title: [Ophthalmoplegia-ataxia-areflexia in pediatrics. Three new patients and review of the literature].
abstract: OBJECTIVE
  The objective of this study was to investigate if pediatric patients with benign brainstem encephalitis (Bickerstaff Syndrome) or with Miller-Fisher Syndrome are the extremes of the same nosological entity which, in adults, has been named ophalmoplegia-ataxia-areflexia syndrome.
  
  
  METHODS
  The subjects included in the study were three patients of our institution and 24 patients found in the revision of the English and Spanish pediatric literature who fulfilled the diagnostic criteria of ophtalmoplegia-ataxia-areflexia syndrome. The topographical location of the lesion in the nervous system was based on previously established criteria by using clinical and complementary studies.
  
  
  RESULTS
  Of the 27 patients included in the study we were able to reach an accurate topographical diagnosis in 9. None had an exclusive involvement of the peripheral nervous system, (6) had exclusively central nervous system involvement and 2 showed involvement of both system. In 12, the topographical location of the lesion could be only ascertained as probable; 3 of them in the peripheral nervous system, 2 in the central nervous system and mixed involvement in 7. In the remaining 7 patients there were insufficient clinical data to allow topographical classification.
  
  
  CONCLUSIONS
  The ophtalmoplegia-ataxia-areflexia syndrome can also be found in pediatric patients. The lesion in the majority of patients in this age group is located in the central nervous system, either alone or combined with peripheral nervous system involvement.
authors.affiliation: Sección de Neurología Infantil, Hospitals Vall d'Hebron de Barcelona.
authors.forename: A
authors.initials: A
authors.lastname: Fargas i Busquets
authors.forename: M
authors.initials: M
authors.lastname: Roig Quilis
authors.forename: M
authors.initials: M
authors.lastname: Gratacòs Vinyola
authors.forename: A
authors.initials: A
authors.lastname: Macaya Ruiz
authors.forename: M
authors.initials: M
authors.lastname: del Toro Riera
authors.forename: A
authors.initials: A
authors.lastname: Fitó Costa
mesh_terms: D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D008297:Male; D019846:Miller Fisher Syndrome / Q000175:diagnosis*
vernacular_title: Oftalmoplejía-ataxia-arreflexia en pediatría. Tres nuevos pacientes y revisión de la literatura.