Page:Brain Volume 31 Part 1.pdf/4

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ORIGINAL ARTICLES AND CLINICAL CASES

condition was confined to the legs and lower trunk; and that these cases were not of a severe type. It is possible that in these three patients the condition was present at birth, and that it became more obvious when attempts were made to support the weight of the body upon the legs.

We are of opinion that several writers have carried this argument too far for the purpose of reducing exceptions in attempting to make clear a new clinical entity, and we are bound upon the present evidence to emphasize a post-natal as well as a pre-natal onset for Oppenheim's disease.

In three cases the condition came on rapidly and reached its maximum within a few days of the onset of an acute illness in a previously healthy child. In one of our cases (13) an attack of acute bronchitis was followed by "complete paralysis" within a week in a child of twelve months of age, who had been up to this time apparently quite healthy. In Leclerq's case a healthy child, aged seven weeks, was seized with broncho-pneumonia and became universally paralysed within a few days, and though slow improvement had gone on all the time, four years later the case was typically one of amyotonia congenita. In Comby's case (15) the amyotonia came on rapidly following an attack of diarrhoea at four weeks, and was almost universal in its extent and severe in degree, but four months later the upper extremities and trunk had completely recovered, but a typical condition of amyotonia still remained in the lower extremities. The history of these three cases hardly admits of the explanation that we are here dealing with the effect of an acute illness in bringing into evidence an amyotonic condition already present in minor degree, but it suggests strongly that an acute toxic process was the cause of the amyotonia.

In the three other post-natal cases—(12) (Collier and Wilson), (21) (Rosenberg), and (22) (Schüller)—the onset was slow and was not preceded by any sign of general ill-health. In one case the power of observation of the mother was so limited as to make it extremely likely that the amyotonia was present at the time of birth, but there is no evidence that this was so in the cases of Schüller and Rosenberg. It seems probable that these are cases of a congenitally installed disease with a slow post-natal development of symptoms which, having lasted for a certain term, gives place to a tendency to marked amelioration.

Sex.—There seems to be a greater incidence of the disease upon males. Of twenty-five cases fourteen were males and ten were females. In the remaining case the sex is not stated.