Does Guillain-Barré Syndrome Recur
Toby A. Ferguson, M.D., Ph.D and Mark J. Brown, M.D.
Department of Neurology, University of Pennsylvania Medical Center, Philadelephia. PA.
Guillain–Barré Syndrome (GBS) is a rapidly progressive illness that affects peripheral nerves. Typical GBS causes weakness, loss of sensation, imbalance and pain. Until recently GBS was considered to be a single condition that resulted from the patient’s own antibodies mistakenly attacking his or her nerve myelin. It is now known that there are at least three other forms of GBS, also thought to be caused by antibodies, where myelin injury is not a prominent feature. In general all forms of GBS follow a predictable pattern; They start with one to three weeks of increasing weakness and sensory loss, progress to a period of one to three weeks with no change in strength or sensation, and end with a recovery period that may extend from weeks to as long as two years. Most patients with GBS ultimately will have a good or functional recovery.
Once the recovery is underway GBS patients begin to wonder whether they will suffer another attack of GBS. For most patients the answer is ”no.” A second attack of GBS is rare, and most patients who have had GBS will not have another, regardless of how the condition was treated. However, a small portion of all patients will have a recurrence.
The medical literature on this subject is not extensive. Published case studies suggest that about one to five percent of individuals who have had GBS will have another episode. The most comprehensive study, reported by Dr.R.H.Kennedy and colleagues at Mayo Clinic in 1978, retrospectively followed 40 GBS patients for up to 42 years after the first attack. In that study only one individual had a second episode of GBS, which occurred four years after the initial one. This is in keeping with the often- quoted figure of one to two percent of recurring GBS.
The literature indicates that most patients with additional episodes of GBS have only one. However, there are reports of patients who have had two or more. Fewer than 20 cases of multiple GBS episodes have been reported. We combined the results of eight major studies of recurring GBS. From a total of 41 such patients there were 31 with two attacks, and 10 with three or more attacks. Thus, a single recurrence of GBS is rare, and multiple recurrences are exceedingly rare. Generally the recurrence comes months to years after the initial episode. The reported range is as short as 4 months and as long as 17 years.
How will the second attack compare to the first one? Respiratory or intestinal infections often precede the second episode, as is the case with isolated GBS. In both single and recurring GBS nerve conduction speed typically slows, there are EMG signs of axonal injury, and spinal fluid protein level rises. In most reported cases of recurrent GBS, the distribution and severity of weakness and sensory symptoms differed from episode to episode. Like single-episode GBS, symptoms progressed over days to weeks and then improved. Patients who had one recurrence of GBS Usually returned to their previous level of function. Patients with multiple recurring episode of GBS accumulated small neurological deficits, resulting in further weakness, additional loss of reflexes, and tremulousness. In summary, isolated GBS and a recurring GBS episode are similar in their presentation, diagnosis, treatment, and recovery.
Much is known about the process that lead to typical and the less common forms of GBS. We know that infections and surgery may trigger GBS. We do not know why a particular trigger will cause one person to develop GBS, while many others in the same situation have no neurological symptoms. Certainly there is an individual susceptibility to developing GBS. This was supported by a recent study by Dr.K.Geleijns and colleagues, published in 2004. The authors described 12 families of susceptible individuals in which at least two family members developed GBS over the preceding 15 years. Given the rarity of GBS, about one case per million persons per month (according to Dr.A.K. Asbury) this is greater than would be expected by chance alone. The underlying host factors causing susceptibility for these families, and for individuals with single-episode and recurring GBS, remain to be discovered.
Patients who are recovering from GBS, or who previously had GBS, may experience symptoms that incorrectly suggest a new episode of GBS. Some patients with GBS have temporary worsening after an initial period of improvement. These relapses may present a variable response to treatment, variability in the course of the progressive phase, or failure of the immunologic process that turns off the GBS process. It is common practise to give these patients additional plasmapheresis or IVIG. Patients with rapidly progressive form of chronic inflammatory demyelinating polyneuropathy (CIDP) may initially appear to have GBS. Two or more relapses of a demyelinating neuropathy within a few months of initial symptoms usually indicate CIDP and not recurring GBS.
Patients who have residual weakness after GBS may experience excessive fatigue, or have weakness of muscles that were especially affected by the initial GBS. These muscles do not have normal reserve endurance under stress. Weakness may appear after vigorous or prolonged exercise, insufficient sleep, or an unrelated medical illness. This new weakness may be mistaken for a second attack of GBS. Also, decades after recovery from GBS patients may notice slowly increasing weakness in a muscle or limb that was weak during the GBS episode. This is similar to weakness that may come years after an episode of poliomyelitis (post-polio syndrome).
In conclusion, GBS rarely recurs. Only about one to two percent of all former GBS patients will have a second episode. Multiple recurrences are highly unlikely. Most patients with recurrent GBS respond favourably to treatment with plasmapheresis or IVIG. Former GBS patients who develop a new weakness or sensory symptoms should consult with their primary physician and neurologist before concluding that the cause is a new episode of GBS. Further research is needed to understand what makes some individuals susceptible to GBS in its various forms, and why a very small fraction of those who contract GBS do so more than once.