BOOK REVIEWS

DOI:10.1111/j.1651-2227.2011.02155.x

Neurological Disorders in Famous Artists, part 1–3. Editedby Bogousslavsky J, Boller F, Hennerici MG, Bazner M,Basetti C. In: Frontiers of Neurology and Neuroscience,volumes 19, 22 and 27. In total: 674 pp., 160 figs., 56 incolor and 16 tabs. Hard cover. List price €78, €88 and €88respectively. Karger, Basel, CH 2005, 2007 and 2010. ISBN978-3-8055-7914-4, 978-3-8055-8265-0 and 978-3-8055-9330-4.

It better be said right away that these are not books on pae-diatrics. Rather they are books about adults who, in addi-tion to their artistic creativity in music, painting orliterature, were noted to exhibit neurological disorders orpeculiar personalities bordering neuropsychiatric dysfunc-tions. However, all artists referred to in these three volumeswere once children, and it may be of interest to look for anysubtle premorbidity that some of them demonstrated duringtheir early childhood. The possible connection betweenneurological or cognitive dysfunctions and artistic creativityhas been a subject of debate during many years. It is trulyremarkable to find how many artists obviously have beenburdened with different kinds of neurological disabilities orneuropsychiatric traits. There are indeed some examples ofearly signs of such trains in this remarkable collection ofartists that the editors have gathered.

The premorbidity of Marcel Proust (1871–1922) mayserve as an example. Both his father and older brother weremedical doctors. Incidentally, this patrimony seems to haveoften been catalytic for a career in writing, Gustav Flaubert(1821–1880), the contemporary Feodor Michailovitch Dos-toyevsky (1821–1881) and Oscar Wilde (1854–1900) toname just a few. Proust’s asthmatic attacks started when hewas 8 years old. They gradually became more severe andresistant to the various forms of treatment including differ-ent forms of fumigation that were prescribed. At this time,asthma was mainly considered a ‘nervous disease’. The lead-ing neurologist Edouard Brissaud (1852–1909) expoundedon this notion in his book The Hygiene of Asthmatics

(1896), which incidentally had a preface written by Proust’sfather. The many different doctors who were consultedincluded consequently some of the most renowned neurolo-gist in Paris at that time. In addition, the young Marcel’smother is thought to have aggravated his symptoms by over-protection, resulting in additional ambiguous ailments fromthe gastrointestinal system, migrating pain in different mus-cles and joints and the constant feeling of being frozen.

The great composer Wolfgang Amadeus Mozart (1756–1791) is referred to as an example of Tourette’s syndrome,which manifested itself in early childhood, but came intofull blow during adolescence. A detailed analysis ofMozart’s letters with statistical analyses has demonstratedan abundance of coprolalia, mostly scatological expressionsand sexual insinuations, which are typical for this clinicalentity. Similar tendencies are also claimed to be foundin the early personality of the writer and author of Dict-ionary of the English Language (1755) Samuel Johnson(1709–1784) as well as the French writer Andre Malraux(1901–1976). Despite this possible trait, Malraux served asMinister of Cultural Affairs during De Gaulle’s entire presi-dency, which is yet another example of how a disability notnecessarily may hamper a civil or artistic career.

The Hungarian composer Bela Bartok (1881–1945) isargued to illustrate an example of mild autism that naturallymanifested itself during childhood. In contrast to his musi-cal performances, he is described by his contemporaries associally awkward with limited social competence. Duringthe rare instances of conversation, his words flew out com-pletely evenly, and he rarely emphasized a particular word.Likewise, the painter and film maker Andy Warhol (1928–1987), the leading figure in the visual art movement knownas pop art, is given as an example with similar personalityand prosodic repertoire. The idea that the young Austrianpainter Egon Schiele (1890–1918) really was affected bymuscular dystonia, which have been suggested based onearly photographs and self-portrays, is however refuted. Theawkward bodily position he demonstrated on these is morelikely to be an example of the expressionistic style of dis-playing extroverted emotions than a neuromuscular disease.

The chronic pain syndrome affecting the pianist ClaraWieck-Schumann (1819–1896) is likewise discussed. Shewas trained as a child prodigy at the age of five for at leastthree hours every day by her father. From the age of eleven,Clara went on regular concert tours which placed her underan enormous stress. Very early in her career she sufferedfrom persistent pain in her arms and hands. There are rea-sons to believe that she indeed had a chronic myofascialpain syndrome aggravated by the strain of practicing andperforming. It is also plausible that her symptoms later inlife in part may be attributed to the burden of upbringing

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eight children and caring for her gradually more mentallydisturbed husband Robert Schumann (1810–1856).

In summary, in spite of the limited examples of paedia-tric disabilities or sufferings, these books are highlyrecommended to anyone interested in the fascinatinginterface between artistic creativity and neurological orneuropsychiatric dysfunctions. The editors have accom-plished an impressive and comprehensive review ofrenowned artist and their specific personalities.

Carl Lindgrencarl.lindgren@actapaediatrica.se

DOI:10.1111/j.1651-2227.2011.02154.x

Genetics of Mental Retardation – An Overview Encom-passing Learning Disability and Intellectual Disability.Edited by Knight Samantha JL. In: Monographs in HumanGenetics, edited by M. Schmid. Vol 18. XII+166p., 31 fig., 15in colour, 12 tab., hard cover. Karger 2010. €168, $US 235.ISBN 978-3-8055-9280-2, e-ISBN 978-3-8055-9281-09.

This is the 18th book in the series Monographs in HumanGenetics which is devoted to genetics of mental retardation.It covers all new techniques in the modern work of clinicalgenetics. The book is divided into 13 different chapters by13 different authors or group of authors. Mental retardationhas scientifically been studied extensively over a long time.During the last years, the technology revolution in the mole-cular genetic field has improved the diagnostic capability inclinical genetics. As a result, gene mutations and structuralrearrangements of the genome are today known to be themost important factor in the aetiology and understanding ofdevelopmental delay. Genetic studies of mental retardationare very important for genetic counselling. They can providethe basis for accurate prognostic information, developmentof guidelines for clinical care and to fulfil and educationalneeds and to be the backbone when considering future pre-ventive and therapeutics regimes.

In Chapter 1, a parent’s perspective of a daughter withmental retardation is presented. It took a long time to getthe correct diagnosis of this girl. The mother stronglydemonstrates the importance of getting a diagnose of the

child for the medical care and for the family planning in thefuture.

In Chapter 2, definition and classification as well as theaetiology of mental retardation are presented. The negativeperceptions associated with the term mental retardation arediscussed, but as there is no better word, the editor didchose to continue with this word in this book. This chapteris the only one that covers the syndromes of mental retarda-tion, which are well known since a long time back, such asDown syndrome and Fragile X syndrome. The value of thepresent book had increased if more efforts had been put tocover the knowledge about those syndromes. There aremedical care programme for most of those syndromeswhich could have been presented here.

In Chapter 3, the technology advances such as subtelo-meric fluorescent in situ hybridization (FISH) and arraycomparative genomic hybridization are described. Advan-tages and disadvantages of FISH, multiplex ligation-depen-dent probe amplification as well as micro array analysis arepresented. Copy number variations are discussed, andboth the benign as well as the pathologic variations arepresented.

In chapter 4, the importance of the genomic architecturein mental retardation is discussed. The genomic featuresassociated with genomic instability and recurrent rearrange-ments are reviewed. In one table, the clinical and genomicfeatures associated with 33 well-known genomic disorders,such as William, Sotos, Angelman and Prader-Willi syn-dromes, are presented.

Chapter 5 covers the clinical evaluation, including thepsychometric tests that are used for diagnosis of mentalretardation. The definition of mental retardation isdescribed as well as the degrees of MR according to DSM-IV. A diagnostic flow chart in patients with MR ispresented.

In Chapter 6, the help one can get from databases for eva-luation of mental retardation is described. By collectingphenotypes with genotypes, it will become possible in thefuture to understand genetic causes of mental retardation. Asummary of relevant databases with web-links is reviewed.In Chapter 7, the new 3D shape analyses of facial dysmor-phology associated with cognitive developmental disordersis described. This method will perhaps in the future becomea valuable method for distinguishing syndromes in patients,but is still not very useful in the daily routine work ofpaediatrics.

In chapter 8, several monogenic causes of mental retarda-tion are presented. Especially X-linked disorders are dis-cussed. To date, more than 80 genes on the X chromosomehave been identified where a mutation results in syndromicor non-syndromic mental retardation. It is likely that muta-tions in more than 1400 genes on the autosomes give rise tomental retardation. Although many genes have been identi-fied, the biological mechanism by which each gene causesdisease is, however, still poorly understood.

In Chapter 9, newly recognized mental retardation micro-deletion ⁄ duplication syndromes are presented and in Chap-ter 10 Mendelian copy number variants (CNVs) causing

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