Excellent GBS, CIDP and variants reference

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Excellent GBS, CIDP and variants reference

Post by RitaA » Wed 22 Apr 2015 23:10

For anyone interested in learning more about Guillain-Barré Syndrome and related disorders, here is an absolutely amazing resource that I just came across.

https://www.gbs-cidp.org/wp-content/upl ... iewENG.pdf
Guillain-Barré Syndrome, CIDP and Variants

Serving patients with GBS, CIDP and variants with support, education, research and advocacy
Not only does it explain GBS, CIDP and variants in easy-to-understand terms, there are also some really great illustrations contained in this document.

Here's just one of many paragraphs that I found especially interesting for a variety of reasons:
It is of interest that literally millions of people are exposed to events such as infections, surgery, and vaccines that have been identified as triggering agents for GBS, yet only a very small number of the people develop GBS. Why only certain people develop GBS is unclear. Might they have some unique genetic predisposition? Since it is rare for more than one member of a family to develop GBS, genetic factors likely do not play a significant role. Yet some research indicates that genetic factors do correlate with severity of disease (Geleijns et al, 2005; Geleijns et al, 2006; van Sorge et al, 2005). Indeed, GBS and variants may reflect a unique interaction between certain strains of an infectious agent (e.g., C. jejuni, Penner strains 0:19 and 0:41) and the genetically determined immune system composition of the patient (Hughes et al, 1999). Future research will hopefully improve our understanding of how and why GBS occurs.

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Re: Excellent GBS, CIDP and variants reference

Post by ChronicLyme19 » Thu 23 Apr 2015 14:52

Thanks for posting this, I definitely want to read through this. My mother was diagnosed with an a-typical GB when I was very little that almost killed her and left her with some severe permanent neurological damage.

From "What is GBS: Historical Background and Clinical Features" section:
The demonstration by Quinke in 1891 of spinal fluid removal by passing a needle into the low back paved the way for three Parisian physicians, Georges Guillain, Jean Alexander Barré and Andre Strohl to report in 1916 the characteristic abnormality found in GBS of increased spinal fluid protein with a normal cell count. Neurologists call this cytoalbuminologic dissociation, meaning that the fluid contains a normal amount of cells, indicated by use of the prefix ‘cyto’ for cells, but the amount of protein or albumin in the fluid is abnormally elevated (Guillain, 1916). Studies have shown that this disorder can affect any of the peripheral nerves mentioned above: motor, sensory and autonomic nerves. GBS is usually self limited; that is, recovery starts spontaneously
So these two things my mom didn't have. Her protein level in her spinal fluid was normal, the nerve damage was particularly long lasting and severe, and it was not self limiting. What eventually stopped it was a few round of plasmapheresis. I need to ask her again if the direction of her nerve damage progressed upwards or downwards.
Half of what you are taught is incorrect, but which half? What if there's another half missing?

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Re: Excellent GBS, CIDP and variants reference

Post by RitaA » Thu 23 Apr 2015 19:28

I can't help but wonder if your mother's atypical GBS was actually CIDP or some other variant. A lot has probably been learned since then and, as you will see from the following, there is quite a bit of variation even within "classic" GBS:

Acute Immune Polyneuropathies
"Classic" Guillain-Barré Syndrome
http://neuromuscular.wustl.edu/antibody ... .html#cidp
Chronic Immune Demyelinating Polyneuropathy (CIDP)
Prognosis worse with
  • Progressive course
    CNS involvement
    Pathology: More Axonal loss
Here's a bit more information regarding prognosis, as well as a reference to Lyme disease. Keep in mind this 2004 article may not reflect the latest GBS/CIDP research:

Guillain-Barré Syndrome

DANA L. NEWSWANGER, LCDR, MC, USNR, National Naval Medical Center, Bethesda, Maryland
CHARLES R. WARREN, LCDR, MC, USNR, Naval Hospital Jacksonville, Jacksonville, Florida

Am Fam Physician. 2004 May 15;69(10):2405-2410d
Characteristic CSF findings consist of elevated protein (higher than 0.55 g per dL [5.5 g per L]) without pleocytosis (abnormal number of cells in the CSF). CSF is often normal when symptoms have been present for less than 48 hours, but by the end of one week the level of CSF protein is elevated. An increased white blood cell count in CSF (10 to 100 per mm3 [10 to 100 ×106 per L]) in a patient with typical GBS symptoms increases the possibility of Lyme disease, neoplasia, human immunodeficiency virus (HIV) infection, sarcoid meningitis, or other diseases.7,11

GBS has five distinct subtypes: acute inflammatory demyelinating polyradiculoneuropathy (AIDP), acute motor axonal neuropathy (AMAN), acute motor sensory axonal neuropathy (AMSAN), Miller Fisher syndrome, and acute panautonomic neuropathy. The axonal forms are generally thought to have poorer prognoses, indicating a need for determining the specific subtype. These subtypes are distinguished electrodiagnostically and pathologically (Table 3).2,12–16

Prognosis and Recovery

Approximately 85 percent of patients with GBS achieve a full and functional recovery within six to 12 months. Recovery is maximal by 18 months past onset. However, some patients have persistent minor weakness, areflexia, and paresthesia. Approximately 7 to 15 percent of patients have permanent neurologic sequelae including bilateral footdrop, intrinsic hand muscle wasting, sensory ataxia, and dysesthesia.7 The mortality rate is less than 5 percent in tertiary care centers with a team of medical professionals who are familiar with GBS management.2 Causes of death include adult respiratory distress syndrome, sepsis, pulmonary emboli, and cardiac arrest.34

Several factors during the acute phase of illness predict subsequent poor recovery. These factors include age older than 60 years; severe, rapidly progressive disease; and low nerve conduction amplitudes on distal stimulation, which suggests axonal loss.6 In addition, prolonged mechanical ventilation for more than one month and preexisting pulmonary disease predict a poor outcome. In general, a poor long-term prognosis is directly related to the severity of the acute episode and delay in onset of specific treatment.
And last, but not least, I found the following snippets on a GBS/CIDP forum. As most of us have learned, there are almost always exceptions to the rules set out in medical textbooks and other reference material written and used by doctors.

https://forum.gbs-cidp.org/topic/has-an ... nal-fluid/
I had my total Protein tested and it was 21 mg/dl. Have the asymetrical sensory and motor decrement. I was probable diagnosed with Multifocal form of CIDP. With this form, a percentage of patients do not have elevated protein

It’s normal to have regular protein levels with CIDP
I have had 2 normal protein lps and 1 abnormally high lp result. 5% of all gbs/cidp have normal lp results.

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Re: Excellent GBS, CIDP and variants reference

Post by RitaA » Thu 30 Apr 2015 7:57

Hi CL19,

I thought you might also be interested in this article:

Autoimmune Diseases Volume 2014 (2014), Article ID 201657, 11 pageshttp://dx.doi.org/10.1155/2014/201657

Review Article

Treatment of Chronic Inflammatory Demyelinating Polyneuropathy: From Molecular Bases to Practical Considerations

Paolo Ripellino,1 Thomas Fleetwood,1 Roberto Cantello,1 and Cristoforo Comi1,2,3

1 Department of Neurology, A.O.U. Maggiore di Novara, Amedeo Avogadro University, Corso Mazzini 18, 28100 Novara, Italy
2 Department of Translational Medicine, Section of Neurology and Interdisciplinary Research Centre of Autoimmune Diseases (IRCAD), Via Solaroli 17, 28100 Novara, Italy
3 Department of Translational Medicine, Amedeo Avogadro University, Via Solaroli 17, 28100 Novara, Italy

Received 29 September 2013; Accepted 13 November 2013; Published 14 January 2014

Academic Editor: Umberto Dianzani

Copyright © 2014 Paolo Ripellino et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.


Chronic inflammatory demyelinating polyneuropathy (CIDP) is an autoimmune disease of the peripheral nervous system, in which both cellular and humoral immune responses are involved. The disease is clinically heterogeneous with some patients displaying pure motor form and others also showing a variable degree of sensory dysfunction; disease evolution may also differ from patient to patient, since monophasic, progressive, and relapsing forms are reported. Underlying such clinical variability there is probably a broad spectrum of molecular dysfunctions that are and will be the target of therapeutic strategies. In this review we first explore the biological bases of current treatments and subsequently we focus on the practical management that must also take into account pharmacoeconomic issues.


CIDP is defined by a slow clinical deterioration that reaches its maximum after more than 8 weeks, differently from GBS, which is an acute and self-limiting disease. That aside, there are many similarities between these two conditions, which may even be variants of the same disease spectrum, with CIDP being the result of prolonged survival of activated T cells, not undergoing apoptosis due to a defective Fas pathway function [18–20], and GBS characterized by a self-limitation likely related to a preserved function of such apoptotic mechanism. In line with this concept, the finding that corticosteroids are effective in CIDP and not in GBS would be related to the known effect of these drugs in restoring T cell apoptosis.


Currently available treatments for CIDP are corticosteroids, immune globulin, plasma exchange (PE), and chronic immunosuppressive agents [21, 25].


4. Conclusions

CIDP is a rare but treatable disease. Clinical experience indicates that about 70% of patients will respond to immunomodulation; there are patients responding to steroids, whereas others, especially with pure motor CIDP, will benefit more from IVIg or PE. It is becoming evident that CIDP is not a uniform disease but includes several variants which might have different response profiles.
I just came across the following tweet from a few hours ago, with the hashtags #LymeDisease and #Neurological:
My wife Jen has Chronic Inflammatory Polyneuropathy Disease from a tick.

CIP (chronic inflammatory polyneuropathy disease) is apparently synonymous with CIDP.

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Re: Excellent GBS, CIDP and variants reference

Post by dlf » Mon 15 Aug 2016 14:33

I recently noticed two more articles that would be appropriate to add to this thread:

Guillain-Barré syndrome
Willison, Hugh J et al.
The Lancet , Volume 388 , Issue 10045 , 717 - 727
Published Online: 29 February 2016

http://www.thelancet.com/journals/lance ... 1/fulltext


Guillain-Barré syndrome is the most common and most severe acute paralytic neuropathy, with about 100 000 people developing the disorder every year worldwide. Under the umbrella term of Guillain-Barré syndrome are several recognisable variants with distinct clinical and pathological features. The severe, generalised manifestation of Guillain-Barré syndrome with respiratory failure affects 20–30% of cases. Treatment with intravenous immunoglobulin or plasma exchange is the optimal management approach, alongside supportive care. Understanding of the infectious triggers and immunological and pathological mechanisms has advanced substantially in the past 10 years, and is guiding clinical trials investigating new treatments. Investigators of large, worldwide, collaborative studies of the spectrum of Guillain-Barré syndrome are accruing data for clinical and biological databases to inform the development of outcome predictors and disease biomarkers. Such studies are transforming the clinical and scientific landscape of acute autoimmune neuropathies.

Mimics and chameleons in Guillain–Barré and Miller Fisher syndromes

Benjamin R Wakerley1, Nobuhiro Yuki2
1Department of Neurology, Gloucestershire Royal Hospital, Gloucester, UK
2Departments of Medicine and Physiology, Yon Loo Lin School of Medicine, National University of Singapore, Singapore
Accepted 25 August 2014
Published Online First 19 September 2014

Guillain–Barré syndrome (GBS) and its variant, Miller Fisher syndrome (MFS) have several subtypes, together forming a continuous spectrum of discrete and overlapping syndromes. Such is the heterogeneity within this spectrum that many physicians may be surprised to learn that these disorders are related pathophysiologically, and therefore share certain clinical features. These include history of antecedent infection, monophasic disease course and symmetrical cranial or limb weakness. The presence of cerebrospinal fluid albuminocytological dissociation (raised protein, normal cell count), antiganglioside antibodies and neurophysiological evidence of axonal or demyelinating neuropathy also support a diagnosis in many cases, but should not be relied upon. Mimics of GBS and MFS can broadly be divided into those presenting with symmetrical limb weakness and those presenting with brainstem signs. MFS and the pharyngeal-cervical-brachial variant of GBS are frequently mistaken for brainstem stroke, botulism or myasthenia gravis, whereas Bickerstaff's brainstem encephalitis is often diagnosed as Wernicke's encephalopathy. Chameleons or atypical presentations of GBS-related disorders include: paraparetic GBS, bifacial weakness with paraesthesias, acute ataxic neuropathy, acute ophthalmoparesis, acute ptosis and acute mydriasis. Many neurologists may also not be aware that deep tendon reflexes remain present or may even appear brisk in up to 10% of patients with GBS. Correct diagnosis of GBS-related disorders helps to avoid unnecessary investigations and allows early immunotherapy if appropriate.

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