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Show BEST CATCH FROM NANOSNET Section Editors: Wayne T. Cornblath, MD, and Preston C. Calvert, MD Editor's note: In this section, we have excerpted and edited some of the choice interchanges that have transpired during the past six months on the NANOS- sponsored electronic mail discussion list known as NANOSNET. Started in 1996, NANOSNET is open to physicians and selected allied professionals who are actively working in neuro- ophthalmology. It is not restricted to NANOS members. A Web page with more information about the NANOSNET list, including instructions on subscribing, is located at: http:// www. nanosweb. org/ members/ nanosnet. htm. We have initiated this section to give neuro- ophthalmologists around the world a sense of how lively ( and sometimes silly) a free- flowing exchange can be. Wayne T. Cornblath, MD, and Preston C. Calvert, MD, the section editors, have made the selections and offer a commentary after each exchange. The names of the participants have been deleted to protect confidentiality. Topic I. Giant Cell Arteritis Around the World Asker: I was under the impression, mistakenly I now know, that patients of Asian background do not get giant cell arteritis ( GCA). I have examined a man today whose origins are from northern China, and he had the full- blown picture: headaches, jaw claudication, shoulder pain, polymyalgia rheumatica, and elevated sedimentation rate. He has a floridly positive artery biopsy, even grossly occluded. I did find one case in the Indian literature- a young woman in her late 40 ' s with biopsy proven GCA. Do any of you have experience with giant cell arteritis in Asians? Responder 1: Some Arabians are Asians. I have seen GCA in my home country of Jordan. Responder 2: Actually, the ancient silk route caused a mingling of HLA types from the Far East through the Middle East ( Ohno reported this as an explanation for shared diseases such as Vogt- Koyanagi- Harada, Behcet's); it is certainly true that geography and religion have determined it otherwise. Asker: Some of the " northern" Chinese in ancient times had links across to Lapland, of all places, according to my learned patient, who has a PhD in Chinese history. He researched GCA and came to the conclusion that he himself had it. Responder 3: We've seen quite a few GCA biopsy-positives among Cubans, notwithstanding the West Coast report ( see " Comments" below). Responder 4: There may have been a discussion of GCA in Asians in " Letters to the Editor" in the New England Journal of Medicine in the late 1970' s. As I recall, there were some other cases reported. PubMed does not list these letters, so a manual search may be necessary. The following reference showed up on my PubMed search: Giant cell arteritis in two Chinese patients. Wing YK, Kay RL, Lai FM. ( Department of Psychiatry, Chinese University of Hong Kong). AustNZJMed 1991; 21: 751- 2. Abstract: Giant cell arteritis is virtually unknown in Orientals. We report two cases of histologically proven giant cell arteritis in two elderly Chinese men, one of whom presented with typical symptoms and the other with occult features of the disease. Responder 5: You spurred me to take a quick look at my recent experience. Looking at 200 biopsies I have done over a 5- year period, I have 30 positive. None has an Asian name. One has a Hispanic name. A Czech physician told me that they don't do temporal artery biopsies because they don't see GCA in the Czech Republic. We see what we look for! Responder 6: One thought about the likelihood of seeing GCA in Asians is the average life expectancy in this group. The other is the dilutional factor of looking for this condition in Asians who happen to live to a ripe old age in the United States. It was not very many years ago that the community wisdom was that GCA did not occur in African Americans, and I do not think anyone believes that now. Responder 7: Many years ago, I co- authored a paper in the American Journal of Medicine on GCA in African Americans. I called the census bureau to try to get race- specific survival rates to ages 60, 70, and 80 years to see if we could make that point with the help of the statisticians. They would not release the data to me! Longevity may be a relevant point in many countries that do not report GCA. Responder 8: I have seen two African American, one Asian Indian, and one Hispanic case out of around 100 GCA- positive biopsies. Responder 9: Our population is mainly Hispanic, and we do see GCA with relative frequency. J Neuro- Ophthalmol, Vol. 22, No. 1, 2002 51 Copyright © Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited. JNeuro- Ophthalmol, Vol. 22, No. 1, 2002 BEST CATCH FROM NANOSNET Responder 10: I practice in a country that has a predominant Chinese population. GCA is very uncommon here, but I did report one elderly Chinese lady with bilateral sequential anterior ischemic optic neuropathy ( AION) with a raised erythrocyte sedimentation rate ( ESR) and a positive temporal artery biopsy. Unfortunately, her vision did not recover. Goh KY, Lim TH. Giant cell arteritis causing bilateral sequential anterior ischemic optic neuropathy- a case report. Singapore Medical J 2000; 41: 32- 3. Comments: An observation and question posed by a physician about giant cell arteritis in an Asian patient led to a discussion of human leukocyte antigen ( HLA) types and ancient migration patterns, sources of demographic bias, and encounters with giant cell arteritis in other ethnic groups. The comment about " West Coast experience with GCA " refers to a paper entitled " The epidemiology of giant cell arteritis: a 12- year retrospective study. " ( Liu NH, LaBree LD, Feldon SE, et al. Ophthalmology 2001; 108: 1145- 9) that asserts " Giant cell arteritis occurs primarily in the white population. None of the Hispanic patients in our study was found to have positive biopsy results. Hispanic persons may have unknown factors that protect them from this disease. " In this instance, the group experience obviously expands on the published information. Two additional responses provide demographic data on an additional 130 or so cases, again providing additional unpublished data. One author provides a reference entitled " Giant cell arteritis in two Chinese patients ", that is directly applicable. An additional two comments raise thoughtful questions about sources of bias in determining the prevalence of an age-related disease. Finally, a neuro- ophthalmologist in Singapore reports his personal experience and a reference. Interestingly, this reference does not appear in Medline when searching epidemiology or ethnology of giant cell arteritis. Topic II. Oligoclonal Bands and Multiple Sclerosis Asker: Is it current wisdom that oligoclonal bands are practically always present in multiple sclerosis ( MS)? To my surprise, a local MS expert downgrades " possible MS" to " unlikely MS" in the absence of oligoclonal bands. Responder 1: The statistics are something like 90% positive, but that is in patients with otherwise confirmed MS, and based on old data, much before routine use of magnetic resonance imaging ( MRI). In my experience, patients very early in their course ( which is the rule rather than the exception these days) may on occasion be oligoclonal- band negative. I usually do not do a lumbar puncture ( LP) routinely unless there is either something atypical, or the diagnosis is not confirmed clinically and with MRI. I had a recent patient with optic neuritis that was followed six months later with hemisensory loss below Tl. His head MRI was negative, as was his cerebrospinal fluid ( CSF) for bands. However, his cervical spine MRI showed a clear de-myelinating plaque, and systemic work- up was negative. I feel he has MS and started him on immunomodulating therapy, but I am sure there are neurologists out there who would differ. I certainly would not use the absence of bands to rule out MS; their absence might just raise my suspicion that it may not be MS. Responder 2: Wasn't it the finding of the Optic Neuritis Treatment Trial ( ONTT) that patients with negative MRI and positive bands were more likely to develop MS than those without bands? Thus, bands seem to have predictive value, just like MRI in a patient with a first neurologic event ( though I don't mean to imply the same statistical predictive value as MRI). Whoever wrote the LP/ ONTT article, please respond! Responder 3: The key article was written by Loren Rolak, MD. I'll forward this message to him and ask him to respond. Responder 4 ( not Dr. Rolak): Depends on the stage of MS. There are no absolutes. MRI and bands are positive in virtually everyone with clinically definite MS. Positive MRI or positive bands have predictive value for development of MRI in patients with a clinically isolated syndrome [ single event, like optic neuritis or internuclear ophthalmoplegia ( INO)]. Conversely, a patient with a single clinical event, negative MRI, and no bands, has a lesser risk of developing MS, but 18% of patients with optic neuritis and a negative MRI will develop MS over time. In patients with two clinical events and an abnormal MRI with everything " typical," most physicians would not do an LP. An LP tends to be used as a ' tiebreaker' or for patients with atypical features. Responder 5: 1 agree with the other opinions. The statistics are misleading. After years, almost all MS patients have bands, but after their first attack, they may not have bands; they may be normal or have a single band. This is a real problem because you do not usually need help to make the diagnosis late in the course. It's early in the course that you need a diagnosis- when you want to start treatment. I like the LP for ischemia versus inflammation, if that is the concern. I figure that a new MS event should have a few cells at least, if the LP is done just after the event. I like the LP for bands/ immunoglobulin G ( IgG) index for the 52 © 2002 Lippincott Williams & Wilkins Copyright © Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited. BEST CATCH FROM NANOSNET JNeuro- Ophthalmol, Vol. 22, No. 1, 2002 MS- like MRI with a bad story and exam, or for the great story/ exam and an atypical MRI. For a single episode of optic neuritis with a normal MR, I tap if I'll treat with interferons, but hold off if I won't. I make this choice by considering the moon phase and the color of the socks I have on that day. Sometimes I use even less rigorous methods to decide on doing LPs! Responder 6 ( Dr Rolak): There were 83 people in the ONTT who had spinal fluid analysis. The spinal fluid findings mirrored the brain MRI findings. Almost every patient with oligoclonal bands in the CSF also had an abnormal MRI. Almost every patient with no bands had no MRI changes. Therefore, positive bands did correlate with subsequent development of MS, but bands add no information over and above the MRI. If you get an MRI you do not need to get bands/ CSF. Incidentally, the CSF never showed any other abnormality in these patients. Responder 7: Having been one of the principal investigators in the ONTT, and having looked at that CSF data, as well as our experience in caring for over 5,000 patients with MS, I find that it is very unusual for us to use CSF findings in our therapeutic decision- making. We save the LPs for the pseudotumor cerebri patients. Responder 8: My interpretation of the ONTT study is that with normal MRI and no bands, none of the 28 patients got MS, while with normal MRI and bands, 18% ( two of 11) got MS. The numbers were small, but the difference seemed helpful. Responder 9: In the group of patients with negative MRI, bands had an estimated 100% sensitivity and 76% specificity for detecting those who developed MS. That means that the positive likelihood ratio is 4.1 and the negative likelihood ratio is infinite. These data provide evidence that favors obtaining CSF in those with negative MRI. In the Rolak paper, of 27 patients with abnormal MRI and positive bands, nine developed MS; and often subjects with abnormal MRI and negative bands, two developed MS. The positive likelihood ratio for bands in those with abnormal MRI is estimated to be 1.2, and the negative likelihood ratio is 1.7. The probability of MS with abnormal MRI is 11/ 37, or 30%. With positive bands, this probability goes up to 33%, and with negative bands, this probability decreases to 20%. Although based on limited numbers of patients, these data do not provide much support for CSF analysis in those with abnormal MRI. In those with normal MRI, the probability of MS is 2/ 39, or 5%. This increases to 2/ 11, or 18%, with positive bands, and decreases to zero with negative bands. Again, the numbers are small, but there is support for CSF analysis in those with normal MRI. I can provide confidence intervals for all of these estimates, but what we really need are more data. Responder 10: We need more data than what these numbers can provide. No serious conclusions can be drawn from 2/ 11 patients in this setting. Comments: A common clinical situation in neurophthalmology is the patient with signs and symptoms suggestive of multiple sclerosis ( MS). While not all neuro-ophthalmologists treat MS with immune- modulating drugs, familiarity with the diagnostic criteria and process is essential. In this interchange, the role of CSF analysis for oligoclonal bands in determining possible versus unlikely MS is queried. A number of authors responded that bands are positive at some point in virtually all MS patients and that positive bands are useful as a " tiebreaker" in atypical cases or clinically isolated syndromes with normal MRI. Interestingly, no one mentioned the recently revised criteria for diagnosis of MS ( McDonald WI, Compston A, Edan G, et al. Recommmended diagnostic criteria for multiple sclerosis: guidelines from the international panel on the diagnosis of multiple sclerosis. Ann Neurol 2001: 50: 12- 17). The new diagnostic categories are " MS", " possible MS," or " notMS". " Unlikely MS, " mentioned in the query, is not an accepted category. In the new criteria, dissemination in space is demonstrated by either three or more MRI lesions ( with specific criteria), or two MRI lesions plus bands. Bands alone cannot be used to make a diagnosis of MS. A question about the predictive value of bands in patients with optic neuritis and a normal MRI seen in the Optic Neuritis Treatment Trial was also brought up. The lead author of the article on bands in that study commented that the presence of bands did not add additional information to the MRI findings. The next two authors advanced the discussion by bringing up a subgroup comparison of patients with normal MRIs and positive versus negative bands, noting that inpatients with abnormal bands, the risk of developing MS was 18% with bands versus 5% without bands. The limitations of these numbers were pointed out by subsequent responders. Topic III. Shaking Eyes Asker: I have a strange case of a 54- year- old woman in good health who, in March 2001, began to have episodes of bilateral horizontal oscillopsia lasting a few seconds, at first unassociated with other symptoms. She claims the frequency of the episodes has increased to many times per day. She states that they are now preceded by a pounding feeling 53 Copyright © Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited. JNeuro- Ophthalmol, Vol. 22, No. 1, 2002 BEST CATCH FROM NANOSNET at the top of her head ( but not pain) that crescendos just before the onset of the visual symptom. During the examination, she had quite a few of these episodes. I did not see any eye movement during the episodes either with the naked eye or on slit lamp magnification. I noticed that when she complained of the " shaking" she " squinted" her lids and had bilateral lower lid myokymia. At no time did she have a convergence movement or miosis during the episodes. When I looked with the direct ophthalmoscope during the episodes, sure enough ( and much to my surprise), she had a very high frequency, very low amplitude horizontal shimmering of the discs in both eyes, that would last for about 10 seconds or so. There was no vertical or torsional component. There was no lateral shift of the disc position that would suggest voluntary nystagmus. I was able to observe her for complete episodes, including the few seconds before and after the episode. Her vision was fine. Ductions, versions, saccades, and pursuit were normal. There was no vestibulo- ocular reflex ( VOR) problem, at least on head shaking during ophthalmoscopy. I forgot to do a head thrust test. There were no nystagmus or cerebellar eye signs. Have any of you seen this with lid myokymia? Is she voluntarily bringing on myokymia that then shakes her eyeballs? Is this some sort of strange epileptiform thing? A party trick? Before I reluctantly start to think of this as a variation on the theme of ocular flutter and do a work up, I have to also tell you that even before I saw her, I had received disability papers on her. If someone out there has seen a similar case or believes that myokymia could cause such findings, I am tempted to try a little Botox to the lower lids to see what happens. Responder 1: I was going to post a similar question on a 52- year- old woman whom I saw two days ago and who has been symptomatic for 3 years. She complains her vision becomes " jiggly" for periods of up to 15 minutes. What she means is that she gets rapid, horizontal oscillations of vision, which subside spontaneously, and are unaccompanied by any other symptoms. She compares this to the TV image from a camera shaking from the wind. This was occurring about every 3 weeks and now occurs several times a day, but only for 10 to 15 seconds. She can " make it go away" by " concentrating" ( not focusing). Her heath is good, she takes no medications, and has no significant past disease. She says she is " low on B12" and gets shots for this. Examination is totally normal except for very low amplitude, high frequency horizontal movement of her eyes on slit lamp, which is not constantly present. I characterized it as a " buzz." She did not complain of oscillopsia at these times. She asks, " How can I continue to work?" She complains of being unnerved or disoriented by the strobe effect of light through trees while driving and walking in a brightly lit supermarket aisle. My concern was alleviated somewhat at the end of the examination when she said she could " make it go away" by pressing on the left occipital prominence. She had normal computed tomography ( CT) and MRI prior to seeing me. My impression is also of a muscular " tone" problem but I was thinking of treating as with superior oblique myokymia. Responder 2: On her MRI, is there a good midline sagittal view and no Chiari malformation? Responder 3: Is she on any medications ( prescribed or otherwise)? Responder 4: I have also recently encountered a middle-aged woman who, upon close questioning, complained of low amplitude horizontal oscillopsia that lasted several seconds. The complaint coincided with quick to- and- fro horizontal oscillations of no more than one quarter of a diameter of the optic disc. After several months, the movement became less frequent and briefer. I presumed that this was a micro flutter of autoimmune or viral origin similar to that of patients who improve after a bout of ocular flutter and truncal ataxia. Any more out there? Responder 5: 1 think that this is a variant of the parlor trick: patients jack up their norepinephrine or fusion or something and make the eyes jiggle. Maybe it is excess anxiety that, you know, has to go somewhere. It's better to have oscillopsia than irritable bowel! I am aware of a nurse who could do this. I'd have her show it to my residents. It's pseudo-voluntary, and can only be sustained briefly. If it were more, I'd think it would last longer. Perhaps you could suggest that you'll support a disability if they can make their ears wiggle too. Responder 6: 1 saw a similar patient last year, except that the shimmering of the fundi was so fine and fast that I could not tell its direction ( she described vertical or diagonal oscillopsia). She also had a long history of recurrent vertigo and had decreased vestibular function on one side. MRI was negative. Her symptoms disappeared on carbamazepine ( Tegretol, Novartis Pharmaceutical Corp., Basel, Switzerland) for over a year, but she returned recently with an exacerbation of " eye fluttering" and vertigo. Is this microsac-cadic flutter? I know most are idiopathic, but it is reported with MS and cerebellar degeneration. 54 © 2002 Lippincott Williams & Wilkins Copyright © Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited. BEST CATCH FROM NANOSNET JNeuro- Ophthalmol, Vol. 22, No. 1, 2002 Responder 7: Is this voluntary nystagmus? Responder 8: Bob Reinecke wrote up a case ( see " Comments") of " nystagmus" secondary to lid myokymia some years ago. Botox to the lid might be diagnostic and therapeutic. Responder 9: Sure sounds like voluntary nystagmus. I frequently see " blepharoclonus" at the onset; this is not true myokymia. Responder 10: When I try to do this, I have to break fixation by accommodating. Do the patient's pupils constrict when the eyes buzz? Responder 11: I have seen similar things in several patients, always binocular, intermittent, small amplitude movements. Some are associated with a history of vestibular dysfunction. Most have responded to either carbamaze-pine or acetazolamide ( Diamox, Wyeth- Ayerst Pharmaceuticals, St. Davids, PA). I presume it is vestibular in origin, but the lack of associated vertiginous symptoms is puzzling. Responder 12: 1 suspect that she does have " voluntary nystagmus" without a significant component of convergence. You can exclude secondary ocular oscillations from lid movements by pulling them away from the eyes with your fingers. Electronic recordings could verify saccadic oscillations consistent with voluntary nystagmus. Responder 13: I believe you might be describing micro-saccadic oscillations ( assuming this is not volitional). Ihave seen one or two of these ( documented with magnetic search coil recordings). I believe one or two of the five patients described below ended up with a diagnosis of MS. Ashe J, Hain TC, Zee DS, et al. Microsaccadic flu tter. Brain 1991; 114: 461- 72. Abstract: Microsaccadic flutter is a rare symptomatic saccadic oscillation that has been reported only twice previously. Here we describe five patients with this disorder. The oscillation is horizontal, has a frequency of 15- 30 Hz, an amplitude of 0.1- 0.5 degrees, and cannot be seen with the unaided eye. It is usually not associated with any underlying neurologic disorder. We hypothesize that microsaccadic flutter is due to malfunction of the brainstem om-nipause neurons. Responder 4: 1 agree this is what it seems to be. I would be interested in more information about the subsequent diagnosis of MS. Responder 13: Of the two patients I have seen, one has been followed for 10 years without development of MS; the other is lost to follow- up. Asker: Thanks to everyone for all the great suggestions. When I've acted on some of them, I'll give you an update. Comments: This thread starts with a query about a patient with oscillopsia, lid myokymia, and very small horizontal ocular movements seen only with the direct ophthalmoscope. The first response is from another physician with a very similar patient. The next two respondents ask about the presence of a Chiari malformation and drug use. The fourth respondent describes a similar case and postulates a postviral or autoimmune origin similar to opsoclonus. Another respondent wonders if these are voluntary movements and if there is a way to distinguish this. Four respondents then continue on the theme of voluntary nystagmus, with questions about the lid movements, pupil changes, and secondary gain. One respondent brings up the possibility of nystagmus secondary to lid myokymia and mentions a reference ( Reinecke RD. Translated myokymia of the lower eyelid causing uniocular vertical pseudonystagmus. Am J Ophthalmol 1973; 75: 150- 1), whereas another respondent wonders about a vestibular abnormality. Recording of eye movements for diagnosis is also suggested. A diagnosis of " microsaddicflutter " is then proffered. The last respondent mentions two additional cases, supplies a reference on microsaccadic flutter ( Ashe J, Hain TC, Zee DS, et al. Microsaccadic flutter. Brain 1991; 114: 461- 72), and wonders about a possible association with multiple sclerosis. In the course of 6 days, a rare ocular motor abnormality is identified, and five to six additional cases are mentioned. The next step would be for the respondents to pool their cases and seek to report them formally. Topic IV. Choroidal folds and hyperopia Asker: I saw a patient today who is a 31- year- old athletic man in good health. He has had myopia of about - 10.00 D OU, but began a progressive hyperopic shift OS 18 months ago. The OS is now - 7.25 D, with OD stable. There is a small area of temporal paracentral metamorphopsia OS as the only other symptom. He has a history of mild migraine disorder, quiescent for several years, up to the present. No recent headaches, pulsatile tinnitus, or transient obscurations of vision. The rest of the past medical history is essentially negative. Examination shows normal visual function except for a small area of relative depression temporal to the blind spot OS on a 30- 2 Humphrey visual field, which is stable compared with a study done last year. He has very prominent choroidal folds around the disk OS in a random, serpiginous pattern, and an area with a more radial configuration extending towards the macula. There are a very few folds OD around the disk. The disks themselves are not elevated. There is no proptosis, with normal 55 Copyright © Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited. JNeuro- Ophthalmol, Vol. 22, No. 1, 2002 BEST CATCH FROM NANOSNET exophthalmometry. Tensions are normal. Fluorescein angiography shows only the folds, no disk leak. Excellent MRI of orbits with contrast, fat suppression, that I have reviewed, is normal, with no mass or abnormal enhancement of orbital structures or globe. MRI of brain is normal with contrast. Here are the questions: 1. Given the report by Griebel and Kosmorsky ( see " Comments"), and the one by Dan Jacobson and others ( see " Comments"), of finding otherwise unsuspected elevated intracranial pressure ( ICP) when a LP is done in patients with " idiopathic" choroidal folds, would you do an LP on my patient? 2. Have you seen regression of such " idiopathic" folds in a patient with elevated ICP after treatment of the ICP? A decrease in the hyperopic shift? Responder 1: According to Cohen and Gass ( Cohen SM, Gass JD. Bilateral radial chorioretinal folds. Int Ophthalmol 1994- 95; 18: 243 5), acquired choroidal folds are most often idiopathic. You have ruled out orbital tumor, thyroid ophthalmopathy, and choroidal neovascularization. One other " eye problem" is posterior scleritis, but your patient does not have the symptoms of this. You might consider doing orbital ultrasonography, looking at the sclera and caliber of the optic nerve sheath ( the " 30 degree" test described by the Bascom Palmer group) before you do an LP. Responder 2: 1 would definitely do an LP. I have seen several patients similar to those described by Greg Kosmorsky; mine have been skinny guys. Granted, my cases did show enlargement of the perioptic space on MRI, but I would still do the tap. Asker: The main reason to do an LP seems to be to know that a further search for systemic causes of choroidal folds is not indicated, and whether my patient needs dural venous sinus imaging. I am left to wonder what else I would do with the data that the ICP is elevated. Does anyone have any experience that suggests remission of the folds, or the hyperopic shift, with ICP- lowering treatment? If not, what is the reason to treat? Responder 3: 1 don't recall any of our patients truly resolving their folds after an LP, but some of the patients stated that the quality of their vision improved anyway. Is this the placebo effect of a big needle? Asker: Thanks. I think I'll recommend the LP, but explain to the patient that it's for the purpose of determining the possible further extent of the evaluation, not to determine therapy. Responder 4: I've had one patient with the same findings who had acetazolamide intolerance, and opted for optic nerve sheath fenestration but the folds persisted. Responder 5: I've almost always seen MRI flattening of the posterior globe at the insertion of the optic nerve, and often, but not always, some distention of the optic nerve sheath and a bit of tortuosity. Does your patient have any of this? If so, these are radiographic signs of intracranial hypertension, and it might lead me to consider an LP for opening pressure. To be honest, though, I'm not sure it matters. I think it is cool to document elevated pressures in these cases ( almost all that I've seen now have mild elevation), but treating the intracranial hypertension has done nothing for the folds or hyperopia in any of the patients I've tried this on. Let us know what the pressure is if you tap him. Responder 6: How high are " mild ICP elevations"? If treatment of pressure doesn't change choroidal folds, are you sure they are related? Responder 5: I don't have the numbers at hand, but my recollection is that they are in the 250- 300 mm range. Sure, the pressures and syndrome could be unrelated, but then you have to reconcile: 1. Why they have the same imaging characteristics ( flat globe, distended optic nerve sheath)? 2. Why Responder 3 found the same thing I did in his study in a number of patients? Responder 7: In a patient with isolated idiopathic bilateral choroidal folds but without papilledema, is an LP needed? Is a scan necessary? You know it's not an orbital tumor if it's bilateral and there is no relative or absolute proptosis or other orbital sign. Has anyone seen or read in the literature any cases of intracranial hypertension caused by a brain tumor presenting as bilateral choroidal folds? Perhaps in Responder 5' s article? Responder 8: I wonder if whatever is causing the globe to shorten could also be causing the elevated pressure, like a problem in collagen affecting arachnoid granulations and sclera ( this is a wild guess). Given that treatment of ICP doesn't change folds, it seems pressure is an epiphenomenon. Comments: In this thread, the asker describes an uncommon clinical condition, idiopathic acquired hyperopia with choroidal folds, first described in 1980 ( Kalina RE, Mills RP. Acquired hyperopia with choroidal folds. Ophthalmology 1980; 87: 44- 50) and amplified in their follow- up article in 1986 ( Dailey RA, Mills RP, Stimac GK, et al. The natural history and CT appearance of acquired hyperopia with choroidal folds. Ophthalmology 1986; 93: 1336- 42). The asker then goes on to note two more recent references ( Jacobson DM. Intracranial hypertension and the syndrome of acquired hyperopia with choroidal folds. J Neuro- Ophthalmol 1995; 15: 178- 85; and Griebel SR, Kosmorsky 56 © 2002 Lippincott Williams & Wilkins Copyright © Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited. BEST CATCH FROM NANOSNET JNeuro- Ophthalmol, Vol. 22, No. 1, 2002 GS. Choroidal folds associated with increased intracranial pressure. Am J Ophthalmol 2000; 129: 513- 16) that describe elevated intracranial pressure ( ICP) in a number of patients with this condition. The group is then asked for opinions on doing a lumbar puncture, an invasive procedure typically disliked by patients, and whether the information gained by the procedure will affect the patient's outcome. The first responder notes that most choroidal folds are idiopathic, but the reference provided describes a case of choroidal folds from occult choroidal neovascularization associated with age- related macular degeneration. The responder then mentions a noninvasive test- orbital ultrasound- that can indirectly show the effects of elevated ICP ( Gans MS, Byrne SF, Closer JS. Standardized A- scan echography in optic nerve disease. Arch Ophthalmol 1987; 105: 1232- 6). Responder 2 recommends the lumbar puncture, without commenting on any effect of treatment, and mentions MRIfindings that can indicate elevated ICP ( Brodsky MC, Vaphiades M. Magnetic resonance imaging in pseudotumor cerebri. Ophthalmology 1998; 105: 1686- 93). The asker then raises the point that knowledge of the elevated ICP could lead to consideration of other diagnoses, namely venous sinus thrombosis, as a cause of elevated ICP. The question of effect of treatment is again asked. Responder 3, author of a paper on the topic, notes that folds and vision did not seem to change with treatment of ICP. The asker then decides to proceed with the LP for diagnostic reasons. Responder 4 adds a case and notes that treatment with optic nerve sheath fenestration did not change vision. Responder 5, another author on the topic, describes additional MRI findings of elevated ICP and also notes little effect of treatment on the folds or hyperopia. Responder 6 asks for clarification on the amount the ICP is increased and, as some do with glaucoma, wonders if the elevated pressure is causally related to the findings of folds and hyperopia. Responder 5 notes the level of the pressure increase and the fact that others have also found increased pressure. Responders 7 and 8 question both the need for an LP and neuroimaging in a patient with bilateral choroidal folds but no papilledema. This thread helps shed light on a difficult topic. Choroidal folds have been described with many different underlying processes, with and without hyperopia. Kalina and Mills described six patients with unilateral or bilateral choroidal folds and " no papilledema", though three patients had disc elevation, though without leakage on fluorescein angiogram. One patient had a lumbar puncture with elevated intracranial pressure. Over time, these patients had a benign course, with the rare patient having resolution of the folds without treatment. Jacobson, and then Griebel and Kosmorsky, described an additional 15 patients, nine with disc swelling and 14 with elevated intracranial pressure, though some elevations were modest. As with the original patients, there seemed to be little change over time; treatment of elevated intracranial pressure did not seem to change the hyperopia or folds. Whether this is a homogeneous or heterogeneous group is not clear. It is not clear whether hyperopia with choroidal folds and disc elevation is the same as hyperopia with choroidal folds and no disc elevation. The relation between hyperopia with choroidal folds and elevated ICP is also not clear. Acquired hyperopia with choroidal folds, with or without disc edema, could be an unusual presentation of elevated ICP, an atypical pseudotumor cerebri. The elevated ICP, reduction of which does not seem to change the eye condition, could merely be an epiphenomenon. Again, material in the literature is brought forward through the interchange. While elevated ICP can clearly be seen with acquired hyperopia with choroidal folds, treatment of the pressure does not appear to change the folds or hyperopia. While adding a magnetic resonance venography ( MRV) to look for venous sinus thrombosis is both reasonable and noninvasive, the LP does not appear to add to diagnosis or treatment in this condition. 57 Copyright © Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited. |