Alzheimer's Disease
Review of: Alzheimer's Disease and Related Conditions. A Ciba Foundation Symposium. Ed. by G. E. W. Wolstenholme and Maeve O'Connor. London, Churchill, 1970.
Journal of Medical Genetics, Vol. 8, October 1970, p. 252.
Alzheimer’s disease is a moderately infrequent form of presenile dementia, with well defined course and clinical picture, and characteristic cerebral pathology. Genetically it is interesting because of the indications of genetic heterogeneity within this clinico‑pathologically delimited syndrome. A fair number of families have been described, in his contribution to the symposium Pratt says "more than a dozen", in which a completely typical form of autosomal dominant inheritance is shown, with equal sex representation, and nearly complete penetrance. In comprehensive series, however, selected on a clinico‑pathological basis, these typical families do not appear; and what one sees instead is a moderately raised familial incidence and a female preponderance of about 3 : 1, i.e. the indications of a polygenic genetical basis. The genetical evidence, therefore, is strongly in favour of regarding the syndrome as being a mixture of at least two different and independent conditions. This is a conclusion which, for the most part, both clinicns and pathologists are unwilling to accept, as is made plain in these papers.
The symposium reported here was predominantly a pathological one, with interesting and original pathological work. Participants came from Belgium, Germany, the Argentine, Sweden, Hungary, the U.S.A. (7) and Britain (10). Active work is going on in histology, histochemistry and electron‑microscopy. Most promising, perhaps, is the study of the ultramicroscopic structure, i.e. the abnormal intracellular microtubules, which differentiate more than on kind of lesion, and may lead to a successful. breaking down of the clinico‑pathological continuum extending into normal senescence, senile dementia and cerebrovascular disease, by which most workers are at present both obsessed and bewildered.
The reviewer would suppose that this book is an important one for the neuropathologist. Its genetical interest is not great. Apart from Pratt's paper reviewing the present state of genetical understanding, main interest lies in the communication by Jacob (Marburg) of a family with six females in three generations showing a form of Alzheimer’s disease in every case, the unusual feature of molouic twitchings appearing the course. Jacob reviews the literature.
The book is well produced, with an abundance of good pathological illustrations, good papers, lively discussions,rather inconclusive results.