OCR Text |
Show NEURO- OPHTHALMOLOGY AT LARGE 41st Annual Meeting of the Japanese Neuro- Ophthalmology Society, Kyoto, Japan, December 12- 13, 2003 This review includes highlights of some of the 72 platform presentations and 22 posters at the 41st Japanese Neuro- Ophthalmology Society meeting held in Kyoto, Japan, December 12- 13, 2003. There were approximately 300 registrants, mostly full- time or part- time practitioners of neuro- ophthalmology in Japan. The meeting president was Satoshi Kashii, MD ( Kyoto). TOXIC OPTIC NEUROPATHY Tomohiro Saijou et al ( Tokyo) reported a case of optic neuropathy presumed to be caused by a side effect of linezolid ( United States brand, Zyvox), an antibacterial agent. A 27- year- old woman had a bone marrow infection with methicillin- resistant Staphylococcus aureus during corticosteroid treatment of lupus. She was started on 1,200 mg linezolid per day, and within three months she noted blurred vision in both eyes and numbness in both legs. Her visual acuity was 20/ 30 OU. Visual fields showed central scotomas in both eyes and funduscopic examination showed bilateral disc edema. She was diagnosed as having optic neuropathy caused by systemic lupus erythematosus and treated with 1,000 mg intravenous methylprednisolone for one day. Visual acuity worsened to 20/ 200 OU. The linezolid was discontinued, and in two months, visual acuity recovered to 20/ 20 OD and 20/ 25 OS. The optic discs returned to normal. This appears to be the second report of optic neuropathy associated with linezolid. LEBER HEREDITARY OPTIC NEUROPATHY Reiko Kubo et al ( Tokyo) reported three cases of Leber hereditary optic neuropathy ( LHON) with a point mutation at 14,484 or 9,804, and showed the clinical differences from the more common LHON with a point mutation at 11,778. The two LHON cases with a point mutation at 14,484 showed some visual improvement and the LHON case with a point mutation at 9,804 showed chronic visual deterioration. Yuzo Nakao et al ( Osaka) compared three LHON 11778 cases in which the mother was the same but the father was different. Visual acuity varied between three families from 20/ 200 to 20/ 25. Considering that LHON cases with different point mutations have a similar clinical course, we do not know why Japanese patients have such clinical variability. ISCHEMIC OPTIC NEUROPATHY AND PAPILLEDEMA It is well known that the eNOS gene plays a major role in controlling vascular contractility. In a study that compared 13 patients with nonarteritic ischemic optic neuropathy ( NAION) and 101 healthy controls, Tutomu Sakai et al ( Tokyo) sought to determine the relationship between NAION and the eNOS gene. Abnormal mutation in the eNOS gene was positive in 15.3 8% of patients with NAION but in only 1% of controls. Tetsuo Ogino et al ( Hokkaidou) reported a case of transient visual loss caused by vitreopapillary traction. A 26- year- old woman noted blurred vision OS. Her visual acuity was 20/ 20 OU. A relative afferent pupil defect was present OS, and a visual field showed an enlargement of the blind spot and a centrocecal scotoma OS. Funduscopic examination showed a blurred optic disc margin with preretinal and subretinal hemorrhage around the left optic disc. One month later, the relative afferent pupil defect disappeared and the visual field defect was improved without treatment. Hideki Chuman et al ( Miyazaki) reported a case of pseudotumor cerebri syndrome secondary to a dural arteriovenous fistula. A 76- year- old man noted chronic blurred vision in both eyes. His visual acuity was 20/ 60 OU. A Goldmann visual field test showed an enlargement of the blind spot in both eyes, and funduscopic examination showed bilateral disc edema. Brain magnetic resonance imaging ( MRI) was normal. Lumbar puncture opening pressure was 300 mm H20 with no abnormality in the fluid constituents. MRI and magnetic resonance angiography ( MRA) denied a dural sinus thrombosis. However, MRA showed a slight enlargement of the left occipital artery, and a catheter cerebral angiogram showed a dural fistula between the left lateral sinus and the left occipital artery. The 170 JNeuro- Ophthalmol, Vol. 24, No. 2, 2004 Neuro- Ophthalmology At Large JNeuro- Ophthalmol, Vol. 24, No. 2, 2004 right sigmoid sinus was 90% occluded. The authors speculated that the fistula created high pressure in the dural venous sinuses, which could not be compensated because of poor flow in the right sigmoid sinus. The fistula was considered too complex to treat, so a stent was placed in the stenotic right sigmoid sinus. After stent placement, cerebrospinal fluid pressure decreased to 5 mm H20, as measured by lumbar puncture, and the papilledema improved. OPTIC NEURITIS Jonathan D. Trobe ( Ann Arbor, MI) delivered an invited lecture on the current management of optic neuritis. His main points were: 1) optic neuritis is usually part of multiple sclerosis ( MS); 2) a normal MRI at the time of the first attack of optic neuritis identifies a group with very low chance of MS; 3) a small minority of patients with optic neuritis, a normal MRI, and a very swollen optic disc have thus far not developed MS in 10 years of follow- up in the Optic Neuritis Treatment Trial conducted in the United States; 4) baseline MRI predicts relapses but not future disability; more sophisticated MRI sequences undergoing study may do better; 5) the best predictor of disability is the pace of the illness; 6) neurologic and visual disability after optic neuritis are relatively mild; 7) corticosteroid treatment at the time of acute optic neuritis hastens recovery slightly but has no long- term benefit; and 8) disease- modifying agents- interferon beta and copolymer- reduce the frequency of clinical relapses and the accumulation of MRI signal abnormalities but do not substantially affect neurologic disability. The upshot of these facts is that the rationale for early treatment of optic neuritis with disease-modifying agents is still not well- founded and the agents themselves are not very effective. Future studies may bring better remedies. FIG. 1. Dignitaries at the 2003 Japanese Neuro- Ophthalmology Society Meeting, Kyoto, Japan. Standing, left to right: Akio Tabuchi, MD, Kawasaki Medical School; Haruki Abe, MD, Niigata University School of Medicine; Satoshi Kashii, MD, Osaka Eye Hospital; Kazuo Nakatsuka, MD, Oita Medical University; Masato Wakakura, MD, Inouye Eye Hospital, Tokyo; Kazutaka Kani, Shiga University of Medical Science; Jonathan Trobe, MD, Ann Arbor, Ml; Avinoam Safran, MD, Geneva, Switzerland; Hideki Chuman, MD, Miyazaki Medical College; Kenji Kitahara, MD, The Jikei University School of Medicine; Motohiro Kiyosawa, MD, Tokyo Medical and Dental University Graduate School. Sitting, left to right: Emiko Adachi, MD, Chiba University; Satoshi Ishikawa, MD, Tokyo University and former president of the Japanese Neuro- ophthalmology Society; Hiroko Yamamoto, MD, Fujita Health University School of Medicine. 171 JNeuro- Ophthalmol, Vol. 24, No. 2, 2004 Chuman SUPRASELLAR TUMORS AND RIDDOCH PHENOMENON Hiroshi Shima et al ( Ishikawa) reported that 83 patients with treated suprasellar tumors and Riddoch phenomenon have a better prognosis for visual field improvement than do patients without Riddoch phenomenon. ORBITAL DISEASE Kimiko Gotou et al ( Tokyo) described the ophthalmic manifestations of Graves disease in teenagers. Among 302 patients ( 36 men, 266 women) between the ages of 13 and 19 years, the authors noted prominent exophthalmos relative to that found in older adults but less prominent motility disturbances and optic neuropathy. CAVERNOUS SINUS In a session on cavernous sinus lesions, two infectious cases led to bilateral blindness, both occurring in a setting of previous chronic corticosteroid treatment. However, a single noninfectious inflammatory case was cured with corticosteroid treatment, emphasizing the importance of distinguishing infectious from noninfectious inflammation in this region. There were three cases of sixth cranial nerve palsy associated with Horner syndrome, reminding us how specific is this combination of findings for cavernous sinus disease. One dural fistula case presented as an isolated fourth cranial nerve palsy, a reminder that cavernous fistula is a consideration in unexplained fourth cranial nerve palsy. INFRANUCLEAR OCULAR MOTILITY DISORDERS In a session devoted to intranuclear ocular motility cases, Taro Nakamori et al ( Aichi) described the first reported case of myasthenia gravis ( MG) combined with Lambert- Eaton syndrome ( LES). The patient was undergoing treatment with D- penicillamine for rheumatoid arthritis. The combination of MG and LES was diagnosed by electromyography. The authors attributed this " double hit" to an immune reaction to D- penicillamine. Shunichi Suzuki et al ( Hokkaidou) analyzed three cases of superior oblique myokymia using a magnetic search coil technique. They found that the fast phases corresponded to the contracting directions of the superior oblique muscle. One sixth cranial nerve palsy case was caused by pansinusitis. Three third cranial nerve palsy cases were caused each by a schwannoma, an inflammatory cavernous sinus lesion, and migraine. In the migraine case, Takayuki Takeshita ( Miyagi) showed MRI enhancement at the root of the third cranial nerve during an attack. Between attacks, there was no enhancement. These findings added further evidence that ophthalmoplegic migraine is an inflammatory cranial neuropathy. Akiko Masuda et al ( Hyogo) reported a case of multiple cranial neuropathies caused by relapsing polychondritis. A 53- year- old woman developed a sixth nerve palsy, which cleared spontaneously; then, she consecutively FIG. 2. Dignitaries at the meeting entrance (/ eft to right): Hideki Chuman, MD; Masato Wakakura, MD, chair of the 16th International Neuro- Ophthalmology Society ( INOS) meeting to be held in Tokyo, November 29 to December 6, 2006; Avinoam Safran, MD, invited speaker; Satoshi Kashii, MD, chair of the 2003 Japanese Neuro- Ophthal-ology Society Meeting; Kazuo Mukuno, MD, School of Allied Health Sciences, Kitasato University, Kanagawa, and current president of the Japanese Neuro- Ophthalmology Society. 172 © 2004 Lippincott Williams & Wilkins Neuro- Ophthalmology At Large JNeuro- Ophthalmol, Vol. 24, No. 2, 2004 developed right twelfth, left third, and left seventh nerve palsies, all appearing separately and disappearing without treatment. All studies were negative except for a tuberculin skin test. NUCLEAR AND SUPRANUCLEAR OCULAR MOTILITY DISORDERS In a session devoted to nuclear and supranuclear motility disturbances, Makiko Takagi et al ( Osaka) reported a case of upgaze deficit and skew deviation caused by damage at the right midbrain between the riMLF and the posterior commissure. Atsuko Nihira ( Hokkaidou) reported a case that showed an upgaze deficit from a single lesion in the medulla. Youji Takahashi ( Iwate) suggested a relationship between skew deviation and vertical smooth pursuit movement. Masaki Kondou et al ( Kyoto) suggested that the downgaze pathway is crossed. Hanako Matsunaga ( Tokyo) reported a case of acquired ocular motor apraxia caused by global hypoperfusion after surgery for aneurysm of the thoracic artery. Noriko Koga ( Tokyo) reported a rare case of alternating skew deviation between upgaze and downgaze in a patient with spinocerebellar degeneration. No family history was present and genetic examination was negative. Ken Jokura et al ( Kanagawa) reported that 27 ( 59%) of 46 patients who entered coma after cardiac arrest had vertical eye deviation. More than 40% of patients with upgaze deviation died; all patients with downgaze deviation developed a persistent vegetative state. VISUAL FUNCTION Takashi Kurachi et al ( Aichi) found that on- center bipolar cells and off- center bipolar cells affect the optokinetic nystagmus. Hiromasa Tsuda et al ( Tokyo) reported a homonymous hemianopia caused by infarction at the lateral geniculate body. Chiharu Tanaka et al ( Hokkaidou) reported a case of cerebral achromatopsia, pure alexia, prosopagnosia, and visual object agnosia caused by infarction of both occipital lobes. Masaki Yoshida et al ( Tokyo) showed how diffusion tensor imaging could visualize anterograde degeneration of nerve fibers after a cerebral hemorrhage. Kunihiko Akiyama et al ( Tokyo) compared the reproducibility of functional MRI from units of different institutes. They found better intra- individual than inter- individual reproducibility. VISUAL PERCEPTION Avinoam B. Safran ( Geneva, Switzerland) delivered an invited lecture entitled " Unperceivable Borders Between Illusion and Reality in Visual Function." He pointed out that we depend on illusions as a normal aspect of life. Because environmental information is often suboptimal, perception must rely on the brain reconstruction of contours or other image features without retinal input. Although illusions generally are beneficial, in some instances they generate misleading percepts because individuals are not aware of their occurrence. Perceptual filling- in is a type of visual illusion with major clinical implications. As a result of filling- in, defects in the visual system may remain unrecognized. This leads to underdiagnosis of visual field defects and delayed treatment. FUNCTIONAL MRI AND VISUAL EVOKED POTENTIALS Takekazu Ohi et al ( Miyazaki) reported a case of mainly upper limb visual ataxia caused by cerebral FIG. 3. Traditional Japanese dinner at the home of Satoshi Kashii, MD. Left to right: Hideki Chuman, Avinoam Safran, Satoshi Kashii, Joan Lo-wenstein ( Ann Arbor, Ml, wife of Jonathan Trobe), and Jonathan Trobe. 173 JNeuro- Ophthalmol, Vol. 24, No. 2, 2004 Chuman infarction. Brain MRI showed bilateral parietal lobe infarction. Using functional MRI, Mari Sakai et al ( Shiga) investigated the differential effect of viewing a solid stereogram and a random- dot stereogram. They showed that viewing the solid stereogram activated anterolateral right upper parietal lobe, whereas viewing the random stereogram activated posteromedial right upper parietal lobe. Keiko Momose et al ( Tokyo) showed the usefulness of the visual evoked potential ( VEP) color contrast response for the objective measurement of contrast sensitivity in congenital vision disturbances. Using multifocal VEP, Yoshida Shimada et al ( Aichi) showed that the visual signal that originates in nasal retina reaches the corresponding visual cortex 5.3 to 6.3 milliseconds earlier than the signal that originates in temporal retina. POSTER SESSION Yuri Ishida et al ( Shimane) reported a case of right hemispatial neglect and right homonymous hemianopia caused by infarction of the left thalamus and left lateral geniculate body. With functional MRI, Kunihiro Asakawa ( Tokyo) reported that processing of color with contour occurred in the ventral part of occipital lobe, whereas processing of color without contour occurred in the dorsal part of occipital lobe. Hisako Hayashi et al ( Kyoto) reported a case of dorsal midbrain syndrome caused by neurosarcoidosis. The patient was a 30- year- old man with light- near dissociation and skew deviation. MRI showed an enhancing area in the dorsal midbrain. Sarcoidosis was diagnosed by skin biopsy. The manifestations resolved completely after corticosteroid therapy. WORKSHOP lonathan D. Trobe, Hideki Chuman, and Satoshi Kashii presented a workshop entitled " The Proper Way to Prepare a Scientific Manuscript for Publication in the Journal ofNeuro- Ophthalmology." The major mistakes made by authors are: 1) the article's title does not convey the main idea; 2) the abstract does not faithfully recapitulate the article and contains unwarranted conclusions; 3) the introduction devotes too much to describing the condition and too little to describing why the project was performed ( the rationale); 4) the methods section fails to include complete data on when, where, and how the experiment was conducted and how subjects were accrued; 5) the results are poorly displayed with inadequate attention to tables, figures, and legends; 6) the discussion repeats the introduction instead of summarizing results and explaining their importance ( how they confirm or depart from available knowledge) and acknowledging the limitations of the study; and 7) the references contain too many errors. Hideki Chuman, MD, PhD Miyazaki University Miyazaki, Japan 174 © 2004 Lippincott Williams & Wilkins |