Page 29 - Read Online

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

cranial nerves abnormalities: less than 10% of patients lower than the lower limit; (C) twenty percent or more
suffer from facial paralysis or opthalmoplegia. In some prolongation of F wave latency than the normal upper
[2]
cases, the cranial nerves innervated bulbar muscles limit. When examining the amplitude of negative phase
are compromised. In even rare cases, there will be wave, which is lower than the lower limit by 20%, the
papilledema; (B) gravis: in most cases of CIDP of F wave latency should be prolonged by 50% or more.
classical type, patients suffer from gravis, which extend In certain cases, there is also absence of F waves;
to both proximal and distal extremities; (C) sensory (D) partial blockade of motor nerve conduction: when
disturbances: most patients also suffer from numbness comparing the proximal and distal end of the normal
of the limbs or occasionally pain. There is sometimes segments of peripheral nerves, the former amplitude of
loss of glove- or sock-like pinprick and deep sensation. negative phase of compound muscle action potential
In severe cases, there is also severe sensory ataxia; (CMAP) decreases by 50% or more. If the reduction
(D) abnormal tendon reflexes: tendon reflexes are is less than 20%, the reliability of this examination is
diminished or even disappeared. In some cases, there not guaranteed; (E) discrete abnormal waveform: the
would be reduction or loss of tendon reflexes in normal duration of CMAP negative phase wave is widened
muscle; (E) autonomic dysfunction: it is expressed for 30% or above. (2) Sensory nerve conduction:
as orthostatic hypotension, sphincter dysfunction, there could be delay of sensory nerve conduction
arrhythmia, etc. and/or decrease of amplitude. (3) Electromyography
with needle electrodes: although it is usually present
Variant CIDP as normal, there could be abnormal spontaneous
(1) Pure motor CIDP: about 10-11%. There is only the potential, prolongation and elevation of amplitude of
numbness without any sensory symptoms; (2) pure potential of motor units, and even loss of motor units
sensory CIDP: about 8-17%. There are only sensory [3]
symptoms, e.g. sensory ataxia, numbness and pain. when there is secondary axonal damage.
However, with the extension of the course, there CSF
may be symptoms of motion expressed; (3) DADS: There are about 80-90% of patients with CSF protein-
numbness and/or sensory impairments limited in
the distal extremities. DADS is onset slower that the cell separation, which is about 0.75-2.00 g/L (in some
classical CIDP, some of which is a category of IgM cases, it can be higher than 2.00 g/L).
monoclonal obulinemia, a subtype of monoclonal Biopsy of sural nerve
gammopathy of unknown significance (MGUS), In the situation that the electrophysiology test result
and associated with peripheral neuropathy. Steroid
therapy does not produce any therapeutic effect. In is not consistent with the clinical symptoms and
contrast, CIDP without IgM monoclonal obulinemia is vasculitic peripheral neuropathy and hereditary
sensitive to steroid therapy; (4) MADSAM: there is limb neuropathy cannot be excluded, biopsy of sural
asymmetrical sensorimotor peripheral neuropathy, nerve is necessary. The main pathological hallmarks
which is clinically similar to multifocal motor neuropathy include segmental demyelination of myelinated nerve
(MMN), but there is also evidence of sensory damages fiber, axonal degeneration, proliferation of Schwann
and no anti-ganglioside GM antibody titer. [4] cell with onion-like formation, mononuclear cell
1 infiltration, etc.
ACCESSARY EXAMINATION
DIAGNOSIS AND DIFFERENTIAL DIAGNOSIS
Electrophysiology
In the motor nerve conduction examination, there Diagnosis
is peripheral nerve demyelination. There is also The current diagnosis of CIDP is in fashion of exclusion.
conduction block at the entrapment site or discrete One can be suspected as CIDP when the following
abnormal waveform, which is useful for diagnosis criteria are fit: (1) chronic progression or remission
for demyelination. Median, ulnar, tibial and common relapse of CIDP associated symptoms over eight
peroneal nerves are commonly diagnosed. Results weeks; (2) numbness of proximal and distal extremities
of electrophysiological tests should be consistent in different degrees in symmetrical manner; however,
with the clinical symptoms. The standardized criteria some are in asymmetrical pattern such as MADSAM.
of electrophysiological diagnosis includes: (1) motor The reduction or loss of tendon reflexes with also
nerve conduction: there is at least one of two testing depth paresthesia; (3) CSF protein-cell separation; (4)
motor nerves with the following abnormalities. (A) Fifty reduction and blockade of peripheral nerve conduction
percent or more prolongation of distal latencies; (B) or discrete abnormal waveform; (5) excluded as other
velocity of motor nerve conduction is 30% or above neuropathies; (6) improved by glucocorticoids. [4,5]
Neuroimmunology and Neuroinflammation ¦ Volume 4 ¦ February 20, 2017 21