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Journal of the American Academy of Dermatology
Volume 72, n° 2
pages 333-339 (février 2015)
Doi : 10.1016/j.jaad.2014.10.033
accepted : 24 October 2014
Original Articles

Generalized lymphedema associated with neurologic signs (GLANS) syndrome: A new entity?
 

Marine Berton, MD a, b, Gérard Lorette, MD a, b, Françoise Baulieu, MD a, c, Emmanuelle Lagrue, MD, PhD a, d, f, Sophie Blesson, MD e, Frédéric Cambazard, MD, PhD g, Loïc Vaillant, MD a, b, f, Annabel Maruani, MD, PhD a, b, f,
a University François Rabelais, Tours, France 
b Department of Dermatology, Centre Hospitalier Régional Universitaire, Tours, France 
c Department of Nuclear Medicine, Centre Hospitalier Régional Universitaire, Tours, France 
d Department of Pediatric Neurology, Centre Hospitalier Régional Universitaire, Tours, France 
e Department of Genetics, Centre Hospitalier Régional Universitaire, Tours, France 
f Inserm U930, Tours, France 
g Department of Dermatology, University Hospital of Saint-Etienne, France 

Reprint requests: Annabel Maruani, MD, PhD, Department of Dermatology, Centre Hospitalier Régional Universitaire Tours–Hospital Trousseau, Avenue de la République–37044, Tours Cedex 9, France.
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.

Limitations

Genetic studies were performed in only 1 case.

Conclusion

These observations suggest that neurologic assessment and electroencephalography are indicated for patients with lymphedema of the limbs and face to identify this syndrome.

The full text of this article is available in PDF format.

Key words : epilepsy, lymphatic, lymphedema, neurology, primary lymphedema, seizure

Abbreviations used : CSF, EEG, ISF



 Funding sources: None.
 Conflicts of interest: None declared.



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