- Disability After "Recovery" From GBS -
Kleopas A. Kleopa, M.D. Neuromuscular Fellow
Mark J. Brown, M.D. Professor
Department of Neurology
University of Pennsylvania
School of Medicine
Philadelphia, PAThis article was published in "Communicator" 2000
Guillain-Barré syndrome (GBS) is the most common cause of acute neuromuscular paralysis in developed countries. Most patients recover and return to productive, independent lives. In a recent representative survey of 140 GBS patients, 70% made a complete neurological recovery within a year, 22% could walk but were unable to run, 8% were unable to walk unaided, and 2% remained bedridden or ventilator-dependent after a year. Thus, despite the good prognosis for recovery, GBS can cause long-term disability.
Persisting disability is largely the result of weakness from the motor nerve injury that occurred during the acute illness. An estimated 25,000 to 50,000 persons in the United States alone are experiencing residual effects from the disease. Most research on GBS has focused on understanding the cause and finding better treatments. Much less attention has been paid to the long-term disability caused by GBS. In addition to the previously mentioned residual weakness, there may be pain, fatigue, psychosocial dysfunction, possible relapses of the illness, and late progression of weakness.
Pain
Moderate to severe pain is a well-recognized symptom during the course of acute GBS. For some patients neuropathic pain, consisting of abnormal painful sensations, may persist after recovery from the disease.
In a recent prospective study of 55 GBS patients followed for up to 24 weeks, pain occurred during the course of the illness in almost 90% of cases. Whereas deep aching back and leg pain were the most common early on, abnormal painful sensations and myalgic-rheumatic type pain were observed during the recovery period.Musculoskeletal pain was common in association with physiotherapy. Painful abnormal sensations in the extremities tended to persist after 8 weeks, and were still present in some patients after 24 weeks. In two cases the pain was severe.
Overall, pain can be effectively relieved with an escalating regimen of analgesic medications, starting with nonsteroidal anti-inflammatory drugs or acetaminophen, and if necessary including oral or parenteral opioids. Even severe pain can be controlled, sometimes with the addition of patient-controlled analgesia. In a large series of GBS patients treated for pain, these medications were generally effective, and no adverse effects on breathing function or narcotic addiction occurred.Chronic fatigue
Fatigue following GBS is underrecognized by neurologists and rehabilitation physicians, because attention is directed toward the more objective weakness and sensory disturbances.
In a recent study of 83 patients recovering from GBS, severe fatigue was reported as one of the three most disabling symptoms by over 80%. The incidence of fatigue did not correlate with age, or motor and sensory residual deficits, but fatigue was more common in women. Fatigue was unrelated to the time since the acute phase of the GBS, a median of 5.2 years in this group.Another study of 123 GBS patients, evaluated 3 to 6 years after the acute illness, concluded that psychosocial functioning, especially in areas such as sleep and rest, alertness, emotional behavior and social interaction, was seriously affected. This was true even when "complete" physical recovery was reached, or only minimal residual deficits were present. Deconditioning and less engagement in physical activities were discussed as possible explanations for persistent fatigue. A supervised training program and low-intensity aerobic exercise may reduce daily fatigue, with improvements in activities of daily living and functional capacity. Specific treatments for other factors associated with fatigue, such as sleep disturbances, pain, and daytime inactivity, are available.
Psychosocial dysfunction
Reports of long-term psychological sequela after GBS are rare, although this issue may be a major factor in psychosocial dysfunction of patients recovering from the disease. Many psychological factors could contribute to chronic fatigue and social dysfunction, including fear of disability, inability to cope with physical limitations, and depression following a major illness.The role of depression in psychosocial dysfunction after GBS is not fully understood. The sickness impact profile of GBS survivors was found to differ from the profile of other patients with depression. Nevertheless, further study of the long-term psychological impact of the disease is necessary, and depression should be considered on an individual basis when appropriate. Both supportive psychotherapy and/or pharmacologic treatment can be effective.
Post-traumatic stress disorder (PTSD) has been reported in a patient following severe GBS with paralysis and a prolonged intensive care stay. The GBS-induced PTSD shared the features of PTSD seen following other traumatic events. Even such profound psychological problems following GBS can be treated with supportive psychotherapy and appropriate medications. They may at least in part be prevented by adequate pain management and the use of a communication system, such as clear lucid letter-board in the event of near complete paralysis.
Better understanding, prevention and treatment of these issues may have a positive impact on the quality of life for GBS survivors. Moreover, it is important for patients and their families to know that their psychosocial problems are also experienced by other patients after GBS.
Recurrence of GBS
Although GBS is thought to be a one-time disease, relapses and chronic recurrent forms can occur. Patients are often concerned about the risk of having additional episodes of GBS.In a study of 220 GBS patients, 15 were found to have a relapsing course, with one to 4 recurrent episodes. The interval between episodes ranged from 3 months to 25 years. Antecedent events such as a viral infection preceded most relapses, and patients presented each time with the typical clinical and laboratory findings of acute GBS. All patients had long asymptomatic periods between the episodes.
In a more recent study of 476 patients following GBS, 2.5% experienced a recurrence of the acute illness, with a mean period of 16 months between the episodes (range 2-47 months). One patient had three episodes. The authors found no relationship between the risk of having a recurrent episode and the severity of the first episode. Furthermore, the severity of the subsequent episode did not correlate with the intensity of the first episode.
Reaching a correct diagnosis may be challenging in these cases. Even GBS experts may find it difficult to separate a "relapsing variant of GBS" from chronic inflammatory demyelinating polyneuropathy (CIDP), especially early in the course. Recurrent episodes of true GBS, although rare, may occur following similar preceding illnesses, and should be treated in the same way as the initial episode. They respond well to the same established treatment modalities.
Delayed progression
Weakness from GBS reaches its maximum during the first two or three weeks of the disease. This is the active or acute phase of the illness. After a plateau period of days or weeks, recovery begins, lasting between weeks and two years. During this time strength improves steadily.
Strength and sensory function plateau after about two years.However, many decades after GBS, recovered muscles once weakened by the disease may again grow weak. This is a slow process that occurs over years, and may at first escape the patient's notice. It is likely that this delayed weakness is the effect of the normal gradual age-related nerve cell loss on muscles that have a reduced reserve nerve supply from earlier GBS.
The same phenomenon has been observed after poliomyelitis ("post-polio syndrome") and other forms of acute nerve injury. The incidence of slowly progressive late weakness in GBS is unknown, but it is rare. When it does occur, the patient's physician must recognize that the new weakness of seemingly recovered muscles does not necessarily indicate a second attack of GBS.For many patients recovering from GBS, residual motor or sensory deficits may be only one aspect of the long consequences of the disease. Other issues described here may have a considerable impact on their quality of life. Effective treatments are available for most of these problems.
References available on request. Contact Dr. Kleopa at: kleopa@mail.med.upenn.edu
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