Diffuse sclerosis (encephalitis periaxialis diffusa) / by L. Bouman ; with 64 illustrations.

  • Leendert Bouman
Date:
1934
Catalogue details

Licence: In copyright

Credit: Diffuse sclerosis (encephalitis periaxialis diffusa) / by L. Bouman ; with 64 illustrations. Source: Wellcome Collection.

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    there is often a slight tremor of the hands. The contractures can easily be relaxed but soon return. A certain amount of spontaneous movement is also now and then possible in the contracted parts. Both legs are a little Hexed (roughly speaking at an angle of 160°) at the hip-joints. Both ankle-joints are markedly adducted and extended, the soles are concave, and the foot is shortened by strong flexion contractures. It is not possible entirely to reduce this ‘club¬ foot/ position, the foot cannot be completely abducted although the plantar llexion can be passively adjusted. There are no spontaneous movements in the legs ; if one lifts the boy up and then tries to put him on his feet, the upper legs are crossed. The boy can see. Ophthalmologic examination : Both pupils equal, react to light and accommodation. Edges of the disc are not clear. Veins thick and tortuous. Signs of congestion in the papillae. Lumbar puncture revealed a pressure of 15 cm., cerebrospinal fluid clear. Du ring the first one and a half months after admission to hospital there was apparently a certain improvement, since the child put on weight ; the friendly grin, and the turning of the head from side to side, became more lively, whereas the frequent movements of the head and upper arms mentioned above were less noticeable. After a diffuse bronchitis accompanied by rise of temperature there was a continued regression ; the contractures in the upper arms became continuously worse, the patient became much thinner, the papillae of the eyes passed into a state of optic atrophy. After the disease had lasted about a year the patient died. At the autopsy it was found that the whole of the cerebrum was less in volume than normal, and there was an increase of fluid in the sub¬ arachnoid space. The pia was in many places opaque. It appeared that the whole of the white matter was unusually hard and resistant, the colour was dull yellow, like old ivory. The grey matter, too, was harder than normal, although its consistency had not been so much altered as that of the white matter ; its colour was pale grey and pale yellowish-grey ; white and grey matter were more sharply defined in relation to each other than in the normal brain. The cerebellum, too, had become harder in consistency, as also the pedunculi cerebri, pons, and medulla oblongata. The dia¬ gnosis of tumour had been made. Heubner says that had he possessed a more accurate knowledge of the symptomatology of diffuse brain sclerosis, he would perhaps have been able to make the diagnosis during the life of the patient. Schmaus describes a child from the Munich paediatric clinic. It was quite healthy up to the age of If years (it had already walked
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