John
van Swieten is well known in the field of dementia and it is his
clear account on the epidemiology
of fronto-temporal dementia (FTD) that forms one of our review
articles in this issue. This condition - which encompasses semantic
dementia and primary progressive aphasia - seems to be heterogeneous
not only in its clinical presentation but its pathology and aetiology,
including known genetic defects in tau and more recently in the
ESCRT3 complex subunit CHMP2B. This review article clearly lays
out the ambiguities of studies attempting to define the prevalence
of FTD as well as delineating the heterogeneity and problems and
challenges that this throws up.
For most of
us (well for me, anyway) once outside the familiar ground of chronic
tension type headaches, migraine and cluster headache, the diagnosis
of different types of recurrent headaches becomes a little bit muzzy.
What for example are hypnic headaches? In this article by Anish
Bahra we are taken on a brief but thoroughly helpful review of unusual
types of headache, many of which are especially responsive to
indomethacin. This clearly written account by such an expert will
now accompany me to clinic so that the next time I see someone with
a walking headache I will know to refer them for an exercise tolerance
test.
Our management
series stays with headache. All in clinical practice are familiar
with the headache following lumbar puncture, indeed in years gone
by it was thought to be helped by not allowing the patient out of
bed for several days. However, spontaneous
intracranial hypotension as a cause of headache is much less
common. In their article Imam and Weatherby explore the pathological
basis of this condition, as well as all of its clinical features,
investigations and management. This article highlights that it is
CSF volume rather than pressure that is critical in the genesis
of this condition which can be difficult to diagnose and treat.
Peter Tucker
addresses a very interesting area of rehabilitation, namely one
of the psychological
sequelae and management of children/ adolescents with acquired brain
injury. As he comments, "a central difference between children
and adults is that while the effects of the injury are immediately
obvious in adults, children's development is disordered after injury
and some deficits may take a considerable time to appear".
This is explored through a series of informative tables, highlighting
the differences in deficits in children and adults and their functional
and educational impact. This is a stimulating review which discusses
an often overlooked area of rehabilitation medicine.
Microarrays
are now emerging as one of the most exciting new tools in biology
as they allow one to look at thousands of genes in a single experiment.
Jo Jones leads us through this new technique highlighting the principles
that underpin it and the differences between spotted and oligonucleotide
Affymetrix gene chips. This is a clear account of an important new
technique which has been criticised on the grounds that it promotes
experiments which are non hypothesis- driven fishing expeditions.
This is true, but fishing expeditions often identify new fishing
grounds to harvest, and as such this is a powerful technique for
doing just that. It does of course generate data that represents
formidable bioinformatic challenges, as well as issues of which
of the changes are of greatest biological significance.
The use of muscle
biopsy in modern day neurological practice is explored by Dr
Leslie Bridges and shown to be central in the diagnosis of many
muscle disorders (see
also Rakowicz). This account takes us through the practicalities
of the biopsy procedure itself, through to its preparation, staining
and interpretation. As with all articles in this series it boasts
a plethora of wonderful illustrations.
In the visual
series Stewart Shipp takes us through the complexities
of the parallel visual pathways. The original scheme of organisation
within the visual pathway devised by Hubel and Wiesel was conceived
of as being serial-hierarchical processing, although in the mid
1960s different functional retinal ganglion cells (XYW system) were
recognised, which led to the possibility that the visual system
was composed of parallel pathways. This appears to have been verified
over the years with three major pathways being recognised projecting
from the retina to a number of targets both subcortically and cortically.
Stewart Shipp takes us through this complex system highlighting
what is known and not known and simplifying what is an immensely
complex field into an easily digestible and illuminating article.
Don Tucker and
colleagues from Oregon have also provided a very thought provoking
article on the use
of EEG to more accurately localise abnormalities in the CNS.
This is a very technical article but nevertheless clearly written
and provides insights into how old techniques can be developed and
modified to provide even greater and more useful information in
modern day neurological practice.
Andrew Larner
takes us to one of literature's
most famous epileptics, Fyodor Mikhailovich Doestoevsky. Andrew
explores the origin of the epilepsy of this wonderful Russian writer,
which encompasses aetiological causes ranging from psychogenic (Freud)
to an onset from a left mesial temporal lobe focus (Rosetti &
Bogousslavsky). This account is peppered with literary references
and will hopefully enthuse you to read his novels - Doestoevsky
that is, rather than Larner!!
Finally we have our usual wide range of journal, book and conference
reviews. We hope you enjoy this new issue and do let us know if
there aspects of neurobiology/neurology/rehabilitation that you
would like to see in ACNR.
Roger
Barker
Roger@acnr.co.uk |