This issue of ACNR boasts two magnificent review articles on different types of motorneuron disease.

We are delighted that Gen Sobue and colleagues have written a very topical and exciting review on spinal-bulbar muscular atrophy (SBMA) or Kennedy’s disease. This disorder is rare but is due to a trinucleotide CAG repeat in the androgen receptor, although the mechanisms that lead to selective cell death as a result of this are unknown. However Sobue and colleagues have recently shown that the translocation of mutant androgen receptors to the nucleus is a critical pathogenic event, and that blocking this process could provide an effective treatment for this disorder. This work which was recently published in Nature Medicine (June 2003; 9:768-773), represents a major step forward in our understanding of this disease, and offers a clear therapeutic target – namely blocking the androgen binding to the receptor in the cytoplasm and by so doing preventing its nuclear translocation. This clearly creates ethical issues in the clinic as it would mean that effective treatment could be used at the cost of infertility.

This beautiful account of SBMA is nicely complemented by the article from Pam Shaw and Alice Brockington on new developments in the treatment of other more common forms of motor neurone disease. This review provides a comprehensive account of what is currently known about the pathogenesis of this condition, and how this may lead to new therapies. Thus whilst standard symptomatic and therapeutic agents are discussed, there is also a section on future directions. These two articles therefore provide a comprehensive and up-to-date account on the pathogenesis and therapeutic options for this fatal neurodegenerative condition.

This issue’s management topic tackles myoclonus with a case of palatal myoclonus (see also our Case Report with video clip). I have tried to take a pragmatic approach to the classification, aetiology and treatment of this movement disorder, which forms the fourth topic in our series on movement disorders.

The rehabilitation article this time presents some fascinating data and possibilities using functional electrical stimulation in cervical cord damage. This account highlights the mechanics of the process and its effect on cardiovascular performance and thus its role in maintaining fitness in patients with limited upper function secondary to cervical cord damage.

This issue also contains the last in our series on peripheral nerves and their neurophysiological investigation by Brian McNamara. This time Brian takes on the brachial plexus, which he describes as “the sceptre that stalks every anatomy student’s nightmares”. As usual it is packed with common sense and we shall greatly miss his no nonsense approach, which has been a hallmark of his series of wonderful articles.

Our historical account this month is by Andrew Larner, and describes Gilles de la Tourette syndrome in Dr Samuel Johnson, Mr Pancks in Dickens’ Little Dorrit and Captain Hardcastle, a schoolmaster to Roald Dahl. As always this account is learned and fascinating, and once more highlights the power of literature to capture neurological conditions.

We also have an interview with the new President of the BNA, Professor Richard Frackowiak. Professor Frackowiak, as many will testify, is a larger than life character who brings great energy and enthusiasm to all that he takes on.He has a formidable track record in research and has very much managed in his work to bridge clinical and basic neuroscience, and is a fine choice for this prestigious post. We wish him all the best, as his appointment reinforces the point that this journal tries to make with each issue - namely that clinical neurology can inform basic neuroscience and vice versa and thus each needs to know what the other is doing.

Finally we have our usual meeting reports and journal review, including a summary of the latest US double blind placebo controlled trial of fetal neural transplantation in Parkinson’s Disease. So we hope you enjoy this issue, and do keep the feedback coming so we know what we are missing and can improve upon.

Roger Barker
AdvancesinCNR@aol.com