| This
issue of ACNR sees us entering our third year of existence and continuing
to link neuroscience to neurology and vice versa. The two review
articles look at very different aspects of the CNS and disease,
the first by Desmond Kidd reviews the neurological complications
of Behcet’s disease and the second by Mike Cheetham and Paul
Chapple explores neurodegenerative processes through the study of
retinitis pigmentosa.
Desmond
Kidd has set about trying to explore this often thought of but rare
disorder, through the recruitment of patients with the help of the
British neurological surveillance unit, and through his work at
tertiary referral centres. He then brings this personal experience
into a survey of the literature to highlight what is known and not
known about this condition. So, for example, he highlights that
the evidence for a vasculitis in Behcet’s is lacking and that
drug trials in this condition are needed if we are to develop any
rational approach to treating what often becomes a chronic, debilitating
disorder.
Mike
Cheetham and Paul Chapple have concentrated on looking at the molecular
mechanisms underlying the photoreceptor degeneration that characterises
retinitis pigmentosa (RP).Whilst this may be seen as a relatively
specialised area with little clinical significance to most neurologists,
the study of the processes underlying it may have wide-ranging implications
for a whole host of common neurological disorders. These researchers
have set about trying to unravel the intracellular processes causing
RP, particularly concentrating on the interaction of mutant proteins
with chaperones and chaperonins, whose normal role is to ensure
the correct processing of proteins within the cell and by so doing
protect it from damage. These proteins are also associated with
the proteosome, which acts to remove misfolded or abnormally processed
protein from within the cell. Disorders in these pathways have recently
been thought to be important in the pathogenesis of a range of common
neurodegenerative disorders such as Huntington’s (see journal
reviews) and Parkinson’s disease. Thus by studying one system
in one condition, inferences can be made that have wide-ranging
implications for a host of other disorders, and this in turn may
lead to common therapeutic strategies.
The
rehabilitation article this month is written by John Shneerson and
provides a marvellous overview of the use of ventilatory support
in diseases of the nervous system. Alex Leff provides an interesting
account on the rather obscure physician of the mid- to late 1800s,
Thomas Laycock. Laycock was the first proponent of cerebral reflexes
- actions which patients cannot restrain or modify and was also
the first person to argue that the anatomical divisions of the CNS
in humans is similar to that seen in other animals and forms part
of a continuum. Alex Leff gives a beautifully clear account of his
life and his contribution to neuroscience, as well as highlighting
that he may have been a greater influence of neuroscience than people
recognise, through his teaching of Hughlings-Jackson.
We
also have our usual collection of regular articles.Wojtek Rakowicz
and Mike Hanna conclude the superb series on muscle disease with
a beautiful summary of a complex area, this time channelopathies.
It is a shame that this is the last in the series, as they have
been a real highlight of recent issues – we move on to movement
disorders next with David Burn opening the bowling on the approach
to such a patient.
Brian
McNamara takes on the common peroneal nerve (CPN) and foot drop
– a condition which often presents diagnostic problems. Whilst
we all confidently tell medical students how to distinguish foot
drop in a CPN palsy from a radiculopathy, we often find ourselves
in clinic struggling to distinguish between the two (or perhaps
it is just me!). Brian takes his usual no-nonsense approach to this
issue through his neurophysiological eyes and provides a useful
summary, including many sentences telling us very appropriately
what steps we need to take to unravel the cause of this common problem.
Malcolm Steiger and Hiliary Tyne continue the series on apomorphine,
this time outlining the role of the neurologist and the need to
educate patients and public alike about this drug and its efficacy,
and Tipu Aziz and Dipander Nandi present their results for the neurosurgical
treatment of patients with intractable pain. We have our usual journal
and book reviews, as well as conference reports.
So
another packed issue, hopefully of the kind of articles that you
want to read, but if not let us know!
Roger
Barker
AdvancesinCNR@aol.com
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