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Essays on kuru
146 Other chapters cover the pharmacokinetics of drugs in the CNS, psychological aspects of sleep pharmacology, statistical considerations in the design of sleep studies and prescription practices i~1 the U.S. This is an important book and a comprehensive, authoritative account of sleep pharmacology is most welcome at this time. It deserves a place in all medical libraries and will be consulted by physicians interested in many different aspects of sleep. C. H. Ashton
Essays on Kuru (Monograph Series of the Institute of Human Biology, Papua New
Guinea, No. 3), by R. W. Hornabrook (Ed.), E. W. Classey, Ltd., Faringdon, Berks., 1976, £ 8.50. This very attractively produced book offers an up-to-date conspectus of kuru and the environment in which it developed and appears now to be passing away. The natural beauty of the Eastern Highlands deaerves good colour illustration, but enough is shown to emphasise the difficulties in contracting cases and in carrying out physical examinations. Each contributor is an acknowledged authority in his field, and most have spent considerable time in the country. Early accounts of kuru (mistaken for "hysteria") by Williams (1923) and Berndt a few years later might have been amplified as might well have been the early, but very instructive, genetic studies with their postulaton of K and k genes. These latter serve as a reminder of Wood Jones' famous paragraph: "Every European who has watched a Chinese cashier manipulating his abacus must have felt admiration both for the man and his i n s t r u m e n t . . . B u t . . . all are agreed that the universal reliance the Chinese place on the abacus h a s . . , hindered the development of a more widely reaching . . . higher mathematics amongst (them). "I have been tempted to wonder if my unbounded admiration for the facility with which the modern geneticist solves his problems by manipulating hypothetical little balls - - called genes - - along the bars of the c h r o m o s o m e s . . , keeps tally of the petty cash transactions of h e r e d i t y . . . ( a n d ) . . . if the great reliance placed upon the workings of the abacus of heredity is not perhaps repressing the development of a possible higher mathematics of inheritance." Insufficient prominence is given to the seminal suggestion made by W. J. Hadlow in 1959 in a letter to the Lancet, calling attention to the "strikingly similar natural history and general clinical aspects of (scrapie and kuru). Hornabrook (p. 53) indeed omits mention of Hadlow's idea, whilst Matthews (p. 85) mentions it en passam, as do Beck and Daniel (p. 120). At that time interested workers were still enmeshed with K and k. The beautifully illustrated contribution by Elizabeth Beck and P. M. Daniel is marred by sins of omission and commission. In their own earlier descriptions of kuru Beck et al. (1964) laid great emphasis on the muscle lesions of scrapie, finding that 24 of 25 representative muscles showed varying degrees of myopathy ranging from fully developed "polymyositis with great destruction of muscle fibres to early changes with slight cellular infiltration and a few necrotic fibres". This theme is a persistence of their early, more intense pathological changes (Bosanquet et al. 1956). Indeed the 1954 description led to a remarkable leading article in the Lancet (1956), in which the muscle findings reported above were held to overthrow all previous "neurological" views of scrapie. Subsequent correspondence made it clear that Bosanquet et al. had had the misfortune to work with sheep also having a myopathy. Yet to this day there has not been (to the writer's knowledge) a written withdrawal of this error by the authors, though more recent descriptions of scrapie-kuru by Beck and Daniel have omitted reference to muscle changes. The reader who may have followed the changing pattern of description of scrapie-kuru-JCD over the years will recall that Gibbs et al. (1968) claimed with reference to the experimental transfer of JCD to the chimpanzee that there were "features not characteristic of kuru that enabled this brain to be easily distinguished from that of the animal with experimental k u r u ' . He wilt notice, too, the gradual withdrawal from the view of serapie-kuru-JCD as a "system" disease, sensu stricto, to that of a widespread degenerative condition in which the ictus is from time to time more pronounced on the cerebeUar system. The sin of commission is the attribution of a relationship between MS and scrapie to Field (1967). The error has been clearly withdrawn in writing (Field et al. 1974; Field 1975), that no relationship exists as indicated by the in vitro MS tests developed. Indeed in the latter article, the Abstract
147 (placed at the head of the paper) begins "Multiple sclerosis (MS) is shown to have no relation to scrapie and the evidence for a "slow infective" aetiology is very meagre" (p. 285). The confusion of JCD with Alzheimer's disease by Gajdusek et al. (p. 138) may puzzle neurologists and psychiatrists, but brings together a definitive view of Dr Gajdusek's multitudinous writings. The book is an excellent survey of the scrapie-kuru-Jacob-Creutzfeldt problem, though relatively few who have not worked in the field will appreciate the fascinating ethnographic and related problems discussed so ably by R. M. Glasse and Shirley Lindenbaum. E. J. Field REFERENCES Beck, E., P. M. Daniel and H. B. Parry (1964) Brain, 87: 153. Berndt, R. M. (1954) Oceania, 24: 190, 255. Berndt, R. M. (1958) Sociologia, 8: 4. Bosanquet, F. D., P. M. Daniel and H. B. Parry (1956)Lancet, 2: 737. Field, E. J. (1967) Dtsch. Z. Nervenheilk., 192: 265. Field, E. J. (1975) Acta neurol, scand., 51 : 285-298. Field, E. J., B. K. Shenton, G. Joyce and D. Buntain (1974) 1RCS, 2: 1041. Gibbs, C. J., D. C. Gajdusek, D. M. Asher, M. P. Alpers, E. Beck, P. M. Daniel andW. B. Matthews (1968) Science, 161: 388-9. Hadlow, W. J. (1959) Lancet, 2: 289. Lancet (1956) Editorial, 2: 767-8. Williams, F. E. (1923) Territory Papua Anthropological Report, Port Moresby, 73: 78. Wood Jones, F. (1943) Habit and Heritage, Kegan Paul, Trench, Trubner and Co., Ltd., London, pp. 98-99.
Dystonia ( A d v a n c e s in N e u r o l o g y , V o l 14), by R . E l d r i d g e a n d S. F a h n (Eds.), xviii q- 491 p a g e s , 136 i l l u s t r a t i o n s , 107 tables, R a v e n Press, N e w Y o r k , 1976, U S $ 35.00. This volume based on an international conference held in 1975 sponsored by the Dystonia Foundation, is intended to draw wider attention to the dystonic states as disease entities. When Oppenheim introduced the term dystonia in 1911, attributing the characteristic involuntary movements and postures to fluctuating hypertonia and hyptonia of muscle, his paper generated considerable controversy which has still to be satisfactorily resolved. Wolfgang Zeman in a scholarly and attractively astringent chapter reviews the history of dystonia and cogently argues that it is preferable to accept the rubric as synonymous with a complex of diseases, formerly termed dystonia musculorum deformans or torsion dystonia, rather than to identify a discrete malady. Although in many instances the disease arises sporadically there is now convincing evidence that it is often inherited by at least two different modes. The available genetic information is critically presented by several contributors and it now seems well established that there is a dominantly inherited form which affects predominantly the non-Jewish population and a recessive inherited form appearing to prevail among those of Ashkenazim stock and is often associated with above average intelligence. The genetic heterogeneity of dystonias becomes evident from the carefully documented family histories and it seems reasonable to conclude that the great variability of expressivity in families with the dominant form, strongly favours the interaction of other genes with the dystonic gene. Despite intensive attention from the numerous investigative disciplines the diagnosis remains essentially clinical and even the seemingly distinct biochemical differentiation between the dominant and recessive forms as determined by the activity of serum dopamine beta-hydroxydase appears not to be as absolute as earlier studies had suggested. This well-edited volume presents lucidly for clinicians and investigators of differing interests a comprehensive statement concerning the extent of our knowledge - - or perhaps more correctly of our ignorance - - about this perplexing and cryptic group of disorders. The editors rightfully draw attention to the anguish and hardship over and above that caused by the disease itself which arises from frequent misdiagnosis of the condition as being the manifestation of a psychiatric ailment. Their hope that the present volume will facilitate accurate diagnosis and stimulate research into the causes and treatment of dystonia and related disorders is well justified. G. M. Stern
Essays on Kuru (Monograph Series of the Institute of Human Biology, Papua New
Guinea, No. 3), by R. W. Hornabrook (Ed.), E. W. Classey, Ltd., Faringdon, Berks., 1976, £ 8.50. This very attractively produced book offers an up-to-date conspectus of kuru and the environment in which it developed and appears now to be passing away. The natural beauty of the Eastern Highlands deaerves good colour illustration, but enough is shown to emphasise the difficulties in contracting cases and in carrying out physical examinations. Each contributor is an acknowledged authority in his field, and most have spent considerable time in the country. Early accounts of kuru (mistaken for "hysteria") by Williams (1923) and Berndt a few years later might have been amplified as might well have been the early, but very instructive, genetic studies with their postulaton of K and k genes. These latter serve as a reminder of Wood Jones' famous paragraph: "Every European who has watched a Chinese cashier manipulating his abacus must have felt admiration both for the man and his i n s t r u m e n t . . . B u t . . . all are agreed that the universal reliance the Chinese place on the abacus h a s . . , hindered the development of a more widely reaching . . . higher mathematics amongst (them). "I have been tempted to wonder if my unbounded admiration for the facility with which the modern geneticist solves his problems by manipulating hypothetical little balls - - called genes - - along the bars of the c h r o m o s o m e s . . , keeps tally of the petty cash transactions of h e r e d i t y . . . ( a n d ) . . . if the great reliance placed upon the workings of the abacus of heredity is not perhaps repressing the development of a possible higher mathematics of inheritance." Insufficient prominence is given to the seminal suggestion made by W. J. Hadlow in 1959 in a letter to the Lancet, calling attention to the "strikingly similar natural history and general clinical aspects of (scrapie and kuru). Hornabrook (p. 53) indeed omits mention of Hadlow's idea, whilst Matthews (p. 85) mentions it en passam, as do Beck and Daniel (p. 120). At that time interested workers were still enmeshed with K and k. The beautifully illustrated contribution by Elizabeth Beck and P. M. Daniel is marred by sins of omission and commission. In their own earlier descriptions of kuru Beck et al. (1964) laid great emphasis on the muscle lesions of scrapie, finding that 24 of 25 representative muscles showed varying degrees of myopathy ranging from fully developed "polymyositis with great destruction of muscle fibres to early changes with slight cellular infiltration and a few necrotic fibres". This theme is a persistence of their early, more intense pathological changes (Bosanquet et al. 1956). Indeed the 1954 description led to a remarkable leading article in the Lancet (1956), in which the muscle findings reported above were held to overthrow all previous "neurological" views of scrapie. Subsequent correspondence made it clear that Bosanquet et al. had had the misfortune to work with sheep also having a myopathy. Yet to this day there has not been (to the writer's knowledge) a written withdrawal of this error by the authors, though more recent descriptions of scrapie-kuru by Beck and Daniel have omitted reference to muscle changes. The reader who may have followed the changing pattern of description of scrapie-kuru-JCD over the years will recall that Gibbs et al. (1968) claimed with reference to the experimental transfer of JCD to the chimpanzee that there were "features not characteristic of kuru that enabled this brain to be easily distinguished from that of the animal with experimental k u r u ' . He wilt notice, too, the gradual withdrawal from the view of serapie-kuru-JCD as a "system" disease, sensu stricto, to that of a widespread degenerative condition in which the ictus is from time to time more pronounced on the cerebeUar system. The sin of commission is the attribution of a relationship between MS and scrapie to Field (1967). The error has been clearly withdrawn in writing (Field et al. 1974; Field 1975), that no relationship exists as indicated by the in vitro MS tests developed. Indeed in the latter article, the Abstract
147 (placed at the head of the paper) begins "Multiple sclerosis (MS) is shown to have no relation to scrapie and the evidence for a "slow infective" aetiology is very meagre" (p. 285). The confusion of JCD with Alzheimer's disease by Gajdusek et al. (p. 138) may puzzle neurologists and psychiatrists, but brings together a definitive view of Dr Gajdusek's multitudinous writings. The book is an excellent survey of the scrapie-kuru-Jacob-Creutzfeldt problem, though relatively few who have not worked in the field will appreciate the fascinating ethnographic and related problems discussed so ably by R. M. Glasse and Shirley Lindenbaum. E. J. Field REFERENCES Beck, E., P. M. Daniel and H. B. Parry (1964) Brain, 87: 153. Berndt, R. M. (1954) Oceania, 24: 190, 255. Berndt, R. M. (1958) Sociologia, 8: 4. Bosanquet, F. D., P. M. Daniel and H. B. Parry (1956)Lancet, 2: 737. Field, E. J. (1967) Dtsch. Z. Nervenheilk., 192: 265. Field, E. J. (1975) Acta neurol, scand., 51 : 285-298. Field, E. J., B. K. Shenton, G. Joyce and D. Buntain (1974) 1RCS, 2: 1041. Gibbs, C. J., D. C. Gajdusek, D. M. Asher, M. P. Alpers, E. Beck, P. M. Daniel andW. B. Matthews (1968) Science, 161: 388-9. Hadlow, W. J. (1959) Lancet, 2: 289. Lancet (1956) Editorial, 2: 767-8. Williams, F. E. (1923) Territory Papua Anthropological Report, Port Moresby, 73: 78. Wood Jones, F. (1943) Habit and Heritage, Kegan Paul, Trench, Trubner and Co., Ltd., London, pp. 98-99.
Dystonia ( A d v a n c e s in N e u r o l o g y , V o l 14), by R . E l d r i d g e a n d S. F a h n (Eds.), xviii q- 491 p a g e s , 136 i l l u s t r a t i o n s , 107 tables, R a v e n Press, N e w Y o r k , 1976, U S $ 35.00. This volume based on an international conference held in 1975 sponsored by the Dystonia Foundation, is intended to draw wider attention to the dystonic states as disease entities. When Oppenheim introduced the term dystonia in 1911, attributing the characteristic involuntary movements and postures to fluctuating hypertonia and hyptonia of muscle, his paper generated considerable controversy which has still to be satisfactorily resolved. Wolfgang Zeman in a scholarly and attractively astringent chapter reviews the history of dystonia and cogently argues that it is preferable to accept the rubric as synonymous with a complex of diseases, formerly termed dystonia musculorum deformans or torsion dystonia, rather than to identify a discrete malady. Although in many instances the disease arises sporadically there is now convincing evidence that it is often inherited by at least two different modes. The available genetic information is critically presented by several contributors and it now seems well established that there is a dominantly inherited form which affects predominantly the non-Jewish population and a recessive inherited form appearing to prevail among those of Ashkenazim stock and is often associated with above average intelligence. The genetic heterogeneity of dystonias becomes evident from the carefully documented family histories and it seems reasonable to conclude that the great variability of expressivity in families with the dominant form, strongly favours the interaction of other genes with the dystonic gene. Despite intensive attention from the numerous investigative disciplines the diagnosis remains essentially clinical and even the seemingly distinct biochemical differentiation between the dominant and recessive forms as determined by the activity of serum dopamine beta-hydroxydase appears not to be as absolute as earlier studies had suggested. This well-edited volume presents lucidly for clinicians and investigators of differing interests a comprehensive statement concerning the extent of our knowledge - - or perhaps more correctly of our ignorance - - about this perplexing and cryptic group of disorders. The editors rightfully draw attention to the anguish and hardship over and above that caused by the disease itself which arises from frequent misdiagnosis of the condition as being the manifestation of a psychiatric ailment. Their hope that the present volume will facilitate accurate diagnosis and stimulate research into the causes and treatment of dystonia and related disorders is well justified. G. M. Stern