Brain, Vol. 127, No. 9, 1913,
September 2004
© 2004 Guarantors of Brain
doi: 10.1093/brain/awh285
Editorial |
Editorial
The current issue of Brain introduces a few minor changes. We include a book review that is longer than our previous notices of recent publications. Neil Scolding tests Myelin Biology and its Disorders, edited by Bob Lazzarini, against some of the heavyweights in glial neurobiology. He records important but not well-known historical details concerning the discovery of oligodendrocytes and microglia. Professor Scolding assigns high but not top marks to Dr Lazzarini and his publishers, suggesting additions and expansions for an improved next edition. As before, the meat of the journal is made up of original publications, all processed and selected by the former editorial team. In our coverage of cognition and brain function, development, neurogenetics, peripheral nerve disease, headache, neurophysiology, functional imaging, and disease mechanisms—from both the experimental and the clinical perspective—we hope there is something of interest for all our readers.Following its introduction in the 1950s (most vigorously by Wilder Penfield at the Montreal Neurological Institute), surgery for epilepsy went somewhat into abeyance for a while, but now there is renewed interest despite the increased availability of effective anti-epileptic drugs. Does surgery work, alone or in combination with drug therapy? Anne McIntosh and colleagues from Melbourne (page 2018) provide follow-up on 325 patients, around 10 years after anterior temporal lobectomy. About half do well within 2 years, and this effect lasts. Freedom from seizures is especially increased for individuals with an identifiable preoperative lesion. Many of those showing improvement in seizure frequency are able to discontinue drug treatment, suggesting that surgery is the effective intervention per se. The more radical procedure of hemispherectomy is described in a much smaller cohort by the Dutch Collaborative Epilepsy Surgery Programme (page 2071). Ron van Empelen and colleagues report freedom from seizures in 75% of 12 children, and with no lasting increase in pre-existing physical and functional motor deficits contralateral to the missing hemisphere—apart from the distal part of the arm (commonly known as the hand?).
Amongst the papers on neurogenetics, Isabelle Le Ber and colleagues describe 10 affected individuals with a new disorder in a single pedigree involving 30 family members from three generations (page 1979). Clinical, electrophysiological and histological features of myotonic dystrophy are combined with the cognitive characteristics of severe frontotemporal dementia, SPECT and structural brain abnormalities, and pathological confirmation, including occasional neuronal and glial tau inclusions, in two individuals coming to autopsy. The absence of mutations for DMPK and ZNF9 exclude DM1 and DM2, respectively. A genome-wide screen maps the gene to 15q21-24, and the authors propose designating this new disorder ‘DM3’.
Clinical neuroscience often progresses by foxtrot—two steps forward and one sideways. Against the background of persistent Notch1/Jagged1 expression in tissue from individuals with multiple sclerosis failing to show significant remyelination, Mark Stidworthy and the group of Robin Franklin (page 1928) seek confirmation that Notch1/Jagged1 signalling also inhibits oligodendrocyte progenitor maturation in a rodent model of remyelination. Not so: despite expression of Notch1 receptors on oligodendrocyte precursors and widespread availability of Jagged1 from a variety of local cellular sources, remyelination is unaffected. Exploiting the observation that remyelination proceeds more slowly in older animals, the team finds no age-related differences in Notch1/Jagged1 expression. Knocking out Notch1 and inducing demyelination with cuprizone in these transgenic mice also fails to influence remyelination. Thus, the jury remains out on whether persistent Notch1/Jagged1 expression prevents tissue repair in demyelinating disease, and (once again) clinical and experimental observations prove difficult to reconcile.
We publish an article by Renaud Du Pasquier and colleagues (page 1970) that correlates the effect of cytotoxic T lymphocytes recognizing the polyomavirus JC, and appearing early in the disease course, on improved prognosis in 26 individuals with progressive multifocal leucoencephalopathy, most of whom were immune-deficient as a result of HIV infection. Progressive multifocal leucoencephalopathy was first described in Brain ...
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