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Edited by William B Svoboda. Cambridge: Cambridge University Press, 2004, £85.00 (hardback), pp 638. ISBN 0-521-82338-2
Given that Alexander the Great, Julius Caesar, Cardinal Richelieu, and Lenin all suffered from epilepsy it is clear that epilepsy does not preclude future career success. The prominence of sufferers within the higher echelons of the creative arts is striking. Dostoyevsky, Flaubert, Moliere, and Byron are just a handful of names that immediately spring to mind. Van Gogh’s most creative period coincided with the time when his epilepsy was at its worst. And yet, we know that epilepsy can have a dramatic and disastrous effect on the cognitive and language abilities of our paediatric patients. It is hard not to be moved by West’s description of how his son regressed following the development of infantile spasms. We hear similar stories time and again in paediatric clinics of how an apparently normal baby arrests developmentally and then regresses coincident with the onset of infantile seizures.
It must be equally distressing to be the parent of a child with Landau-Kleffner syndrome (LKS). One day you have a previously chatty 5 year old who suddenly is unable to understand what you are saying to them. Their speech and behaviour deteriorates and, to cap it all, they develop seizures.
We do not understand the relation between epilepsy and the cognitive, behavioural, and linguistic disorders so evident in these and other paediatric epilepsy syndromes. In both LKS and West’s syndrome they may have chaotic status-like electroencephalograms. We postulate that such chaos must be interrupting the formation of critical neural synapses and pathways. However, the resolution of such electrographic disorder and clinical seizures may not, unfortunately, coincide with any cognitive or language improvement.
We search for effective treatments for these disorders. The breadth of different treatments used suggests that we are uncertain where to target our therapeutic approaches. For example, in LKS, steroids often improve the situation, but is it because they are modifying some infectious or autoimmune process or through their action at the GABA-A receptor? Indeed our treatments may exacerbate the situation. Virtually all the anticonvulsant drugs have been associated with behavioural and cognitive problems.
Of course, I am exaggerating the state of confusion in this area … but only slightly. I turned to Professor Svoboda’s book on this subject, looking for some clarity and direction. It is a veritable goldmine of anecdote and case reports. A lifetime of reading and clinical experience are condensed here. It would be wrong to say that this is an evidence-free area. Svoboda documents a wealth of studies, references, and data. However, at the end of the book I longed for some critical appraisal of the evidence he had marshalled together. He gives no indication, for example, of the relative benefits of steroids, immunoglobulins, anticonvulsants, and sub-pial transection in the treatment of LKS.
In fact, this book is a testament to the lack of knowledge that exists. There is a pressing need for a good evidence base about the aetiology and treatment of these disorders. Which treatments improve cognitive outcome in infantile spasms? Is surgery preferable to medical therapy in LKS? Does treatment alter the prognosis of so-called benign focal epilepsies of childhood? The answers to such questions need to be unearthed but they are not to be found or hinted at in this book.