Page:Brain Volume 31 Part 1.pdf/3

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AMYOTONIA CONGENITA
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  1. There was complete loss of electrical excitability in the affected muscles.
  2. All the cases ended fatally within a few weeks.
  3. None of the above features have occurred in any of the cases here included as cases of Oppenheim's disease.

Our best thanks are here expressed to Dr. Beevor for his permission to make use of two patients under his care in the National Hospital; to Dr. Whait, of Hampstead, for the early notes of the patient who had been under his care for several years, and for his kindness in affording us every facility to examine and photograph his patient: and to Professor Oppenheim, who was good enough to examine one of our patients and to give us the advantage of his valuable opinion and comments upon the case.

Causation.

A careful search through the history of the recorded cases for any possible antecedents and factors in causal relation with this strange malady have thrown no light upon its etiology. Certain negative facts, however, are of considerable importance, especially in the distinction of this malady from other forms of paralysis occurring in the earliest years of childhood.

Age.—In nearly all cases the paralysis has been obvious at the time of birth or it has been noticed so few hours after birth as to make it certain that the condition has been pre-natal. This was so in eighteen out of twenty-five cases, but in four of the remaining cases it seems certain that the paralysis either appeared or became much aggravated many months after birth. These cases demand special consideration, for they form exceptions to one of the most characteristic features of this disease. In Schüller's case it is certain that the child was generally weak from birth, but it is also certain that, having learned to sit and stand at ten months of age, the child became much worse as regards weakness and atonia and lost the power of standing and sitting up. In Rosenberg's case the child seemed normal at birth: it learnt to sit up at seven months and the paralysis and flaccidity of the legs were not noticed by the parents till the child was eleven months old. In one of our cases the child seemed natural to its parents until it was nine months old, when weakness of the legs became rapidly manifest.

We are justified in agreeing with other authors that in some of these cases the condition became obvious just at the time when some attempt is usually made by parents to get a child upon its legs; that the