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Show ]. CJin. Neuro-ophthalmol. 1: 77-79, 1981. 1. Monocular amaurosis fugax is a frequent problem in practice. Usually due to migraine in younger patients, and to carotid atheromatous disease in older ones, there are at times unusual causes. An interesting case occurred in a 23-yearold woman with S-C hemoglobin and lupus erythematosus and responded dramatically to steroids (Shaw, H.E. et aI., Am.]. Ophtha/mol. 87: 281-285, March 1979). 2. Another important paper by Dr. Lanning B. Kline and Dr. c.L. Kelly, "Ocular Migraine in a Patient with Cluster Headaches," appeared in Headache 20(5): 253-257, Sept. 1980. An exactingly documented case with beautiful pictures is presented of a 48-year-old man with a long-standing history of cluster headaches who had repeated transient episodes of profound visual loss in only one eye. Fluorescein angiography during an attack was performed. Although a delay in disc arterial perfusion was evident, the prominent finding during the attack on disc photos was marked narrowing of the retinal veins. Dr. Mark Daily of Wheaton, Ill., kindly sent me a beautiful set of fundus pictures taken of a patient before and after an episode of monocular visual loss. These pictures documented beautifuly that the arteries appeared of same size in both sets of pictures, but profound venoconstriction was striking during the attack, which was presumably migrainous in origin. Dr. Daily should formally report his case, too. For a reprint of this paper write: Dr. L.B. Kline, 1720 Eighth Ave., S., Birmingham, Ala. 35233. 3. A new "gem" in the management of myasthenia gravis pOPl?ed out of Dr. Norman Schatz on a recent trip to Philadelphia. He has about 22 patients with myasthenia gravis now under good medical control on a combination of Mestinon and [muran. Most of these patients had preViously had thymectomies. As a rule, they have severe generalized myasthenia (not simply ocular myasthenia), are in the older age groups, and, although frequently controlled on mestinon and steroids, are developing very serious side effects from prolonged steroids. The dosage employed and regimen used will be detailed in a subsequent issue of Neuro-ophthalmology Tapes, but this has allowed Dr. Schatz to get all but three of these patients (who had to be terminated because of side effects from Imuran) completely off steroids, and many are doing very well. This is an important alternative in myasthenia gravis therapy! March 1981 New Pearls Checklist J. L. SMITH, MD. 4. Remember: cyproheptadine has been helpful in Nelson's syndrome. In case you don't know it, cyproheptadine is simply your old friend, "Periactin"! (N. Engl. ]. Med. 302(8): 453, Feb. 21, 1980.) 5. Downbeat nystagmus is most often a sign of cervicomedullary junction disease. Careful neuroradiologic study should be given to this area to rule out a surgical lesion there. However, downbeat nystagmus can also definitely occur with mesencephalic level lesions. Hereditary spinocerebellar degenerations can cause downbeat nystagmus and should prompt you to look at all family members when you see such a case. Dr. John Costin now has documented four patients of downbeat nystagmus in which oscillopsia was the initial presenting symptom due to alcoholism. (Ann. Ophthalmol. 12(10): 1127-1131, Oct. 1980.) 6. Years ago, Dr. W. F. Hoyt predicted that Duane's syndrome could be explained by anatomical anomalies of the ocular motor nerves (Am. ]. Ophthalmol. 60: 443-448, 1965). His prediction was beautifully confirmed by Dr. Hotchkiss et al. (Arch. Ophthalmol. 98: 970, 1980). The best overall paper on Duane's syndrome I have read in recent years was in the Japanese Journal of Ophthalmology (23: 354-468,1979). These papers are discussed in Annals of Ophthalmology (12(8): 906-913, Aug. 1980). 7. A super issue of Surgical Neurology was volume 14, no. 3, published in September 1980. A good paper by Dr. S. E. Rawe discussed 30 patients who had trans-sphenoidal surgery for prolactinomas. He concluded that surgical treatment in patients with microadenomas (less than 1 cm in diameter) is comparable to bromocriptine therapy. In patients with macroadenomas, bromocriptine is more effective than surgery. Radiation therapy was reserved postoperatively for those patients with macroadenomas with suprasellar or parasellar extension. Other good papers in that issue discuss nasopharyngeal tumors, dural arteriovenous fistulas, a fantastic case of bilateral intracavemous aneurysm treated by bilateral carotid ligation with a great result, arachnoid cysts, and several others! 8. Rapid developments are occurring in neuroradiology. In San Francisco we saw beautiful reconstructions in the sagittal, parasagittal, and oblique sagittal planes of computed tomographic scans made on the GE 8800 scanner. Thus, with proper equipment, cuts made in the conventional axial or coronal planes can be reprogrammed and 77 New Pearls Checklist presented to the observer in these other planes and can really give a fantastic new dimension to the pictures! 9. Another new approach in neuroradiology is to use intravenous injections of contrast agent and to combine that with digital video subtraction so as to visualize the cervical and intracranial vessels! In other words, they are on the way to performing intracranial angiography and to studying the great vessels in the neck angiographically-yet without sticking the patient in an artery at all! This holds an exciting promise for the detection of carotid atheromatous disease in the neck (see Am. ]. Neuroradiol. 1(5): 379, Sept.-Oct. 1980). We have had no personal experience with this yet, but keep your eye on this! When they develop computed tomographic scans of the neck to the degree that we can see those lesions in the great vessels without any contrast agent, then we'll really have something valuable! 10. A nice paper on septo-optic dysplasia appeared in the American Journal of Neuroradiology (1(5): 443, Sept.-Oct. 1980). De Morsier's syndrome (septo-optic dysplasia) consists of hypoplasia of the optic discs, agenesis of the septum pellucidum, and hypopituitarism is frequent in these patients as well. 11. We must now include hematomas of the pons in the differential diagnosis of patients presenting with a cerebellopontine angle syndrome! An important paper on this has been published (Surg. Neurol. 14: 115, Aug. 1980) by Dr. J. Vaquero and associates from Madrid, and the reason this is important is that they had fantastic surgical cures in two cases! 12. Spastic paretic facial contracture is an unusual but very important clinical entity! To understand it, simply look at page 343 in Walsh and Hoyt (vol. 1). You see a patient with an asymmetric face who presents with unilateral twitches on one side resembling hemifacial spasm. However, the "moment of truth" comes when you ask the patient to close both eyes as tightly as he can! You will then find that the orbicularis oculi is weak on the side of the spastic facial twitches! This is evident on inspection by simply seeing fewer "crow's-feet" around the eye on the involved side, or by opening the eye with one's finger while the patient tries to keep it shut. This combination is always a sign of intrinsic disease of the dorsal pontine tegmentum and nearly always points to a tumor there. Drs. Dejong and Hicks illustrated the syndrome nicely in a long-standing vascular brain-stem malformation in Neurology (30: 995-997, Sept. 1980). We have recently had further experience with this syndrome and it is so important that I hope Dr. James Mitchell will formally report on it. It is important because by simple inspection and using 78 your finger you can diagnose an intrapontine mass lesion in 10 seconds in the office! 13. The best medical regimen for essential blepharospasm we know of at this time is Clonopin -it seems to help at least a fourth to a third of these patients. The others may come to bilateral differential section of the facial nerves if they are severe. 14. A 30 diopter base left Fresnel prism on the left lens of a young woman with a total left homonymous hemianopia secondary to occipital arteriovenous malformation was advised by Dr. Ira Weiner. She loved it! We are now using some of these "hemianopic prisms" as a substitute for hemianopic mirrors! 15. Fluorescein angiography can be helpful in differentiating the optociliary shunt vessels on the disc that occur in patients with optic nerve sheath meningiomas from those occurring secondary to central vein occlusion. At first blush, you might think this is unnecessary because the total clinical picture is different in these two entities. I agree with Dr. Hoyt, however, that the triad of progressive monocular visual loss in a young to middleaged woman plus optic disc pallor plus optociliary shunt vessels is extremely helpful in diagnosis of an optic nerve sheath meningioma. Optociliary shunt vessels, however, are a rather late sign in optic nerve sheath meningioma, and usually only occur a few years after the patient has come under scrutiny. When seen, however, they are helpful. See the paper by Dr. Boschetti et al. in this issue for further comment on this differentiation. We recently have seen a patient with optociliary shunt vessels, however, that was rather difficult to call (i.e., between meningioma and vein occlusion), and this "pearl" on fluorescein angiography was helpful then! 16. Dr. Olson reports on stellate ganglion blocks as helpful in relieving the pain on ophthalmic herpes zoster (in this issue). The real problem, however, is not with acute or subacute herpes zoster, but with the poor patients who develop postherpetic neuralgia. Dr. Olson tells me they have tried this treatment on two such cases, without success. However, he did have two patients with rather late recurrences of pain that did respond to this treatment. His paper is published, therefore, to see if this form of therapy could help reduce the incidence of developing postherpetic neuralgia in the older patients. Dr. Olson and Dr. Ivy wrote a letter to the editor on this point (Arch. Ophthalmol. 98: 1656, Sept. 1980). Since we have no effective therapy to offer patients with postherpetic neuralgia at this time, we published the entire paper, as any reasonable approach to try and lower the incidence of this problem should be carefully studied! Journal of Clinical Neuro-ophthalmology 17. Your editor has recently been on a sabbatical trip and has interviewed Drs. Schatz, Lessell, Wray, and Hoyt on tape. Several new "gems" popped out that probably should not even appear in such a short overview section as this, becausE' they <He rushing into press in some cases. We will at least March 1981 Smith let you hear them on Neuro-ophthalmology Tapes and will do our best to keep you up to date in subsequent issues of this journal. Please let us hear from you with suggestions as to the types of articles and features that will be the most helpful to you in your practice. 79 |