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Classification of Hennermann et al for a Patient with Glycine Encephalopathy (GE, Nonketotic Hyperglycinemia)

Description

Hennermann et al classified patients with glycine encephalopathy into prognostic groups. The authors are from Charite Universitatsmedizin Berlin, Medizinische Einrichtungen des Bezirks Oberpfalz, Max-Delbruck Center for Molecular Medicine, University of Colorado and Katholieke Universiteit Leuven.


Patient selection: glycine encephalopathy

 

Parameters:

(1) intelligence quotient (IQ) as a measure of developmental outcome

(2) age of onset

 

Intelligence Quotient

Onset

Group

< 20

during first week of life (neonatal)

severe neonatal

< 20

during infancy (after first week of life)

severe infantile

>= 20

during first week

attenuated neonatal

>= 20

during infancy (after first week)

attenuated infantile

 

A patient with the severe phenotype had a poor outcome. A patient with an attenuated phenotype might show a less severe outcome over time.


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