Chronic diffuse and focal leptomeningitis.
The microscopic examination confirmed the diagnosis of paresis. The hypertrophic nodules were of special interest. They were found to be overlain by a characteristic though thin exudate of lymphocytes and plasma cells, together with pigmented cells. The nodules appeared to be supplied with an unusual number of vessels of small calibre, about which were a few lymphocytes. The large vessels and those with well developed adventitiæ were surrounded by more numerous lymphocytes and by more focal accumulations of pigmented cells. The cortex in the middle of a nodule had almost lost its characteristic cortical layering. The cortex was here reduced (specimen from temporal lobe) to about one-quarter of its normal thickness, and was found to be composed largely of expanded neuroglia cells and vascular tissue, with a few nerve elements, small, shrunken, and dark-staining. The destructive process appeared to have borne hardest on the layer of internal large pyramids and the fusiform layer. There was, however, nowhere any evidence of focal necrosis such as ought to characterize a true gumma. The sections stained by the Marchi method failed to show evidence of fatty degeneration within the focus, although there was a marked diffuse accumulation of fatty granulations along the nerve fibres in the underlying white matter. A special study of the cerebellar material was made by one of the authors.[[4]] Occasional Purkinje cells showed the characteristic binucleate condition, which has frequently been noted in recent literature.
The cerebellum of this case was perhaps the most markedly diseased of all portions of the nervous system. As noted, the cerebellar tissue was exceedingly firm. How far the notable incoördination of the case (he was observed on staff rounds characteristically curled up in a heap, showing quite an unusual degree of general incoördination) was due to the cerebellar lesions, it is perhaps not possible to say.
Summary: John Lawrence, Juvenile Paretic Neurosyphilis, is a foil to Case 3 (James Dixon), paretic neurosyphilis due to acquired syphilis.
Both showed Cerebral Atrophy, but Lawrence the more markedly because of hypoplasia incidental to the congenital origin of his condition.
Whereas Dixon gave little or no sign of stigmata, Lawrence (besides being under-sized, having suspicious teeth, and showing at autopsy a persistent thymus) showed a Hydromyelia and curious trefoil shape to the spinal cord. Dixon on the other hand had liver lesions and arterial lesions of the leg.
The suggestion of Tuberous Sclerosis in Lawrence is not found in Dixon; but we have not found it elsewhere. Bourneville did not describe tuberous sclerosis as syphilitic.
Binucleate Purkinje cells emphasize the congenital source of the lesions in Lawrence.
Plasmocytosis and Lymphocytosis, Perivascular, and (less marked) Meningeal, are found in both the congenital and the acquired cases, as also parenchymatous changes, both nerve cell losses and gliosis. Both also show granular ependymitis.
It is clear that, over and above the factors of destruction evident in both Lawrence and Dixon, the congenital case, Lawrence exhibits also the effects of arrest (in brief not merely atrophy but also hypoplasia). Early treatment is, therefore, theoretically indicated in the juvenile group, which means early diagnosis. Early diagnosis and treatment are still more to be recommended because these juvenile cases progress often very slowly at first.