OCR Text |
Show Journal of Clinical Neuro-ophthalmology 8(4): 247-248, 1988. Editorial Comment Ischemic 'Optic Neuropathy of the Young © 1988 Raven Press, Ltd., New York Ischemic optic neuropathy of the young (AIONY) is a rare entity (1,2) characterized by recurrent attacks of segmental pallid swelling of the optic disc, associated with visual field defects, usually arcuate bundle in nature (altitudinal). Sequential attacks progressively involve areas of normal disc and eventually leave the patient with devastating bilateral visual loss. Thorough medical, including immunologic and neuroradiologic, investigations have failed to reveal any recognizable underlying disease process. The original appelation was used because of the similarity of each acute attack to that occurring in the more common socalled nonarteritic "anterior schemic optic neuropathy" (AION) as defined initially by Kurz (3,4) and subsequently by Francoise et al. (5) and by Miller and Smith (6). Each acute event is marked by the occurrence of pallid swelling of the optic nerve associated with the development of arcuate bundle defects, often altitudinal in nature (7-13). Hayreh has shown experimentally that altitudinal defects, as reflected by the angiographic blood supply to the optic disc, are due to compromise of one of the major long posterior ciliary arteries, stating, "Horizontal localization of the blood supply exists only in the optic disc ... and not anywhere else in the optic nerve." (7) The implied underlying vascular pathogenesis is certainly in accord with the increased incidence of hypertension, diabetes mellitis, myocardial infarction, and stroke reported in the literature (8,10-13). A further similarity between the two entities is the recognition of what appears to be a "disc at risk" that predisposes an individual to develop such an attack. Previous studies have shown an association between typical nonarteritic ischemic optic neuropathy and a smaller than average cupdisc ratio in the involved fellow eye (14-16). The study of disc photographs in all five reported patients with recurrent optic neuropathy of the young noted in Table 1 of the paper by Hamed et al. (1) shows a small or absent cup. It has been the observation of one of us (R.M. B.) that a similar disc structure is found in patients with the papillopathy 247 associated with insulin dependent juvenile diabetes mellitus (17-19), also of a presumed vascular nature. The question that becomes immediately apparent is this: "00 these occurrences represent two distinct entities, or do they represent a continuum?" Although in neither case is the underlying pathoetiology known, the clinical course and prognosis are so different that a distinction is warranted on clinical grounds. Wherein both nonarteritic AION and AIONY are marked by bilaterality, a single reoccurrence in a previously affected eye in the former is extremely rare (12). In the latter, multiple recurrences in previously affected eyes is almost universal. Thus, prognostic information given to an adult with anterior ischemic optic neuropathy can almost guarantee a sighted, if somewhat compromised, lifestyle, whereas in a younger individual, no such reassurance can or should be given. At this point in time, a reexamination of the descriptive name given to this entity may be in order. First of all, as pointed out by Ellenberger et aI. (20) in discussing the garden variety of anterior ischemic optic neuropathy, the course (and histopathology) (21) is that of infarction, not ischemia (reversibility). Although for the purist, anterior optic nerve infarction would be the more appropriate designation, studying the course of such previous debates would suggest that the appellation "anterior ischemic optic neuropathy" is permanently fixed in our vocabularies. In the case of AIONY, it might well be better to term this disease entity "anterior optic neuropathy of the young," avoiding the implication of reversibility, which certainly is not the case. Jeffrey M. Josef, M.D. Ronald M. Burde, M.D. Department of Ophthalmology Albert Einstein College of Medicine Montefiore Medical Center Bronx, New York 248 EDITORIAL COMMENT REFERENCES 1. Dutton J], Burde RM. Anterior ischemic optic neuropathy of the young. JClin Neuro-opthalmoI1983;3:135-144. 2. Hamed LM, Purvin V, Rosenberg M. Recurrent anterior ischemic optic neuropathy in young adults. JClin Nezlroophthalmol 1988;8:239-246. 3. Kurz 0, Uber X. Papillitis arteriosclerotica. Opthalmologica 1948;116:281-5. 4. Kurz O. Vascular opticopathy. Cocum Ophthalmol 1969; 26:582-91. 5. Francoise J, Verriest G, Baron A. PseudopapiIIitis vasculaires. Bull Soc Ophthalmol Fr 1956;69:36--57. 6. Miller GR, 5mith ]1. Ischemic optic neuropathy. Am JOphthalmol 1966;62:10:>--15. 7. Hayreh 55. Blood supply of the optic nerve head and its role in optic atrophy, glaucoma and edema of the optic disc. Br JOphthalmol 1969;53:721-48. 8. Hayreh 55. Pathogenesis of visual field defects. Role of the ciliary circulation. Br JOphthalmoI1970;54:289-311. 9. Hayreh 55. Posterior Ciliary arterial occlusive disorders. Trans Ophthalmol Soc UK 1971;91:291-303. 10. Hayreh 55. Anterior ischemic optic neuropathy: I. Terminology and pathogenesis. Br JOphthalmol 1974;58:955-63. 11. ~oghen DR, Glaser IS. Ischemic optic neuropathy. The clinICal profile and natural history. Brain 1975;98:680-703. •· .. 1 N,. 4.1'188 12. Repka MX, Savino PI, 5chatz N], 5ergott RC. Clinical profile and long term implications of anterior ischemic optic neuropathy. 13. Guyer DR, Miller NR, Aver CL, Fine SL. The risk of cerebrovascular and cardiovascular disease in patients with anterior ischemic optic neuropathy. Ophthalmology 1985;103: 1136-42. 14. Beck RW, Savino PJ, et al. Optic disc structure in anterior ischemic optic neuropathy. Opthalmology 1984;91:1334-7. 15. Feit RH, Tomsak RL, Ellenberger C. 5tructural factors in the pathogenesis of ischemic optic neuropathy. Am J Ophthalmol 1984;98:105-8. 16. Green GJ, Lessell S, Lowenstein JI. Ischemic optic neuropathy in chronic papilledema. Arch Ophthalmol 1980;98: 502-4. 17. Lubow M, Markley TA. Pseudopapilledema of juvenile diabetes mellitis. Arch Ophthalmol 1971;85:417-22. 18. Barr CC, Glaser IS, Blankenship G. Acute disc swelling in juvenile diabetes. Arch Ophthalmol 1980;98:2185--92. 19. Pavan PR, Aiello LM, Wafai Z, Briones ]C, Sebestyen JG, Bradbury M]. Optic disc edema in juvenile onset diabetes. Arch Ophthalmol 1980;98:219:>--5. 20. Ellenberger C, Keltner ]L, Burde RM. Acute optic neuropathy in older patients. Arch Neural 1973;28:182-5. 21. Henkind P, Charles NC, Pearson J. Histopathology of ischemic optic neuropathy. Am JOphthalmol 1970;69:78--90. |