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Show IN OTHER JOURNALS Editor's Note: This section contains brief reviews of articles that have appeared in other journals within the past six months. From a comprehensive list of clinical and scientific medical journals, each reviewer has selected about 30 titles and reviewed the most pertinent articles. The March and September issues include reviews from ophthalmology and medicine journals; the June and December issues will include reviews from neuroclinical and neuroscience journals. Ophthalmology Journals Reviewer: Dan Boghen, MD I. Optic Nerve Protection Miller NR. Optic nerve protection, regeneration, and repair in the 21st century: The LVHIth Edward Jackson Memorial Lecture. Am J Ophthalmol 200l; B2: m-$. This transcription of Dr. Miller's lecture to the American Academy of Neurology is an excellent state- of-the- art summary of a subject outside the immediate experience of most clinicians. Neuroprotection, neurorepair, and the use of stem cells for the restoration of sight are clearly discussed. Neuroprotection consists mainly in the prevention of the death of retinal ganglion cells by blocking apoptosis: a physiologic, programmed process leading to cell death after optic nerve injury. Strategies that can be used to prevent apoptosis include the use of substances such as glutamate and nitric oxide inhibitors, alpha- 2- adrenoreceptor agonists, nerve growth factors, and heat shock proteins, as well as vaccination with certain proteolipid proteins or glycoproteins. Neurorepair seeks to restore optic nerve function after injury. Ways in which this could be accomplished include providing a permissive environment for regeneration by countering the effect of substances such as the products of myelin breakdown that interfere with neurorepair or by providing external growth factors. Another experimental approach consists in activating a neuronal program of gene expression capable of inducing regeneration by means of cyclic adenosine monophosphate. Stem cells have the potential to differentiate into retinal ganglion cells and could restore optic nerve- related visual loss after transplantation into the eye as well as by other means. This article is the recommended starting point for an understanding of this important area of developing research. n. Optic Nerve Inflammation Purvin V, Kawasaki A, Jacobson DM. Optic perineuritis: clinical and radiographic features. Arch Ophthalmol 2001; 119: 1299- 306. Optic perineuritis is thought to be " a form of orbital inflammatory disease in which the specific target tissue is the optic nerve sheath." Although it may be the result of specific infections or inflammatory disorders such as Wegener's granulomatosis, it is most frequently of idiopathic origin. The clinical and radiologic features of 14 patients were reviewed. Eye pain, generally exacerbated by eye movement, was present at onset in all the patients. The majority of patients had visual loss consisting, most often, of paracentral or arcuate field defects and, more rarely, of diminished visual acuity. Optic disc edema was present in 10 eyes. Diagnosis was based on the presence of enhancement of the optic nerve sheath on magnetic resonance imaging. This condition can easily be mistaken for optic neuritis. The authors discuss the distinguishing clinical features of the two conditions. Optic perineuritis affects an older age group and is characterized by a slower tempo of visual loss, a tendency to spare central vision, and an excellent response to corticosteroid treatment. Ultimately, however, the diagnosis is based on the magnetic resonance imaging findings. It is very likely that optic perineuritis is often misdiagnosed as optic neuritis. It should be considered in all patients with optic neuritis, particularly those with atypical clinical features, such as sparing of central vision and relapse after the discontinuation of corticosteroid treatment. CHAMPS Study Group. Interferon ( 3- la for optic neuritis patients at high risk for multiple sclerosis. Am. J Ophthalmol 2001; 132: 463- 71. The finding that clinically definite MS will develop in 50% of patients with optic neuritis and magnetic resonance imaging ( MRI) changes compatible with multiple sclerosis ( MS), evidence that initial treatment with intravenous methylprednisolone reduces the rate of development of clinically definite MS for 2 years, and studies showing that interferon ( 3- la has a beneficial effect on the clinical and radiologic course of MS led to the CHAMPS study, which was designed to determine whether there is benefit in initiating treatment with interferon ( 3- la in patients who experience a first attack of optic neuritis, brainstem demyelina-tion, or spinal cord demyelination and have abnormalities on MRI consistent with a diagnosis of MS. In this multicenter study of 383 patients, there was a reduction in the rate of clinically definite MS by about 50% o nJNeuro- Ophihqlmol,. Vol. 22, No. lJ, 2002n ,„,.,.. . . DOI: 10.1097/ 01. WNO. 0Q00028903.09.257.53 , 247 , Copyright © Lip pincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited. JNeuro- Ophthalmol, Vol. 22, No. 3, 2002 IN OTHER JOURNALS and a decreased likelihood of progression of MRI lesions in patients treated with interferon ( 3- la. In this article, the authors analyze separately the 192 patients in whom optic neuritis was the initial event. All patients were treated with intravenous and oral corticosteroids and were randomly assigned to receive weekly injections of interferon or placebo. The analysis yields results similar to those of the entire CHAMPS study, namely, that the rate of development of MS and the aggravation of the MRI abnormalities were significantly lower in treated patients. The findings support the initiation of interferon ( 3- la treatment patients with a first episode of optic neuritis who have substantial MRI signal abnormalities compatible with demyelination. HI. Ischemic Optic Neuropathy Hayreh SS, Podhajsky PA, Zimmerman B. Ipsilateral recurrence of nonarteritic anterior ischemic optic neuropathy. Am J Ophthalmol 2001; 132: 734^ 42. The purpose of this study was to discover the prevalence of recurrence of nonarteritic anterior ischemic optic neuropathy ( NAION ) in the same eye and to determine the possible risk factors. Recurrence was documented in 45 ( 7.5%) of 594 patients, with a median follow- up of 3.1 years. Thirty- two ( 71%) of patients with recurrence had bilateral NAION, and 4 of them had multiple recurrences in both eyes. There was no correlation between recurrence and the presence of systemic conditions such as hypertension and diabetes. The major risk factor for recurrence was nocturnal diastolic hypotension, which was greater in patients with recurrence. Drawing on data from their previous studies, the authors state that aspirin is not beneficial in preventing NAION recurrence in the same eye or its occurrence in the fellow eye. Based on findings in this and previous studies, they emphasize the importance of avoiding the aggressive treatment of hypertension and avoiding the use of hypotension- producing drugs in NAION. Sadda RS, Nee M, Miller NR, Biousse V, Newman NJ, Konzis A. Clinical spectrum of posterior ischemic optic neuropathy. .4m J C^ Ma/ mo/ 2001; 132: 743- 50. Posterior ischemic optic neuropathy ( PION) is uncommon relative to anterior ischemic optic neuropathy ( AION). Most reports are limited to single cases. In this study, the records of 72 patients with PION examined over a 22- year period were analyzed retrospectively and, whenever possible, a follow- up determination of visual function and comorbid states was obtained. In 28 patients, the condition occurred perioperatively; in 6 patients, it was associated with temporal arteritis; in the remaining 38 patients, it was attributed to nonarteritic vascular disease. Among the patients with perioperative PION, 50% had undergone spinal surgery, and 70% of them had bilateral involvement. Patients in the perioperative and arteritic groups had the poorest visual function both at onset and during follow- up. The visual prognosis for nonarteritic PION was similar to that of patients with nonarteritic anterior ischemic optic neuropathy ( NAION). The poorest prognosis was in those with a history of carotid and cerebrovascular disease. This important study reviews the visual and systemic findings in a large number of patients with a rare condition. It draws attention to the important association of PION with surgical procedures, particularly those that involve the spine. IV. Amaurosis Fugax Jehn A, Dettwiler BF, Fleischhauer J, Sturzenegger M, Mo-jon DS. Exercise- induced vasospastic amaurosis fugax. Arch Ophthalmol 2002; 120: 220- 2. The authors describe three patients with exercise-induced visual disturbances. Patient 1, a 65- year- old man free of any known illness, experienced visual field constriction in the OD during jogging or biking. The visual disturbance would progress to complete uniocular blindness if he persisted with the exercise. A fundus photograph obtained during an attack convincingly illustrated an ipsilateral central artery occlusion. In patient 2, there was a single 30- minute episode of temporal hemianopsia in the OS after " heavy" physical activity. This patient also suffered from exercise- induced headache and abdominal pain preventable by prior administration of aspirin. Patient 3 had " graying-out" episodes in his OD on exercise. After the attacks, mild constriction of the visual field of the involved eye could be demonstrated. The attacks ceased after the administration ofnifepidine. Patient 1 is cited as evidence that visual disturbances provoked by exercise can occur in older patients. Whereas vasospasm is thought to have been the mechanism in all 3 of these cases, this was clearly established only in the first patient. V. Temporal Arteritis Hayreh SS, Jonas JB. Optic disc morphology after arteritic ischemic optic neuropathy. Ophthalmology 2001; 1088: 1586- 94. On the basis of a study of 29 patients, the authors assert that arteritic anterior ischemic optic neuropathy ( AAION), like glaucoma, causes loss of the neuroretinal „ 248 . , „.. „ . . © 2002 Lippincott Williams & WUkins , Copyright © Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited. IN OTHER JOURNALS J NEURO- OPHTHALMOL, VOL. 22, No. 3, 2002 rim, cupping of the optic nerve head ( ONH), and parapap-illary atrophy. This finding is unlike what occurs in nonar-teritic ischemic optic neuropathy ( NAION) and in compressive and other optic neuropathies. The article seeks to explain these findings from a clinical, neuropathologic, and experimental viewpoint. The authors suggest that the ONH abnormalities in both AAION and glaucoma are mainly due to vascular factors. The lack of an ample physiologic optic disc cup and the much milder and more transient ischemic phenomena explain the lack of these findings in NAION. According to the authors, the main difference in the optic disc cupping caused by glaucoma and that caused by AAION is the presence of pallor of the neuroretinal rim in the latter. The reason for this difference is not discussed. This is a thorough and thoughtful reflection on an important subject. VI. Visual Cortex Mejico LJ, Bergloeff J, Miller NR. Peripheral homonymous scotomas from a cavernous angioma affecting fibers subserving the intermediate region of the striate cortex. Am J Ophthalmol 2001; 132: 440- 3. The subject of this study was a patient with a left homonymous scotoma from an occipital cavernous angioma. Correlation of the location of the angioma as determined by MRI with the field defect reveals that none of the previously proposed maps of cortical representation of the visual field are accurate. The oldest of these maps ( Holmes and Lister), according to which central vision occupies only 25% of the striate area, would situate the scotoma more peripherally. The two more recent maps, which attribute 37% ( Wong and Sharpe) to 60% ( Horton and Hoyt) of the striate cortex to central vision would situate the scotoma more centrally. This paper demonstrates the value of a well- studied single case and shows that further research is required to definitively resolve the issue of the cortical representation of the visual field. VII. Ocular Motor Phenomena Donahue SP, Lavin PJM, Mohney B, Hamed L. Skew. Deviation and inferior oblique palsy. Am J Ophthalmol 2001; 132: 751- 6. Damage to the oculovestibular pathway disrupts the normal effect of the otolithic stimulation on eye and head position and causes an ocular tilt reaction ( OTR). The latter consists of a triad of vertical ocular misalignment, bilateral ocular torsion, and head tilt. The head and the superior poles of both eyes are rotated toward the hypotropic eye. The term " skew" refers to the vertical ocular misalignment, which is the most easily observed feature of the OTR. The authors describe 6 patients in whom the ocular tilt reaction was the result of posterior fossa disease. In 5 of these patients, the ocular motility pattern was consistent with inferior oblique palsy, except for the cyclotorsion, which was in the opposite direction ( the eye was excyclo-torted) from that observed in an inferior oblique palsy. It seems likely that a significant number of cases diagnosed as inferior oblique palsy are cases of OTR. Mielke C, Alexander MSM, Anand N. Isolated bilateral trochlear nerve palsies as the first clinical sign of a metastatic bronchial carcinoma. Am J Ophthalmol 2001; 132: 593^ 1. This is a report of a patient with an isolated bilateral fourth nerve palsy from a metastasis of bronchial carcinoma to the dorsal midbrain area. The authors state that they were unable to find a previous report in which such a palsy occurred as the sole manifestation of metastatic lung cancer. Acquired isolated bilateral trochlear nerve palsy of simultaneous onset is rare and most commonly is due to head trauma. The value of this article is to emphasize the fact that bilateral trochlear nerve palsy occurring in the absence of head trauma is likely to be due to a potentially serious structural lesion of the midline dorsal midbrain. VII. Orbit Smith JR, Rosen baum JT. A role for methotrexate in the management of non- infectious orbital inflammatory disease. Br J Ophthalmol 2001 ; 85: 1220- 4. This is a retrospective study of the usefulness of methotrexate in the treatment of noninfectious orbital inflammation. The indication for treatment was the inability to taper corticosteroids or to tolerate their prolonged systemic use. Many of the patients had previously been treated with orbital irradiation, a few had had orbital decompression, and some continued taking corticosteroids at the same time as methotrexate. Of the 14 patients initially treated, 10 were considered to have had an adequate trial. Of the 10, most had nonspecific orbital inflammation or Graves' disease, 1 had sarcoidosis, and 1 had the Tolosa- Hunt syndrome. Nine patients benefited from the treatment. Of these, 6 were ultimately able to discontinue methotrexate, and 5 of the 6 using corticosteroid therapy were able to cease this medication. The median duration of treatment of the nine responders was 25 months. Gastrointestinal disturbance and fatigue were the most common side effects, each affecting 50% of the 14 patients initially treated. Copyright © Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited. JNeuro- Ophthalmol, Vol. 22, No. 3, 2002 IN OTHER JOURNALS The limitations of the study are its retrospective nature, the small number of patients, and the heterogeneity of diagnoses and treatments. A more systematic approach is required to confirm the impression that methotrexate is an effective treatment of noninfectious orbital inflammation. Thorne JE, Volpe, NJ, Wulc AE, Galetta SL. Caught by a masquerade: sclerosing orbital inflammation. Surv Ophthalmol 2002; 47: 50- 54. This case presentation draws attention to sclerosing orbital inflammation, an entity that may be difficult to distinguish from an orbital tumor. A 42- year- old woman had had a 6- month history of right orbital pain, lid swelling, and visual loss that fell to hand movements. There was restriction of OD movement with a positive forced duction test and 2 mm of right proptosis. Fundus examination revealed right optic disc edema, venous stasis retinopathy, and opto-ciliary shunts. Magnetic resonance imaging ( MRI) showed an enhancing mass that surrounded the optic nerve and extended into the cavernous sinus, encasing the carotid artery. The extraocular muscles on the right side were enlarged. Treatment with corticosteroids did not improve the vision. A biopsy was performed to confirm a clinical suspicion of optic nerve meningioma. Three different pathologists who examined the specimen diagnosed optic nerve glioma. Histopathologic examination of a later biopsy specimen revealed the correct diagnosis. The features that initially led to the diagnosis of glioma were thought be of reactive origin. The discussion reviews the clinical, radiologic, and neuropathologic features of sclerosing orbital inflammation and discusses the difficulty in distinguishing this entity from a tumor. Whereas the condition was initially thought to represent the end stage of orbital pseudotumor, it is currently believed to be a type of idiopathic fibrosclerosis that also affects other regions such as the mediastinum or the retroperitoneum. Gorman CA, Garrity JA, Fatourechi V, et al. A prospective, randomized, double- blind, placebo- controlled study of orbital radiotherapy for Graves' ophthalmopathy. Ophthalmology 2001; 108: 1523- 34. Forty- two of 53 consecutive patients with moderate symptomatic Graves' ophthalmopathy ( GO) were randomized to receive 20 Gy of external beam radiation to one orbit and sham therapy to the other. Six months later, the therapies were reversed. The parameters studied included the volume of the extraocular muscles and orbital fat, proptosis, range of extraocular muscle motion, area of diplopia fields, and lid fissure width. The effect of treatment was assessed by comparing the findings in the same orbit before and after treatment and between the two orbits at baseline and at 6 and 12 months. No clinically significant difference was observed for the main outcome measures between the treated and untreated orbits at 6 months. Of marginal clinical significance was a slightly positive change from baseline at 12 months in the orbit treated first. In the authors' view, the value of radiotherapy in GO requires reassessment. In an accompanying editorial ( Feldon SF. Radiation therapy for Graves' ophthalmopathy: trick or treat. Ophthalmology 2001; 108: 1520- 2), the author wondered whether the inclusion of patients with inactive disease or previous treatment with steroids may have contributed to the negative results. VIII. Blepharospasm and Hemifacial Spasm Drummond CT, Hinz BJ. Botulinum toxin for blepharospasm and hemifacial spasm: stability of duration of effect and dosage over time. Can J Ophthalmol 2001; 36: 398- 403. This retrospective study addressed the question of the duration of benefit and dosage requirement over time in patients with essential blepharospasm ( BSP) and hemifacial spasm ( HFS) treated with botulinum toxin. Of the 28 patients studied, 17 had BSP and 11 had HFS. The patients were treated over a 5- year period, starting in 1989. All patients had at least 6 treatments; 17 had 12 or more treatments, and 7 had more than 20 treatments. The conclusion of the study is that the duration of benefit and dosage requirements remain stable over time for at least 20 treatments. The results were similar when the patients were analyzed as a group and when BSP and HFS were analyzed separately. These findings offer a measure of reassurance to patients with these conditions who wonder about what the future may bring. As reported by others, the duration of benefit was slightly longer in the patients with HFS ( an average of 15 weeks for HFS compared with 12 weeks for BSP). IX. Facial Nerve Keega D J, Geerling G, Lee JP, Blake G, Collin JR, Plant GT. Botulinum toxin treatment of hyperlacrimation secondary to aberrant regenerated seventh nerve palsy or salivary gland transplantation. Br. 1 Ophthalmol 2002; 886: 43- 6. This is a thorough study of lacrimation before and after the administration of botulinum toxin ( Dysport) tran-scutaneously into the lacrimal gland of 3 patients with gustatory reflex hyperlacrimation ( crocodile tears) and into the ^ 250 „.. „ . . © 2002 Lippincott Williams & WUkins , Copyright © Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited. IN OTHER JOURNALS J NEURO- OPHTHALMOL, VOL. 22, No. 3, 2002 submandibular gland of 1 patient with epiphora after the treatment of dry eyes by autologous submandibular gland transplantation to the temporal fossa. Severity of tearing was evaluated subjectively and objectively ( by means of a Schirmer test and a special tear clearance test) in the presence and absence of a tear secretion stimulus ( chewing, exercise) and before and after the administration of Dysport. In 3 of the 4 treated patients, there was marked reduction in tearing at 2 weeks. The beneficial effect persisted for 3 to 4 months, after which re- injection became necessary. Tear duct obstruction rather than inappropriate parasympathetic innervation was the cause of excessive tearing in the 1 patient who did not benefit from Dysport. Side effects were transient and consisted of upper lid ptosis in 2 patients, 1 of whom also had paresis of the superior rectus. The findings in this small group of patients are in keeping with other positive reports on the treatment of crocodile tears with botulinum toxin. Because of the difficulties in converting Dysport units into the equivalent units of Botox, the dosage used in this study cannot serve as a guide for physicians practicing in countries where only the latter preparation of botulinum toxin is available. Medicine Journals Reviewer: Martin ten Hove, MD I. Cerebrovascular Disease Lubin J, Capparella J, Vecchione M. Acute monocular blindness associated with common carotid artery dissection. Ann Emerg Med' 2001; 38: 332- 5. The authors describe a 49- year- old man with acute monocular blindness caused by a spontaneous dissection of the common carotid artery. The man was otherwise healthy, with no history of trauma, neck manipulation, or connective tissue disease. He did have controlled hypertension and hypercholesterolemia. The results of examination were notable only for a central retinal artery occlusion with no evidence of emboli. The patient later recalled that he had experienced transient scintillating scotomas during the previous week. Doppler ultrasonography demonstrated occlusion of the carotid siphon and ophthalmic artery. Subsequent magnetic resonance angiography ( MRA) revealed a dissection in the distal portion of the common carotid artery. Although there are a few other reported cases of CRAO due to carotid artery dissection, this case differs from those reported because of the absence of associated trauma, neck or jaw pain, or significant medical history. Although the dissection is reported to be in the distal common carotid artery, the occlusion of the carotid siphon indicates that it may have extended to involve the internal carotid artery. No mention is made of the presence ( or absence) of clinical features suggestive of Horner's syndrome. The use of MRA in the evaluation of an otherwise unexplained CRAO is supported by this case. Westwood ME, Kelly S, Berry E et al. Use of magnetic resonance angiography to select candidates with recently symptomatic carotid stenosis for surgery: systematic review. BMJ 2002; 324: 1- 5. The current literature describes little rigorous research on the performance of magnetic resonance angiography ( MRA) in evaluating cervical carotid artery stenosis. Unfortunately, there are no studies that compare conventional angiography with MRA for surgical decision making or outcomes. The authors sought to determine whether there was sufficient existing evidence to support the use of MRA as a means of selecting patients with symptomatic high-grade cervical carotid artery stenosis. The authors systematically reviewed the literature, evaluating the diagnostic performance of MRA versus conventional angiography at thresholds set by the North American Symptomatic Carotid Endarterectomy Trial ( NASCET) ( 70 to 99% stenosis) and European Carotid Stenosis Trial ( ECST) ( 50 to 99% o stenosis). Of the 7,183 articles that were reviewed, only 26 met the main inclusion criteria, and only 8 met all the inclusion criteria. By combining the reported sensitivities and specificities, the authors plotted a summary receiver operating characteristic curve ( a method described by the Cochrane Work Group for meta- analysis of screening and diagnostic tests). The point on the curve where the combined sensitivity and specificity were equal was determined ( Q*). For a diagnostic threshold of 70 to 99% o carotid artery stenosis, the Q* was 99% ( 95% CI: 98- 100). MRA technique was not a significant variable in the multiple linear regression analysis. For a diagnostic threshold of 50 to 99% o carotid artery stenosis, the Q* fell to 90% ( 95% CI: 81- 99). On the basis of these results, the authors state that MRA is highly sensitive and specific for diagnosing a 70 to 99% o stenosis but caution against selecting surgical candidates with 50 to 99% stenosis using MRA alone. Although there is a trend toward improved diagnostic performance with MRA, further research is needed at this threshold. Until then, users of MRA are advised to ensure that audits are in place, including feedback from surgeons and quality control comparisons with another noninvasive imaging modality such as ultrasonography. Copyright © Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited. JNeuro- Ophthalmol, Vol. 22, No. 3, 2002 IN OTHER JOURNALS Mohr JP, Thompson JLP, Lazar RM, et al. A comparison of warfarin and aspirin for the prevention of recurrent ischemic stroke. NEnglJ Med 2001; 345: 1444- 51. First ischemic stroke has a cardiogenic source in approximately one third of patients with a first ischemic stroke ( and should be treated with warfarin) or are candidates for carotid endarterectomy. The rest are candidates for antiplatelet therapy, which has been shown to reduce the recurrence of stroke by 30%. Many physicians have tried to improve on this prevention rate by prescribing warfarin with little evidence- based data to support this practice. The authors investigated whether warfarin was superior to aspirin in the prevention of recurrent ischemic stroke in patients with a prior noncardioembolic ischemic stroke. This randomized double- blind study involved 48 centers recruiting 2,206 patients over seven years. The patients were divided into two treatment groups: one receiving aspirin 325 mg daily and placebo, the other receiving a warfarin dose adjusted to achieve and maintain an INR in the range of 1.4 to 2.8. Patients in the aspirin group followed the same schedule of visits to the clinic for drawing blood, monitoring the medication, and adjusting the dose. False INR values for patients in the aspirin group were sent to the monitoring physician to ensure blinded assessments. Patients were monitored for 2 years with monthly telephone assessments and quarterly clinical evaluations. The primary end point was death resulting from any cause, or recurrent stroke. Recurrent ischemic stroke was defined as a new lesion detected by neuroimaging or clinical findings consistent with stroke that lasted for more than 24 hours. Overall, the end point occurred in 16.9% of patients. There were no significant differences between the warfarin and aspirin groups in time to the end point or in the number of patients reaching the end point ( P = 0.25). Warfarin was expected to confer a small reduction in the risk of recurrent stroke; instead, it was associated with a nonsignificant 13% increase in recurrent stroke risk. Rates of major hemorrhage during the study were low in both groups, again with no significant difference between them. This important study tells us that aspirin is a well-justified choice for the prevention of recurrent ischemic noncardiogenic stroke and that warfarin does not offer any additional benefit. For those patients taking warfarin for other established reasons, this study provides evidence of its safety. Perez- Vega C, Narvaez J, Vilaseca J. Giant cell arteritis presenting as transient vertebrobasilar ischemic attacks. Am J Med 2001; 111: 578. The authors report a case of giant cell arteritis ( GCA) presenting as transient vertebrobasilar ischemic attacks in an otherwise healthy 76- year- old man. The episodes consisted of 5- to 10- minute attacks of ataxia, diplopia, dysarthria, and unilateral facial weakness. He did not have any of the usual symptoms of GCA, including jaw claudication, visual loss, headache, and polymyalgia. The results of all investigations for cardiac and atherosclerotic disease were negative, including electrocardiogram, 24- hour Holter monitor, carotid Doppler ultrasonography, cholesterol, and blood pressure measurements. The diagnosis was confirmed by positive result of temporal artery biopsy and further supported by an elevated erythrocyte sedimentation rate ( ESR) ( 70 mm/ h) and platelet count ( 534 x 103 uml). Despite numerous case reports of stroke and transient ischemic attacks due to GCA, intracranial involvement is uncommon. This case is unique in that none of the usual GCA symptoms were present, and the diagnosis was suspected on the basis of an elevated ESR and mild thrombocytosis. II. Headache Vinson D. Treatment patterns of isolated benign headache in U. S. emergency departments. Ann EmergMed 2002; 39: 215- 36. The author analyzed the treatment of adult patients with isolated headache who sought treatment at United States emergency departments in light of practice guidelines in the United States and Canada. Data were extracted from the 1998 National Hospital Ambulatory Medical Care Survey, which sampled all visits from 398 emergency departments in the United States over a 4- week period. The migraine headache and unspecified headache cohorts included 811,419 and 604,977 patients respectively. On average, patients received 1.8 medications from a pharmacopeia of 36 drugs. The most common drugs used for the migraine cohort were meperidine ( 35.7%), promethazine ( 29.4%), IV ketorolac ( 16.6%), and prochlorperazine ( 15.6%). Parental medications were given to 85% of migraine patients. These facts need to be interpreted in light of the fact that 35% of the migraine patients reported moderate headache and only 20% reported severe headache. The American Academy of Neurology recommends an antiemetic ( particularly prochlorperazine) plus dihydro-ergotamine as the therapy of choice for aborting an acute migraine episode. The Canadian Headache Society's algorithm for the treatment of severe migraine headache states that the agent of choice is a dopamine- antagonist antiemetic followed by dihydroergotamine or sumatriptan. The treatment of patients in this study diverged from these recommendations in two ways. First, only one fourth of patients with migraine received a non- opioid agent as part of their drug regimen. Second, opioids were more commonly used as first- line drugs than as second- line drugs reserved for patients who do not respond to non- opioid treatment. The ^ 252 . _ „ , . © 2002 Lippincott Williams & WUkins , Copyright © Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited. IN OTHER JOURNALS J NEURO- OPHTHALMOL, VOL. 22, No. 3, 2002 author questioned the heavy reliance on meperidine, because its shorter half- life and potential for dependency make the drug " suboptimal" and a " last resort" choice for therapy, according to the Canadian Headache Society. The study is limited by the absence of data detailing prior medications tried by these patients, as well as the efficacy and tolerance of the medications. There are likely to be patient and physician factors that explain why current practice in emergency rooms differs from the recommended therapy. Stafstrom CE, Rostasy K, Minister A. The usefulness of children's drawings in the diagnosis of headache. Pediatrics 2002; 109: 460- 72. The authors conducted an entertaining study designed to determine whether drawings can aid in the differential diagnosis of headaches in children. Before any formal history was obtained, 226 children ( aged 4 to 19 years, mean 11.4 years) with a chief complaint of headache were asked: " Please draw a picture of yourself having a headache. Where is the pain? Are there any other changes that come with your headache that you can show me in your picture?" No leading questions were asked. With a pencil and a single piece of paper for each headache type, all children complied with the request. Two pediatric neurologists, who were blinded to the clinical history, analyzed the drawings independently. Pictures were graded as either migrainous or nonmigrainous. Specific features of the drawings that were considered to represent migraine included depiction of severe pain with a pounding/ hammering quality, nausea or vomiting, sensitivity to light and/ or sound, desire to sleep, headache exacerbation by exercise or movement, or clear- cut unilaterality. Overall, 57.5% of the children were given a clinical diagnosis of migraine or mixed headache with a prominent migraine component. The remaining headaches were diagnosed as nonmigraine for a wide variety of causes. Inter-rater agreement was excellent, with a Kappa score of 0.92. The drawings had a sensitivity of 93 %> and a specificity of 83%> when compared with the " gold standard" of the clinical diagnosis. The results were then stratified into three groups according to age. Surprisingly, there were fewer false positive and false negative results in the youngest age group. This study adds to the growing literature on migraine art. The illustrative examples in this paper must be seen to be fully appreciated. Asking children to draw their headache is a simple and accurate adjunctive aid for headache differential diagnosis in the clinical setting. Drawings can be completed in the waiting room, with instructions given by a nurse or receptionist. For most children, this becomes an enjoyable exercise that also serves as a useful clinical adjunct. III. Miscellaneous Koul R, Chacko A, Ganesh A, et al. Vigabatrin- associated retinal dysfunction in children with epilepsy. Arch Dis Child 2001; 85: 469- 73. Many studies have described the ocular changes and visual field changes associated with vigabatrin in the adult population. However, comparatively little information from children has been reported. The authors report ocular changes in 21 children ( aged 1- 16 years) who were treated with vigabatrin. The children were being treated for epileptic syndromes with infantile spasms. The vigabatrin dose varied from 25 to 114 mg/ kg/ day ( mean 55.8 mg/ kg/ day) for a duration of therapy of 6 to 85 months ( mean 35.7 months). Retinal changes developed in four children ( 19% o), and optic atrophy also developed in one of these children. Visual evoked potentials ( VEP) were abnormal in 16 children, 2 of whom had abnormalities before starting the drug. All children required sedation with chloral hydrate for fundus examination and VEP testing. Visual field testing could not be performed. The results are not surprising but support the recommendation that children receiving vigabatrin be monitored every 3 to 6 months. Evaluation should include visual field testing whenever possible; if that is not possible, sedation may be required to perform a proper funduscopic examination and VEP testing. The authors do not report electroret-inogram ( ERG) results because this test was not available to them. They do suggest that ERG is superior to VEP in its ability to detect vigabatrin toxicity. Brex PA Ciccarelli MD, O'Riordan JI, et al. A longitudinal study of abnormalities on MRI and disability from multiple sclerosis. N Engl.) Med 2002: 346: 158- 64. The authors monitored 68 patients with isolated syndromes suggestive of multiple sclerosis ( MS) ( including optic neuritis) and reported the correlations between early magnetic resonance imaging ( MRI) lesion volume, change in MRI lesion volume, and long- term disability. Not surprisingly, 50% o of the isolated syndromes were optic neuritis. The patients were assessed at 12.5 to 16.8 years ( mean 14.1 years) after diagnosis, when their mean age was 45 years. Only one patient received long- term interferon therapy. Clinically definite MS developed in 48 patients ( 68%). The median EDSS score was 3.25 ( with 31 %> of patients scoring > 6.0). The EDSS score at 14 years correlated moderately with the change in lesion volume on MRI over Copyright © Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited. JNeuro- Ophthalmol, Vol. 22, No. 3, 2002 IN OTHER JOURNALS the first 5 years ( r = 0.61) and the lesion volume at 5 years ( r = 0.60). The change in lesion volume over the first 5 years correlated moderately with EDSS scores, but changes in lesion volume from 5 to 10 years and 10 to 14 years showed a much weaker correlation. The number of lesions on MRI at baseline correlated moderately with the EDSS score at 14 years ( r = 0.47), as did the number of lesions at 5 years ( r = 0.55) and 10 years ( r = 0.45). This study is an important natural history study, and similar studies are unlikely now that agents that substantially modify lesion volume on MRI are used to treat relaps-ing- remitting MS. The study suggests that the number of lesions and the lesion volume on MRI in patients with early MS are moderately predictive of long- term disability. Given only moderate correlation, the authors point out that MRI lesion volume alone should not be used as the sole determinant when deciding about disease- modifying treatments. Weaknesses of this MRI- based study include the change in magnetic field strength ( baseline study used 0.5 Tesla, and follow- up study used 1.5 Tesla) and change in image slice thickness ( 10 mm at baseline and 5 mm in follow- up). Herdman SJ, Schubert MC, Tusa RJ. Role of central preprogramming in dynamic visual acuity with vestibular loss. Arch Otolaryngol Head Neck Surg 2001; 127: 1205- 10. The vestibulo- ocular reflex ( VOR) is augmented by the pursuit/ optokinetic ( OKN) system, the cervico- ocular reflex, central programming, and anticipatory intent. When the vestibular system is impaired, these mechanisms are still present and serve to minimize retinal slip and visual degradation during head movement. The authors studied the ability of central programming to augment the VOR by comparing dynamic visual acuity ( DVA) during predictable ( active) and unpredictable ( passive) head rotation. They hypothesized that DVA would be significantly worse with unpredictable head rotation compared with predictable head rotation irrespective of the vestibular of the individual, and that subjects with bilateral vestibular loss would have the greatest decrement in DVA scores during unpredictable ( passive) head rotation. DVA was recorded in 26 subjects with normal vestibular status, 20 subjects with unilateral vestibular impairment, and 7 subjects with bilateral vestibular impairment. Vestibular loss was confirmed by clinical examination, rotational chair testing, and caloric testing. Predictable and unpredictable head movements were generated by the subject and examiner, respectively. Standardized optotypes were projected only during head rotation with velocities between 120 and 180 degrees/ s. Significant differences were found between DVA-predictable and DVA- unpredictable scores in all three groups ( P < 0.02 for all three groups). The largest decrement in DVA did indeed occur in subjects with bilateral vestibular impairment during unpredictable head rotations. This is the first study to show a significant difference between VOR gain measured during active and passive head rotation in healthy subjects. The authors reason that this is because previous studies used passive head rotations that were predictable and allowed for central programming to augment the VOR. DVA can be readily used in the clinic to distinguish unilateral and bilateral vestibular loss from normal patients. This paper suggests that this test is best performed with unpredictable passive head rotations. Pan CC, Chen PC, Wang LS, et al. Thymoma is associated with an increased risk of second malignancy. Cancer 2001; 92: 2406- 11. Thymomas occur frequently in patients with myasthenia gravis. The authors examined the association between thymoma and a second malignancy. They retrospectively reviewed 192 consecutive patients with thymoma and compared the incidence of second malignancy with that in two different " control" groups. The first control group consisted of 206 patients undergoing thymectomy for non-thymomatous reasons. The second control group consisted of 1,426 patients with nasopharyngeal carcinoma. The mean follow- up period for the three groups was 74, 72, and 70 months, respectively. A second malignancy occurred in 8% of patients in the thymoma group, 2% of patients in the nonthymoma group ( OR 3.81, CI 1.05- 13.81), and 2% of patients in the nasopharyngeal carcinoma group ( OR 4.89, CI 22.26- 10.53). It is of interest that 73 of the 192 patients in the thymoma group and 102 of the 206 patients in the nonthymoma group had myasthenia gravis. However, the presence of myasthenia was not a significant risk factor for the development of a second malignancy. Among those in whom a second malignancy did develop, no consistent pattern of ex-trathymic sites was identified. Although the authors' choice of a " control" group may be questioned, this paper still lends support to the recommendation that clinicians should be aware that second malignancies occur more commonly in patients with thymomas ( including those who also have myasthenia gravis). ^ 254 „.. „ . . © 2002 Lippincott Williams & WUkins , Copyright © Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited. |