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Alpers disease

Alpers' disease, also called progressive infantile poliodystrophy, is a progressive degenerative disease of the central nervous system that occurs in infants and children. It is an autosomal recessive disorder that is sometimes seen in siblings. First signs of the disease, which include intractable seizures and failure to meet meaningful developmental milestones, usually occur in infancy, after the first year of life, but sometimes as late as the fifth year. more...

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Primary symptoms of the disease are developmental delay, progressive mental retardation, hypotonia (low muscle tone), spasticity (stiffness of the limbs) possibly leading to quadriplegia, and progressive dementia. Seizures may include epilepsia partialis continua, a type of seizure that consists of repeated myoclonic (muscle) jerks. Optic atrophy may also occur, often leading to blindness. Deafness may also occur. And, although physical signs of chronic liver dysfunction may not be present, many patients suffer liver impairment leading to liver failure. While some researchers believe that Alpers' disease is caused by an underlying metabolic defect, no consistent defect has been identified. Pathologically, there is status spongiosus of the cerebral grey matter.

Treatment

There is no cure for Alpers' disease and, currently, no way to slow its progression. Treatment is symptomatic and supportive. Anticonvulsants may be used to treat the seizures. However, caution should be used when selecting valproate as therapy since it may increase the risk of liver failure. Physical therapy may help to relieve spasticity and maintain or increase muscle tone.

Prognosis

The prognosis for individuals with Alpers' disease is poor. Those with the disease usually die within their first decade of life. Liver failure is usually the cause of death, although cardiorespiratory failure may also occur.

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Book reviews -- Human Biology in Papua New Guinea: The Small Cosmos edited by Robert D. Attenborough and Michael P. Alpers
From Human Biology, 10/1/94 by Tracer, David P

Human Biology in Papua New Guinea: The Small Cosmos, edited by Robert D. Attenborough and Michael P. Alpers. Oxford, England: Oxford University Press, 1992. 427 pp. $98.00 (cloth).

Upon glancing at the table of contents of this edited volume, I had the initial impression that its contributed chapters were about as diverse as the country that is their focus. The 22 chapters that constitute Human Biology in Papua New Guinea are indeed disparate; for the most part the studies were carried out independently rather than as pieces of some larger research project, and they deal with everything from archeology to linguistic diversity to allelic frequencies of various class I and II HLA types throughout the country. Yet, as I read this volume, I was impressed with the extent to which its individual chapters largely succeeded in presenting a detailed and coherent picture of the biology and biocultural adaptability of Papua New Guinea's (PNG's) indigenous human populations.

In the opening chapter the editors point out that PNG is notable for its rare combination of small size and tremendous ecological and biocultural diversity. It is, in their words, a "small cosmos" that has allowed human biologists to carry out, in an area just slightly bigger than the state of California, research on physiological and genetic adaptations to thermal stress, endurance work, endemic disease, nutritional insufficiency, and, more recently, the effects of colonial intrusion and modernization on the health and social fabric of the indigenous populations.

The introduction is followed by two chapters that present overviews of the geography (B.J. Allen) and demographic patterns (I.D. Riley and D. Lehmann) of the country. These are largely descriptive and serve as useful introductions for those not familiar with the terrain, production systems, and fertility and mortality situation in various regions of PNG. The third chapter, by J.W. Wood, begins by presenting a summary of regional variation in total fertility rates; Wood makes the interesting observation that fertility in the highlands regions is uniformly lower than that in other regions of the country. The rest of the chapter examines the potential causes of this lower fertility in the highlands through a detailed case study of one particular population, the Gainj. Wood concludes with an appropriately cautious remark about the generalizability of the Gainj findings to other populations, but the analytical methods used are clearly applicable to other populations, and thus this chapter may serve as a useful resource for those interested in field-based research on fertility and reproductive biology.

Chapters 5 and 6 deal with social anthropology (D.S. Gardner and J.F. Weiner) and linguistic variation (W.A. Foley), respectively. Although each chapter provides excellent coverage in its subject area (which is notable, given the tremendous sociocultural and linguistic diversity in PNG) and one can learn a great deal by reading them, neither chapter concentrates specifically on issues pertaining to human biology, though brief allusions to the importance of research "combining sociological and biological skills" (p. 129) are made. Thus these otherwise excellent chapters remain oddly out of place in this volume.

Chapters 7 and 8 are concerned with the origins of PNG's native populations, in one case using archeological evidence (I. Lilley) and in the other, genetic markers (R.L. Kirk). Kirk considers the extent of concordance between the major linguistic stocks (Austronesian vs. non-Austronesian language speakers) and genetic markers; his chapter is a natural lead-in to the subsequent chapter on population genetics in PNG (S.W. Serjeantson, P.G. Board, and K.K. Bhatia). This chapter presents data on the frequencies of various red blood cell defects and HLA types and reminds us that such frequencies have been shaped as much by the selective forces intrinsic to different ecological zones as by population movements and sociolinguistic "relatedness."

Chapter 10 (by P.F. Heywood and N.G. Norgan) provides data on growth and development and recent secular trends in growth in several regions of the country. The chapter is notable not only for the valuable data it provides but also for its consideration of the relative contributions of genetics, nutrition, altitude, and disease to the diversity of growth patterns found in PNG. The subsequent chapter by P.F. Heywood and C. Jenkins presents a closer examination of nutrition and food procurement. It considers not only the myriad systems of production practiced by different PNG populations but also the potential adaptive mechanisms by which these populations cope with seasonal and more chronic nutritional shortfalls.

The sections on human growth and nutrition are followed by a consideration of general physiological adaptability in PNG (J. Lourie, G. Budd, and H.R. Anderson). This chapter presents an authoritative summary of the well-known studies conducted at Lufa and on Karkar Island as part of the International Biological Programme and includes several previously unpublished results of these studies. A broad array of topics in human adaptability is treated, including thermal stress, blood pressure, work capacity, and high-altitude adaptation.

The next nine chapters form a somewhat unified section of the volume and concern various acute, chronic, and endemic diseases found in PNG. These include respiratory infections (I.D. Riley, D. Lehmann, and M.P. Alpers), chronic lung disease (H.R. Anderson and A.J. Woolcock), malaria (J.A. Cattani), kuru (M.P. Alpers), infection by Clostridium perfringens ("pigbel") (G. Lawrence), intestinal parasitism (G. Barnish), iodine deficiency (P.F. Heywood), diabetes (H. King), and cardiovascular disease (P.F. Sinnet, I.H. Kevau, and D. Tyson). All these chapters are at least adequate, many are much better than that, and two in particular are standouts--the chapters on kuru and diabetes.

Michael Alpers's chapter on kuru is a fine summary of the detailed work that led to the discovery of its etiology and is as engrossing to read as any detective story. He does an excellent job of showing how the epidemiological and behavioral pieces of the kuru puzzle were ultimately fitted together and provides (although not overtly) one of the better arguments for why the various subdisciplines of anthropology should not succumb to the forces threatening to tear them apart. He also provides interesting notes on diseases related to kuru, such as Kreutzfeld-Jakob syndrome, which is transmissible through the developed world's analog to cannibalism: organ transplants derived from human cadavers.

Hilary King's chapter provides us with intriguing data showing that non-insulin-dependent diabetes mellitus (NIDDM) is exceptionally rare in PNG except among coastal-dwelling Austronesian-language speakers. To explain this finding, King first reviews the thrifty genotype hypothesis and then considers the suggestion that because of the early adoption of agricultural practices in PNG, especially in the highlands, non-Austronesian speakers may not have been subject to cycles of feast and famine or to the resultant selection for the thrifty genotype that predisposes its possessor to NIDDM.

The volume concludes with a chapter by C. Jenkins on medical anthropology in PNG. It stresses the need for continuing attention to elements of traditional culture as well as biology in attending to the problems of population growth, disease, nutritional insufficiency, and health care delivery in PNG.

Although uneven in places, as is the case with most edited volumes, Attenborough and Alpers have done a fine job of integrating some of the newest, best, and most intriguing studies on human biology in Papua New Guinea. As such, this volume will be a welcome addition to the library of human biologists and anthropologists interested in the small cosmos that is Papua New Guinea.

Copyright Wayne State University Press Oct 1994
Provided by ProQuest Information and Learning Company. All rights Reserved

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