| Affiliation |
(AGL) Chairman, Department of Ophthalmology, The Methodist Hospital, Houston, Texas; Professor of Ophthalmology, Weill Cornell Medicine, New York City, New York; (KL) Baylor College of Medicine, Houston, Texas |
| Transcript |
Okay, so today we're going to be talking about what a neuro ophthalmologist needs to know about familial hyperlipidemia syndromes and it could be increased triglyceride increased cholesterol, or both. As you know, these familial hyperlipidemias are classified by Type: I, II, III, IV, and V. Each has its own lipoprotein problem. Lipoproteins, as you know, are the carrier proteins that carry the cholesterol -a triglyceride absorbed in the gut passed to the liver and then make their way around the blood circulation to be delivered to their definite targets. The size kind-of matters. The very big thing, chylomicron, and then you've got various sized densities of lipoproteins: a very low-density lipoprotein, and in between one intermediate-density lipoprotein, a high-density lipoprotein and a low-density lipoprotein. Of course this is also known as the bad cholesterol because he's carrying it around in the blood. Then you've got the good cholesterol he's a good guy because he is actually carrying the lipid back to the liver to get it out of your system and metabolized. The LDL, also a bad guy. Chylomicron, also a bad guy also. So you need them to carry the stuff but some are worse than others and the higher your HDL -that's the good Cholesterol. Type I, II, III, IV, V: The most common are Type II and Type 4. Type II actually has two types: Type IIa and Type IIb. This is the familial hypercholesterolemia. The problem in this one is LDL, and in the Type IIb it's both -LDL and VLDL. This one has hypercholesterol. This one has hypercholesterol, but also high triglyceride. In contrast the familial hypertriglyceridemia is the Type IV, that's just the VLDL. So these two are the main ones you need to be worried about. Type I is chylomicrons. Type III is the intermediate-density lipoprotein. Type V is kind of a combination of Type I and Type IV which is the LDL and chylomicrons. These are very uncommon and so really we should really be concentrating on II and IV and between the II: IIa and IIb, IIb is really way more common. This comes to us in various ways: it can come to us as arcus in the cornea -a ring of white or yellow. And that is the deposition of the lipid, usually bilateral and symmetric in a younger person. Arcus, in an older person that's called ‘arcus senilis' -but you should check their cholesterol, if they have no risk factor or they're young. We're gonna be looking for xanthelasma which are on the lid and so their yellow plaque-like on the lid that is also cholesterol. If they're elsewhere in the body we call those a xanthoma but they're the same stuff. In the back of the eye, lipemia retinalis which instead of the retinal blood column being red, it's milky-white because it's so full of chylomicrons. So arcus, xanthelasma, xanthom, lipemia retinalis. You should be thinking about familial hyperlipidemias in patients who have had stroke and heart attack ischemic thirds non-artery KION. We also want to check their cholesterol. If we've got big numbers like 290...300 kind of cholesterols, those are the people we really should be thinking about the familial hypercholesterolemia, or the familial hypertriglyceridemia, or the combined hypercholesterolemia and hyper triglyceridemia. So, be on the lookout for the familial hyperlipidemia syndromes in the cornea, arcus xanthelasma, lipemia retinalis and in patients who have stroke and the young. |
