Congenital malformations of the brain and skull, part II

Congenital malformations of the brain and skull, part II

201 Early Human Development, 1978, 2/2,201-202 o Elsevier/North-Holland Biomedical Press BOOK REVIEW Congenital Malformations of the Brain and Sk...

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Early Human Development, 1978, 2/2,201-202 o Elsevier/North-Holland Biomedical Press




of the Brain and Skull, Part II

Handbook of Clinical Neurology, volume 31 P.J. Vinken and G.W. Bruyn (eds.), in collaboration poulos (632 pages, 310 illustrations, 90 Tables) North-Holland, Amsterdam, 1977 Dfl. 245.~-/US$ 99.25

with N.C. Myriantho-

This volume of the well-known Handbook of Clinical Neurology is the second of a three volume series. The title is somewhat misleading as far as this particular book is concerned, for the topics included mainly involve generalized or widespread abnormalities in which central nervous defects form but one aspect, rather than specific malformation of the cranium or central nervous system. The twelve chapters fall into two separate groups. The first eight constitute individual subjects which bear little relationship to each other. These include the phakomatoses, congenital tumours of the brain, congenital arachnoid cysts, congenital aneurysms and arteriovenous malformations, syndromes with infectious pathogenesis, syndromes of skeletal abnormalities with CNS malformations, growth deficiency syndromes, and syndromes with known or probable metabolic aetiology. The last four chapters, which comprise the second half of the book, are devoted to a comprehensive coverage of chromosomal abnormalities. The individual articles of the first half of the book are in general useful reviews of their subjects of a convenient length for reference purposes, with adequate illustrations and extensive bibliographies. Catherine Haberland’s chapter on the phakomatoses with which the work commences is devoted to discussion of the five major conditions of this group, tuberous sclerosis, Von Recklinghausen disease, Von Hippel-Lindau disease, Sturge-Weber disease, and ataxia telangiectasia. The remaining rare conditions are dealt with in tabular form. There are large, well-produced illustrations, and references up to 1976 are included. The contributions on congenital tumours of the brain by’Tonio Ohta and colleagues, and congenital arachnoid cysts by Cheng Mei Shaw and Ellsworth C. Alvord are similarly useful. In contrast, I found that the chapter on Syndromes of Skeletal Anomalies with CNS Malformations by Robert J. Gorlin and Heddie 0. Sedan0 a depressing example of pure syndromology. There are descriptions and differential diagnoses of 21 different syndromes but no hint as to what the clinician may do to relieve any of the effects of these distressing conditions or indeed


where he may find the appropriate information. The account of congenital aneurysms and arteriovenous malformations by Jorge C. Lagos is marred for this reviewer by the inaccuracy and inadequate references given when discussing intraventricular haemorrhage and intracerebral haemorrage in the newborn among the differential diagnoses of congenital vascular anomalies. One hardly expects Craig’s 1938 paper on intracranial haemorrhage in the newborn to be quoted as the definitive work on the subject in a book published in 1977! However, the most important part of this volume is the second half. This opens with a chapter on the human chromosomes and their aberrations by David M. Cox and Manoranjan Ray. The chapter outlines the history of modem cytogenetics, the main techniques in use, the nomenclature of chromosome bands and that used in describing aberrations and the various mechanisms by which chromosome abnormalities occur. The text is illustrated by informative diagrams and good photomicrographs. This particular contribution provides in 24 pages all the background information on cytogenetics that the average clinician could want. The chapter on Down syndrome by Hans Zellweger is an important and comprehensive loo-page monograph covering all aspects of the subject. The author disarmingly dismisses the 750 odd references as a selected bibliography. Chromosomal aneuploidies other than Down syndrome are covered in a separate article by a team of authors under Dr. Zellweger in similar comprehensive detail. The two chapters constitute valuable reference works giving full descriptions of the many mosaic forms and mixed trisomies relating to the various conditions and the problems of differential diagnosis. References are given up to the end of 1974. The final article in this section on chromosomal deletions and ring chromosome syndromes by Marie Odile Rethore is less successful as it contains excessive detail of individual case reports which swamp the main features of each syndrome described. The mixture of subjects covered in this work are all ones on which the paediatrician or paediatric pathologist may require a readily available work of reference providing more information than is to be found in a general paediatric textbook. For such a purpose, particularly in relation to the chromosomal abnormalities, this work can be thoroughly recommended. It is, however, apparent from a volume such as this how few medical authors can write a comprehensive review of their subject in a readable style. The only chapter which I found a pleasure to read was that by Cox and Ray referred to above, which is a synopsis of the most complex subject covered in the work, rather than a comprehensive review. If all contributions had been as well planned and written, I suspect that the entire contents of the three volume series might have been encompassed in a single book of this size. What a work that could have been! Jonathan