BACKGROUND: @entity4269 in @entity1 , especially generalized disease with @entity2652 , is rare. OBJECTIVE: We sought to report 3 childhood cases of @entity4269 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 @entity1 had @entity4269 of all 4 limbs and the face. This was confirmed by lymphoscintigraphy, which showed @entity509 of vessels and hypofixation of lymph nodes. They had nonspecific @entity16 and electroencephalography abnormalities, without intellectual deficit. Neuroimaging revealed normal findings. Microcomparative genomic hybridization in 1 @entity1 revealed no cytogenetic anomaly. The outcome was fatal in 1 case with development of @entity10478 and @entity772 . LIMITATIONS: Genetic studies were performed in only 1 case. CONCLUSION: These observations suggest that neurologic assessment and electroencephalography are indicated for @entity1 with @entity4269 of the limbs and face to identify this syndrome.

Generalized XXXX associated with @entity16 : A new entity?

@entity1 :: ('9606', 'Species') :: ['children', 'patients', 'patient'] @entity10478 :: ('OMIM:155310', 'Disease') :: ['visceral lymphedema'] @entity2652 :: ('MESH:D005148', 'Disease') :: ['facial involvement'] @entity4269 :: ('MESH:D008209', 'Disease') :: ['Primary lymphedema', 'lymphedema', 'primary lymphedema'] @entity16 :: ('MESH:D009422', 'Disease') :: ['neurologic disorders'] @entity772 :: ('MESH:D003128', 'Disease') :: ['coma'] @entity509 :: ('MESH:C538429', 'Disease') :: ['hypoplasia']
@entity4269 :: (MESH:D008209,Disease) :: ['lymphedema']
