BOOK REVIEWS
Lipid Storage Diseases. Enzymatic Defects and Clinical Implications, by J. BERNSOHN AND H. J. GROSSMAN (Eds.), ix + 316 pages, 59 illustrations, 38 tables, Academic Press, New York, 1971, US $ 9.50. During the past decade or two immense advances have been made in many diseases which since Garrod's writings early this century have been grouped under the general term of inborn errors of metabolism. Perhaps those receiving most attention recently are included in the title of the book under review. This volume presents the proceedings of an American conference at Chicago in 1970 where a group of interested and extremely able scientists gathered to discuss some of the latest features of these disorders, including a new one called lactosyl ceramidosis. There are three main sections of which the first deals with the morphological and clinical aspects. Dr. R. D. Terry discusses in his usual lucid manner the general histological as well as ultrastructural characteristics with special reference to the membranous cytoplasmic bodies, as typically seen in Tay-Sachs disease. He has presented evidence as to physical qualities and the place lysosomes play in the disorders. Niemann Pick's disease and Hurler's disease are shown by Dr. A. C. Crocker, who has had unrivalled experience, to have many features in common; yet there are also marked biochemical differences. Drs. R. Y. Moore and N. J. Lenn discuss some of the clinical and pathological aspects of a number of lipid diseases illustrating their thoughts with a number of case records. These three chapters are well presented and can easily be understood even by a neurochemist. Each is followed by a record of an interesting discussion relative to each topic. Following this introduction there are six chapters which deal with the biochemistry of the enzyme defect and the nature of the biochemical lesion in a number of lipid diseases. Apart from one paper by Dr. N. S. Radin each is the result of collaborative effort in presentation--although Dr. Radin would be the first to acknowledge how many of the
Brain Hypoxia (Proceedings of the International Symposium on Brain Hypoxia, August 26-28, 1970, at the Medical Research Council Laboratories, Carshalton, Surrey). (Clinics in Developmental Medicine, No. 39/40, Spastics International Medical Publications), by J. B. BRIERLEY AND B. S. MELDRUM (Eds.), with a preface by P. E. POLAN1, xvi + 320 pages, 195 illustrations, 37 tables, Heinemann, London, 1971, £ 4.50. This book is composed very largely of the papers of contributors with only very scanty reporting of
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studies reported by him .are the work of the team he leads in his studies on brain cerebroside about which he spoke. The enzymes reported upon include those involved in globoid (cell) and metachromatic leucodystrophy, in Fabry's disease and the newly characterised condition already mentioned. These six chapters are full and detailed reviews of the position of the various enzymic abnormalities illustrated by records of biochemical investigation in specific cases and achieve a very high academic standard. They may be tough going for the clinician but would be well worth the trouble involved. One important practical point raised concerns the relative ease with which diagnosis can be made in early life as well as by the examination of amniotic fluid during pregnancy. In the chapter on cerebroside metabolism some interesting points are made relative to lysosomal disorders, steroids and multiple sclerosis. Lastly there are three final chapters relating to future research, partly by reference to that already done. Dr. J. S. O'Brien and his colleagues consider ganglioside storage diseases, and their various enzymic abnormalities, while Dr. R. O. Brady presents another of his typical writings concerning complex lipid metabolic changes. The final chapter on genetic engineering by Dr. B. S. Strauss is a fascinating and thought-provocating essay..The idea of producing gene change in man as can be done in bacteria could be of great practical value. The suggestions made include specific mutation, replacement of genes and addition of new gelaetic material. Yet at the end consideration is given to diagnosis with subsequent abortion as one method in the management of genetic disease. This volume is worthy of being read and studied by neurochemists and by neurologists and it will repay the time spent. It is well prepared and documented with few positive errors. However, it must be recorded that the literature quoted is almost, although no quite, entirely from North America, thereby failing on a number of occasions to give correct priority to studies made by others, and in some cases even failing to mention nonAmerican sources. J. N. CUMINGS
subsequent discussion and comment. Contributors are 47 in number from seven nations coming from the fields of neuropathology, neurosurgery, neurology, neurophysiology, neuropsychiatry and anaesthesia. A part of the book is devoted to each of seven sessions on cerebral blood flow, electron-microscopy, some biochemical aspects of cerebral metabolism in hypoxia, post-hypoxic brain swelling, atmospheric decompression, hypoglycaemia and epilepsy. An account of the concluding discussion is again brief.
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BOOK REVIEWS
The section on cerebral blood flow collects together, in papers on experimental studies on primates and classical necropsy investigations, morbid anatomical data well known to neuropathologists. Marshall and his colleagues present some of the results of their isotope studies in subarachnoid haemorrhage, expressing the hope that this may eventually prove to be of value in the management of individual clinical cases. The chapter by Brock on cerebral blood flow and intracranial and tissue pressure changes in brain hypoxia is the least tangible in the whole book. His treatment of these problems is largely theoretical, but raises many interesting points for discussion, the report of which is severely curtailed. The second section on electron-microscopy features a satisfying verbal account of ultrastructural changes marred, as is often the case, by poorly presented electron-microphotographs. The chemical aspects of cerebral metabolism at low perfusion pressures, in arterial hypoxaemia and during anaesthesia, presented in Part 3, have been very generously pitched at a level which the nonbiochemist can understand and indeed enjoy. The next section, on post-hypoxic brain swelling, is lively. It is obvious that the pathogenesis of this change is still not understood, but the zealous application of the efforts of several competent investigating teams, as evidenced by their papers presented here, gives promise that this problem --which is of no inconsiderable clinical im-
portance--may soon be solved. Sections on atmospheric decompression will be of increasing importance as aerospace and supersonic engineering proliferates. Neuropathological. behavioural and physiological data on baboons exposed experimentally to decompression are presented in five papers. What was evidently a vigorous discussion is again reported in a tantalizingly brief fashion. In the final two parts, on hypoglycaemia and epilepsy, similar biochemical, physiological and neuropathological material is presented, The stresses produced in these experimental models also lead to the disappearance of affected neurons. There is an obvious association with the perinatal problems of hypoglycaemia, ischaemic anoxia and trauma. The value of the experimental approach to these problems is well demonstrated. Although much of what is written has appeared in the scientific literature before and since the Symposium, its being gathered together in one place, spanning the disciplines, is invaluable. The editors must be applauded. The literary style is all through workmanlike rather than elegant, information being given out at a steady pace. Only the introduction is a bit congested and reads like an effort of Gibbon's. The index and author index are adequate. Only two figures, those on pages 164 and 167 did I find unintelligible. This book can be highly recommended to neurological workers of all denominations. PETER O. YATES
Mental Health of Adolescents and Young Persons (Report on a Technical Conference, Stockholm, 9-13 June, 1969) (Public Health Papers, No. 41), by A. R. MAY, J. H. KAHN AND B. CRONHOLU (Eds.), 72 pages, 1 illustration, 7 tables, World Health Organization, Geneva, 1971, 50 p. ; US $ 1.75; Sfr 5.00.
surmise that this apparent superficiality is a reflection of the multidisciplinary nature of the conference which may have precluded any technical depth discussion. In parts it provides interesting background reading, but mostly it is too superficial for anyone seriously concerned with the problems of adolescence. However, it may well appeal to sophisticated lay readers, especially those wanting a brief account, including epidemiology of alcohol and drug abuse in adolescents which is the one area in which there is reasonable cover. 1. KOLVIN
This is a report of the working papers and discussion at a WHO technical conference. It covers a wide range of topics but only in general terms and contains little that is new or original. One can only
J. neurol. Sci.,1972, 16:499-502