Dr. James R. Allen describes his personal encounter with Guillain-Barre Syndrome and reviews the topic. This inflammatory polyradiculoneuropathy may have a number of different etiologies, but essentially appears to be an auto-immune process. The most striking symptoms are those of muscle weakness but respiratory and autonomic problems account for a five percent mortality. No specific treatment is available as yet but plasmapheresis offers hope of arresting the auto-immune process in some cases.
Timely diagnosis and adequate treatment are essential in Guillain-Barre Syndrome (GBS) to reduce the mortality as well as the severity of symptoms and prolonged course which result from nerve root demyelination. Having experienced this syndrome firsthand, I thought perhaps the combined perspective of a neurologist-patient might be useful to other physicians in recognizing GBS and determining an appropriate treatment plan.
As a 48-year-old physician in excellent medical health, it was my habit to run about two miles and carry out 20 push-ups, sit-ups, leg raises and other exercises daily. In early September 1984, following a particularly hectic Labor Day weekend on call, I began noticing aching of both thighs along with fatigability. About two to three weeks before this, there had been a bout of severe headaches and sore throat which had rapidly cleared without any apparent residuals.
Early on, it was primarily repetitive activity such as walking and stair climbing that brought on muscle fatigue and weakness.
I consulted my personal physician on two occasions and had a complete physical examination as well as extensive laboratory work, all of which proved to be within normal limits. This included testing the various muscles for single maximal contractions.
However, it was primarily with repetitive activity such as walking, stair climbing and exercising that the muscle fatigue and weakness occurred. There was no evidence of an acute febrile illness, neoplasm, metabolic abnormality, polymyositis or collagen disease. The diagnosis of Guillain-Barre Syndrome was entertained, but there was not sufficient evidence to make the diagnosis at that point in time.
Stair climbing became increasingly difficult with heavy reliance on the hand rail. Negotiating the long hallways and tunnels at the hospital also became a formidable task. My wife and I had planned a trip to the Montreal Neurological Institute and had decided, unwisely as it turned out, to proceed with that plan. We drove our Suburban truck, and stayed at campgrounds along Lake Superior and Lake Michigan. The fall color was beautiful but my lack of ability to hike even short distances resulted in more of a trial than an adventure.
Allen: By now, I could only walk about one block at a time, very slowly, and then would need to sit on a window ledge or lean against something to keep from falling while the muscles recuperated enough to continue another short distance.
Montreal (unfortunately, named after Mount Royale) is a beautiful, charming, historic old city but with very steep streets as well as steep stone staircases inside and outside of buildings. By now, I could only walk about one block at a time, very slowly, and then would need to sit on a window ledge or lean against something to keep from falling while the muscles recuperated enough to continue another short distance.
I attended the first two days of meetings but realized my condition was much worse and consulted the neuromuscular specialist at the Montreal Neurological Institute who confirmed the diagnosis of GBS and advised me to fly home immediately. As the flights to Minneapolis involved going through Detroit and Chicago, it appeared that a better alternative might be a rapid trip home on the road.
We therefore arose at 5:00 a.m. and made our way out of Montreal through the fog and rain. It is a 1200 mile drive with Sault Ste. Marie at the halfway point. We had planned to stay overnight there but all of the motels were full of fall color enthusiasts. About 100 miles out onto the Upper Peninsula of Michigan, we found a small motel off the main road and were able to rent the last room after prying the proprietor out of the local tavern. There was no heat in the room and it was 26 degrees Fahrenheit outside with snow on the ground.
We set off for Twin Cities. I spelled my wife at the wheel but went through a stop sign, unable to move my right leg to apply the brake. After that, she drove. Every 1 to 2 hours another muscle group would become paralyzed, particularly in the lower extremities.
By this time, I could scarcely walk and was unable to squeeze the toothpaste out of the tube. The walls were paper-thin in the motel and the young couple next door had better things to do than sleep. Therefore, at 4:00 a.m. we loaded up and set off for the Twin Cities again. I tried to spell my wife at the wheel but went through a stop sign because I was unable to move my right leg to apply the brake. After that, she did all the driving.
About every one to two hours, another muscle group would become paralyzed, particularly in the lower extremities. In addition, there were brief, sharp, stinging pains in the extremities, as though one were receiving an injection of Penicillin or tetanus toxoid into the palm of the hand or sole of the foot. This would ache for a few minutes afterward and then disappear. There were other vague sensory symptoms but the muscle weakness and fatigability predominated.
After arriving home I entered the hospital. Over the next 24 hours, the proximal lower extremities became totally paralyzed for all directions of movement and the right arm could not be held up. The left side of the face became weak and the vital capacity dropped from 110 percent to 80 percent. Other autonomic symptoms appeared including coldness of the extremities, diaphoresis, and heaviness of the chest with a right bundle brach block on the EKG.
On arrival home, I entered hospital. Over the next 24 hours my proximal lower extremities became totally paralyzed; my right arm could not be held up. Lung vital capacity dropped from 110% to 80%. It was difficult to sit up in bed for even a few minutes without experiencing autonomic symptoms such as diaphoresis.
At this point, we decided to proceed with plasmapheresis and three of these were carried out over a four day period. 14,000 cc of plasma were removed and replaced with five percent albumin. The progression of the symptoms stopped after the first treatment and very slow improvement began to take place. I went into shock while trying to sit up during one of the plasma exchanges, with a marked drop in blood pressure, blurred vision, slurred speech, nausea, etc., but this rapidly cleared.
The only other problem associated with plasmapheresis came about on two occasions when calcium was being given to replace the amount lost during the procedure. A slow infusion had been prescribed but inadvertently this was flushed in rapidly, producing a pronounced sensation of heat for several minutes in the trunk and perineal area. However, the results of plasmapheresis were well worth the inconvenience.
In the hospital, it was difficult to sit up in bed for even a few minutes at first without experiencing autonomic symptoms, but as improvement occurred, I was able to sit in a chair, walk with crutches or two canes for short distances and return home after two weeks.
Just to get over the threshold into the house was a struggle and to carry out routine activities such as taking a shower, brushing my teeth, shaving, dressing, etc., produced such fatigue that I would need to lie in bed for a half hour or so just to summon enough strength to walk to a chair in the family room. The improvement was slow and somewhat erratic, but I was able to return to work after three and one-half months from the onset.
Plasmapheresis halted my decline. In two weeks I was discharged. Fatigue persisted; recovery was erratic. When the condition was at its worst, even my speech and interest in my surroundings fatigued quickly. After morning grooming, I spent ½ hour in bed summoning the strength to walk to a chair in the family room.
The fatigability persisted with certain activities such as prolonged standing, stair climbing, or walking more than 40 or 50 yards. When the condition was at its worst, even my speech and interest in my surroundings would fatigue quickly. Muscle twitches, presumably myokymia, were quite severe for two to three months, but then lessened and eventually cleared.
An excellent bit of advice came from a former patient with GBS who called and discussed the similarities between our cases and then suggested, to avoid discouragement, that I plan on improvement about every two weeks rather than looking for it on a day-by-day basis. With that advice and with the joy that comes from the return to good health, I have been able to cope with this very well. I have also developed an even greater appreciation of our health care system and the importance of the support of family, friends and colleagues.
Guillain-Barre Syndrome (GBS) has gradually evolved into a fairly well recognized clinical entity. It has been given a number of names (Table 1) but consists essentially of an inflammatory polyradiculoneuropathy.
In 1859, Landry offered the first description of ascending symmetric paralysis (Table 2) and in 1916, Guillain-Barre and Strohl pointed out the elevated protein and normal cell count in cerebrospinal fluid of most patients. Haymaker and Kernohan described the inflammatory reaction which was maximal in the proximal nerve roots but in peripheral nerves as well. As larger series of patients have been reported, we are now better able to grasp the clinical spectrum of this syndrome and avoid some of the arbitrary exclusions of earlier authors.[2-6]
In Guillain-Barre, the cerebrospinal fluid shows elevated protein, but a normal white-cell count (termed albumin-cytologic dissociation). The inflammatory reaction, maximum in the proximal nerve roots, extends to the peripheral nerves.
There may be some merit in dividing these into primary and secondary cases since approximately two-thirds are preceded by another illness (Table 3), surgery or other condition. The Swine Flu vaccination is perhaps the most recent to be implicated. The syndrome involves males twice as frequently as females, but affects all ages and races. It occurs at a rate of approximately 1.5 cases per 100,000 population.
Most persons feel GBS is an auto-immune disease with both cellular hypersensitivity and serum antibodies involved in response to an antigen. These apparently then attack the myelin of the peripheral nervous system. Viral infection is most common precursor, usually upper respiratory or gastrointestinal, approximately one to four weeks prior to GBS onset.
The clinical symptoms (Table 4) usually begin in the lower extremities with paresthesias or weakness. Myalgia of the back or other muscles occurs in about one-third and headaches in one-fifth of all cases. Cranial nerve involvement is seen in approximately 75% of cases, with facial diplegia in 50%. This can therefore be a very helpful sign in making the diagnosis. Loss of deep tendon reflexes is also an excellent finding if present, though this may not occur early.
Autonomic symptoms (Table 5) have been recognized in recent years as being a rather frequent accompaniment to GBS. There can be increased or decreased sympathetic or parasympathetic activity or a combination of the two. There is usually no fever in an uncomplicated case of GBS. Blood pressure and respiratory symptoms are the most frequent autonomic changes. Fortunately, autonomic problems tend to disappear in approximately ten days after their onset.
Autonomic dysfunction is often seen in Guillain-Barre. The most common signs are blood pressure fluctuation, and respiratory symptoms. Lung vital capacity needs to be monitored frequently: 20% to 40% of cases will require a respirator.
Generally, laboratory (Table 6) and microbiology studies are normal in the uncomplicated GBS case, unless there is an underlying condition such as porphyria, infectious mononucleosis or collagen disease. The spinal fluid protein is often elevated, maximum at two to eight weeks following the onset, and sometimes can be markedly elevated. Usually, the CSF cell count is less than ten cells per mm with predominant mononuclears.
EMG findings can be helpful if present but can remain normal. Approximately 20 percent of persons have normal EMG's throughout their illness. Slowed conduction velocities, delayed distal latencies and F waves can suggest the diagnosis. If severe denervation is found, the prognosis for a full recovery is quite poor. It is best to test a large number of nerves for conduction velocity since the condition does not affect all nerves equally and simultaneously.[10-13]
Evolution of GBS symptoms (Table 7) is usually complete in over 90 percent of patients by four weeks from the time of onset. There is some evidence to suggest that persons with slower evolution of symptoms do not recover as well. After the symptoms reach a maximum level, there is a stable period followed by the recovery phase. Approximately 85 percent have a good long-term recovery, but the remainder are left with long-term disability, usually sensory or motor problems of the distal lower extremities. Respiratory failure requires the use of a respirator in about 20–40 percent of cases.
Prognosis (Table 8) can be difficult to predict. As indicated earlier, most persons eventually obtain a satisfactory level of function. A normal EMG at five weeks from onset suggests a good prognosis but severe denervation indicates a prolonged and perhaps incomplete recovery. A stable plateau longer than 18 days often suggests an incomplete recovery. The mortality rate for untreated persons is approximately 30–40 percent, but even with the best of treatment, there remains a five percent or greater mortality in most series.[2-4] The majority of deaths result from respiratory failure but autonomic dysfunction and thromboembolism account for most of the remainder.
Plasmapheresis can arrest GBS if applied during the immunologic and inflammatory stage prior to actual demyelination of the nerve roots.
In the early stage, physical therapy should be discouraged, or used very sparingly: relapse can occur with too vigorous therapy.
Since there is no specific etiology known for GBS, no specific treatment (Table 9) is available. Particular attention to respiratory care is most important, along with excellent nursing care and a team approach by various physicians to prevent or correct complications.
The vital capacity needs to be checked frequently during the progressive phase of the illness, with respirator intervention if it drops to a level of about one-third the expected normal.
Physical therapy should be discouraged in the early phases or used very sparingly since relapses have been reported where too vigorous therapy has been applied. Comfort medications such as Indocin or Tegretol are sometimes required for myalgia or paresthesias. Steroids have not been found to be helpful in GBS according to most authorities, and in some cases may even produce harm by causing a steroid myopathy. There is a variation of GBS known as chronic relapsing polyneuropathy which appears to respond to steroid medication.
Plasmapheresis appears to have considerable potential in arresting the course of GBS, particularly if applied during the immunologic and inflammatory states prior to actual demyelination of the nerve roots. There is stilI a question as to whether there may be sub-populations of responders and non-responders and also as to the number of treatments and other parameters of treatment.
Based upon the GBS literature, experience with a number of GBS patients, and now my own encounter with the syndrome, I would like to advance several observations and suggestions:
Although I have had a special interest in GBS for a number of years, my understanding of the intricacies of the syndrome has greatly deepened with this personal experience. My enthusiasm for research into the etiology, early diagnosis and treatment of this condition is far more intense and urgent as a result of my encounter.
|Acute idiopathic polyneuropathy|
|1859||Landry||First description: ascending symmetric paralysis with absent tendon reflexes, peripheral sensory deficit, respiratory death.|
|1916||Guillain-Barre and Strohl||Noted CSF albuminocytologic dissociation|
|1949||Haymaker and Kernohan||Pathologic description PNS changes|
|Two-thirds of cases preceded by underlying illness or condition. Examples: viral or bacterial infection, vaccination (e.g. swine flu), allergic and immune states, metabolic or endocrine disturbances, toxic exposures and neoplasms.|
|All ages, seasons and races.|
|1.5 cases per 100,000 population|
|Clinical Symptoms and Signs|
|Weakness||Mild <—> total paralysis.
Usually begins in lower extremities.
Loss of deep tendon reflexes.
|Cranial Nerve Involvement||In 75% of patients. All cranial nerves except olfactory have been involved. 7th most frequent.|
|Facial Diplegia||In 50% of cases. Bulbar palsy not uncommon.|
|Fisher's Syndrome||GBS plus opthalmoplegia.|
|Miscellaneous||Headaches, myalgia, respiratory distress and autonomic dysfunction.|
|Hypertension and hypotension.|
|Diminished cardiac output with edema, tachycardia, bradycardia.|
|Paresis of bronchial smooth muscle – reduced secretions, atelectasis.|
|Disturbance of thermal and sweating regulation.|
|Sphincter Abnormalities – bowel, bladder and sexual problems – infrequent.|
|Inappropriate ADH Secretion – vagal defect?|
|Normal lab and microbiology unless underlying condition such as porphyria, infectious mononucleosis or collagen disease.|
|CSF – elevated protein (max. at 2–8 weeks); usually less than ten cells, predominant mononuclears.|
|EMG – findings helpful when present. Slowed conductions and delayed distal latencies, F Waves. Later denervation may occur, and if severe, suggests a poor prognosis for a full recovery.|
|Evolution of symptoms complete in > 50 percent after two weeks; > 80 percent after three weeks; > 90 percent by four weeks.|
|A brief stable period follows.|
|Recovery phase then begins and is satisfactory in 85 percent of cases by four to six months.|
|Respiratory failure requiring use of respirator occurs in 20–40 percent of cases.|
|No specific predictors of consistent value, e.g. severity of disease, respiratory involvement, level of CSF protein.|
|Normal EMG at five weeks suggests good prognosis; severe denervation indicates a prolonged and sometimes incomplete recovery.|
|After symptoms have peaked, a plateau longer than 18 days usually heralds an incomplete recovery.|
|Majority (75–85 percent) of cases make a good functional recovery. Mortality rate with optimal treatment is approximately five percent, untreated approximately 30–40 percent.|
|No specific treatment.|
|Respiratory care – most important.|
|Excellent nursing care and total team approach to prevent complications.|
|Physical Therapy – gentle or none at first; as tolerated later to prevent relapse.|
|Plasmapheresis – valuable in some cases, especially early in the course or with respiratory complications.|
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