Journal of Intellectual Disability Research

volume 49 part 9

pp

692

–

693 september 2005

©

2005

Blackwell Publishing Ltd

692

Blackwell Science, LtdOxford, UKJIRJournal of Intellectual Disability Research0964-2633Blackwell Publishing Ltd, 200549

9692693

Book Review

Book reviewBook review

Book review

Intellectual Disabilities: Genetic, Behaviour and Inclusion

Edited by Jean A. Rondal, Robert M. Hodapp, Salvatore Soresi, Elizabeth M. Dykens, Laura Nota

WHURR, Publishing for professionals, 2004. 200 pp., £29.50 (pbk). ISBN 1 86156 350 7

Advancing knowledge in the field of genetics and also the epigenetic environment has led to a better under-standing of intellectual disability (ID) both as a whole and in particular in terms of aetiology. This book looks at the relationship between specific genetic syndromes and both their behavioural pheno-types and their cognitive characteristics. Down’s, Prader–Willi and Williams syndromes are all dis-cussed in part. Some behaviours related to the aeti-ology of ID will be unique to the specific syndromes, whereas other, non-aetiology-related behaviours may be displayed in one or more of the genetic syndromes.

The authors claim that what is currently known of the behaviour associated with genetic ID represents only the tip of iceberg and that in reality we still know very little about even syndromes such as Down’s – the most extensively researched and comprehensively understood genetic cause of ID. As an example, the links between Prader–Willi syndrome and its food related characteristics are discussed with the mention of pronounced feeding–sucking difficulties during infancy superseded by hyperphagia and food seeking behaviour during childhood. Coupled with a whole range of impulsive behavioural traits including tem-per tantrums, aggression and emotional labiality, the level of care demanded is out of proportion to the, usually mild, degree of ID.

The book refers to the variability of language within a number of relatively common genetic causes of ID and relates this to the differences in neurode-velopment between the syndromes. For example, in Neurofibromatosis type

1

(NF

1

), remarkably, moderate to profound language problems may be observed in children, in whom there is only very mild learning disability. The neurological basis of language impairment of NF

1

has not yet been established, but the range of language deficit is large, representing both expressive and receptive disability, including stammering, abnormal fluency, hypernasility, abnor-mal rate of articulation, mispronunciations, incorrect sequencing, voice problems (pitch, hoarseness). These problems may be demonstrated in

30

–

40

% of patients with NF

1

and it has been demonstrated that the language skills of a child with NF

1

fall behind those of an unaffected sibling, despite early thought that no such language disorders existed in children with NF

1

. As the neurological basis is not under-stood, it is difficult to explain, but certainly there is a large variation in the extent of language disability even within this one syndrome. In addition, there is a huge discrepancy between the syndromes and this implies an aetiological role in language variability within the population with learning disabilities.

The Italian law is discussed relating to issues for school inclusion. Inclusion is a complex process requiring careful planning and training with the involvement of teachers, social workers and the par-ents of both children with and without ID. Social and community inclusion is also discussed. Deinstitution-alization has progressed and much work now focuses on community and work inclusion. Community inclusion requires a number of actions to be fulfilled

Journal of Intellectual Disability Research

volume 49 part 9 september 2005

Book review693

©

2005

Blackwell Publishing Ltd,

Journal of Intellectual Disability Research

49

,

692

–

693

such as leisure and free time contexts, community living, occupational contexts, personal satisfaction with daily activities, housing and social networks.

In order that successful work inclusion would take place, the environment must be adjusted appropri-ately, and support services need to be in place.

This book requires rather high levels of existing understating in the field of behavioural phenotypes and is perhaps suitable for someone who has worked

in the field and has a reasonable grasp on the topic. There are some long-winded, with long, complex sentences each carrying a number of points that lack clarity. The text would have be benefited by some illustration and tables. The reference to the Italian law would have been more useful if it was presented in a comparative way with the law in other countries.

Manga Sabaratnam