Tag: Chronic inflammatory demyelinating polyneuropathy

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.

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).

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.

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.

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

________________________________________________________________________

Why not check out everything CIDP on Neurochecklists:

• Clinical features of CIDP
• Conditions associated with CIDP
• Investigations of CIDP
• Treatment of CIDP
• Lewis Sumner syndrome (MADSAM)
• Distal acquired demyelinating symmetric neuropathy (DADS)