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. Author manuscript; available in PMC: 2016 Nov 1.
Published in final edited form as: Pediatr Neurol. 2015 Aug 10;53(5):379–393. doi: 10.1016/j.pediatrneurol.2015.08.001

TABLE 4.

Commonly held assumptions and facts in G1D

Assumption Fact References
The blood-brain barrier is defective Synaptic abnormalities are independent of the blood-brain barrier [9, 22]
G1D is an energy failure syndrome The tricarboxylic acid cycle is preserved in the mouse G1D brain [7]
CSF/blood glucose ratio is diagnostic The denominator is excessively dependent upon non-disease related factors
CSF glucose derives from brain tissue CSF glucose is primarily excreted by the choroid plexus [47]
Genotype – phenotype correlations indicate a continuum of a single syndrome Phenotypes diverge widely and constitute in effect separable syndromes
Seizures are manifest EEG can reveal a large number of nonapparent seizures [118]
Ketone bodies (betahydroxybutyric and acetoacetic acids) are a complete alternative energetic source These ketone bodies supply energy but do not refill tricarboxylic acid precursors lost in the course of normal metabolism [15]
FDG-PET reflects glucose uptake FDG-PET represents radioactive atom accumulation (influx minus effux)