It can start with a simple tingling feeling in the legs that progressively creeps up the body, but within a matter of hours that sensation can lead to a serious, life-threatening situation.
An often-misconstrued and relatively uncommon neurological disorder, Guillain-Barré syndrome occurs in about 1 out of every 100,000 Americans. That’s about as common as finding that one person in a packed Ohio State-Michigan rivalry game at The Big House, the largest stadium in the United States.
Despite its rarity, doctors and physical therapists in our area have dealt with its effects and recognize its tell-tale symptoms. Dr. Joseph Ashbury, a neurologist at Western Baptist Hospital, studied under one of the leading researchers in the disease and has encountered several cases himself.
Sometimes synonymously referred to as acute inflammatory demyelinating polyneuropathy, Guillain-Barré is an autoimmune disease that attacks parts of the myelin sheaths protecting the peripheral nervous system.
Similar to multiple sclerosis in its attack on nerve coverings, Guillain-Barré’s onset tends to be more sudden than MS, which can take sometimes months or years to develop.
“Likely what happens is there’s an overall acute assault on nerves at the same time,” Ashbury said. “Someone has numbness in their toes at breakfast and by early afternoon they’re paralyzed from the waist down.”
The quickness of the ascending paralysis associated with the disorder is what can make Guillain-Barré so life-threatening. Once paralysis begins affecting the nervous system in the mid-neck, breathing signals from the brain to the diaphragm are interrupted and immediate intubation is required.
“These people become debilitated within hours and it can ultimately be fatal,” Ashbury said.
Typically patients are stabilized in an intensive care unit and connected to a respirator. Weakness progressively moves throughout the body, commonly reaching the greatest period of weakness within three weeks, according to the National Institute of Neurological Disorders and Stroke.
Doctors are still unsure about the catalyst that leads to Guillain-Barré, but most frequently, onset happens several days or weeks after a patient experienced a respiratory or gastrointestinal viral infection.
Treatment comes in the form of either plasmapheresis or intravenous immunoglobulin therapy, Ashbury said. Plasmapheresis removes blood from the body and extracts immune cells from the blood, while IVIg therapy consists of introducing countermeasures into the body that confuse harmful antibodies.
For patients, weeks of treatment leads to months of rehabilitation and physical therapy. Carlos Cochran, a physical therapist at Lourdes hospital, said therapy can last anywhere from three weeks to as much as a year or longer, depending on the severity of the case.
“No single case is alike when dealing with this disease,” Cochran said.
Generally, the physical therapy process begins with essential breathing techniques and core-muscle strengthening. Once the trunk muscles are rehabilitated enough to sit up in bed, patients begin to work on extremities through isometric exercises.
“It can be very difficult,” Cochran said. “We take for granted lying in bed, then being able to sit up on the side of it, but that’s something you can work for months to achieve.”
As is particularly the case with Guillain-Barré, Cochran said the key to a successful recovery is to gradually rehabilitate the patient and not over-fatigue their muscles to the point of setting them back several weeks or even months.
Generally in the United States, about 80 percent of people diagnosed will recover completely with only about 30 percent experiencing further weakness after three years, according to the Guillain-Barré Syndrome and Chronic Inflammatory Demyelinating Polyneuropathy Foundation International.
The low-mortality rate of the disorder in the United States can directly be attributed to the advances in hospital intensive care units, Ashbury said, while Guillain-Barré remains a killer throughout much of the world.
“Guillain-Barré syndrome is a pretty bad prognosis in other countries, but it’s one of the true success stories here in the U.S.,” he said.
Call Will Pinkston, a Sun staff writer, at 270-575-8676.