title: Generalized lymphedema associated with neurologic signs (GLANS) syndrome: a new entity?
abstract: BACKGROUND
  Primary lymphedema in children, especially generalized disease with facial involvement, is rare.
  
  
  OBJECTIVE
  We sought to report 3 childhood cases of lymphedema with associated neurologic findings and to provide a pathophysiologic explanation for this association.
  
  
  METHODS
  Clinical observations, electroencephalography, and neuroimaging studies were evaluated. Microcomparative genomic hybridization was performed in 1 case.
  
  
  RESULTS
  The 3 children had primary lymphedema of all 4 limbs and the face. This was confirmed by lymphoscintigraphy, which showed hypoplasia of vessels and hypofixation of lymph nodes. They had nonspecific neurologic disorders and electroencephalography abnormalities, without intellectual deficit. Neuroimaging revealed normal findings. Microcomparative genomic hybridization in 1 patient revealed no cytogenetic anomaly. The outcome was fatal in 1 case with development of visceral lymphedema and coma.
  
  
  CONCLUSIONS
  Genetic studies were performed in only 1 case.
  
  
  CONCLUSIONS
  These observations suggest that neurologic assessment and electroencephalography are indicated for patients with lymphedema of the limbs and face to identify this syndrome.
authors.affiliation: University François Rabelais, Tours, France; Department of Dermatology, Centre Hospitalier Régional Universitaire, Tours, France.
authors.forename: Marine
authors.initials: M
authors.lastname: Berton
authors.affiliation: University François Rabelais, Tours, France; Department of Dermatology, Centre Hospitalier Régional Universitaire, Tours, France.
authors.forename: Gérard
authors.initials: G
authors.lastname: Lorette
authors.affiliation: University François Rabelais, Tours, France; Department of Nuclear Medicine, Centre Hospitalier Régional Universitaire, Tours, France.
authors.forename: Françoise
authors.initials: F
authors.lastname: Baulieu
authors.affiliation: University François Rabelais, Tours, France; Department of Pediatric Neurology, Centre Hospitalier Régional Universitaire, Tours, France; Inserm U930, Tours, France.
authors.forename: Emmanuelle
authors.initials: E
authors.lastname: Lagrue
authors.affiliation: Department of Genetics, Centre Hospitalier Régional Universitaire, Tours, France.
authors.forename: Sophie
authors.initials: S
authors.lastname: Blesson
authors.affiliation: Department of Dermatology, University Hospital of Saint-Etienne, France.
authors.forename: Frédéric
authors.initials: F
authors.lastname: Cambazard
authors.affiliation: University François Rabelais, Tours, France; Department of Dermatology, Centre Hospitalier Régional Universitaire, Tours, France; Inserm U930, Tours, France.
authors.forename: Loïc
authors.initials: L
authors.lastname: Vaillant
authors.affiliation: University François Rabelais, Tours, France; Department of Dermatology, Centre Hospitalier Régional Universitaire, Tours, France; Inserm U930, Tours, France. Electronic address: annabel.maruani@univ-tours.fr.
authors.forename: Annabel
authors.initials: A
authors.lastname: Maruani
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D004569:Electroencephalography; D004827:Epilepsy / Q000175:diagnosis*; D005121:Extremities; D005145:Face; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D008209:Lymphedema / Q000151:congenital / Q000175:diagnosis* / Q000628:therapy; D008297:Male; D009422:Nervous System Diseases / Q000175:diagnosis; D059906:Neuroimaging; D012893:Sleep Wake Disorders / Q000175:diagnosis*; D014057:Tomography, X-Ray Computed
keywords: epilepsy; lymphatic; lymphedema; neurology; primary lymphedema; seizure