Looking on helplessly as a wave of weakness increases one’s body from the ankles upward can lead to dismay. This is what happens in Guillain Barre (pronounced GHEE-on bah-RAY) syndrome, known more formally as acute inflammatory demyelinating polyradiculoneuropathy. Occurring in just a couple of people annually at a population of 100,000, Guillain Barre syndrome makes up for its rarity by taking people by surprise and immediately disabling them.
Acute inflammatory demyelinating polyradiculoneuropathy is about as bulky and awkward a name because there is, but the language has the endearing characteristic of encoding the disease’s essential features. Starting from the rear end and working forward, “-pathy” means illness;”neuro” states the peripheral nerves are involved; “radiculo” means the spinal nerves arising from the spinal cord are also affected; “poly” means it is a widespread process; “demyelinating” means that the nerve-fibers are stripped of the sheath-like myelin coverings; “inflammatory” means a local tissue response to physical or biochemical irritation; and “severe” means that the disease develops rapidly over a few days.
Despite the lesson in medical terminology offered by the entire title, it’s easy to understand why the condition often goes by the names of AIDP or Guillain Barre Syndrome (GBS). Georges Guillain and Jean-Alexandre Barre described cases of the illness among French soldiers in the First World War. It’s noteworthy that the problem is labeled a”syndrome,” rather than a disorder, since it’s very likely that multiple disease-processes can create exactly the identical pattern of clinical illness (syndrome).
Diagnosing GBS involves recognizing the normal routine of progressing symptoms where a reduction of power works its way up the legs and often even into the arms and breathing muscles. The symptoms quickly worsen over a few days, even hours, and the weakness typically peaks within 2-3 weeks of the onset of symptoms. Although the peripheral nerves and spinal nerves also run messages regarding bodily feeling, sensory loss in GBS is typically a minor element, while weakness — due to disturbance of nerves carrying messages to muscles — predominates.
The physical examination confirms the muscle fatigue and, when present, the related numbness. Another classic finding on examination is a reduction of (rubber-hammer-type) tendon reflexes. Supplemental tests which help confirm the diagnosis — or, depending on their results, point in a different direction — are nerve conduction studies and cerebrospinal fluid analysis. Nerve conduction studies check the electrical characteristics of the peripheral nerves. In GBS the neural impulses tend to be slowed or blocked on their way from one part of the nerve to another.
Let’s understand it
Cerebrospinal fluid is the watery liquid bathing the outside of the brain, spinal cord and spinal nerves. It’s obtained for analysis by way of a lumbar puncture, also called spinal tap. In GBS the protein content of the fluid is raised with no corresponding gain in the numbers of white or red blood cells in the fluid. The cause of GBS is unknown, but since it often follows a disease or other barrier to the human body’s immune system and also involves inflammation, it appears likely that GBS is caused by an overactive immune system. In that case, GBS is one of many so-called autoimmune diseases where the body’s immune system mistakenly attacks a part of the human body, in this case the myelin coverings of human nerve-fibers.
Other examples of autoimmune disease are rheumatoid arthritis, where the immune system attacks the joints, and psoriasis, in which the immune system attacks the skin. A case series refers to a group of consecutive cases sharing agreed-upon capabilities. Analyzing a case series offers insight into just how variable the illness can function and which features are more continuous. Between 1995 and 2003 researchers at the Aga Khan University Hospital in Karachi, Pakistan, gathered a case series of 34 patients with GBS.
The ages of these patients ranged from 3 to 70, and 62 percent were male. In 35 percent of the cases there was a previous gastrointestinal disease and in another 26% of the cases there was a previous respiratory disease. Breathing failed in 56 percent of the cases, requiring mechanical ventilation. Despite the often devastating nature of GBS, most patients improve, albeit slowly. Compiling another case series, researchers at the Centre for Rehabilitation Research in Orebro, Sweden, monitored the progress of 42 patients with this illness.
Mechanical ventilation was necessary in only 21 percent of their cases. At 2 weeks, 1 year and 2 years after the onset of symptoms, 0%, 38% and 45% of patients had completely regular strength. At precisely the exact same time points, 38%, 90% and 93% could walk 30 feet without help. Treatment is available for patients with GBS. Needless to say, when patients can not breathe by themselves, using a mechanical ventilator to support respiration is a form of therapy and is generally life-saving.
Two other treatments are demonstrated by randomized, controlled trials — the gold standard way of assessing a remedy — to hasten recovery in GBS. One is plasmapheresis, also called plasma exchange, where the liquid part of the blood (plasma) is separated from the blood cells. The blood cells are then returned to the individual’s body, and the body produces more plasma on its own to replace the plasma which was eliminated. The reason why plasmapheresis works is unclear, but it probably eliminates harmful antibodies in the bloodstream. Infusing immunoglobulin to the patient’s blood is another treatment of proven effectiveness. The immunoglobulin preparation comprises antibodies pooled from a high number of healthy donors. These healthful antibodies presumably counteract the injurious radicals generated from the GBS patient. One may think that two remedies — plasmapheresis and immunoglobulin infusion — administered together or in series will be better than just one, but this isn’t the case. A study revealed that the two remedies in combination were no better in hastening healing than 1 treatment.