[Pediatric Guillain-Barré syndrome. Experience in the Neuropediatrics Unit of a Portuguese hospital]. / Síndrome de Guillain-Barré en edad pediátrica. Experiencia de la Unidad de Neuropediatría de un hospital portugués.

INTRODUCTION: Guillain-Barré syndrome (GBS) is an acute inflammatory ascending polyradiculoneuropathy. Autoimmune mechanisms play a role in the demyelinating process. Clinically, progressive symmetric loss of motor strength, areflexia, sensitive and autonomic manifestations are observed. Albuminocytological dissociation and electrophysiological signs of demyelination are frequently found. It is the most common cause of acute flaccid paralysis in children. PATIENTS AND METHODS: Retrospective review of all children with GBS admitted to Garcia de Orta Hospital in a 10 year period (1994-2003). RESULTS: 17 children (18 months to 14 years) were admitted during this period. Respiratory or gastrointestinal prodrome was identified in 15 children, with positive serologic studies in six. The main clinical manifestations were disturbances of gait, progressive muscular weakness, sensitive manifestations (pain, paresthesias) and osteotendinous areflexia. 13 children had albuminocytological dissociation and acute demyelinating neuropathy was identified in 12.64% of children were treated with immunoglobulins (2 g/kg). Clinical evolution was favourable in 16 cases, with a death secondary to autonomic dysfunction. CONCLUSIONS: Clinical presentation may be unspecific, particularly in young patients, with pain as a primary complaint, preceding muscular weakness and areflexia. Increased cerebrospinal protein and abnormal electrodiagnostic studies may be absent in the early course of GBS. Immunoglobulin therapy was efficacious and well-tolerated.

Síndrome de Guillain-Barré en edad pediátrica. Experiencia de la Unidad de Neuropediatría de un hospital portugués / Pediatric Guillain-Barré syndrome. Experience in the neuropediatrics unit of a portuguese hospital

Introducción. El síndrome de Guillain-Barré (SGB) es unapolirradiculoneuropatía aguda de naturaleza autoinmune que semanifiesta mediante quejas sensitivas, debilidad muscular progresivasimétrica ascendente y arreflexia. Presenta habitualmente unaumento de la proteinorraquia sin pleiocitosis y los estudios electrofisiológicosdocumentan la existencia de una neuropatía aguda desmielinizantesubyacente. Constituye una urgencia neurológica en laedad pediátrica, y requiere una evaluación y orientación terapéuticaadecuadas. Pacientes y métodos. Estudio retrospectivo de los casosclínicos del SGB admitidos en el Servicio de Pediatría del HospitalGarcia de Orta entre enero de 1994 y diciembre del 2003. Resultados.Durante este período se ingresó a 17 niños, con edades comprendidasentre 18 meses y 14 años. Existió pródomo infeccioso en15 niños, con incremento de los niveles serológicos en seis de ellos.Las manifestaciones clínicas fueron alteraciones de la marcha, disminuciónde la fuerza muscular, manifestaciones sensitivas con predominiodel dolor y arreflexia. La disociación albuminocitológica sedocumentó en 13 niños y se encontraron niveles electrofisiológicosde neuropatía aguda en 12. Se suministró inmunoglobulina endovenosa(2 g/kg) a 11 niños. La evolución clínica fue globalmente favorable,aunque se registró un óbito por disfunción autonómica. Conclusiones.La presentación clínica del SGB puede ser inespecífica alinicio de la enfermedad, especialmente en los grupos de edad másjóvenes, en los que la debilidad muscular y la arreflexia son más difícilesde evaluar. Los exámenes complementarios presentan limitaciones,sobre todo en la primera semana de la enfermedad. El uso deinmunoglobulina fue eficaz y quedó exento de efectos secundariossignificativos

Introduction. Guillain-Barré syndrome (GBS) is an acute inflammatory ascending polyradiculoneuropathy. Autoimmunemechanisms play a role in the demyelinating process. Clinically, progressive symmetric loss of motor strength, arreflexia,sensitive and autonomic manifestations are observed. Albuminocytological dissociation and electrophysiological signs ofdemyelination are frequently found. It is the most common cause of acute flaccid paralysis in children. Patients and methods.Retrospective review of all children with GBS admitted to Garcia de Orta Hospital in a 10 year period (1994-2003). Results.17 children (18 months to 14 years) were admitted during this period. Respiratory or gastrointestinal prodrome was identified in15 children, with positive serologic studies in six. The main clinical manifestations were disturbances of gait, progressivemuscular weakness, sensitive manifestations (pain, paresthesias) and osteotendinous arreflexia. 13 children had albuminocytologicaldissociation and acute demyelinating neuropathy was identified in 12.64% of children were treated with immunoglobulins(2 g/kg). Clinical evolution was favourable in 16 cases, with a death secondary to autonomic dysfunction. Conclusions.Clinical presentation may be unspecific, particularly in young patients, with pain as a primary complaint, preceding muscularweakness and arreflexia. Increased cerebrospinal protein and abnormal electrodiagnostic studies may be absent in the earlycourse of GBS. Immunoglobulin therapy was efficacious and well-tolerated