Re:
so Jeffrey west gets goofy sleepy confused dazed every single time he eats anything, sugars worse than other, he has live on rice and beans, they say he is also severely hypoglycemic. 20 and under. mayo couldn't figure it out, he has been to sleep studies, mri, blood up the kazoo no answer. probably stimulating the vagus nerve.
It's hard to know if his encephalopathy is a primary symptom or the result of hypoglycemia. Anyway — was thinking through weird causes of hypoglycemia. Best way to think about it is:
Either his body doesn't know how to regulate basic metabolism (reactive hypoglycemia)
He has fasting hypoglycemia
He's under-producing glucose
There are only these three paths (above) to hypoglycemia. I've excluded the common stuff or the "not likely" stuff since I'm sure Mayo has already tried those things.
If his hypoglycemia is postprandial (after meals), then there are some very real things to think of in the category of reactive hypoglycemia — some are straightforward mechanical and the others are errors of metabolism:
Postoperative rapid gastric emptying (alimentary hyperinsulinism)
Galactosemia
Leucine intolerance
His diet sounds varied to me. Beans and rice means protein and carbohydrates are tolerated to some degree. If it is a primary structural issue (like a glucose transporter gene mutation in the brain GLU-1 or GLU-3) or simple sugar or carbohydrate intolerance, you'd think he would do really well on a ketotic diet, like super Atkins. Has he tried that?
You gotta wonder too about what happens when he fasts. Is that when he gets hypoglycemic? Cancer seems unlikely since he's had this his whole life. Antibodies or weird islet cells could be the case.
Carnitine is really interesting, and he can buy it over the counter and give it a try.
Islet cell disorders (adenoma, nesidioblastosis, cancer) seem unlikely given chronicity
Antibodies to endogenous insulin
Carnitine deficiency
Finally, he might be under-producing glucose. There are tons of tests for this and I bet Mayo has run most of them.
Glycogen metabolism (glycogen phosphorylase, glycogen synthetase)
Hexose metabolism (glucose-6-phosphatase, fructose-1,6-biphosphatase)
Glycolysis, Krebs cycle (phosphoenolpyruvate carboxykinase, pyruvate carboxylase, malate dehydrogenase)
I guess to start we'd need to know:
...When is he hypoglycemic?
...Has he tried a protein / ketotic diet?
...Has he tried supplementation with carnitine?
...Do any of these things look familiar to him?
...Does he get googly eyes after eating? Or even while sleeping?
Interesting case. Between you and I — I bet we can figure it out.
