We have included these cases in our list as they were certainly the first to which attention was drawn in this country. Our four cases have shown certain features to which attention has not hitherto been drawn, and the object of this paper is to point out these features, to call attention to Oppenheim's malady as a distinct and important clinical entity, and to put before the reader a complete account of all the cases which have been recorded up to the present time.
The essential diagnostic characters of Oppenheim's disease that will serve to distinguish it at once from all other diseases may be stated in a few words, which are here emphasized:—
"A condition of extreme flaccidity of the muscles associated with an entire loss of the deep reflexes, most marked at the time of birth and always showing a tendency to slow and progressive amelioration. There is great weakness but no absolute paralysis of any muscle. The limbs are most affected, the face is almost always exempt. The muscles are small and soft, but there is no local muscular wasting. Contractures are prone to occur in the course of time. The faradic excitability in the muscles is lowered and strong faradic stimuli are borne without complaint. No other symptoms indicative of lesions of the nervous system occur."
Under the name of congenital muscular atonia Sorgente [24] has described two cases occurring among the children of the same mother (vide p. 42). These cases seem to us to depart too widely from the type of Oppenheim's malady, which in all other reported cases has been so regular, to be justifiably included in this description in the present state of our knowledge. But since we have ourselves had the opportunity of observing a case of Sorgente's familial type, and since a complete pathological investigation was made by Dr. Batten, we shall here exclude these three cases from our general consideration of Oppenheim's disease, and we shall describe them in detail separately, leaving subsequent investigation to determine their position in relation to the usual type of congenital amyotonia. Our reasons for excluding these cases are the following:—
- The malady was familial. In Sorgente's cases two children of the same mother were affected; in our case five children of the same mother were affected.
- The paralysis was complete.
