Prolonged Guillain-Barré syndrome

• Published in: Anales de pediatría (Barcelona, Spain : 2003) Spain : 2003), 2003-01, Vol.58 (1), p.74-76
• Main Authors: Criado Molina, A, Pérez Navero, J L, Frías Pérez, M A, Antón Gamero, M, Ibarra de la Rosa, I
• Format: Article
• Language: Spanish
• Subjects: Child; Disease Progression; gamma-Globulins - therapeutic use; Guillain-Barre Syndrome - drug therapy; Humans; Male; Time Factors
• ISSN: 1695-4033

Outcome in Guillain-Barré syndrome (GBS) in childhood is usually favorable although prolonged and severe forms may develop. We report the case of a 12-year-old boy with rapidly ascending, slowly-progressive GBS with severe cranial nerve and autonomic dysfunction. Diagnosis was based on clinical presentation, electromyography, cerebrospinal fluid analysis and plasmatic antiganglioside GM1 antibodies. Early treatment with an initial loading dose of 2 g/kg of intravenous gammaglobulins over 5 days was given with a significant but slow clinical improvement. A repeat infusion of gammaglobulins was given two months later. Response was satisfactory with motor, sensory and autonomic dysfunction recovery. A third course of immunoglobulins 28 days later produced little benefit. At the present time, 5 months after the onset of the disease, flaccid paralysis of the lower limbs and the distal portion of the upper limbs persists but autonomic instability has disappeared. The patient has been decannulated and is following an ambulatory rehabilitation program. Although repeated intravenous immunoglobulins may be useful in the treatment of prolonged GBS, the key issue is excellent intensive care unit management. Further research examining potentially more effective treatment such as beta-interferon and immunosuppressive agents is required.