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Welcome to
the treatment section of the CIDP web site.PAGE-2 |
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High-Dose Immunosuppression for
Refractory CIDP?
Chronic inflammatory demyelinating polyneuropathy
(CIDP) is an immune-mediated neuropathy for
which treatment with plasma exchange,
intravenous immunoglobulin, or corticosteroids usually
is beneficial. However, one third of CIDP patients
do not respond to these conventional
therapies, and many who respond initially
relapse and develop substantial disability after several
years. Compelling data suggest that both B
and T cells mediate the immune attack on
peripheral nerve, thus providing a biologic
rationale for using more aggressive immunosuppressive
regimens.
These researchers report their experience with
high-dose cyclophosphamide (200 mg/kg over 4
days) in 4 patients with severe CIDP that was
refractory to other therapies. All patients were
disabled (Rankin scores of 4 or 5) and had
failed numerous trials of standard therapies.
Interestingly, 3 patients also had failed to
improve after previous treatment with intravenous
cyclophosphamide (1 g/m2).
All patients' Medical Research Council strength
scores and Rankin disability scores improved;
2 patients improved by 3 Rankin grades.
Febrile neutropenia and transient renal insufficiency
occurred in 2 patients, and another developed line
sepsis, but there was no long-term morbidity
or mortality.
Comment:
Current
therapies for CIDP are inadequate for many
patients. In this study, a 70-kg patient would
have received 14 g of cyclophosphamide, only
slightly more than the "immunomodulating"
dose (1 g/m2) of 1.5 to 2.0 g
administered as a pulse monthly for 6 months
(a regimen that many centers already have adopted
empirically). Therefore, the intensity of
immunosuppression may be a more relevant
factor than the total cumulative dose or the
duration of therapy. The
benefits of this approach must
be weighed
against high risks for systemic infection and for
other serious complications.
The risk-benefit
balance may be
in favor
of high-dose immunosuppression if patients experience
sustained remission and discontinue prednisone or other
chronic
immune (and
potentially toxic) treatments.
— Kenneth C. Gorson, MD
Dr. Gorson is Associate Professor of Neurology, Tufts
University School of Medicine, Boston.
Published in Journal Watch Neurology August
22, 2002
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
excess para-protein.
To conclude, CIDP is a very variable condition. It is 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 have shown
promise. ..........cidpusa
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[High-dose intravenous immunoglobulin in the
treatment of sensory ataxic neuropathy with Sjögren's syndrome:
a case report]
Taguchi Y, Takashima S, Takata M, Dougu N, Asaoka E, Inoue H.
The Second Department of Internal Medicine, Toyama Medical &
Pharmaceutical University, 2630 Sugitani, Toyama 930-0194,
Japan.
We report herein a case of sensory ataxic neuropathy with
Sjogren's syndrome (SS-SAN) who became dramatically improved in
response to high-dose intravenous immunoglobulin treatment
(IVIg). An 81-year-old man began to feel numbness in his hands
and feet in August 2002. Because he became unsteady and could
not do skillfull movement, he was admitted to our hospital in
May 2003. On neurological examination, all tendon reflexes were
absent. His vibratory and position senses were severely impaired
to knees and elbows. Touch, temperature, and pinprick sensations
were mildly disturbed in a glove-stocking distribution.
Coordination was clumsy in all limbs because of sensory loss. He
had gait ataxia with Romberg sign. Nerve conduction study
revealed that sensory nerve action potentials were absent. He
was diagnosed as having SS-SAN because Schirmer test, Saxon test
and both SS-A and SS-B antibodies were positive. Thereafter, Mg,
400 mg/kg daily for 5 days, was administered. His sensory
impairment began to improve 2 days after Mg. Subsequently, he
could walk steadily without ataxia. It is considered that IVIg
may be an effective treatment for SS-SAN.
PMID: 15279200 [PubMed - indexed for MEDLINE]
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Diabetic demyelinating polyneuropathy
responsive to intravenous immunoglobulin therapy.
Sharma KR, Cross J, Ayyar DR, Martinez-Arizala A, Bradley WG.
Department of Neurology, University of Miami School of Medicine,
1150 NW 14th St, Room 603, Miami, FL 33136, USA. ksharma@med.miami.edu
BACKGROUND: There is growing evidence that idiopathic chronic
inflammatory demyelinating polyneuropathy (CIDP) and
polyneuropathy in patients with diabetes mellitus (DM) that
meets the electrophysiological criteria for CIDP (DM-CIDP) have
many similarities. OBJECTIVE: To evaluate whether DM-CIDP
responds to intravenous immunoglobulin (IVIG) therapy. PATIENTS
AND METHODS: Twenty-six patients (mean [SD] age, 64 [8.9] years;
age range, 40-80 years) with type 2 DM (n = 25), who met the
electrophysiological criteria for CIDP, were given IVIG therapy
(400 mg/kg body weight per day for 5 days) in a prospective
open-label pilot study. All patients had quantitative evaluation
using the Neuropathy Impairment Score at baseline and at the end
of 4 weeks from the initiation of IVIG therapy. RESULTS: The
mean Neuropathy Impairment Score improved significantly from
baseline (mean [SD], 61.5 [26.0] points) to the end of the
fourth week (33 [29.6] points; P<.00l). This clinically
significant improvement occurred in 21 (80.8%) of the 26
patients. Conduction block occurred in 11 (42.3%) of the 26
patients; improvement in the Neuropathy Impairment Score was
more frequent in patients who had a conduction block (11 of 11
patients) than in those who did not (10/15 [66.7%]; P =.03).
Adverse reactions to IVIG included reversible renal dysfunction
in 3 patients, flulike symptoms in 5, headache in 5, and chest
pain and shortness of breath in 1. CONCLUSION: Although IVIG
therapy seemed to improve DM-CIDP in this uncontrolled trial, a
controlled trial is required for confirmation of our findings.
Publication Types:
PMID: 12020256 [PubMed - indexed for MEDLINE]
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Diabetic Lumbosacral Polyradiculoneuropathies.
Amato AA, Barohn RJ.
Department of Neurology, Brigham and Women's Hospital, Harvard
Medical School, 75 Francis Street, Boston, MA 02115, USA.
The pathogenic basis and treatment of diabetic
polyradiculoneuropathy is a source of recent controversy as
there may be two or more distinct forms of diabetic
polyradiculoplexopathy. We believe that the following two
categories of diabetic polyradiculoneuropathy can be made on the
basis of clinically differences: 1) the more common asymmetric,
painful polyradiculoneuropathy; and 2) the rare symmetric,
painless, polyradiculoneuropathy. The asymmetric, painful form
(also known as diabetic amyotrophy) may have an autoimmune
basis, but the etiology is not clear. The natural history for
diabetic amyotrophy is spontaneous improvement. Nevertheless,
various immunotherapies (eg, corticosteroids and intravenous
immunoglobulin (IVIg) have been tried with subsequent
improvement in symptoms. Treatment is reserved only for patients
with severe ongoing pain, given the significant side effects of
these medications in those patients with diabetes. Prednisone
and IVIg may help alleviate the pain associated with diabetic
amyotrophy. Relief of pain can help patients begin physical
therapy earlier, however, there are no prospective, blinded,
controlled studies that demonstrate that these treatments lead
to an earlier and better recovery of muscle strength compared
with the natural history of the disorder. The symmetric,
painless form of diabetic polyradiculoneuropathy may in fact
represent chronic inflammatory demyelinating polyneuropathy
(CIDP) occurring in a patient with diabetes mellitus (DM).
Patients with idiopathic CIDP may improve various
immunomodulating therapies, including corticosteroid treatment,
plasma exchange (PE), and IVIg. In this regard, patients with
the symmetric, painless, proximal diabetic
polyradiculoneuropathy may also respond to corticosteroids,
plasma exchange, IVIg, azathioprine, or cyclophosphamide.
However, as with diabetic amyotrophy, some patients improve
spontaneously without treatment. In still other patients, the
neuropathy appears unresponsive to immunotherapy. In such
patients, this polyradiculoneuropathy might be caused by
metabolic dysfunction associated with DM. Unfortunately, from a
clinical, laboratory, and electrophysiologic standpoint, it is
impossible to distinguish the patients with a symmetric,
painless diabetic polyradiculoneuropathy who might respond to
therapy. A trial of PE can be useful in identifying patients who
might have a polyradiculoplexopathy that is responsive to
immunotherapy. If patients respond to PE, they may continue to
receive intermittent exchanges or be switched over to prednisone
or IVIg.
PMID: 11180751 [PubMed - as supplied by publisher]-
| Brain. 1996 Aug;119 ( Pt 4):1067-77. |
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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.-
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J Neurol Neurosurg Psychiatry. 2005
Aug;76(8):1115-20. |
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Intractable chronic inflammatory demyelinating
polyneuropathy treated successfully with ciclosporin.
Odaka M,
Tatsumoto M,
Susuki K,
Hirata K,
Yuki N.
Department of Neurology, Dokkyo University School of Medicine,
Kitakobayashi 880, Mibu, Shimotsuga, Tochigi 321-0293, Japan. m-odaka@dokkyomed.ac.jp.
BACKGROUND: Chronic inflammatory demyelinating polyneuropathy (CIDP)
is a heterogeneous disorder and both clinical course and response to
treatment vary widely. Because of the propensity for relapse, CIDP
requires maintenance therapy after the initial response to
treatment. There is no consensus regarding this in the published
literature.Present report: A patient with CIDP was treated with oral
prednisolone and cyclophosphamide pulse therapy but required
repeated plasma exchange and intravenous immunoglobulin (IVIg).
Treatment with ciclosporin freed the patient from repeated IVIg
administration. Therapeutic responses in 14 subsequent cases
including three patients who showed improvement with ciclosporin are
also presented along with an algorithm of the authors' suggested
protocol for treatment. CONCLUSION: Ciclosporin should be considered
for patients with intractable CIDP who require repeated IVIg.
PMID: 16024890 [PubMed - in process]
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ProcalAmine is a intravenous protein
infusion. . Well
he treated and diagnoses a lot of CHF and FMS. A
neurlogist had quite a
success with ProcalAmine...... He has even written a paper on this
except it has not been accepted yet.
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www.cidpusa.org
www.cidpusa.org/P/ivig.htm
http://www.cidpusa.org/disease.html
http://www.cidpusa.org/Lahore.html
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