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Intravenous immune globulin (IVIG) has also been found to be
beneficial. IVIG is usually given in divided doses over 4 or 5
consecutive days. Maintenance doses are often needed at variable
intervals to maintain clinical response. Serious side effects of
IVIG treatment are rare (fatal anaphylaxis in IgA-deficient
patients; potential nephrotoxicity, especially in patients with
pre-existing renal disease).
Those patients who do not respond to IVIG can be tried on
Plasmapheresis. Other treatments that a doctor can prescribe are
Cytotoxic eg (Imuran, Cyclosporin). Steroids can also be used .
The clinical diagnosis is based on a criteria which was set out by
an American committee in 1991. It is a progressive or relapsing
neuropathy and the progressive phase lasts for more than two
months. There is no clear antibody test to prove the diagnosis,
and it is reached by the following methods:-
- Nerve conduction tests
- Blood tests (to exclude things like diabetes, B12
level, which may mimic or even cause it)
- X-rays to ensure there is no underlying inflammatory reason, or
even cancer
- Spinal fluid testing because the protein is usually raised in
CIDP
- Careful family histories, as hereditary neuropathy can mimic
CIDP
Nerve biopsies are not usually necessary.
There are many different types of GBS and 3% of patients have
recurrent attacks. There is also a form where onset develops over a
period of four weeks or so and a sub-acute type that develops over
4-8 weeks. CIDP is only named if it progresses very chronically. It
is usually symmetrical and affects both the motor and sensory
nerves, but there are many different sorts of CIDP and a type known
as MADSAM (multifocal acquired demyelinating sensory and motor
neuropathy) is very asymmetrical and involves different nerves. CIDP
is usually motor, but is sometimes just sensory. A purely motor
form, known as MMN (multifocal motor neuropathy) is a separate
condition where conduction is not slow, but actually blocked at
multiple sites.
The cause of CIDP is that its a autoimmune disease triggered by
infections, toxins, vaccination, injury. Antibodies to
gangliosides have been found in only a small number of patients. It
is believed to be an auto-immune condition as it has been found to
resemble an experimental chronic auto-immune neuritis, but also
because it responds to immuno suppression.
A study involving 40 patients with CIDP was looked at, with the
following results:-
- More men than women were affected (as in GBS)
- Mainly middle age and elderly people were affected, and it was
rare in children
- Preceding infections were only documented in 25% of patients
(much more common in GBS)
- There were no examples of it being triggered by immunisations
- There were no examples of it being triggered by cytomegalovirus
Antibodies to various different myelin proteins have been looked at
and Carolyn Gabriel produced a test for antibodies to a protein
known as "PMP22", showing it to be positive in 41% of patients with
CIDP. This is so far the closest they have got to finding antibodies
consistently to any particular antigen and could be part of the
story. More recently, the Sydney Group has shown antibodies to
another protein, known as "PO", in 28% of patients. This is yet to
be confirmed, but it is now possible to look for cell responses to
those two proteins, though the jury is still out as to whether they
are really responsible.
CIDP is usually treatable the first line of treatment is IVIG if a
patient does not benefit from IVIG then the physician should
consider other treatments like plasmaphersis or steroids,
cyclosporon, avonex, enbral.
Neurology. 2002 Dec 24;59(12 Suppl 6):S33-40. |
|
Intravenous gammaglobulin (IVIg) for treatment of
CIDP and related immune-mediated neuropathies.
Brannagan TH 3rd.
Peripheral Neuropathy Center, Department of Neurology and
Neuroscience, Weill Medical College of Cornell University, New York,
NY 10021, USA.
Intravenous immune globulin (IVIg) is considered an effective and safe
treatment for autoimmune neuropathies, especially in comparison to
the alternative treatments such as corticosteroids, chemotherapy,
and plasmapheresis. Patients are frequently given a standard
induction dose of 2 g/kg, which may be followed by maintenance
therapy as needed. Mild infusion-related reactions are frequent but
these can often be controlled by slowing the infusion rate or by
symptomatic medications. Serious adverse effects are rare and can
include thromboembolic events, renal failure, anaphylaxis, or septic
meningitis. Patients with IgA deficiency are at risk for
anaphylaxis. Immobility, increased serum viscosity, and preexisting
vascular disease can increase the risk for thromboembolic events.
Preexisting renal insufficiency or the use of sucrose-containing
IVIg preparations can increase the risk for renal failure, and
patients with migraine are at risk for development of aseptic
meningitis. Screening patients for risk factors that predispose to
development of adverse events may reduce the incidence of
complications.
For those with multi-focal neuropathy with conduction block the
rules for treatment are different. Those patients get worse with
steroids and do not do very well with plasma exchange, but do
respond well to IVIg.
Some patients with CIDP have a para-protein in the blood, which is
an abnormal protein produced by the bone marrow. These patients may
respond to CIDP treatments, but may also require treatment for the
para-protein.
To conclude, CIDP is a very variable condition. It is almost
certainly auto-immune and is treatable. It is exceptional for CIDP
not to respond to at least one of the available drugs. The first
choice of treatment is either prednisolone or IVIg. The diagnosis is
difficult, much less so than GBS, depending critically on the nerve
conduction studies and neuro-physiologies. Trials for
immuno-suppression are badly needed to find out which of these drugs
really work and which are the best to use. The research is exciting
and funding is badly needed for it. ..........
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Brain. 1996 Aug;119 ( Pt 4):1067-77. |
|
Intravenous immunoglobulin treatment in chronic
inflammatory demyelinating polyneuropathy. A double-blind,
placebo-controlled, cross-over study.
Hahn AF, Bolton CF, Zochodne D, Feasby TE.
University of Western Ontario, London, Canada.
Thirty patients with definite or probable chronic inflammatory
demyelinating polyradiculoneuropathy (CIDP) of chronic
progressive (16 patients) or relapsing (14 patients) course were
randomly assigned to receive intravenous immunoglobulin (IvIg)
0.4 g per kg body weight or a placebo treatment on 5 consecutive
days in a double-blind, cross-over trial. Neurological function
was monitored by serial quantitative assessments [neurological
disability score (NDS); clinical grade (CG) and grip strength
(GS) measurements] and by electrophysiological studies before
and after each treatment period. Twenty-five patients completed
both treatment periods. A comparison of the observed changes in
clinical outcome measures revealed statistically significant
differences in favour of IvIg, with (mean +/- SD) improvements
in NDS by 24.4 +/- 5.4 points (P < 0.002) in CG by 1 +/- 0.3
points (P < 0.001) in GS by +6.3 +/- 1.7 kg (P < 0.005), whereas
scores were unchanged or worse with placebo. A secondary
two-groups analysis of the first trial period included all 30
patients; 16 patients had been randomly assigned to IvIg and 14
to placebo treatments. Again significant differences in favour
of IvIg were observed in all the clinical end-points:
improvement in NDS was 35.6 +/- 25 points (P < 0.0001), in CG it
was 1.3 +/- 1.9 points (P < 0.002) and in GS +9.8 +/- 7.7 kg (P
< 0.001), whereas all scores worsened with placebo. Of the 30
patients, 19 (63%) improved with IvIg treatments; nine out of 16
patients (56%) with chronic progressive CIDP, and 10 out of 14
patients (71%) with relapsing CIDP (differences were not
statistically significant). A placebo response was seen in five
patients. Comparison of paired electrophysiological measurements
before and 4 weeks after IvIg treatments revealed statistically
significant improvements in the summed motor conduction
velocities (sigma MCV; P < -0.0001) and in the summed compound
muscle action potentials (CMAP) evoked with proximal stimulation
(sigma proximal CMAP, P < 0.03) of median, ulnar, peroneal and
tibial nerves. Eight of nine IvIg responders with chronic
progressive CIDP improved gradually to normal function with a
single 5 day course of IvIg; in five of these, small doses of
prednisone were prescribed during follow-up. In 10 IvIg
responders with relapsing CIDP, improvements lasted a median 6
weeks (range 3-22 weeks) and was reproducible with open label
treatments. All 10 patients have been maintained and stabilized
with IvIg pulse therapy of 1 g per kg body weight or less, given
as a single infusion prior to the expected relapse. A beneficial
response to IvIg was found to be most likely in patients with
acute relapse or with disease of one year or less. Patients with
predominantly sensory signs did not improve.