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Über einen Fall von Panarteriitis der Hirngefäße mit ausgedehntem Markverlust

Acta Neuropathologica, ISSN: 0001-6322, Vol: 6, Issue: 3, Page: 220-230
1966
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Article Description

A 4 year-old boy suffered from generalized epileptic seizures, progressive rigidity with tetraplegia, blindness with papillary atrophy, deafness and deterioration and impairment of psychic efficiencies including speech. After about 2 years' duration of the progressive disease he expired in an apallic state. Neuropathological examination revealed: 1. generalized panarteriitis of the cerebral vessels with vascular focal lesions; 2. widespread total demyelination of cerebral and cerebellar white matter and cortex with 3. correspondent diffuse sclerosis; 4. accumulation of concrements, and sudanophilic decomposition. Arteriitic lesions in other organs were not to be found. In this case a chronic inflammatory process is of principal importance, mainly consisting in vascular lesions of the type of severe generalized cerebral panangiitis. The widespread myelin destruction in the whole CNS with diffuse sclerosis shows all criteria of vascular focal lesions though the large extent and expansion of the demyelinating foci cannot be explained by this fact. Sequelae of chronic edema and primary encephalitic reaction of the nervous parenchyma are suggested to have modified the complex pathological syndrome. An autoallergic process could be taken into consideration, the etiology of the process, however, being unknown. © 1966 Springer-Verlag.

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