Table 1.

Summary of clinical data for the patients in this study.

Patient	Age (at Time of Study Conduction)	Clinical Presentation *	Brainstem Evoked Auditory Potential–BEAP	Visual Evoked Potential–VEP	EEG Findings	Radiological Studies (MRI or CT)	Treatment
1	3 Years	Neonatal seizures, mental retardation, developmental delay	No response bilaterally	Normal	Generalized epileptiform discharges	Normal MRI	Failed medical therapy, surgical intervention performed
2	8 Years	Neonatal seizures	Normal	Normal	Normal	Normal MRI	Responded to medical therapy
3	8 Years	Neonatal seizures, mental retardation, developmental delay	Normal	Normal	Severe abnormal epileptiform discharges	Normal MRI	Failed medical therapy, surgical intervention performed
4	4 Years	Neonatal seizures, mental retardation, developmental delay	Normal	Normal	Slow background activity	Normal MRI	Failed medical therapy, surgical intervention performed
5	11 Years	No additional clinical symptoms	Normal	Normal	Not done	None done	Responded to medical therapy
6	6 Months	Neonatal seizures, developmental delay	Not done	Not done	Generalized epileptiform discharges	Normal MRI	Failed medical therapy, surgical intervention performed
7	14 Years (Twin)	Neonatal seizures, mental retardation, developmental delay	Normal	Normal	Normal	Normal MRI	Failed medical therapy, surgical intervention performed
8	14 Years (Twin)	Neonatal seizures, mental retardation, developmental delay	Normal	Normal	Normal	Normal MRI	Failed medical therapy, surgical intervention performed
9	2 Years	Developmental delay	Not done	Not done	Not done	Normal CT	Responded to medical therapy
10	3 Years	Neonatal seizures, mental retardation, developmental delay	Not done	Not done	Generalized epileptiform discharges	Normal MRI	Responded to medical therapy
11	9 Years	Neonatal seizures, mental retardation, developmental delay, deafness, blindness	No response bilaterally	No response	Generalized epileptiform discharges	MRI: Severe brain atrophy	Failed medical therapy, surgical intervention performed
12	7 Years	Neonatal seizures, mental retardation, developmental delay, deafness, blindness	No response bilaterally	No response	Generalized epileptiform discharges	MRI (brain): Delayed Myelination	Some response to medical therapy - Surgery refused by family
13	6 Years	Neonatal seizures, mental retardation, developmental delay, decreased hearing and vision	No response bilaterally	Normal	Generalized epileptiform discharges	Normal MRI	Failed medical therapy, surgical intervention performed
14	7 Years	Developmental delay	Normal	Not done	Not done	None done	Failed medical therapy, surgical intervention performed

* In addition to neonatal persistent hypoglycemia and Intolerance to fast.