Abstract book 6th RMS 16.indd

(alpha-synuclein and LRKK2) and three
recessive, usually causing young onset
“PD” (Parkin, PINK1 and DJ1). In addition
heterozygotes for the recessive Gaucher’s
disease with glucocerebrosidase gene
mutations have an increased risk of PD.
The three main neurodegenerative
diseases mistaken for PD are the alphasynucleinopathy
multiple system atrophy
(MSA), and the tauopathies progressive
supranuclear palsy (PSP) and corticobasal
degeneration (CBD).
Dopamine receptor blockers or dopamine
depletors commonly cause parkinsonism,
and vascular disease a poor mimic of
PD, but both can also often aggravate or
modify underlying PD.
There are many other causes of
parkinsonism, most ofthem genetic.
Probably the two most important are the
recessive Wilson’s disease (WD), because
it is treatable and without treatment it is
fatal, and the dominantly inherited dopa
responsive dystonia (DRD, Segawa disease)
because its signs and symptoms are almost
completely controlled by L-dopa.
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Anterior Clinoidal Meningiomas
Luis A. B. Borba MD (Brazil)
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Treatment of Parkinson’s Disease
Niall Quinn MD (UK)
Anticholinergcs were the first drugs used
to treat PD, followed in the late 60’s by
L-dopa and amantadine.
The dopa decarboxylase inhibitors
benserazide and carbidopa (in Madopar
and Sinemet respectively) were introduced
in the early 70’s, and controlled release
versions in the 80’s.
The dopamine agonist era began in
the md-70’s with bromocriptine, later
joined by the other ergoline oral agonists
pergolide and cabergoline (all now rarely
used because of lung, retroperitoneal
and cardiac valve fibrosis), and then the
non-ergolineropinirole and pramipexole
and the transdermal rotigotine. Prolonged
release oral formulations of ropinirole and
pramipexole have also been introduced.
Recently the frequency (13-14%), range
and severity of impulse control disorders
(including hypersexuality, compulsive
gambling, shopping and eating, and
morbid jealousy) in patients taking
dopamine agonists has been increasingly
recognised
Selegiline and rasagiline inhibit MAOB
(which metabolises dopamine) and
entacapone and tolcapone inhibit COMT
(which metabolises L-dopa).
Whether rasagiline has disease modifying
properties (as was originally claimed for
selegiline) is the subject of controversy,
and to date there is no conclusive evidence
that any drug used to treat PD has
neuroprotective effects.
More invasive drug treatment includes
subcutaneousinjections or infusion
ofapomorphine, and the intra-jejunal
infusion of L-dopa-carbidopa gel
(Duodopa).
On the surgical front, lesioning has given
way to deep brain stimulation (DBS), and
the preferred target has moved from
thalamus through globuspallidus to
subthalamic nucleus.
Fetalnigral grafts are still being investigated,
and in the future there will be safety and
efficacy trials using stem cells.
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Tumors of Jugular Foramen
Luis A. B. Borba MD (Brazil)
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Hyperkinetic Movement Disorders
Niall Quinn MD (UK)
In contrast to the akinetic-rigid syndromes,
in which there is too little movement,
hyperkinetic movement disorders cause
unwanted, excessive, movement.
Step one in diagnosis is to identify which
category of abnormal movement can
be seen in the patient. Most abnormal
movements can be described and identified
using just 5 terms:- tremor, dystonia
(athetosis is distal mobile dystonia), and
three types of jerk – tics, myoclonus and
chorea (ballism is severe proximal chorea).
Some patients display only one of the
above, e.g. isolated tremor in essential
tremor (ET). However, commonly a patient
can present more than one movement
disorder e.g. akinesia, rigidity, dystonia
and chorea often coexist in Huntington’s
disease, and tremor, myoclonus and tics
can sometimes also be seen. An important
consideration whenever one sees a
mixed movement disorder is whether
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