Duke Neurologist Receives Grant to Investigate Origins of Chronic Inflammatory Demyelinating Polyneuropathy

According to a publication from Duke University, Dr. Karissa Gable, a neurologist at the University, has received a one-year research grant from the GBS|CIDP Foundation International to study the activity of chronic inflammatory demyelinating polyneuropathy (CIDP) in affected patients.

About Chronic Inflammatory Demyelinating Polyneuropathy

CIDP is a highly rare neurological disorder characterized by the inflammation of the peripheral nerves and the progressive destruction of their myelin coatings. Peripheral nerves are the network of nerve cells linking the brain and spine to the rest of the body. When they are damaged, it can severely hamper or outright sever the brain’s ability to communicate with (and therefore control) muscle groups.

Unlike other demyelinating conditions like acute inflammatory demyelinating polyneuropathy (AIDP) or Guillain Barré syndrome (GBS), the symptoms of CIDP are chronic and continuously progressive — most often slowly getting worse and worse without plateau or improvement without ongoing treatment. CIDP is sometimes considered to be a chronic form of AIDP.

Symptoms of CIDP present slowly, typically over the course of two or so months. Symmetrical weakness of muscles around the hips, shoulders, hands, or feet is “highly suggestive” of CIDP if nerve damage is confirmed to be the cause. Nerve and muscle dysfunction are frequently reported, which can lead to fatigue, burning sensations, and changes in sensory perception and coordination.

The causes of CIDP are uncertain, but much of the existing evidence suggests an autoimmune origin. Autoimmune diseases are caused by malfunctioning immune systems that mistakenly target healthy tissues and organs (and potentially neurons, in this case) as though they were hostile pathogens.

Currently there is no cure for CIDP. Its worst symptoms are managed through immune suppresssion, in addition to the application of corticosteroids and plasma exchange. Physical therapy may be employed in parallel to mitigate the residual muscle weakness experienced by some.

The prognosis for CIDP is unique to each patient. Some may experience only a short, relatively limited episode. Others may have recurring bouts with only partial recovery between episodes. Early and aggressive treatment is recommended to preserve as much of the peripheral nerves as possible.

Gable Receives Grant

Dr. Gable and her colleagues are particularly interested in the role of the immune system in CIDP. Although immunosuppressive treatment provides effective results in some cases, it can be difficult to wean patients off them without accidentally triggering flare-ups of CIDP.

Gable and her research team plan on examining the role of Th17 in determining the severity of CIDP disease activity. Th17 is a cytokine of the immune system that plays an important role in the induction of tissue inflammation and destruction that are commonly experienced in inflammatory immune diseases. Gable’s team will also run tests to evaluate if Th17-induced inflammation specifically can be inhibited through the use of targeted therapeutics.

Better understanding of T and B cell function, Gable believes, may help physicians better identify aggressive cases of CIDP and develop “…potential targets for new treatments.”

The nature of many rare diseases is still uncertain or only theorized. Why is it important to understand the activity of a disease before attempting to formulate cures? Share your thoughts with Patient Worthy!

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