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Diagnosis and treatment of
chronic inflammatory demyelinating polyneuropathy.
De Sousa EA,
Department of Neurology, Weill Medical College of
Cornell University, 635 Madison Avenue, Suite 400,
New York, NY 10022, USA.
Chronic inflammatory demyelinating polyneuropathy (CIDP)
is an immune-mediated acquired polyneuropathy that
may lead to disability. CIDP is characterized by an
autoimmune attack against peripheral nervous system
myelin, by cellular and humoral mechanisms. Early
diagnosis and treatment may yield better functional
recovery, probably by minimizing secondary axonal
loss from a primary demyelinating insult.
Intravenous immunoglobulin and plasmapheresis are
considered standard-of-care therapy in CIDP, based
on randomized, double-blinded, placebo-controlled
evidence. Corticosteroids, despite less robust
evidence, are also considered standard therapy for
CIDP. Other nonstandard therapies may work in
refractory patients.
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These include azathioprine,
cyclophosphamide, cyclosporine A, etanercept,
interferon-alpha 2a, mycophenolate mofetil, and
tacrolimus. Emerging therapies include
interferon-beta 1a, rituximab, and high-dose
cyclophosphamide without stem-cell rescue. Because
most patients will require prolonged therapy,
long-term side effects are important considerations.
PMID: 16464406 [PubMed]
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- ALS & CIDP LOOKS
SIMILAR
Rinsho Shinkeigaku. 2001 Dec;41(12):1210-3.
[New trends in neuropathy practice: clinical approach to
CIDP].
Baba M.
Source
Department of Neurological Sciences, Hirosaki University
School of Medicine.
Abstract
Our recent study showed that the overall prevalence of
CIDP was estimated as 2.2 per 100,000 population in
Aomori Prefecture, in Northan Honshu of Japan. In our
series of more than 80 cases with CIDP, a chronic
acquired inflammatory demyelinating polyneuropathy,
nearly 30% showed clear laterality of weakness, and
electrophysiologic laterality or multifocality was
apparent in almost all cases. Nearly 90% of patients
were able to walk without walking aids or other
assistance. Sixty% showed distal dominant muscular
weakness. In 12 patients with age of onset under 15, pes
cavus deformity was seen in 5. Two thirds complained
numbness in the extremities during progressive phase.
Four cases initially showed severe sensory ataxia
associated with motor conduction block. It should be,
thus, reminded that clinical spectrum of CIDP is
enormously wide: chronic acquired demyelinating multiple
mononeuropathy showing asymmetric involvement
(Lewis-Summer syndrome) should be put on one side of the
clinical presentation of CIDP. Multifocal motor
neuropathy (MMN) is, on the other hand, an unique
syndrome mimicking amyotrophic lateral sclerosis (ALS).
There may be, however, true association syndrome of CIDP
and ALS presenting both peripheral nerve demyelination
and pyramidal sign with progressive bulbar involvement.
Recently, several atypical varieties of CIDP showing
only one-limb involvement, upper limb weakness rather
than lower limb power loss, or proximal weakness, etc
... have been reported in the literature. To realize
such clinical variations of chronic acquired
demyelinating neuropathy is important for early
diagnosis and commencement of treatment of CIDP.
Clinical guideline for suspicion of CIDP could be useful
for general physicians and neurologists unfamiliar to
peripheral neuropathies. |
