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The Pathology of Acute Infantile Cerebral Diplegia

Department of Pathology, Midland Centre for Neurosurgery, Smethwick

Part of this work was done while holding a Travelling Fellowship from the British Postgraduate Medical Federation.

ABSTRACT

1. The characteristic features of Acute Infantile Cerebral Diplegia were illustrated by clinico-pathological reports on three cases. The disease typically affects infants and young children who are suffering from fever with dehydration or suppuration in the head or neck. The onset is sometimes, but not always, ushered in by convulsions, which may be bilateral or unilateral. Coma may ensue. On recovery, the patient is di-, tri-, or quadraplegic and may show other manifestations of cerebral degeneration, such as blindness, aphasia or dementia. The chronic phase may persist for many years until death ensues from intercurrent infection.
   The distinction from Little's Disease, i.e. Congenital Cerebral Diplegia, in which the disability is noticed at or soon after birth, was emphasized.
   
2. The changes in the brains from the cases studied were remarkably uniform and consisted of a diffuse sclerosis of the white matter of the dorsal parts of both cerebral hemispheres, with less extensive laminar sclerosis or ulegyria of the cortex. Softening was not seen in the cortex or white matter and the demyelination shown by the latter was less marked than the sclerosis.
   It was considered that the changes in the white matter were typical of the sequelae of oedema and that the cortical degeneration was due to an accompanying, but less widespread, anoxia, such as is seen in cerebral venous thrombosis. In view of the topography of the changes, it was considered that their most probable cause was a thrombosis of the superior longitudinal sinus. The clinical histories of the reported cases were shown to be consistent with this concept.