| “If
the cell body were about thirty yards across the axon of a typical
motor neuron would be about the size of a tube train tunnel connecting
London and New York”. So writes Ammar Al-Chalabi of the
motor neuron, the demise of which underlines motor neurone disease.
In his short review Ammar lays out a logical, thorough, yet pragmatic
approach to this condition highlighting recent advances as well
as a range of useful statistics on its frequency and prognosis.
Neurodegenerative
disorders of the CNS are viewed as conditions where the primary
deficit is neuronal loss. Whilst there is no doubt that this
is the case, this is a relatively late event with the early manifest
problems being a consequence of more subtle abnormalities in
the neuron at the level of neurotransmission and synaptic plasticity.
In
this issue Tony Hannan and Nektarios Mezarakis explore this concept
in murine transgenic models of Huntington’s disease
highlighting the extent to which such an abnormality exists and
can be modified by manipulation of the environment. This is a succinct
account in an exciting area of neuroscience by acknowledged experts
in this area who have done much to throw light on the significance
of gene environment interactions in neurological disorders.
In
a third review article, Thomas Bak takes us through the differing
cognitive profiles of parkinsonian syndromes – an area
to which he has made a substantial contribution. In this review,
Thomas makes the point that documenting the profile of cognitive
deficits in atypical parkinsonian syndromes is not only a useful
exercise in its own right, but can be used to help define and refine
the diagnosis. Indeed, as he writes, “with growing understanding
of fronto-striatal connections it seems likely that at least some
cognitive and motor symptoms are different manifestations of the
same underlying pathology”.
Special
care dentistry forms the topic for the Rehabilitation article.
This “forgotten
strand in rehabilitation” covers
the dentistry needs of people with ‘special needs’ and
the article by June Nunn provides a clear and informative narrative
on how to prevent and recognise problems and their optimal management
which will significantly impact on my practice with such patients.
The
identification of intracranial metastases carries with it a poor
prognosis and often it is not clear what treatment should be
offered. In the Neurosurgery series, Peter Whitfield and oncologist
Steve Kelly take us through the various different approaches and
the evidence for their adoption or use in different clinical scenarios.
This is a clear account based on a detailed analysis of the published
literature and highlights the merits of surgery and whole brain
irradiation in selected, affected, groups of individuals.
We
are extremely privileged to have writing for us in this issue
Professor Raymond Adams, still active at the age of 95! In his
article Professor Adams lays out the vast number of neurological
conditions to which he has made seminal contributions. Thus it
was difficult to know what to ask Professor Adams to write about
given this, but we settled on central pontine myelinolysis, and
he takes us through the original description of this case outlining
how the syndrome came to be recognised in collaboration with his
colleague Maurice Victor. This is an absolutely fascinating article
which I am sure many will find of great intellectual interest
as well as being of huge historical significance.
We
also have a short article on a new initiative in MS, the Atlas
of MS, organised by the Multiple Sclerosis International Federation
(MSIF). This resource is designed to highlight and report on
different aspects of MS across the globe and looks to be a very
useful resource.
Finally
it would not be right as we enter November 2006 not to have some
mention of Alois Alzheimer – as it is now a 100 years
since he described the first case of his disease, patient Auguste
D, Andrew Larner delivers his usual scholarly account of this
historical event and reassuring tells us that his presentation “prompted
no comments or
reaction from the audience”.
We
have our usual regular articles, including journal and book reviews,
and as always we are keen to hear from you as to what we can
do to improve and expand the journal that now has a circulation
of over 4500.
Roger
Barker
Roger@acnr.co.uk |