Institute of Genomic Medicine and Rare Disorders
Semmelweis University
Head of department: Prof. Dr. Mária Judit Molnár
25-29. Tömő str., Budapest, Hungary, H-1083
Tel:+36-1-459-
1483
Email:
[email protected] mmelweis-univ.hu
Immun mediated neuropathies
Next supervion expected: 2017.06.01.
Definition and background
Immun mediated neuropathies represent a broad spectra of periferial nerve lesions that has common
entities like good response to immun-suppressive agents and mostly demyelinating type of nerve lesions.
They can be classified depending onset, time course, distribution, type of nerve fibers involved, etc. One of
the most important aspect is the onset. Onset is acut in Guillaine-Barré syndrome (GBS) and subacut or
chronic in CIDP. In GBS after a 4-6 weeks progression steady state and gradual improvement can be
observed. While in CIDP the onset in most of the cases subacut and the time course is slowly progressive
over mounths. Both (GBS and CIDP) have different subtype regarding the nerve fibers predominantly
involved and the distribution of nerve lesions throughout the body (Table 1.). Different subtypes have
different prognostic perspectives and in certain cases different treatment approach. In all immun
mediated polyneuropathy the diagnose lies upon the triad of clinical presentation, electrophysiological
studies and CSF analysis. Other causes of etiology have to be excluded.
Table 1. Main subtypes of acut and chronic immun mediated
polyneuropathies
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Institute of Genomic Medicine and Rare Disorders
Semmelweis University
Head of department: Prof. Dr. Mária Judit Molnár
25-29. Tömő str., Budapest, Hungary, H-1083
Tel:+36-1-459-
1483
Email:
[email protected] mmelweis-univ.hu
Acut immun mediated neuropathies
Guillain-Barré syndrome is an acut, immun mediated, inflammatory multifocal polyneuropathy. Typically it
manifests with an ascending pattern beginning in the lower extremities. Main symptomes are muscle
weakness, sensory deficits due to large myelinated fibers lesions, pain common but autonomic
disturbances are minor and loss of deep tendon reflexes. Miller-Fisher subtype characterized by
ophtalmoplegia, ataxia and loss of deep tendon reflexes. Electrophysiological studies reveal demyelinating
(or in certain subgroups axonal) lesions. In the cerebrospinal fluid there is cytoalbuminaer dissociation
(elevated protein level without cells).
2
Institute of Genomic Medicine and Rare Disorders
Semmelweis University
Head of department: Prof. Dr. Mária Judit Molnár
25-29. Tömő str., Budapest, Hungary, H-1083
Tel:+36-1-459-
1483
Email:
[email protected] mmelweis-univ.hu
Chronic immun mediated neuropathies
Chronic inflammatory demyelinating polyneuropathy (CIDP) is a chronic, acquired, immune-mediated
condition affecting the peripheral nervous system. The classic form of the disorder is characterized by: (1)
progressive limb weakness, usually with a predilection for proximal muscles, sensory loss, and areflexia
with a relapsing or progressive course; (2) electrophysiological features of demyelination, including
prolonged distal motor and F-wave latencies, reduced conduction velocities, and conduction block and
temporal dispersion; (3) laboratory features of albumino-cytological dissociation in the cerebrospinal fluid.
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Institute of Genomic Medicine and Rare Disorders
Semmelweis University
Head of department: Prof. Dr. Mária Judit Molnár
25-29. Tömő str., Budapest, Hungary, H-1083
Tel:+36-1-459-
1483
Email:
[email protected] mmelweis-univ.hu
Mutiplex motor neuropathy (MMN) is a subroup of CIDP and it is of particular importance because of only
reacting to IVIG treatment. In MMN there is an asymmetrical involvment of only motor nerve fibers.
References
Krónikus immunmediált neuropathiák. In: Csépány Tünde és Illés Zsolt Klinikai Neuroimmunológia. 2.
kiadás Professional Publishing Hungary Kft., Medical Tribune Scientific Divízió; 2014.p. 181-198.
Kenneth C. Gorson An update on the management of chronic inflammatory demyelinating polyneuropathy
Ther Adv Neurol Disord. 2012 Nov; 5(6): 359–373. doi:
10.1177/1756285612457215
Helmar C. Lehmann et al. Pathogenesis and Treatment of Immune-Mediated Neuropathies Ther Adv
Neurol Disord. 2009 Jul; 2(4): 261–281. doi: 10.1177/1756285609104792
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