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When you have a rare disease, you may find you have more questions than answers.
That’s certainly true for many people with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). CIDP is an autoimmune condition that affects the protective sheath that lines the nerves in the arms and legs.
The condition tends to elude diagnosis, especially when someone has one of the less common types, says Divyanshu Dubey, M.B.B.S., a neurologist specializing in CIDP treatment at Mayo Clinic.
“Getting an accurate diagnosis is crucial,” says Dr. Dubey.
That’s because the earlier someone is diagnosed, the more likely treatment is to be effective. In addition, not all types of CIDP respond to the same treatments. In other words, before you can get treated and start feeling better, you first need to know your CIDP type.
Typical CIDP
Sometimes referred to as classical CIDP, this more common form of CIDP accounts for up to 50% to 60% of all cases. It affects both sides of the body equally, leading to weakness and numbness in the arms, hands, legs and feet. Symptoms tend to gradually worsen over weeks or months.
If you have this form of CIDP, you may struggle to:
- Climb or descend stairs.
- Rise from a seated position.
- Lift objects overhead.
- Walk without tripping or swaying.
- Complete fine motor tasks like buttoning or opening jars.
This type of CIDP responds well to intravenous immunoglobulin (IVIg). These antibodies help to reset the immune system, training it to work correctly. Eventually, the immune system stops attacking the nerves, allowing them to heal. When treated early enough with IVIG, many people with the typical form of CIDP go into remission. Not only do most of their symptoms resolve, but they also can eventually stop undergoing treatment.
Atypical CIDP
Sometimes called “variants,” atypical types of CIDP “look a bit different and need to be treated a bit differently,” says Dr. Dubey.
The standard tests used to diagnose typical CIDP can sometimes miss a few of the atypical variants, says Dr. Dubey.
Therefore, in addition to a test that measures your nerve function, your healthcare professional also may recommend that you undergo blood tests. These tests, developed by Mayo Clinic Laboratories, can help identify specific antibodies involved in some of the atypical CIDP variants. Atypical variants include:
- Neurofascin-155 (NF155).
- Contactin-1 (CNTN1).
- Myelin-associated glycoprotein (MAG).
If tests reveal an atypical variant, it will affect your treatment plan.
Multifocal CIDP
Also called Lewis-Sumner syndrome, this CIDP subtype accounts for 5% to 10% of CIDP cases. Unlike the typical form of CIDP:
- Only one arm or leg is affected.
- People experience nerve pain in addition to numbness.
Focal CIDP
This rare form of CIDP affects just one limb or nerve on one side of the body.
Pure motor CIDP
Also a rare form of CIDP, this variant involves the motor nerves used during movement. People may struggle with movement or coordination. However, because this variant spares the sensory nerve fibers, people with pure motor CIDP often do not experience numbness, tingling or pain.
Pure sensory CIDP
With this form of CIDP, the immune system attacks only the sensory nerve fibers. As the nerve fibers erode, people notice tingling and uncomfortable sensations. Unlike with the pure motor CIDP variant mentioned above, the motor nerve fibers are mostly not affected with pure sensory CIDP. However, because people can’t sense where their limbs are in space, they often struggle with movement and daily tasks.
Distal and sensory predominant CIDP (DADS)
This subtype is sometimes called distal-acquired demyelinating symmetric neuropathy (DADS). It progresses more slowly than other types, affecting the legs first. Symptoms in the arms appear a year later. In addition to affecting movement, this CIDP variant also may lead to cramping, fatigue and tremors. Nearly 50% of the DADS cases are positive for the MAG-IgM antibodies mentioned earlier.
Autoimmune nodopathy
If your bloodwork reveals you have the neurofascin-155 (NF155) or contactin-1 (CNTN1) antibodies, you likely have this variant. Autoimmune nodopathies affect nodes or gaps in myelin tissue. Autoimmune nodopathies aren’t considered true CIDPs. However, autoimmune nodopathies share clinical and diagnostic features that overlap with CIDP.
Chronic immune sensory polyradiculopathy (CISP)
This type of demyelinating neuropathy primarily affects the sensory roots of the nerves. If you have this autoimmune disorder, you’ll feel off balance when walking as well as experience sensory symptoms like tingling or numbness. If you have this condition, common diagnostic tests like nerve conduction studies may not indicate nerve damage. To receive an accurate diagnosis, you’ll need more-specialized testing.
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