Therapy of Guillain-Barre syndrome

Guillain-Barre syndrome (GBS) is clinically defined as an acute peripheral neuropathy causing limb weakness and disorder of sensation that progress over a time period of several days to 4 weeks. GBS is considered to be an autoimmune disease triggered by a preceding bacterial or viral infection. Immune system responses to target epitopes in Schwann cells or myelin result in demyelinations. In acute motor axonal neuropathy, a type of GBS, immune responses cross-react with axolema causing axonal degeneration. The prognosis is generally favorable, yet it is a serious disease with a mortality of 1.5%-10%, whereas severe neurologic deficits persist in some 20% of patients. Treatment of GBS is divided into two arms: 1) management of paralyzed patients with intensive care and vetilatory support, prevention of deep venous thrombosis and contractures, management of pain and autonomic dysfunction; and 2) immunomodulating therapy to reduce the progressive course of GBS, presumably by limiting nerve damage. Plasma exchange reduces the duration of disability in GBS in comparison with standard supportive treatment. Many neurologists use a protocol where a total of 200-250 ml/kg body weight are exchanged over 7-10 days. Two plasma exchanges are sufficient in mild cases, whereas four exchanges are preferable in patients with moderate and severe disease. Plasma exchange is most effective when it is used within 7-15 days of the disease onset. Intravenous immunoglobulins accelerate recovery as much as plasma exchange. Patients should be treated with immunoglobulins (0.4 g/kg body weight/day for 5 days) within 14 days of the onset of weakness. Intravenous immunoglobulins administered after plasma exchange failed to provide better results than plasma exchange alone. The mehanisms by which immunoglobulins exert their beneficial effect may include a combined effect of complement inactivation, neutralization of idiopathic antibodies, cytokine inhibition, and saturation of Fc receptors on macrophages. Corticosteroids, prescribed for many inflammatory and autoimmune disorders, did not prove efficacious in GBS. A combination of intravenous immunoglobulins and intravenous methylprednisolone showed no significant outcome difference. Yet, corticosteroids may be useful in treating severe nerve trunk pain.