Physician explains best ways to approach optic neuritis cases

Karl C. Golnik, MD, discusses various causes, presentations and treatments of the condition.

John A. Hovanesian, MD: Today we’re joined by Karl C. Golnik, MD, a professor of ophthalmology, neurology and neurosurgery at the University of Cincinnati and the Cincinnati Eye Institute. Dr. Golnik, thanks for joining us.

John A. Hovanesian

Dr. Golnik: My pleasure.

Dr. Hovanesian: We are going to talk about optic neuritis, with emphasis on new information in treating this disorder. Dr. Golnik, is optic neuritis one condition, or is there more than one presentation or a variety of this type of condition?

Dr. Golnik: I usually split these patients up into what I would call typical optic neuritis and atypical optic neuritis. You are going to encounter many more of the typical optic neuritis patients, but it is important to realize that not all optic neuritis is typical optic neuritis.

Examination

Dr. Hovanesian: How would you approach a patient with typical optic neuritis?

Dr. Golnik: In either group, I think you are going to examine the patient to make sure that they have an optic nerve problem, of course. You will look at visual acuity, color vision and presence of a relative afferent pupillary defect. Some form of perimetry is important to document the level of optic nerve involvement. In the patient with typical optic neuritis, you expect to see either retrobulbar optic neuritis with no disc swelling or mild, at most, disc swelling in about one-third of patients.

Dr. Hovanesian: And so the disc swelling is one of the features that would differentiate typical from atypical.

Dr. Golnik: I think that the atypical optic neuritis patient may look much like the typical patient. But when you look at the fundus, the one good clue to remember is if you see more than just mild optic disc swelling, that is, swelling with hemorrhage or exudate, or moderately or worse swollen nerve, you have to really think this is not the typical optic neuritis that we encounter frequently. And then you have to think about other diagnostic possibilities.

Dr. Hovanesian: For the work-up of a patient with fairly typical findings of reduced vision, perhaps a mild afferent pupillary defect, perhaps mild disc swelling and not a great deal of hemorrhage or exudate, what sort of work-up beyond what you mentioned would you recommend?

Dr. Golnik: One other point I would mention is, it used to be thought that patients with optic neuritis had to have loss of central vision. I think it is important to note that you can certainly have optic neuritis and have a field defect and not have loss of central acuity, so don’t use that as a necessary diagnostic criteria. The evaluation would then be to order an MRI of the brain and orbit with administration of gadolinium to look for an enhancement of the optic nerve, which typically is present in optic neuritis, and to look for plaques, which are white spots in the white matter of the brain.

Dr. Hovanesian: The purpose for scanning the orbits as well is to just focus in on the enhancement of the nerve itself?

Dr. Golnik: It is. In the typical scenario of acute or subacute visual loss in a patient of this age, it would be rare to find something other than enhancement of the nerve. In other words, you are not really doing it to rule out tumor in that setting. It would be rare to find something other than optic neuritis, but you do expect, in most cases, to see enhancement of the nerve.

Dr. Hovanesian: Would you perform an MRI in any patient with a suspected diagnosis of optic neuritis?

Dr. Golnik: Yes.

Dr. Hovanesian: What about patients who have had optic neuritis in the past?

Dr. Golnik: It depends a bit, but most likely, I would. Now, that gets into a little bit about whether the patient has been diagnosed with multiple sclerosis or not. Are they being treated for MS and now having another episode? It is sort of a tough question to answer. I think if the patient comes in with a second episode and has been followed by a neurologist and is being treated for MS, then it depends a bit on when the last MRI was done. It gets a little bit more difficult to make a blanket recommendation.

Dr. Hovanesian: In other words, you are looking with an MRI in a patient with MS for progression of the MS.

Dr. Golnik: Sure. Or sometimes we are looking at someone who had optic neuritis in the past. Let’s say they had a normal MRI and don’t have a diagnosis of MS. An MRI 6 months down the road and after another episode showing plaques would be telling and will most likely lead then to a diagnosis of MS if they are new plaques.

Dr. Hovanesian: Any other work-up you would recommend for typical optical neuritis?

Dr. Golnik: There really isn’t. The Optic Neuritis Treatment Trial looked at ancillary testing, chest X-ray and blood tests in the setting of a healthy patient with optic neuritis. Those tests were not shown to be of any help.

Dr. Hovanesian: What you are calling atypical optic neuritis, is that a presentation that suggests the possibility of other diagnoses besides optic neuritis?

Dr. Golnik: Yes. If it initially looks like optic neuritis, and you look at the fundus and find a moderately swollen nerve and/or hemorrhagic exudate or exudate in the macula, immediately you need to switch your diagnostic approach because this patient does not have MS or idiopathic optic neuritis. The patient probably has something such as sarcoidosis or cat scratch disease. They would be the two most common entities, at least that you would see in the United States, that might cause that picture.

Dr. Hovanesian: Hypertensive optic neuropathy or retinopathy also would.

Dr. Golnik: Of course, but usually bilateral. But yes, you are going to want to check blood pressure in the patient.

Dr. Hovanesian: Any other diagnostic suggestions for the approach to the patient with atypical findings?

Dr. Golnik: I think the other thing would be that not every patient who has that presentation has optic neuritis. As I mentioned earlier, most people will have optic neuritis, but if it is atypical and you do not have a good answer for the optic neuritis, you are going to want to get an MRI as well to look at the optic nerve because certainly a compressed lesion in the orbit, for instance, can cause optic swelling. Also look at the patient’s history. It may seem that there is an abrupt loss of vision, but maybe it has been going on for a while and this was more of an abrupt recognition of the loss of vision. So I think there is a place for imaging in the atypical optic neuritis patient if you don’t get an obvious answer from your initial blood testing and chest X-ray looking for sarcoid and cat scratch. I think the imaging of the orbit is, of course, important as well.

Treatment

Dr. Hovanesian: When it comes to treatment of optic neuritis, how has the Optic Neuritis Treatment Trial changed our ways of thinking?

Dr. Golnik: Back before the Optic Neuritis Treatment Trial started, surveys were done to poll neurologists and ophthalmologists as to how they treated patients with optic neuritis. About 30% of ophthalmologists treated patients with oral prednisone, and about 90% of neurologists treated patients with oral prednisone. The Optic Neuritis Treatment Trial, which was completed in the early ’90s, looked at three arms: intravenous Solu-Medrol (methylprednisolone sodium succinate, Pfizer) at 1 g per day for 3 days followed by a 2-week oral prednisone taper vs. just oral prednisone vs. oral placebo. And what researchers found in that study was that none of the treatments helped the ultimate recovery of vision in these patients. The intravenous steroids did help the rate of recovery, but if you look at people even a couple of months later, there was no difference in their recovery of vision in the group treated with IV steroids vs. oral placebo. They also showed a rather unexpected finding, which is that oral prednisone alone was actually contraindicated and resulted in more frequent attacks of optic neuritis. The reasoning behind that is unclear, but the data has sort of been consistent at the 5-, 10- and 15-year follow-up points of the trial. So we feel at this point that oral steroids alone are contraindicated in the treatment of typical optic neuritis.

Dr. Hovanesian: But they are used following an initial pulse with IV steroids?

Dr. Golnik: That’s right. We don’t really know why that is, but it is OK to use the oral steroids if they are preceded by Solu-Medrol 1 g per day for 3 days. There is a Japanese study that has looked at large doses of oral steroids, and that shows promise. So maybe it is not just the administration route, but the actual dosage that is initiated.

Dr. Hovanesian: Do you have a protocol for treatment that you apply to your patients who are diagnosed with optic neuritis?

Dr. Golnik: Yes. The first question is, are you going to treat them? There are two primary reasons that you are going to treat patients with typical optic neuritis. One is if the patient wants their vision to recover more quickly, then I offer them intravenous Solu-Medrol. I do this through home health care. I have never admitted a patient for IV steroids. They are usually young and healthy, so we treat them with home health care. They get 1 g of Solu-Medrol over 30 to 60 minutes per day for 3 days and then an oral prednisone taper, which is about 1 mg per kg per day, and then the last for 10 days followed by a rapid taper over 4 days, usually 20 mg per day for 1 day, 10 mg per day for 1 day, nothing for 1 day, 10 mg per day for 1 day and then stop.

Dr. Hovanesian: So a taper over a fairly short period of time.

Dr. Golnik: Right. The other reason we get the MRI of the brain is to look for these plaques because we know from the Optic Neuritis Treatment Trial that the presence of even one plaque greatly increases the risk of developing MS. In fact, if you look at the 15-year data from the Optic Neuritis Treatment Trial, if there was just one plaque, 75% of those patients went on to develop MS in that 15-year period, whereas if there were no plaques, 25% of the patients developed MS. So the other reason to give the intravenous steroids is that the Optic Neuritis Treatment Trial showed that the patients who get the intravenous Solu-Medrol initially are less likely to develop MS over the subsequent 2 years. That effect wears off at year 3 and on, but for the first 2 years after the attack, people were less likely to develop MS. And although 2 years is not a long period of time in someone’s life, if you tell patients, “This might decrease your risk of developing MS over the next 2 years,” the patient usually wants the medicine.

Dr. Hovanesian: Are you more prone to recommend intravenous steroid treatment in patients with more severe optic neuritis, or is your treatment recommendation the same?

Dr. Golnik: My treatment recommendation is pretty much the same.

Dr. Hovanesian: MS presents with a variety of severity and develops over many years. How do you counsel patients who have optic neuritis, with or without a plaque on their MRI, as to their future risk? What general advice do you give them?

Dr. Golnik: Certainly you tell them that having the plaque puts them at high risk, about 75%, of developing MS. There are multiple studies that have looked at the immunomodulating agents, such as beta interferons and glatiramer acetate, and have shown that if you treat patients who are at high risk of developing MS — high risk would be one episode of optic neuritis and two or more plaques on an MRI, which is the way the protocols in the studies were done — that those patients also are less likely to develop MS. So now, we have a patient with optic neuritis who is presenting to the ophthalmologist who gets an MRI and it shows a couple of plaques. That patient needs to be referred to a neurologist for a discussion of MS and a discussion of these neo-modulating agents. These patients will often be started on a medication for MS to try to prevent MS from occurring.

Dr. Hovanesian: Based on the new information in recent years from the Optic Neuritis Treatment Trial and elsewhere, what general recommendations would you give to an ophthalmologist? How would it affect the clinical practice of a general ophthalmologist?

Dr. Golnik: I think there are a few big things. No. 1, don’t treat these patients with oral steroids alone. That is contraindicated. That is not something that we do any longer. The second thing, when I was in training, the big question was whether or not we should even tell patients about MS because there was no treatment. Nowadays, we have treatment, and so the question is, if you have somebody with an isolated optic neuritis who has a plaque on their MRI, do you start them on these expensive neo-modulating agents? And so I think that the big change has been that all of these patients need to see a neurologist, especially if they have one or more plaques on their MRI because the neurologist may well recommend one of these neo-modulating agents. I think that is something the comprehensive ophthalmologist needs to keep in mind, that they definitely need a referral and a discussion with the neurologist about potential treatment, to either decrease the risk of developing MS or to treat them for the MS they already have.

Dr. Hovanesian: That sounds like great advice. Dr. Golnik, thanks very much for joining us.

• Karl C. Golnik, MD, can be reached at Cincinnati Eye Institute, 1945 CEI Drive, Cincinnati, OH 45242; 513-984-5133; fax: 513-984-4494; e-mail: karl.golnik@uc.edu.
• John A. Hovanesian, MD, FACS, can be reached at Harvard Eye Associates, 24401 Calle De La Louisa, Suite 300, Laguna Hills, CA 92653; 949-951-2020; fax: 949-380-7856; e-mail: drhovanesian@harvardeye.com.