Guillain-Barre Syndrome

 

What is Guillain-Barré Syndrome?

Guillain-Barré (Ghee’-yan Bah-ray’) syndrome (GBS) is an inflammatory disorder of the peripheral nerves outside the brain and spinal cord. It’s also called Acute Inflammatory Demyelinating Polyneuropathy (AIDP) and affects 1 out of every 100,000 people. GBS is characterized by the rapid onset of numbness, weakness, and often paralysis of the legs, arms, breathing muscles, and face. Paralysis is often ascending, meaning that it travels up the limbs from fingers and toes towards the torso. Loss of reflexes or diminished reflexes are a characteristic feature. Other symptoms may include numbness, tingling, and pain in the arms and legs. The cause is unknown, but GBS is thought to be an autoimmune condition. About 50% of cases occur shortly after a microbial infection (viral or bacterial), some as simple and common as the flu or food poisoning.  GBS symptoms may worsen to the point of complete paralysis and will then gradually improve over the next several weeks. Severe cases may take longer to recover and residual symptoms of weakness and sensory loss can occur In severe cases, GBS symptoms can affect a patient's ability to breathe on their own, requiring monitoring in the intensive care unit (ICU) and possibly a ventilator.

How is Guillain-Barré Syndrome diagnosed?

To diagnosis GBS, doctors will take a perform a thorough clinical exam and obtain a detailed history of symptom onset and associated symptoms. Tests, such as a lumbar puncture or electrodiagnostic tests can help confirm a GBS diagnosis. 

How is Guillain-Barré Syndrome Treated?

Plasma exchange (a blood “cleansing” procedure) and high dose intravenous immune globulins are often helpful to shorten the course of GBS. The natural history of GBS is a peak of symptoms approximately at 4 weeks from symptom onset with no progression of symptoms beyond 8 weeks. Patient care involves the coordinated efforts of a team such as a neurologist, physiatrist (rehabilitation physician), internist, family physician, physical therapist, occupational therapist, social worker, nurse, and psychologist or psychiatrist. Some patients require speech therapy if speech muscles have been affected.

Video - https://youtu.be/4omfTbiB0kk

The GBS CIDP Foundation International has publications for patients and their caregivers related to CIDP including:

• An of Overview for the Layperson
• Guidelines for Physical and Occupational Therapy
• A Handbook for Caregivers
• Caring For A Child with GBS or CIDP 

In addition, the GBS CIDP Foundation International provides information about chapter meetings and connecting to local liaisons for support.

See also the AANEM endorsed AAN Guideline on IVIg in Neuromuscular Disorders 

More Information

American Chronic Pain Association
National Institute of Neurological Disorders and Stroke
Neurology Forum
American Autoimmune Related Diseases Association, Inc 
Chronic Inflammatory Demyelinating Polyneuropathy Page

What is Chronic Inflammatory Demyelinating Polyneuropathy?

Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) is a rare disorder of the peripheral nerves characterized by gradually increasing sensory loss and weakness associated with loss of reflexes. The number of new cases per year of CIDP is about 1-2 per 100,000 people, but as the disease can be present in a person for years prior to diagnosis, the prevalence reflecting the accumulation of cases over time may be as high as 9 per 100,000 in some areas.

CIDP is caused by damage to the coating of the peripheral nerves, called myelin. It can start at any age and is more frequent in men than women. The most common average age of onset is age 50-70 years of age.

Who gets Chronic Inflammatory Demyelinating Polyneuropathy?

People of any age and either sex can get CIDP. However, the peak period patients develop CIDP is in the 5th or 7th decade of life and the disorder is twice as common in men. There does not seem to be a genetic link to CIDP and there is no know clear trigger. The incidence of CIDP each year is estimated to be between 1.5 and 3.6 million in the population. Approximately 30,000 patients in the US are affected at any one time.

How is Chronic Inflammatory Demyelinating Polyneuropathy diagnosed?

Diagnosis of CIDP is based on the symptoms of the patient:

Symptoms such as loss of sensation (numbness), abnormal sensation (tingling and pain), loss of reflexes, and weakness (difficulty walking, foot drop) that progress beyond 8 weeks in duration. This is the most common type of CIDP. There are also variants.

Tests such as nerve conduction and EMG (show a  demyelinating neuropathy), spinal fluid analysis (usually showing elevated protein with normal cell count), blood and urine tests (to rule out other disorders that may cause neuropathy and to look for unusual proteins.) It is recommend to reference the electrodiagnostic criteria is the EAN/PNS 2021 guidelines in order to ensure the diagnosis is correct.

How is Chronic Inflammatory Demyelinating Polyneuropathy treated?

Many people with CIDP benefit from  treatment.  Although there is a wide spectrum of how severely people with CIDP can be affected, some with the condition require assistance to perform daily tasks. In the more severe cases devices like wheelchairs or walkers may be needed for mobility. One of the goals of early diagnosis and proper treatment is to avoid this level of disability and to improve the long-term outlook. Risks and benefits of treatment options should be discussed with a physician on an individual basis to find the best option.

Treatments available for CIDP:

• Corticosteroids (Prednisone, Prednisolone) are similar to naturally occurring anti-inflammatory hormones made by the body, and can be used as an initial treatment. Corticosteroids often improve strength, are conveniently taken by mouth, and are inexpensive. Si
• Intravenous Immune Globulins (IVIG)  IVIG contains naturally occurring antibodies obtained from healthy volunteers. IVIG is given through a vein over the course of several hours. There are also newer treatments available with preparations of immunoglobulin that can be given under the skin (subcutaneous) now available for CIDP patients. 

See “Understanding a Self-Infused CIDP Therapy Option” for more information

• Plasma Exchange (PE), or Plasmapheresis (PLEX), is a process by which some of the patient’s blood is removed and the blood cells returned without the liquid plasma portion of the patient’s blood. It may work by removing harmful antibodies contained in the plasma.
• Efgartigimod is the first and only plasma-free treatment approved recently by the FDA for CIDP, and works differently than other CIDP treatments. Efgartigimod can be self injected subcutaneously once weekly by a health care professional or self-administration. 

Per the EAN/PNS guidelines, there are some recommended medications for second-line use for refractory CIDP. These are used in combination or as monotherapy when the above standard treatments fail, cause significant side-effects, or the clinical response is not optimal.  These drugs are largely not tested in randomized controlled trials, but their use is supported by case series from the medical literature

Post-treatment life can be improved if the disease is diagnosed and treated in a timely manner. Patients respond in various ways. One third of patients can achieve medication free remission. However, the chronic nature of CIDP requires long-term care of patients and long term treatment for two -thirds of patients with the diagnosis. Accommodations in the home may be needed to facilitate daily living activities.

Video - https://youtu.be/WJVVYk1sg0I?si=eNeTqCQe5XFs3JbK 

The GBS CIDP Foundation International has publications for patients and their caregivers related to CIDP including:

• An of Overview for the Layperson
• Guidelines for Physical and Occupational Therapy
• A Handbook for Caregivers
• Caring For A Child with GBS or CIDP 

In addition, the GBS CIDP Foundation International provides information about chapter meetings and connecting to local liaisons for support.

See also the AANEM endorsed AAN Guideline on IVIg in Neuromuscular Disorders 

More Information

GBS CIDP Foundation International 
Foundation for Peripheral Neuropathy
American Chronic Pain Association
National Institute of Neurological Disorders and Stroke
Multifocal Motor Neuropathy Page

What is Multifocal Motor Neuropathy?

Multifocal Motor Neuropathy (MMN) is a rare disorder in which focal areas of multiple motor nerves are attacked by one’s own immune system. Typically, MMN is slowly progressive, resulting in asymmetrical weakness of a patient’s limbs. The upper extremities are often commonly affected.  Patients frequently develop weakness in their hand(s), resulting in dropping of objects or sometimes inability to turn a key in a lock. The weakness associated with MMN can be recognized as fitting a specific nerve territory. There is essentially no numbness, tingling, or pain. Patients with MMN can have other symptoms, including twitching, or small random dimpling of the muscle under the skin which neurologists call fasciculations.

The prevalence of this very rare disease is estimated to be 0.6 cases in every 100,000 people. 

Who gets Multifocal Motor Neuropathy?

Men are more often affected with multifocal motor neuropathy than women. Symptoms usually appear before age 45.

How is Multifocal Motor Neuropathy diagnosed?

The diagnosis of MMN is a clinical one that depends on demonstrating that the patient:

• has a purely motor disorder affecting individual nerves
• there are no UMN (upper motor neuron) signs such as brisk reflexes at the knees or ankles or spasticity in the limbs
• there is no difficulty speaking or swallowing
• there are no sensory deficits
• there is evidence of focal areas of nerve in which electrical impulses are slowed or blocked (conduction block) which can be detected on electrophysiology tests

These criteria are designed to differentiate MMN from ALS, (Lou Gehrig’s disease), Lewis-Sumner Syndrome (a multifocal inflammatory nerve disease with motor and sensory symptoms), and vasculitis or inflammation of small blood vessels in the peripheral nerve.

A neurologist is usually needed to determine the diagnosis, which is based on the history and physical examination. Tests include:

• an electrodiagnostic study, which includes nerve conduction studies (NCS) and needle electromyography (EMG).
• Laboratory testing for IgM GM1 antibodies is also frequently done. The absence of these antibodies, however, does not exclude the diagnosis.
  

How is Multifocal Motor Neuropathy treated?

It is now established that intravenous immunoglobin (IVIg), a preparation of antibodies obtained from healthy volunteers, can be readily given through an arm vein and provides benefit to patients with MMN. It is the only treatment for this disorder that is approved by the Federal Drug Administration (FDA) and regulatory agencies in Europe and Canada. IVIg can lead to improved motor function in most patients with MMN, with the response varying from minimal to very large. Early treatment shortly after symptom onset is always more effective. The treatment usually does not completely reverse all of the symptoms, and those patients who do respond will require repeated treatments to maintain their improvement. Patients usually require re-treatment every 2-5 weeks and over time may need increased doses of IVIg or increased frequency of treatment over time. Subcutaneous immunoglobulin is also an option.

IVIg is not a cure for MMN, but currently no other therapy has proven to be widely effective. In a limited number of patients a cancer chemotherapy drug, cyclophosphamide, is temporarily effective. However, its use is limited by the toxic side-effects and risks that accrue with chronic use. Other immunosuppressive treatments such as corticosteroids and plasma exchange are ineffective and can actually make the disease worse.

Video - https://youtu.be/dhv4haxM_1Q 

More Information

Foundation for Peripheral Neuropathy
American Chronic Pain Association
Johns Hopkins Department of Neurology