What are the First Symptoms of CIDP?

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a rare autoimmune disease that can cause progressive or recurrent symptoms affecting the peripheral nervous system. 

Catching CIDP early is important, because treating the disease immediately can play a key role in limiting or slowing its progression. 

In fact, the prognosis for CIDP is usually good. According to Mayo Clinic, a study that followed up on people with this disease found that with appropriate treatment, 90% were living independently after a decade, and had recovered well.

In this post, we will tell you about the first symptoms of CIDP, as well as what you should know about diagnosing and treating this disorder.

CIDP is a rare autoimmune disorder, wherein the immune system attacks the myelin sheaths around your nerves. This can lead to peripheral nerve damage and symptoms. 

CIDP is often compared to Guillain-Barre syndrome, which is an acute disorder. While CIDP is usually chronic, lasting many years, there is a monophasic form that only lasts 1-3 years.

Classic early symptoms of CIDP include tingling and numbness in the arms and legs. You may notice that even a pinprick does not produce a sensation. 

Over time, your arms and legs may become weaker. 

The tingling, numbness, and weakness caused by CIDP can lead to problems with balance, reflexes, and walking. Foot drop is also possible in CIDP, which can further affect your gait. We will discuss foot drop in more detail later on in this guide.

Chronic inflammatory demyelinating polyneuropathy (CIDP) can be classified into different subtypes based on clinical presentation, electrophysiological findings, and response to treatment. The two main categories are typical CIDP and atypical CIDP.

Typical CIDP, also known as classic CIDP, is the most common form, accounting for around 50-60% of cases. It is characterized by:

• Symmetric weakness and sensory deficits in both proximal and distal muscles of the arms and legs
• Gradually progressive or relapsing-remitting course
• Absent or reduced deep tendon reflexes
• Elevated cerebrospinal fluid (CSF) protein levels with normal white blood cell count
• Demyelinating features on nerve conduction studies
• Good response to immunomodulatory treatments like intravenous immunoglobulin (IVIg), corticosteroids, or plasma exchange

Patients with typical CIDP usually present with a chronic progressive or relapsing course over 2 months or more. Sensory symptoms like numbness, tingling, and impaired proprioception often accompany the weakness.

Atypical CIDP variants differ from typical CIDP in their clinical presentation, electrophysiological findings, or treatment response. Around 40-50% of CIDP cases are atypical forms. The main atypical CIDP subtypes include:

1. Multifocal Acquired Demyelinating Sensory and Motor (MADSAM) neuropathy or Lewis-Sumner Syndrome

• Asymmetric, multifocal weakness and sensory deficits
• Affects distal limbs more than proximal
• Conduction block is a prominent feature on nerve conduction studies
• May be confused with multifocal motor neuropathy (MMN)
• Usually responsive to IVIg

1. Distal Acquired Demyelinating Symmetric (DADS) neuropathy

• Symmetrical sensory and motor deficits predominantly in distal limbs
• Spares proximal muscles until late stages
• Slowly progressive course
• May be confused with Chronic Axonal Polyneuropathy
• Response to treatment is often less robust compared to typical CIDP

1. Sensory Predominant CIDP

• Presents with sensory deficits and sensory ataxia with minimal or no weakness
• Large fiber sensory involvement is predominant
• May be confused with sensory neuronopathies or ganglionopathies
• Variable response to immunomodulatory treatment

1. Focal CIDP

• Presents as an asymmetric neuropathy involving a single limb or nerve trunk
• May progress to diffuse CIDP over time
• Electrophysiology and CSF are compatible with CIDP
• Treatment is similar to typical CIDP

Recognizing these different forms of CIDP is important for accurate diagnosis, appropriate treatment selection, and prognosis. Atypical CIDP variants may have a more indolent course and can be challenging to diagnose due to their similarities with other neuropathies. However, most forms are still responsive to standard CIDP treatments, although response may be variable or incomplete in some atypical cases compared to typical CIDP.

If you do not receive treatment for CIDP, your nerves may become damaged due to the loss of the protect sheaths around them.

This damage may become permanent in some cases, which could lead to lasting symptoms even after treatment begins.

Some patients who do not receive treatment eventually lose their ability to walk.

It is also possible for depression, anxiety, and memory issues to co-occur with CIDP. 

Long term damage of this nature can typically be prevented through rapid and suitable treatment. 

A number of different tests can be used to diagnose CIDP. Here are some of the diagnostic testing options your doctor might recommend. 

• Spinal tap: Also called a lumbar puncture, this procedure uses extraction of cerebrospinal fluid (CSF) to check for abnormal protein levels within the CSF.
• Nerve conduction test and needle electromyography: Mild electrical currents are used to see how responsive and functional your muscles and nerves are. 
• Blood tests: These may be done to look for certain antibodies and also to rule out other possibilities (see the section below). 
• MRI: This test can be used to check for nerve root inflammation. 
• Nerve biopsies: This is one of the least common tests used to diagnose CIDP; it is used more when other measures are not diagnostic and when the results of it will affect treatment decisions.

There are some other conditions that may be mistaken for CIDP and vice versa,o, it is important to get a correct diagnosis. Here are a few examples:

• Guillain-Barré syndrome (GBS)
• Multifocal motor neuropathy (MMN)
• Lewis-Sumner syndrome
• Charcot-Marie-Tooth disease
• Multiple sclerosis (MS)
• Peripheral neuropathy
• Other types of neuropathy

Please note that this is not a comprehensive list of all possible conditions that could be confused with CIDP. 

There are a variety of treatments available for CIDP. Here are some of the treatment options you can consider. 

• Plasma Exchange: A plasma exchange can be used to filter antibodies out of the blood that are contributing to the damage to the nerves. 
• Corticosteroids: Using corticosteroids can slow the disease’s progression and help curb inflammation. 
• Intravenous Immunoglobulin: This procedure, usually abbreviated as IVIG, suppresses the immune system through the infusion of immunoglobulin. 
• Stem Cell Therapy: Healthy stem cells can be used to change how your immune system functions. 
• Physical Therapy: If the condition has caused you to lose strength, balance and coordination, you may need physical therapy. 
• Immunosuppressants: A variety of other immunosuppressant medications may be used to treat CIDP.

You can discuss these and other treatment options with your doctor, and work together to create a treatment plan that is right for your individual condition and needs. 

If you believe you may have CIDP, it is important to seek a diagnosis as early as possible. That way, you can begin treatment right away. This can slow the progression of the disease.

According to Johns Hopkins Medicine, if you do not treat CIDP, there is a 1 in 3 chance of needing a wheelchair.

So, to give yourself the best chance at recovery and prevention of further damage, you need to act quickly. 

In some cases, your doctor may recommend that you begin physical therapy (PT). This may be necessary if you have experienced a reduction in strength, balance, coordination, or range of motion. It may also be needed if you have begun walking with an unnatural gait.

CIDP patients may sometimes experience a condition called foot drop. If you develop foot drop, you will have a difficult time fully lifting the front parts of your feet when you are walking. This leads to an unnatural gait where your feet slap down on the ground as you walk. It can also cause difficulties pushing down pedals in a car.

Your physical therapist will give you exercises that can help you recover a natural gait. You also will be encouraged to practice walking regularly, which can aid in your recovery.

Trying to walk with CIDP may sound daunting. But most CIDP patients are able to do it. 

You do need to be extra careful when walking with this condition. An unnatural gait is not as healthy as a natural one, and may lead to straining yourself. Both the unusual gait and the numbness could also make it more likely that you will lose stability, and/or trip over an obstacle and fall.

Your doctor may suggest that you try wearing a type of brace called an ankle-foot orthosis (AFO). The brace can improve stability and hold your feet in their ideal positions as you walk, preventing the slapping-down motion that comes with foot drop.

You can also benefit from wearing shoes that are designed for people with foot drop and other conditions that impact gait. 

Cadense Original Adaptive Shoes for men and women are AFO-compatible, so you can wear them comfortably with a brace for foot drop.

Here are some other features of Cadense Original Adaptive Shoes that make them an excellent choice for patients with CIDP:

• These shoes feature patented variable friction technology. That technology lets the wearer “glide” smoothly over uneven surfaces. This can prevent tripping and stumbling due to being unable to fully lift the front parts of the feet. As a result, you can walk more safely with CIDP and foot drop. 
• Ample cushioning provides a soft, comfortable feel and a snug, secure fit, maximizing stability. A wide base also contributes to keeping the feet stable while walking.
• Lightweight, breathable materials keep feet cool and reduce fatigue, allowing the wearer to walk longer distances and even stay comfortable in hot weather.

These shoes have a stylish design, and are available in multiple colors, making it easy to match them to a range of personal styles and preferences.