5 Things to Know About Chronic Inflammatory Demyelinating Polyneuropathy
By Ariel Harsinay
September 20, 2019
Chronic inflammatory demyelinating polyneuropathy (CIDP) may be a rare disease, but it is one of the most common forms of neuropathy and the most common form of chronic autoimmune neuropathy. Roughly 30,000 Americans are currently diagnosed with CIDP. Unlike many other neuropathies, CIDP is treatable, but its similarities with other nerve disorders often make it extremely difficult to diagnose. Here are five things to know about CIDP.
1. CIDP is an autoimmune disorder that attacks the peripheral nervous system.
CIDP is an autoimmune, neurologic condition in which the myelin sheath surrounding the axons of neurons is attacked by the immune system, causing demyelination. Myelin normally serves as axonal insulation, allowing neurons to rapidly transmit action potentials and communicate with neighboring neurons. The demyelination seen in CIDP impairs this process, resulting in motor impairment, numbness, difficulty walking, and general weakness and fatigue.
The immune response causing CIDP involves the activation of T cells as well as the expression of cytokines, tumor necrosis factor, interferons, and interleukins. Immunoglobulin and complement are also implicated in CIDP pathogenesis.
2. CIDP presentation can vary considerably.
Fifty to sixty percent of CIDP cases are so-called “typical,” presenting with symmetric symptoms of both proximal and distal limbs in which motor impairment is more prominent than sensory symptoms. A diagnosis requires that a patient’s symptoms have progressed gradually for at least 8 weeks, although some patients present with either acute or relapsing-remitting forms of the disease. While CIDP symptoms can usually be managed throughout life, long-term disability is not uncommon.
Some 20%-30% of CIDP cases are idiopathic, although it is frequently seen in conjunction with a variety of other illnesses, including HIV, diabetes, lupus, hepatitis, lymphatic cancer, and restless legs syndrome. It also may present as a comorbidity of infection or as a side effect of various cancer and HIV drugs.
CIDP can affect anyone but is most commonly diagnosed in individuals in their 50s and 60s and is twice as common in men. The global yearly incidence of the disorder is 1.5-3.6 million, with around 30,000 people affected in the United States at any given time. In patients with type 2 diabetes the incidence rises to over 26%.
3. A variety of tests can confirm a CIDP diagnosis.
Nerve conduction studies and electromyography are diagnostic standards in CIDP they; can reveal the demyelination and resulting slowed neuromuscular transmission characteristic of disease. Nerve biopsy can also be helpful, providing pathologic evidence of nerve damage and inflammation.
Often physicians will also order a lumbar puncture in suspected CIDP cases, looking for elevated spinal fluid protein without elevated white cells. In atypical cases, in which electrophysiologic tests may be inconclusive, MRI can help confirm a diagnosis. Blood tests may also be used to detect common comorbidities such as diabetes and lupus.
And, Dr. Cameron has written how one woman with CIDP actually had Lyme disease: https://danielcameronmd.com/chronic-inflammatory-demyelinating-polyneuropathy-cidp-case-resolved-antibiotics/
Lyme disease has been ruled out in other conditions based on negative serologic tests only to seroconvert to positive serologies on follow-up. [1,2] This was demonstrated in the case of a 41-year-old woman with chronic inflammatory demyelinating polyneuropathy (CIDP), according to Perronne from the Infectious Diseases Unit, University Hospital Raymond Poincaré, APHP, Versailles Saint Quentin University, Garches, France.
The woman, who was then treated successfully with 6 weeks of doxycycline and hydroxychloroquine, showed “a dramatic clinical improvement” with a complete disappearance of neurologic signs, according to Perronne.
The authors advised against automatically ruling out Lyme disease based on negative serologic tests.
The woman presented with asthenia (loss of strength), weakness, and diffuse paresthesias (abnormal skin sensations). All of which are hallmark Lyme symptoms.
A word of warning, however. The pdf mentions the success of corticosteroids, but Lyme literate doctors have discovered this to be counter productive unless antimicrobials are used concurrently as catabolic steroids depress the immune system allowing the infection(s) to proliferate.
Regarding treatment for CIDP, the pdf states that “up to eighty percent of CIDP patients respond to one or more therapies that modulate the immune system. Treatments shown to work in clinical trials include prescribing corticosteroids, blood plasma exchange or plasmapheresis, and intravenous immunoglobulin or IVIG. All three treatments are highly effective and lead to improved strength and function in CIDP patients. Each treatment has its advantages and disadvantages that should be discussed with the physician.”
If you’ve been diagnosed with CIDP but suspect tick borne illness, please share this with your medical practitioner. Also, point out the initial seronegativity on Lyme tests by patients with CIDP who were found to seroconvert later.