Infantile cerebral degeneration : with symmetrical changes at the macula / by E. C. Kingdon and J. S. Risien Russell.

  • Kingdon, E. C.
Date:
1904
Catalogue details

Licence: In copyright

Credit: Infantile cerebral degeneration : with symmetrical changes at the macula / by E. C. Kingdon and J. S. Risien Russell. Source: Wellcome Collection.

Provider: This material has been provided by UCL Library Services. The original may be consulted at UCL (University College London)

    11/48 (page 7)
    December 26th, 1893.—The child being now seven and a half months of age, was again seen, and admitted to the hospital, where it remained until its death. The general appearance, nutrition, and muscular weakness corresponded to that of the first case ; but she was more fretful and less apathetic than her brother. Ophthal- moscopic examination revealed the typical changes in the fundus of each eye, similar to those met with in the boy. Hearing was acute, and the sense of taste preserved. The muscles at the back of the neck and intra-scapular region were obviously atrophied. The electrical reactions of the muscles were not tested. Cutaneous sensibility was appa- rently normal. Her weight was 15 lbs. The appetite was good, the bowels regular, and there was no sickness. The heart, lungs, urine, and temperature v^ere normal. During the first fortnight after admission, the patient gained 1^ lbs. in weight, and on January 25th, 1894, was shown at a meetiog of the Ophthalmological Society in London. After this temporary increase in weight no further improvement occurred; on May 2nd she still only weighed 16 lbs., and the subcutaneous fat, as well as the muscles generally, had wasted. Rigidity of the latter was now evident, the head was retracted, the left forearm pronated and partially flexed, with flexion of the fingers and thumb of the left hand, both the thighs were ad- ducted, the legs exteuded, and the great toes extended at the metatarso-phalangeal and flexed at the inter-phalangeal joint. During June the general aspect of the patient grew worse, the wasting and rigidity of the body increased, and the face wore a greater cerebral expression; the appetite remained good, and the temperature normal. During July and August there were occasional attacks of vomiting and diarrhoea, and food was given with diffi- culty. The rigidity of the lower limbs was extreme, so that they could not be bent. The thighs were flexed on the abdomen, rotated inwards and adducted until the knees