10 things we now know about CIDP associated with anti NF155 antibodies

Chronic inflammatory demyelinating polyneuropathy (CIDP) is a neurological disorder which causes loss of the fatty myelin covering of large nerves (demyelination). This slows down the speed at which the nerves can transmit electrical impulses. People with CIDP develop weakness and sensory disturbances, but not always in equal measure. CIDP is a pain for the afflicted, and a veritable nightmare for the neurologist.

 

By AjimonthomasOwn work, CC BY-SA 4.0, Link

The diagnostic process for CIDP includes some rather uncomfortable tests such as nerve conduction studies and lumbar puncture (spinal tap). CIDP is however a most rewarding disease to treat because many people respond to immune treatments such as steroids, intravenous immunoglobulins (IVIG), or plasma exchange (PE).

PRED SOV 5. Leo Reynolds on Flikr. https://www.flickr.com/photos/lwr/3300474346

The diagnosis of CIDP is however not straightforward. The results of the tests are not always clearcut, and a lot of sifting and sorting goes into nailing the diagnosis. And even when the diagnosis is eventually made, there is a very long list of potential causes of CIDP which often require treatment on their own merit. Worryingly, some of these conditions make the treatment of CIDP difficult. And this is where IgG antibodies play a nasty role in CIDP.

By Database Center for Life Science (DBCLS), CC BY 3.0, Link

Neurologists are now recognising that a subset of people with CIDP have IgG4 antibodies which greatly influence the clinical presentation and the treatment of CIDP. Anti-contactin antibody is one such antibody, but by far the most important is anti-neurofascin 155 (NF155). What do we know about this antibody? How does it influence the course of CIDP? To answer these questions, below are 10 important things we now know about CIDP associated with anti-NF155.

By BruceBlausOwn work, CC BY-SA 4.0, Link

1. Anti-NF155 is an antibody to paranodal structures

2. The antibody is present in 7-14% of people with CIDP

3. CIDP with anti-NF155 usually affects young subjects

4. Anti NF155 antibody CIDP is usually severe

5. Anti NF155 may cause central nervous system inflammation 

6. It causes a very high protein level in the spinal fluid

7. It causes very severe changes on nerve conduction studies

8. It responds poorly to intravenous immunoglobulins (IVIg)

9. It may respond to steroids and plasma exchange

10. Treatment-resistant cases may respond to Rituximab
By Oguenther at de.wikipediaOwn work mit Jmol auf Basis RCSB PDB: 2OSL​., Public Domain, Link

________________________________________________________________________

Why not check out everything CIDP on Neurochecklists:

Should people with myotonic dystrophy be counselled on the risk of cancer?

A population-based survey of risk for cancer in individuals diagnosed with myotonic dystrophy Abbott D, Johnson NE, Cannon-Albright LA Muscle Nerve 2016; 54:783-785. Abstract Introduction: The risk of cancer in patients diagnosed with myotonic dystrophy (DM) is reported for the homogeneous Utah population. Methods: Clinical data accessed from the largest Utah healthcare providers have been […]

via Should people with myotonic dystrophy be counselled on the risk of cancer? — Neurochecklists Updates

What factors predict the development of MS after the first attack of inflammation?

Multiple biomarkers improve the prediction of multiple sclerosis in clinically isolated syndromes Martinelli V, Dalla Costa G, Messina MJ, et al. Acta Neurol Scand 2017; 136:454-461. Abstract Objectives: Since its introduction, MRI had a major impact on the early and more precise diagnosis of multiple sclerosis (MS), and the 2010 diagnostic criteria even allow a […]

via What factors predict the development of MS after the first attack of inflammation? — Neurochecklists Updates

The innovative treatments relieving the burden of people with multiple sclerosis

The drug armoury for multiple sclerosis (MS) is ever-expanding. This topic has been extensively covered by our sister blog, The Neurology Lounge, in the blog posts titled Which are the remarkable drugs which have transformed the treatment of MS, Multiple sclerosis treatment: new kids on the block, The emerging progress from the world of MS, and How good is […]

via The innovative treatments relieving the burden of people with multiple sclerosis — Neurochecklists Updates

The 7 most ruthless bacterial infections of the nervous system

This is a follow up to our previous post, the 7 most devastating viral neurological infections. The list of bacteria that invade the nervous system is endless, but some stand out because of the fear they evoke, and the peril they pose. Here then are the 7 most horrifying bacterial infections that threaten the nervous […]

via The 7 most ruthless bacterial infections of the nervous system — Neurochecklists Updates

Does thrombolysis add any value to mechanical thrombectomy after stroke?

Mechanical thrombectomy outcomes with and without intravenous thrombolysis in stroke patients: a meta-analysis. Mistry EA, Mistry AM, Nakawah MO, et al. Stroke 2017; 48:2450-2456. Abstract Background: Whether prior intravenous thrombolysis provides any additional benefits to the patients undergoing mechanical thrombectomy for large vessel, acute ischemic stroke remains unclear. Methods: We conducted a meta-analysis of 13 studies obtained through PubMed and EMBASE database searches to determine whether functional […]

via Does thrombolysis add any value to mechanical thrombectomy after stroke? — Neurochecklists Updates

Is head injury a risk factor for multiple sclerosis?

Concussion in adolescence and risk of multiple sclerosis Montgomery S, Hiyoshi A, Burkill S, Alfredsson L, Bahmanyar S, Olsson T. Ann Neurol 2017; 82:554-561 Abstract Objective: To assess whether concussion in childhood or adolescence is associated with subsequent multiple sclerosis (MS) risk. Previous research suggests an association, but methodological limitations included retrospective data collection and […]

via Is head injury a risk factor for multiple sclerosis? — Neurochecklists Updates