Keigo Shikishima

Baseline Features of Idiopathic Optic Neuritis as Determined by a Multicenter Treatment Trial in Japan

BACKGROUND An optic neuritis treatment trial was conducted at 30 clinical centers in Japan using the same protocol. Patient participation was based on: age range of 14-55 years; acute symptoms indicative of unilateral optic neuritis of unknown or demyelinating origin; visual symptoms of 14-day duration or less; relative afferent pupillary defect in affected eye; and normal or swollen optic disc of affected eye. CASES Initially, 102 patients qualified for participation; baseline data were obtained for analysis from 70 of these patients. Demographic characteristics of Japanese patients with optic neuritis were clarified and compared with those in a US study. OBSERVATIONS The incidence of ocular or periocular pain and the presence of periventricular plaques were noted to be lower, and the incidence of disc swelling higher, in the Japanese patients, suggesting racial differences in the characteristics of the disease. Such differences may possibly be related to the lower incidence of multiple sclerosis in Japanese patients. The results of visual function tests were virtually the same in both studies. The nonaffected eyes of more than half the patients showed abnormal mean deviation in Humphrey field analysis, as also noted in the US study. CONCLUSIONS The baseline clinical features of optic neuritis in the Japanese patients have been defined. Some racial differences in the characteristics of the disease may exist.

Multicenter Clinical Trial for Evaluating Methylprednisolone Pulse Treatment of Idiopathic Optic Neuritis in Japan

BACKGROUND A randomized, controlled clinical trial was conducted in 1991 to compare an intravenous megadose of methylprednisolone with a control drug (mecobalamin) for treating acute idiopathic optic neuritis. CASES Sixty-six cases from 22 clinical centers throughout Japan were examined to evaluate the treatment on visual function parameters, such as visual acuity, visual field, color vision, contrast sensitivity, and critical flicker frequency. OBSERVATIONS The methylprednisolone pulse treatment group showed faster recovery of visual function, particularly the visual acuity at 1 week (P<.05), Humphrey field analyzer mean deviation at 3 weeks (P<.05), and color vision at 1 week (P<.05). Recovery of contrast sensitivity at several different spatial frequencies was significant in the pulse treatment group at 1 (P<.01), 2 (P<.05), and 4 weeks (P<.05) after the start of treatment. Visual function test results at 12 weeks and 1 year were essentially the same in the two treatment groups. Side effects appeared more frequently in the pulse treatment group than in the control (P<.05). CONCLUSIONS Pulse treatment does not appear effective for idiopathic optic neuritis even though visual function in the pulse treatment group of this trial recovered more quickly during the initial phase compared to the controls. More effective and specific treatment should be established for optic neuritis.

Endothelial nitric oxide synthase gene polymorphisms in non-arteritic anterior ischemic optic neuropathy

BackgroundTo examine the association between the polymorphisms of the endothelial nitric oxide synthase (eNOS) gene and the occurrence of non-arteritic anterior ischemic optic neuropathy (NAION).MethodsWe studied 15 patients with NAION (mean age, 62 years; 60% male). We investigated two polymorphisms of the eNOS gene, Glu298Asp polymorphism of exon 7 and T(–786)C polymorphism of the promoter region. The genotype distribution in NAION was compared with the control (mean age, 63 years; 63% male) distribution.ResultsThere was no significant difference in the genotype distribution of the Glu298Asp polymorphism between the NAION and control groups (P=1.000), whereas the genotype distribution of the T(–786)C polymorphism varied significantly between the patients with NAION and control subjects (P=0.002). After adjusting on covariates, individuals with the CC genotype of the T(–786)C polymorphism were more likely to develop NAION compared with those with TT genotype (odds ratio=0.09: 95% CI 0.01–0.86).ConclusionsWe found an increased prevalence of T(–786)C polymorphism of the eNOS gene in patients with NAION. Our data suggest that the T(–786)C polymorphism of the eNOS gene may be an important risk factor in the development of NAION in Japanese subjects.

Metastasis to the orbit from transitional cell carcinoma of the bladder

PurposeTo demonstrate the pathological features of the extremely rare metastatic transitional cell carcinoma (TCC) from the bladder to the orbit, and to review the literature on metastatic TCC to the orbit.MethodsA 74-year-old man experienced 2 weeks of red eye, proptosis, diplopia, pain, and visual loss in the right eye. Three years previous to the current presentation, the patient had undergone a transurethral resection for superficial and moderately differentiated TCC of the bladder. A transseptal anterior orbitotomy was performed.ResultsHistopathological examination of the orbital lesion revealed nests of carcinomatous cells. Atypical pleomorphic cells with vacuolated cytoplasm were evident. The cellular morphology of the orbital lesion was identical to that of the primary TCC. There have been 12 previously reported cases of metastases to the orbit from TCC of the bladder, with the time from onset of primary TCC to observation of ocular symptoms ranging from 3 weeks to 11 years. Mean survival after orbital metastasis developed from TCC was 3.0 months.ConclusionThis study presents a detailed description of the pathological features of metastatic TCC in the orbit. In cases of orbital metastasis from TCC, patient prognosis is very poor. Jpn J Ophthalmol 2006;50:469–473

Pathological evaluation of orbital tumours in Japan: analysis of a large case series and 1379 cases reported in the Japanese literature

Purpose:  To review epidemiological features of orbital tumours in Japan.

Anti-aquaporin-4 antibody-positive familial neuromyelitis optica in mother and daughter

BackgroundCases of anti-aquaporin (AQP)-4 antibody-positive familial neuromyelitis optica (NMO) in mothers and daughters are described.ParticipantsThe demographic, clinical, neuroimaging, and anti-AQP-4 antibody status were investigated in four patients from two Asian families with anti-AQP-4 antibody-positive NMO.ObservationsNMO was diagnosed in both mothers and daughters using the latest diagnostic criteria. All patients were anti-AQP-4 antibody-positive, and only one had an autoimmune background. The Japanese family presented with a poor visual outcome due to multiple occurrences of optic neuritis, whereas the Korean family presented with a good visual outcome. Disease onset occurred at different ages, even within the same family.ConclusionsThese cases may enhance the understanding of the genetic contribution to NMO. Our findings suggest that familial history must be carefully examined in patients with NMO.

A case of malignant myoepithelioma in the lacrimal gland

BACKGROUND A case of malignant myoepithelioma in the lacrimal gland is reported. CASE The patient, a 77-year-old male with increasing proptosis in the left eye was referred to us. Steroid therapy was not effective, therefore lateral orbitotomy was performed. RESULT On pathological examination, the proper structure of actini was normal, but the cord of actini revealed the destruction layer tapering into the surrounding area. The tumor was composed of sarcomatoid changes with spindle-shaped cells, collagen fibers, and myxoid pattern. immunohistochemically, tumor cells were positive for epithelial membrane antigen (EMA) and alpha-smooth muscle actin (alpha-SMA). Electronmicroscopy showed desmosomes in intercellular junctions. CONCLUSION Recently, there have been attempts to distinguish myoepithelioma from pleomorphic adenoma on the basis of cellular organization. In our case, duct formation was less than in pleomorphic adenoma. Therefore we diagnosed this tumor as malignant myoepithelioma.

A limited form of neuromyelitis optica with a lesion of the fourth nerve nucleus.

ing pressure on repeat lumbar puncture was 44 cm H2O. At this point, the patients mother reported that the child had OSA, which was diagnosed 2 years previously. Polysomnographic studies had demonstrated severe OSA with a respiratory disturbance index of 21 events per hour. The patient was irregularly using a continuous positive airway pressure machine. On physical examination, the patient was found to have hypertrophic tonsils and adenoids with complete obstruction of her nasopharynx. She underwent adenotonsillectomy and postoperatively reported immediate relief of her headaches. At her last visit, she had complete resolution of all symptoms referable to IIH and subjective improvement in her sleeping pattern. Recent studies have suggested a potential relationship between IIH and OSA (2–6). The nocturnal hypoxemia and hypercarbia present during apneic episodes cause cerebral vasodilation and secondary increased ICP (7). OSA may be a risk factor in the worsening of IIH, the importance of which is compounded by the fact that the 2 conditions frequently coexist in patients with obesity. Screening for papilledema is recommended in all patients with OSA who have visual symptoms (8,9). In children who are found to have OSA and IIH, airway obstruction caused by adenotonsillar enlargement should be considered. We are not aware of previously reported cases where the removal of adenotonsillar tissue led to significant improvement in OSA and resolution of intracranial hypertension.

A case of neuromyelitis optica developing into myelitis 25 years after optic neuritis

1. Chu JK, Cho YJ. A case report of human thelaziasis. Kisaengchunghak Chapchi 1973;11:83–86. 2. Hong ST, Lee SH, Shim YB, Choi JS, Choe JK. A human case of thelaziasis in Korea. Kisaengchunghak Chapchi 1981;19:76–80. 3. Choudhury AR. Thelaziasis. Am J Ophthalmol 1969;67:773–774. 4. Schultz GR. Intraocular nematode in man. Am J Ophthalmol 1970;70:826–829. 5. Zakir R, Zhong-Xia Z, Chioddini P, Canning CR. Intraocular infestation with the worm, Thelazia callipaeda. Br J Ophthalmol 1999;83:1194–1195.

Interpretation of visual field defects respecting the vertical meridian and not related to distinct chiasmal or postchiasmal lesions

Hemianopia respecting the vertical meridian generally results from a disturbance in the optic chiasm or the postchiasmal visual pathway. We present five unusual patients with visual field defects respecting the vertical meridian that were not related to distinct chiasmal or postchiasmal lesions, as determined by magnetic resonance imaging (MRI). Optic neuritis, plus the influence of the testing algorithm for perimetry, was a possible cause in two cases. The symptoms of one patient with homonymous hemianopia were a functional deficit. In the two other cases, the visual field defects may have been caused by optic disc abnormalities. Although visual field defects respecting the vertical meridian, and without any evidence of distinct disease, are uncommon, neurologists and neuro-ophthalmologists should consider the differential diagnosis if MRI is negative for distinct lesions in the optic chiasm or the postchiasmal visual pathway.