Spongy Degeneration of the Brain in Infancy

Fülszöveg

This monograph contains the first com-plete description of the originál observa-tions made on a new degenerative familial disease described by the authors in 1949. Since that date the disease has been iden-tified all over the world in overthirty pu-blished and verified cases. The authors have never made known their originál cases in their entirity, as they had always hoped to be able to complete their clinical and histopathoiogical description by a multidisciplinary approach and by a de-tailed pathogenic study. The roots of the disease remain unknown. The interest of this book rests in the fact that it contains the first documentation in extenso both of the full dissussion of differential diagnosis and of the study of other neuroiogical ailments, either infan-tile or infanto-juvenile, which on account of their spongy character have certain similarities with recent cases. One of the authors' main aims is to show that the first case described by Canavan, which has led certain... Tovább

Fülszöveg

This monograph contains the first com-plete description of the originál observa-tions made on a new degenerative familial disease described by the authors in 1949. Since that date the disease has been iden-tified all over the world in overthirty pu-blished and verified cases. The authors have never made known their originál cases in their entirity, as they had always hoped to be able to complete their clinical and histopathoiogical description by a multidisciplinary approach and by a de-tailed pathogenic study. The roots of the disease remain unknown. The interest of this book rests in the fact that it contains the first documentation in extenso both of the full dissussion of differential diagnosis and of the study of other neuroiogical ailments, either infan-tile or infanto-juvenile, which on account of their spongy character have certain similarities with recent cases. One of the authors' main aims is to show that the first case described by Canavan, which has led certain Anglo-Saxon authors to put forward the narne of "Canavan type diffuse sclerosis", is not recog-nised by the present authors as a new entity but, indeed, as a periaxial ence-phalitis of Schilder. Neither did Canavan himself insist upon the very particular glial reaction which characterises the illness. The authors describe t'ne genetic character of the disease and its very frequent though not exclusive incidence in Jewish groups, especially those coming from eastern Poland.
This new disease with its completely baffling pathological mechanism will cer-tainly attract the attention of paediatri-cians, geneticists, pathologists and neu-rologists. Vissza