What is Chronic Inflammatory Demyelinating Polyneuropathy?

Jul 29

Chronic inflammatory demyelinating polyneuropathy (CIDP) is a rare disorder of the nervous system. In CIDP, the immune system becomes dysregulated and attacks peripheral nerves.

For reasons that remain poorly understood, the immune system mistakenly attacks myelin, a protective insulation that covers nerve fibers. When myelin is damaged or destroyed, communication between the body and the brain becomes impaired, leading to both motor and sensory symptoms.

If left untreated, CIDP can cause permanent damage to the peripheral nervous system.

How Common is Chronic Inflammatory Demyelinating Polyneuropathy?

CIDP is a rare disorder, with an overall prevalence estimated at 1.9 to 7.7 cases per 100,000 people. CIDP can affect anyone, but is most commonly found after the age of 50 and is two times more likely to occur in males than females (28).

What Causes Chronic Inflammatory Demyelinating Polyneuropathy?

Healthy peripheral nerve fibers are wrapped in an insulating sheath known as myelin. This insulation allows electric impulses to travel rapidly and efficiently along the nerve fiber. Myelin also plays an important protective role in maintaining the health of peripheral nerves. When myelin is degraded, nerve fibers are more vulnerable to cell death and degeneration.

CIDP is a demyelinating disorder. It is also an autoimmune condition, in which the immune system mistakenly identifies myelin as foreign to the body and targets it for destruction. CIDP can be triggered by a number of different conditions that promote immune dysregulation. The immune system directly attacks myelin, leading to degeneration and death of peripheral nerve fibers.

Distal symmetric polyneuropathy (DSPN) is the most common neuropathy to occur in diabetes mellitus. However, patients with diabetes can also develop inflammatory neuropathies, the most common and most treatable of which is chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). Whether diabetes is a risk factor for CIDP remains under debate.

What are the Symptoms of Chronic Inflammatory Demyelinating Polyneuropathy?

CIDP can cause gradual weakness and a loss of feeling in your arms and legs. It affects proximal and distal muscles of all extremities, with absent or reduced reflexes in all four limbs. Other symptoms may include numbness and tingling beginning in the toes and fingers, and pervasive fatigue.

CIDP may be relapsing and remitting, but is generally progressive, and can spread to involve multiple parts of the body. Gait and balance can become challenging, and some people living with CIDP may become disabled. Quality of life can become significantly impaired with CIDP (27).

How is Chronic Inflammatory Demyelinating Polyneuropathy Diagnosed?

Nerve conduction studies are considered essential for a definite diagnosis of CIDP. Nerve ultrasound and MRI may be helpful in making a CIDP diagnosis. Whereas typical CIDP is relatively easy to diagnose, atypical variants with distinct phenotypes can be a diagnostic challenge (7).

Diagnosis of CIDP is based on symptoms such as loss of sensation and numbness, or abnormal sensation such as tingling and pain. It may involve loss of reflexes, and limb weakness, with difficulty walking, or with foot drop.

Tests may include nerve conduction and electromyography studies, usually showing a demyelinating neuropathy. Spinal fluid analysis (usually showing elevated protein with normal cell count), and blood and urine tests (to rule out other disorders that may cause neuropathy and to look for unusual proteins) are also frequently employed.

How Does Chronic Inflammatory Demyelinating Polyneuropathy Affect the Brain?

Central sensory changes have also been shown to take place in CIDP, rendering this not necessarily a disorder restricted to the peripheral nervous system. Visual and auditory evoked potential testing measures conduction velocity of information from the eyes and ears through the brain. Both of these have been shown to be delayed in CIDP, with the severity of central damage increasing along with the degree of peripheral nerve impairment (8).

In many cases, the demyelination from CIDP can attack central nervous system myelin as well, similar to what is seen in multiple sclerosis. Periventricular lesions similar to those observed in MS are often seen in the brains of CIDP patients. Demyelinating disorders lead to the formation of sclerotic plaques of tissue that can impair a host of different types of brain function (4).

Demyelinating features of peripheral nerve damage have been shown in 74% of suspected cases central and peripheral inflammatory demyelinating disease, with demyelinating lesions on brain MRI that meet criteria for multiple sclerosis present in 46% of cases. This implies that the current diagnostic criteria for MS and CIDP may not fully encompass the spectrum of possible manifestations of demyelinating disease states (9).

How is Chronic Inflammatory Demyelinating Polyneuropathy Usually Treated?

Early diagnosis before severe nerve damage can take place is the best treatment for CIDP. Unfortunately, most people will not be diagnosed until their symptoms have been present for weeks to months, by which time considerable nerve damage may have already taken place. The immune dysregulation can be halted by the use of corticosteroids, IVIG infusions, plasma exchange and other immune cell treatments. The earlier treatment is initiated, the better the outcome.

IIVIG, corticosteroids, and plasma exchange are effective immunotherapies, but maintenance treatments are often required for years, and medication dosages must be carefully monitored and adjusted as appropriate (7).

How is the NeuroRescue Program Different?

First and foremost, we ensure that all of our patients with peripheral neuropathies are not suffering from CIDP. Early intervention to stop the demyelinating process is critical for recovering function. If we suspect you may be dealing with CIDP, we will immediately refer you for the appropriate diagnostic testing, and coordinate with your care team to ensure that you are started on medication to limit the tissue destruction as rapidly as possible. Research shows that early intervention with immune modulation therapy can be very effective in CIDP (2).

One unfortunate consequence of autoimmunity is that many people may be forced to remain on medication to modulate their immune systems in the long term. These medications are absolutely necessary, however they always come with a side effect profile that may lead to additional negative consequences.

Getting our patients off medication is not our goal in these cases, however if we can find other ways to modulate the immune system their medication burden may be reduced. Specific dietary strategies have been shown to help limit autoimmune demyelination (10), and vitamin D status appears to be critically important in autoimmune polyneuropathies (11). Gut microbiome status and intestinal barrier permeability also play a significant role in autoimmune diseases (12), as does production and recycling of the critical antioxidant glutathione (13). Supplementation with curcumin (14), resveratrol (15), and various flavinoids have all been shown to help dampen autoimmune responses (16).

Once your immune status has stabilized, we address all aspects of your condition, including central and peripheral dysfunction. The loss of peripheral nerve function can lead to significant problems with gait and balance. CIDP patients have been shown to have prolonged dual leg stance and with shorter stride length with gait testing, as well as increased variability of gait parameters. This predisposes them to instability and falls (17). Non-inflammatory peripheral neuropathy patients walk with reduced trunk sway and slower gait speed, however CIDP patients do not show this compensatory strategy, likely due to a greater deficit in motor nerve fibers. CIDP patients thus have reduced ability to decrease trunk sway with slower gait speed, creating increased risk of falls (18).

Fall risk is mediated not just by peripheral nerves, but also by the inner ear vestibular system. Your brain uses inputs from muscles and joints to control balance, but it also requires proper function of vestibular and visual pathways. Vestibular function has been shown to be decreased in CIDP, and in some cases, severely so (19). In cases of combined central and peripheral demyelination, visual pathways can also become negatively affected (20). This raises the possibility that every major system your body uses to make sense of where you are in the world can be compromised all at the same time in CIDP. In every NeuroRescue Program, we quantify and rehabilitate visual, vestibular, and proprioceptive deficits and imbalances, to help you restore balance and mobility while remaining safe at all times.

We use a wide variety of electrical therapies and neuromodulation strategies to restore peripheral nerve function in your NeuroRescue Program. We may use a somatosensory evoked potential (SSEP) device, which uses repetitive polarizing electrical stimulus to activate peripheral nerves. We may also employ a device called the Rebuilder, which stimulates a peripheral nerve’s entire affected sensory field. TENS therapy has also been shown to be very helpful for reducing painful neuropathy symptoms (3). There are different indications for various types of electrical therapy, but all of our electrical therapies have been shown to be effective in neuroscience research.

We may employ additional therapies, such as laser photobiomodulation. Both low-level and high-intensity laser therapy has been shown to be effective in controlling pain from diabetic neuropathies (21, 22).

We may also employ other advanced therapies, such as transcranial magnetic stimulation to reduce neuropathic pain (23), or hyperbaric oxygen therapy to improve healing (24).

We may employ supplementation strategies to improve peripheral nerve regeneration. Substantial evidence exists for the use of antioxidants like alpha-lipoic acid, and flavinoids like luteolin and quercitin in the treatment of peripheral neuropathies (26).

And as the central changes in visual and vestibular systems that develop from central demyelination can conspire with your loss of sensation from your CIDP, we put all of our patients through some form of vestibular rehabilitation and gait retraining to reduce their fall risk and prevent injury (25).

Every nervous system is different, and so is every NeuroRescue Program. We do not take a cookie-cutter approach to complicated neurological conditions. Your protocol will be determined by the specifics of your neurodiagnostic findings and the unique factors of your case.

How Does the NeuroRescue Program Work?

We design your unique NeuroRescue Program to be among the most comprehensive diagnostic and therapeutic protocol available today. We create individual NeuroRescue Programs based on a comprehensive analysis of every relevant neurological system and pathway, using gold-standard, cutting edge neurodiagnostic technologies and examination procedures and state-of-the-art therapies.

We begin with your Discovery Day, wherein we perform a comprehensive history of not only your condition, but your life on a timeline. This allows us to dive deeply into your case and see all of the factors that led to where you are now. It helps us uncover hidden problems and associated conditions that may be making it difficult for you to move your recovery forward.

Our examination allows us to identify the areas and pathways of your brain that are involved in your CIDP. In all of our cases of assumed peripheral neuropathy, we begin by making certain that there are no central nervous system factors contributing to your symptoms. We do this by precisely quantifying the function of your visual, vestibular, and proprioceptive systems through computerized analysis of your eye movements, your inner ear reflexes, and your balance in a host of different sensory conditions.

We employ technologies including Videooculography and Saccadometry to measure several classes of eye movements. We use Video Head Impulse Testing to measure the function of your inner ear, and Computerized Dynamic Posturography to assess your balance in different sensory conditions.

We use NeuroSensoryMotor Integration testing to evaluate hand-eye coordination and cognition, and Virtualis testing to assess dynamic eye tracking and perception of vertical in a virtual reality environment.

We combine all of this with a comprehensive physical and neurological examination of your sensory, motor, autonomic, and cognitive systems. We review any relevant laboratory testing, radiological imaging, and prior neurodiagnostic testing, and integrate that information with our findings.

We use this information to identify which parts of your nervous system are working properly, which systems are struggling, and the precise point at which your systems fatigue.

We can then design a NeuroRescue Program that is unique and specific to your brain, and yours alone. Your NeuroRescue Program works to rejuvenate and reintegrate the damaged neurons and pathways in your central and peripheral nervous systems. It works to improve energy, endurance, and functional capacity within your involved fragile systems.

We use our technologies and procedures to not only see what we need to address, but also when it is time to stop and let you rest. We address your impaired neurological function from multiple angles of therapy, and provide metabolic support to improve neurological recovery.

While we cannot bring back neurons that have been lost, your NeuroRescue Program allows us to take the pathways that remain and maximize their efficiency and endurance. And by focusing on the integration of systems, we can do more than just get pathways working better, we can get them working together again. This gives us our best opportunity to return you to living a healthy, vibrant, and fulfilling life.

Your Next Best Step:

Living with CIDP can be challenging, but there is hope for functional improvement. To see if the NeuroRescue Program is right for you, contact one of our patient care coordinators to schedule your Discovery Day.

And remember, it’s never too late to start getting better.