The
two review articles in this issue both take antibodies as their
theme. Camilla Buckley introduces us to the disorders
associated with antibodies to the shaker family of voltage gated
potassium channels. The autoimmune disorders targeting this
channel include the peripheral and central nervous systems ranging
from mild cramp fasciculation syndrome to limbic encephalitis and
Morvan’s fibrillary chorea. This article serves to educate
and update us on these not uncommon disorders and is written from
a perspective of great authority, coming as it does from the leading
UK centre involved in the discovery and definition of these conditions.
The second review
article tackles the controversial area of anti
basal ganglia antibodies and their significance. Gavin Giovannoni
takes us through this controversy and the work from his laboratory
showing how these antibodies may underlie a number of neurological
conditions, including Sydenham’s chorea and encephalitis lethargica.
This is a beautifully written and balanced account, which lays out
the arguments for the clinical relevance of these antibodies (see
also Journal
reviews for more on auto-antibodies!).
The neuropathology
article by Professor Jeanne Bell and Dr Iain Anthony gives us a
comprehensive and beautifully illustrated review of the neuropathology
of HIV and in particular a comparison of this prior to and following
the adoption of HAART in 1997. In the pre-HAART era, the onset of
AIDS was associated with HIV encephalopathy and/or opportunistic
infections in 50% of cases with a similar proportion developing
a spinal cord vacuolar myelopathy. Nowadays if the CD4 count can
be restored with HAART, the chances of developing such neuropathology
is significantly reduced. Interestingly, however, other aspects
of the disease are becoming more common - such as the HIV associated
dementia and the less severe minor cognitive motor disorder (MCMD).
This “emerging” neuropathology may relate to the increased
longevity of the life span of patients carrying HIV, and as such
may represent the new challenge to the treatment of HIV in the developed
world. This is a discussion that sadly is not to be had in the developing
world, where HAART is not so readily available.
Charcot has
made many contributions to our current neurological practice and
this includes his work on MS. This contribution to ‘sclérose
en plaques’ was based on only 30 patients, a topic discussed
by Professor Alastair Compston – the current editor of Brain
and renowned international authority on MS and historical neurology.
As one would have anticipated, this article is beautifully written
and lavishly illustrated with quotations from the original texts.
It is once more a reminder of the power of clinical observation
to expose and inform our understanding of disease pathogenesis,
and how so much can be learnt by reading the works of the founders
of modern day neurology.
The rehabilitation
topic in this issue revolves around the very interesting concept
of the neuroprotective
effects of progesterone, especially in the context of traumatic
brain injury (TBI). Don Stein from Atlanta, Georgia begins his discussion
with the initial observations back in 1987 on the differential effects
of TBI in female rats depending on whether they are in the follicular
or luteal phase of their cycle. This has now been investigated and
developed to the point of clinical trials, and as such may impact
on the treatment of this common neurological problem (see also Journal
reviews on pituitary dysfunction in TBI).
The cognitive
primer by Cipolotti and van Harskamp is on dyscalculia,
and we are fortunate to have such experts take us through the types
of deficits that occur in this acquired disorder. This article beautifully
dissects the different problems of number processing and calculation
as well as outlining the logical assessment and approach to such
patients, most of whom will have damage in the region of the left
parietal lobe. Peter Whitfield and Devindra Ramnarine outline the
presentation, management and natural history of acoustic/vestibular
schwannomas. These tumours have an incidence of about 1:100,000
per year and have become more “common”with the advent
of MRI. This article lays out the three major therapeutic approaches
to these tumours – namely a conservative one (especially for
small tumours in elderly patients) or treatment with stereotactic
radiosurgery or neurosurgery itself.
Drugs
in neurology focuses on the new potent irreversible MAO-B inhibitor,
rasagiline (AZILECT®). This drug has recently been launched
in the UK and has received much media attention, given its potential
neuroprotective effects and value in more advanced cases of Parkinson’s
disease. In this article Professor David Brooks discusses the properties
of rasagiline and the trial data supporting the above claims for
the drug.
For those going
to the World Congress of Neurology in Sydney, look out for the special
issue of ACNR. This contains some of our recent articles as well
as a wonderful account by Dr Fisher on his variant of Guillain-Barre
syndrome. This is a truly wonderful account of how this disorder
was first recognised, as well as highlighting the problems of having
a surname as a forename.We are very grateful to Dr C Miller Fisher
for writing this article for us, which is a great inspiration for
all those involved in neurological practice. It is another reminder
of the value of keeping notes and details on all the cases that
one encounters, especially those without a diagnosis. We will be
reproducing this article in the UK edition at the end of the year,
so don’t worry - you won’t miss out on his inspiring
and personal account.
Roger
Barker
Roger@acnr.co.uk |
