RehabGBs: Rehabilitation in People With Guillain-Barré Syndrome

Guillain-Barré syndrome (GBS) is an acute inflammatory demyelinating polyradiculoneuropathy and is the most common cause of acute flaccid paralysis. Its annual global incidence is between 0.8 - 1.9 cases per 100,000 people.

GBS is usually preceded by infection or other immune stimulation that induces an aberrant autoimmune response targeting peripheral nerves and their spinal roots. It is characterized by rapid progressive bilateral muscle weakness of the lower limbs and/or arms, in combination with hyporeflexia or areflexia, and most patients reach their maximum disability within 2-4 weeks.Various phenotypes have been described, including acute inflammatory demyelinating polyradiculoneuropathy, acute motor axonal neuropathy, acute motor sensory axonal neuropathy and Miller-Fisher syndrome.

Overall, the clinical course, severity and outcomes of people with GBS are highly heterogeneous. Significant disability, including incomplete recovery of motor (i.e., ambulation) and sensory function, as well as fatigue, pain, respiratory insufficiency and psychological distress might exist after the acute phase. But long-term significant impacts, two years after the onset, on daily activities, work, social activities and health-related quality of life have also been reported. The most often feature is an impairment of the motor capacity, but respiratory muscle weakness is a common complication in people with GBS and may result in decrease vital capacity, chest wall expansion and coughing strength, causing atelectasis and pulmonary infections. Therefore, it is generally accepted that respiratory muscle weakness causes increased morbidity and mortality, owing to the combination of poor airway protection, inadequate secretion clearance and hypoventilation. Previous studies already identified these clinical manifestations in acute GBS. However, prevalence of hypoventilation and inspiratory muscle weakness in subacute phase of GBS is still unknown. Early recognition and treatment of respiratory muscle weakness is important in people with GBS and physiotherapy interventions may be useful to successfully manage these patients.

Inspiratory muscle training (IMT) is a cheap and free of side-effects therapeutic modality, which consists of breathing exercises using a pressure threshold device, that has been applied in several diseases. In patients with chronic obstructive pulmonary disease, IMT improve inspiratory muscle function, exertional dyspnoea and exercise capacity. In patients with neuromuscular disease, has been suggested that IMT increases inspiratory muscle strength and lung volumes, especially in those with muscular dystrophies. In addition, a randomized control trial concluded that IMT in acute patients increases inspiratory muscle strength and quality of life. However, there are no studies about rehabilitation approaches, such IMT, to improve symptoms (i.e. dyspnoea or fatigue), pulmonary function, functional status and quality of life in people with GBS.

Rehabilitation, namely inpatient rehabilitation programmes, is therefore key for the recovery of symptoms, functional outcomes, community participation and quality of life of these highly complex patients. Nevertheless, insufficient high-quality literature, still precludes definitive conclusions about the effects of rehabilitation in people with GBS. Additionally, the interpretation of the magnitude of its effects is challenging due to the lack of minimal clinically important differences (MCIDs) for most outcome measures used in rehabilitation of people with GBS. A MCIDs is the smallest change in a measure that will be perceived as an important improvement for the patient. Such values constitute thresholds for clinical meaningfulness. Therefore, establishing MCIDs is essential to determine effectiveness of rehabilitation and guide clinical decision-making in the management of people with GBS.