NEUROBIOLOGY OF DISEASE Edited by Dennis Choi and Jacques Mallet Blackwell Science Bi-monthly, Pounds 47.50 (institutions), Pounds 22.50 (individuals) ISSN 0969 9961
It is an exciting time for those working in the neurosciences. Knowledge has proliferated exponentially over the past decade and surely no one can predict where we may be in another five (let alone ten) years' time. We have come out of an era where the causes of neurological diseases were largely unknown and the conditions untreatable. New investigative procedures such as magnetic resonance scanning are revolutionary and widely appreciated by the public but our understanding of neurological diseases comes from all the neuroscience disciplines.
As a neurologist I have seen over relatively few years how genetics, in particular, has considerably altered the way we now diagnose and manage various neurological diseases. This influence is likely to increase in the future. Recently there have been therapies for common conditions like migraine and epilepsy; new treatments for multiple sclerosis and motor neurone disease seem to be just around the corner.
There are many fine journals that cover the neurosciences. In general they are of two types. One is read predominantly by the basic scientist and the other predominantly by the clinician. But there is a natural desire among basic scientists to work in areas that have clear clinical relevance and a natural desire among clinicians to be interested in research. Surely any journal that proposes to appeal to both basic scientists and clinicians must promote our understanding of diseases and the development of new treatments.
Neurobiology of Disease is a new international bi-monthly journal aimed at delivering top-quality research papers at the interface between basic and clinical neuroscience. Its scope is therefore wide and includes molecular and cellular definitions of disease mechanisms, the neural systems involved in behavioural disorders of higher cortical function, ageing in the nervous system, the genetics of neurological and psychiatric diseases and research into new therapies. Important goals as stated by the editors are rapid review and publication times and the highest-quality printing and figure reproduction. The contents may include occasional review articles, eight papers of less than eight printed pages each, followed by an author and subject index. But the raison d'etre of the new journal is to contribute to scientific progress by specifically facilitating an exchange of ideas about the nature of nervous system disease between the clinician and basic scientist.
The first review article is "Motor neurone disease and animal models". Most people have heard about this wretched condition. It is a good example of how clinical and basic research act synergistically. Genetic factors in the aetiology of motor neurone disease have been identified, animal models developed, hypothetical pathogenetic mechanisms have been proposed and tested and promising therapeutic approaches are being explored. This review article includes a brief summary of the human motor neurone disease clinical syndromes, known aetiological factors and neuropathology followed by detailed descriptions of the various animal models, including the mutant superoxide dismutase 1 (SOD1) transgenic mouse model. These mice develop limb weakness at an early age which might be relevant to some cases of familial motor neurone disease where there is also a mutation in their SOD1 gene. Review articles of this sort will certainly be of general interest to neurologists but seem detailed enough to be helpful to people working in the field. The above review has over 100 references.
Of the 17 original papers available for review, speaking as a clinician I found the fact that rats featured in only three pleasing.
As is to be expected not all the papers will be of interest to a clinical neurologist. There is considerable variety. Some papers are very genetically orientated ("APP gene family: unique age associated changes in splicing of Alzheimer's beta A4-amyloid protein precursor"), others neurophysiological ("Scrapie infection of transgenic mice leads to network and intrinsic dysfunction of cortical and hippocampal neurones") and others metabolic ("Infection of sympathetic and sensory neurones with herpes simplex virus does not elicit a shut-off of cellular protein synthesis: implications for viral latency and herpes vectors"). I found the paper "Huntington's disease CAG trinucleotide repeats in pathologically confirmed post-mortem brains" particularly interesting and helpful, having diagnosed a case only recently.
Neurobiology of Disease is a glossy and well-presented journal. It will be well read by many neuroscientists of differing backgrounds. If it manages to achieve its goal of integrating basic science with clinical practices, then it will be a great success and deservedly so.
Paul Davies is a consultant neurologist, Northampton General Hospital.