Scores of Hennermann et al for Evaluating a Patient with Glycine Encephalopathy (GE, Nonketotic Hyperglycinemia): Outcome Score

Description

Hennermann et al developed 2 scores for evaluating patients with glycine encephalopathy. The outcome score is associated with the clinical outcome. 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, age > 18 months

Parameters:

(1) smiling

(2) head control

(3) grasp

(4) sitting

(5) walking

(6) speaking

Parameter	Finding	Points
smiling	absent	0
	present	1
head control	absent	0
	present	1
grasp	absent	0
	present	1
sitting	absent	0
	present	1
walking	absent	0
	present	1
speaking	absent	0
	present	1

where:

• The authors stipulate that at least 5 of the 6 parameters should be reported.

severity score =

= SUM(points for all 6 parameters)

Interpretation:

• minimum score: 0

• maximum score: 6

• The higher the score the better the outcome.

• Children with severe glycine encephalopathy had a low outcome score (range 0 to 3).

• Children with attenuated glycine encephalopathy had a higher outcome score.

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Scores of Hennermann et al for Evaluating a Patient with Glycine Encephalopathy (GE, Nonketotic Hyperglycinemia): Outcome Score

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