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Presse Med 2013 PDF
discussions, stats, and author profiles for this publication at: http://www.researchgate.net/publication/263935465
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6 AUTHORS, INCLUDING:
Christa Walgaard Md
Bart C Jacobs
Erasmus MC
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IMMUNE-MEDIATED NEUROPATHIES
Correspondence:
Available online: 28 April 2013
Pieter A. van Doorn, Erasmus MC, Department of Neurologys-Gravendijkwal 230, 3015CE Rotterdam, The Netherlands.
p.a.vandoorn@erasmusmc.nl
Summary
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PA van Doorn
EMG examination
EMG is especially helpful when it shows signs of a polyneuropathy in clinically not yet involved areas, for example when it
Figure 1
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IMMUNE-MEDIATED NEUROPATHIES
Box 1
Box 3
Box 2
Features that should raise doubt about the diagnosis
Anti-ganglioside antibodies
Anti-ganglioside antibodies, as monomer or as complexes, can
be found in a proportion of GBS patients, however especially in
tome 42 > n86 > juin 2013
Intracranial/spinal cord:
Peripheral nerves:
Neuromuscular junction:
Muscle:
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PA van Doorn
Table I
Spectrum of Guillain-Barr syndrome (GBS) and serum antiganglioside antibodies
GBS subgroup
Antibodies
Unknown
Miller-Fisher syndrome/GBS
overlap syndrome
Treatment
Importance of general care in Guillain-Barr
syndrome (GBS)
Patients with GBS especially need excellent multidisciplinary
care to prevent and manage the potential fatal complications.
This indicates the need for careful monitoring of cardiac and
Table II
Criteria for Guillain-Barr syndrome (GBS) for vaccinations or epidemiological research [21]
Items that are fulfilled/requested
5. Cytoalbuminologic dissociation # (i.e elevation CSF protein level and CSF white cells < 50 106/L)
+/#
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IMMUNE-MEDIATED NEUROPATHIES
Box 4
Box 4 (Continued)
Diagnosis:
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PA van Doorn
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Figure 2
Guillain-Barr syndrome (GBS), treatment-related fluctuations
(GBS-TRF) and acute onset CIDP (A-CIDP)
IMMUNE-MEDIATED NEUROPATHIES
A-CIDP patients:
no ventilatory support;
more demyelinating features on EMG;
when three or more exacerbations;
when deterioration occurs > 2 months from onset.
Autonomic failure
Autonomic dysfunction, like labile blood pressure, tachycardia
or heart rate disturbances, often occurs in GBS patients. Recognition of autonomic dysfunction is important, because it may
be an important cause of death in GBS. Three to 10% of patients
die, presumable partly due to (sudden) autonomic failure. It is
yet not well possible to predict which patients will develop
serious autonomic failure and therefore need continue monitoring [47]. From observational studies, it seems that patients
not only die while being admitted on an ICU due to serious
autonomic failure or pulmonary problems, but also the first
period after discharge from an ICU can be a high-risk period
especially when a patient has a tracheostoma and stasis of
sputum.
Future directions
New treatment options in GBS are absolutely necessary
because the prognosis in a large group of GBS patients is
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Box 5
PA van Doorn
still far from good. One option in the acute phase could be a
second IVIg treatment in patients with a poor prognosis. The
SID-GBS RCT is going on in The Netherlands, and the international second-dose IVIg study (I-SID-GBS) has been started.
Recent experiments indicate that agents that interfere with
complement activation are potentially attractive candidates to
be tested in a very early phase of GBS [52]. Since it is now
possible to predict outcome in individual patients more accurately, new drugs like eculizumab or other regimens could be
tested especially in a restricted GBS population with a poor
prognosis. Focus also on the pathophysiological effect of treatment, such as studying the mechanism of action of IVIg, potentially could also lead to more personalized treatment once it is
shown that some patients require other IVIg dosages or treatment regimens. Treatment trials, especially in rare diseases,
usually require a long period of time to include sufficient patients.
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