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Cui et al. Treatment guidelines of chronic inflammatory demyelinating polyneuropathy in China

Differential diagnosis significant increase of CSF protein, which leads to
(1) POEMS syndromes, or Crow-Fukase syndrome, misdiagnosis of CIDP. The critical diagnostic criterion
demyelination based polyneuropathy, organomegaly is the significant elevation of plasma level of phytanic
of liver, spleen and lymph nodes, endocrine acid. [2,6]
abnormalities such as diabetes, hypothyroidism and
etc., M protein (mainly IgG type with elevation) and The diagnosis or confirmation of CIDP needs to
darkening skin. Systemic multi-system examination is be differential with other factors leading to chronic
needed for diagnosis; (2) MMN: it is a kind of motor polyneuropathy, such as metabolism, drug interaction,
specific asymmetrical chronic acquired dymelinating toxicology, and connective tissue diseases. For
polyneuropathy (CADP), which usually onset in adult adolescent patients, it is essential to eliminate the
male. MMN is onset with asymmetrical numbness opportunities of different hereditary demyelinating
of distal of upper extremities, which spreads to peripheral neuropathy, such as Charcot-Marie-Tooth
the proximal ends of upper extremities and lower. disease.
However, in some cases, onset could be initiated
at lower extremities. Most of the affected muscle TREATMENT
distributes as in a mononeuropathy. However,
electrophysiology indicates multifocal distribution of Immunotherapy
motor transmission blockade. MMN does not highly (1) Glucocorticoid: this is usually the primary mediation
differ from classical CIDP, but it is similar to MADSAM. for CIDP. For instance, intravenous administration
The major difference of these two conditions is of methylprednisolone (500-1,000 mg/day) for 3 to
as followed: MMN is not with any sensory related 5 consecutive days will be prescribed, followed by
symptoms but anti-ganglioside IgM, GM , is found a gradual reduction of dose or oral administration of
1
in serum. Intravenous immunoglobulin, IVIg, or prednisone (morning) for 1-2 months, which will also be
cyclophosphamide (CTX), but not glucocorticoid, reduced in dose accordingly. Alternatively, intravenous
could improve the symptoms. For MADSAM, there is administration of dexamethasone (10-20 mg/day) for
not anti-ganglioside IgM in serum but glucocorticoid 7 consecutive days will also be prescribed, shifted to
is efficacious in treatment; (3) cancers causing 1 mg/kg prednisone (morning). After 1 to 2 months of
peripheral neuropathy (Paraneoplastic syndromes): treatment, dose could be reduced or changed to oral
it is a non-metastatic peripheral neuropathy but administration of prednisone (1 mg/kg for morning)
it could onset before, synchronized and after the for 1 to 2 months followed by a gradual reduction of
carcinogenesis. It is found usually in patients at dose. The mentioned oral therapy by prednisone
middle or elder ages, which a progressive disorder could be reduced to 5-10 mg and last for more than 6
not treatable by glucocorticoid. This can be diagnosed months; afterward, the treatment could be terminated
by comprehensive examination and identification according to the conditions. During the glucocorticoid
of tumor; (4) MGUS associated with peripheral therapy, supplementary calcium and potassium, and
neuropathy: CADP could be seen in MGUS with gastric mucosa protected may need to be considered;
unknown etiological reasons, mainly IgM type. Unlike (2) intravenous immunoglobulin (IVIg): the treatment
to CIDP, MGUS associated peripheral neuropathy course consists of intravenous perfusion of 400 mg/kg
demonstrated more sensory symptoms than motor and Ig for 3 to 5 days, which is repeated once in a month
more significant at the distal extremities. About 50% for 3 months. If necessary, the treatment could be
of patients are with positive result in the test of anti- extended to months; (3) plasma exchange: for CIDP
myelin-associated glycoprotein antibody. This disorder patients who are applicable with, plasma could be
cannot be efficiently treated by immunosuppressive or exchanged for 30 mL/kg each time. The course, once
immunomodulatory agents, but rituximab is potent in a month only, consists of 3 to 5 exchanges, each of
the treatment. In some cases, the MGUS in IgG or which separated by 2 to 3 days. Cautiously, plasma
IgA types are associated with CADP, which is with exchange is only allowed 3 weeks after the treatment
similar clinical symptoms and electrophysiology. The of IVIg; (4) other immunosuppressants: when the
key diagnosis highly relies on the positive finding of M aforementioned approaches failed, or in condition
protein in immunofixation electrophoresis; (5) refsum of hormonal dependence or intolerance, other
disease: it is a genetic problem of motor and sensory immunosuppressants, such as azathioprine, CTX,
peripheral neuropathy caused by the phytanic oxidase cyclosporine and methotrexate, could be considered.
deficiency, which leads to deposition of phytanic acid. Azathioprine, clinically common for CIDP, could be
This is usually found in adolescents and adults with prescribed in 1-3 mg/kg, orally administered in 2-3
symptoms as peripheral neuropathy, ataxia, deafness, times, with also the monitor of hepatic and renal
retinitis pigmentosa, scaling skins, etc. There is also functions and blood biochemistry. [5,7-9]
22 Neuroimmunology and Neuroinflammation ¦ Volume 4 ¦ February 20, 2017