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Clinical characteristics of Japanese children with optic neuritis.

The clinical characteristics of children with optic neuritis have been reported to be different from that of adults and to vary among different races. To determine the clinical characteristics of Japanese children with optic neuritis, we examined the medical records of 41 children who were diagnosed with optic neuritis at the Department of Ophthalmology of Chiba University Hospital between January 1979 and December 2001. Information on the sex, age of onset, laterality, initial visual acuity, final visual acuity, recent infections and immunizations, and presence of systemic neurologic diseases, such as multiple sclerosis or acute disseminated encephalomyelitis, was obtained. These findings revealed that the clinical features of optic neuritis in children were similar to those reported earlier. The percentage of optic neuritis cases that developed multiple sclerosis in children was similar to that for children in Europe and North America, and this percentage was not lower than that in adults in our clinic. These findings can be taken as the characteristics of optic neuritis of children in Japan.

Adolescent↗

Optic neuritis: characteristics and visual outcome.

OBJECTIVE: To determine clinical characteristics of patients with optic neuritis and visual outcome after intravenous methylprednisolone treatment. METHOD: A total of 81 patients with optic neuritis were reviewed retrospectively with regard to their clinical characteristics by dividing into two groups as follows: group I had isolated optic neuritis and group II had optic neuritis with demyelinative disease. The visual outcome in these patients before and after intravenous methylprednisolone treatment was analyzed. RESULTS: Of 81 patients with optic neuritis, 63 patients (77.8%) had isolated optic neuritis and 18 (22.2%) patients were optic neuritis with demyelinative disease. The ages of the patients ranged from 16 to 59 years (mean = 35.3 years) in patients with isolated optic neuritis and from 16 to 73 years of age (mean = 35.8 years) in patients with optic neuritis with demyelinative disease. After treatment, 45 patients (52 eyes) with isolated optic neuritis and 14 patients (25 eyes) with optic neuritis with demyelinative disease who were followed-up for more than 10 days were studied. After treatment, 60 per cent of the isolated optic neuritis patients and 24 per cent of the optic neuritis patients with demyelinative disease had a visual acuity of 6/12 or better respectively. The isolated optic neuritis who had an onset interval to treatment of less than 8 days had a visual acuity better than 6/9 in 75 per cent. CONCLUSION: The final visual outcome in patients with isolated optic neuritis who received earlier treatment was better than those who received treatment later.

Adolescent↗

Current concepts of the pathogenesis of optic neuritis associated with multiple sclerosis.

Optic neuritis may occur as an isolated entity or as a manifestation of multiple sclerosis (MS), a widespread central nervous system demyelinating disease. Clinical, electrophysiological, magnetic resonance imaging and neuropathologic data support the hypothesis that "idiopathic" optic neuritis represents a restricted form of MS. The current evidence for viral, cell mediated, and antibody-induced etiologies of MS and optic neuritis are reviewed.

Autoantibodies↗

[Recovery of visual field defects in ischemic optic neuropathy and idiopathic optic neuritis].

Fifty-four eyes of 41 patients with optic nerve disease demonstrating acute visual field defects without any traumatic, compressive, or other known etiology were classified into four categories. Those showing poor recovery of visual field defects were ischemic optic neuropathy which was subclassified into either anterior ischemic optic neuropathy (AION) or posterior ischemic optic neuropathy (PION) according to the ophthalmoscopic changes in the optic nerve head. Those showing good recovery of visual field defects were idiopathic optic neuritis which was subclassified into either papillitis or retrobulbar neuritis according to the ophthalmoscopic pathology of the optic disc. Patients with ischemic optic neuropathy were significantly older than those with optic neuritis. All eyes with optic neuritis showed good recovery of vision, whereas those with ischemic optic neuropathy showed varying outcomes of vision. With regard to the pattern of field defect, central or paracentral scotoma was predominant in all but eyes with AION in which altitude defect predominated. Pale swelling of the optic nerve head and angiographic evidence of circulatory disturbance in the optic disc or adjacent choroid were common findings in eyes with AION, whereas such findings were never observed in eyes with papillitis. The amplitude of pattern visual evoked potential was significantly lower in eyes with PION than in those with retrobulbar optic neuritis. Four patients classified as optic neuritis developed into multiple sclerosis in the follow-up study. It was concluded that poor recovery of visual field defect is one of the most convincing evidences for the diagnosis of ischemic optic neuropathy.

Adult↗

Atopic optic neuritis.

PURPOSE: To report a case of optic neuritis associated with atopic dermatitis, which may represent an optic nerve counterpart of atopic myelitis. METHODS: Clinical examination, MRI, blood investigations. RESULTS: A 34-year-old man with severe atopic dermatitis developed a steroid-responsive bilateral optic neuritis. Serum IgE was highly elevated, with high titers of mite-specific IgE. Clinical ophthalmological fluctuations paralleled those of atopic dermatitis. CONCLUSIONS: The clinical features and parallel fluctuations strongly suggest that the patient's optic neuritis was related to atopic dermatitis. This atopic optic neuritis may represent the optic nerve equivalent of atopic myelitis.

Adult↗

Optic neuritis during lactation.

The condition called "lactation optic neuritis" has been previously considered a clinical entity of its own. Four women, who developed optic neuritis within 1-12 months while breast-feeding their infants, were investigated ophthalmologically and neurologically in order, to find specific clinical features for this condition. The course of the disorder was similar to classic optic neuritis without lactation. The clinical history and laboratory findings in three of the four patients suggested a demyelinating disorder. It is possible that the decreased immunosuppressive activity just after pregnancy induces the manifestation of an underlying demyelinating disease. The existence of "lactation optic neuritis," however, is questioned as a separate entity of its own. Lactation together with decreased immunosuppression may merely act as a provocateur in the onset of optic neuritis, which in many cases is the first clinical manifestation of incipient multiple sclerosis.

Adult↗

Correlation between optic disc atrophy and aetiology: anterior ischaemic optic neuropathy vs optic neuritis.

BACKGROUND: The morphologic features of swollen disc in the acute stage of optic neuritis and anterior ischaemic optic neuropathy (AION) have been extensively investigated in contrast to the morphologic features of optic disc atrophy after these events. OBJECTIVE: : A prospective study to evaluate the morphologic features of optic disc atrophy 6 months or more after optic neuritis and nonarteritic AION. PATIENTS AND METHODS: A total of 35 optic discs after nonarteritic AION (n=27) and 24 after optic neuritis (n=19) in otherwise healthy subjects have been evaluated by direct fundoscopic examination with a +90 diopters lens and optic disc photography. The average age of patients at the onset of AION was 57.8 years (range: 38-80) and at the onset of optic neuritis was 32.6 (range: 19-46). The female:male ratio was 18 : 17 in the former and 15 : 9 in the latter. The evaluated parameters included: degree of rim pallor (0 to +3), location of rim pallor, height of rim above the retina, depth and width of cup, peripapillary retinal artery to vein (A : V) ratio, and peripapillary pigment epithelial atrophy. A comparison was made also with 17 age-matched normal discs of 17 patients. Statistical significance was calculated with chi(2) and Fisher's exact test. RESULTS: Most of the discs after AION were paler (+2: 70%, +3: 26%) than after optic neuritis (normal colour: 8%, +1: 58%, P< or =0.007). Rim segmental involvement after AION was usually either superior 'altitudinal' (53%) or inferior 'altitudinal' (29%), whereas after optic neuritis, it was usually either temporal-central (papillomacular) (42%) or diffuse temporal (42%, P<0.0001). Discs had lower A : V ratio (1 : 3, 40%) after AION compared with optic neuritis (1 : 3, 8%) (P=0.007). There were no significant differences between the two groups in height of the rim, cupping, and peripapillary atrophy. CONCLUSIONS: : A combination of the degree of rim pallor, location of rim pallor, and A : V ratio may be of value in assessing the aetiology of optic disc atrophy when no previous clinical data are available and a compressive lesion has been ruled out.

Acute Disease↗

Optic nerve head circulation in nonarteritic anterior ischemic optic neuropathy and optic neuritis.

PURPOSE: To quantify optic nerve head circulatory abnormalities in patients with unilateral nonarteritic anterior ischemic optic neuropathy (NAION) or optic neuritis (ON), and to assess the potential of such measurements to differentiate NAION from ON. DESIGN: Prospective, cross-sectional, observational study. PARTICIPANTS: Thirty consecutive patients with unilateral NAION, 22 consecutive patients with unilateral ON, and 50 healthy control subjects. METHODS: All subjects underwent a complete neuro-ophthalmologic evaluation. The widths of Doppler-broadened frequency spectra, which are directly proportional to the speed of blood cells flowing through the capillaries of the optic nerve head, were measured at multiple sites in both eyes of each subject. The variation of Doppler broadening (DB) with age was determined in the control subjects. Doppler broadening values in the patients were compared between similar sites in affected and contralateral eyes, and between both affected and contralateral eyes and the age-adjusted values determined in the control subjects. MAIN OUTCOME MEASURES: The differences in DB between (1) the affected and contralateral eyes of the patients, (2) the patients and the control subjects, and (3) the patients with NAION and those with ON. RESULTS: In NAION, DB was decreased at both temporal (-20.2% and -18.5%) and nasal (-12.8% and -12.4%) sites of the nerve head in affected eyes compared with contralateral eyes or eyes of control subjects. In ON, DB was also decreased at temporal sites (-11.3% and -9.2%) in affected eyes compared with contralateral or control eyes. At nasal sites, there were no significant differences in DB in affected eyes of ON patients compared with contralateral or control eyes. The DB decreases were significantly greater in NAION patients than in ON patients. CONCLUSIONS: Optic nerve head circulatory abnormalities are present in patients with NAION or ON. This is the first demonstration of such abnormalities in ON, a finding consistent with the recent attention given to the phenomenon of axonal loss in this disease. Although there are differences in the circulatory abnormalities between the 2 diseases that provide insights into the pathophysiological mechanisms at play, they are not large enough to enable the clinician to distinguish between ON and NAION in an individual patient.

Adolescent↗

Acute Optic Neuritis.

In acute monosymptomatic optic neuritis, treatment with oral prednisone alone should be avoided. Therapy with intravenous methylprednisolone (1 g/day for 3 days) followed by 11 days of oral prednisone (1 mg/kg with a short taper) should be considered instead. This is particularly true if a patient considers accelerated visual recovery to be particularly urgent or if magnetic resonance imaging (MRI) demonstrates three or more signal abnormalities consistent with demyelination. In patients without a diagnosis of clinically definite multiple sclerosis (CDMS), an MRI should be considered to assess the prognosis for developing multiple sclerosis (MS) and to eliminate other causes of optic neuropathy. Foregoing an MRI or steroid treatment is an acceptable option. Chest x-ray, blood tests, and lumbar puncture are not necessary in evaluating patients with typical clinical features of optic neuritis. These tests may be appropriate, however, for patients who are about to undergo corticosteroid therapy, which could complicate or mask an unrecognized condition.

Journal Article↗

[Visual functions of optic neuritis in children caused by demyelinating disease].

Optic neuritis in childhood caused by demyelinating disease is very rare, and its effect on visual function has not been well documented. The visual function of 11 patients treated in the last 11 years was retrospectively studied and the results were compared with 15 cases of optic neuritis of unknown etiology. Optic neuritis in children with demyelinating disease was characterized as follows. 1. abnormal visual evoked cortical potentials (VECP) and color vision were found not only in the affected eye but also in the non-affected fellow eye, 2. VECPs became normal long after the recovery of visual acuity, 3. full recovery of visual function took longer, 4. at onset, vision was more severely affected, but showed a good outcome. These findings provide useful information to make a diagnosis of multiple sclerosis even at the first attack of optic neuritis.

Adolescent↗

Lesion discrimination in optic neuritis using high-resolution fat-suppressed fast spin-echo MRI.

Fast spin-echo (FSE) is a new sequence with acquisition times currently down to one-sixteenth of those obtained with conventional spin-echo sequences, which allows high-resolution (512 x 512 matrix) images to be acquired in an acceptable time. We compared the higher resolution of FSE with the medium resolution of a short inversion-time inversion-recovery (STIR) sequence in depicting the optic nerves of healthy controls and patients with optic neuritis. Optic nerve MRI examinations were performed in 18 patients with optic neuritis and 10 normal controls. Two sequences were obtained coronally: fat-suppressed FSE (FSE TR 3250 ms/TEef 68 ms, echo-train length 16, 4 excitations, 24 cm rectangular field of view, 3 mm interleaved contiguous slices, in-plane resolution 0.5 x 0.5 mm) and STIR (TR 2000 ms/TE 50 ms/TI 175 ms, in-plane resolution 0.8 x 0.8 mm, slice thickness 5 mm). FSE demonstrated much more anatomical detail than STIR, e.g. distinction of optic nerve and sheath. Lesions were seen in 20 of 21 symptomatic nerves using FSE and in 18 of 21 using STIR. Nerve swelling or partial cross-sectional lesions of the optic nerve were each seen only on FSE in 3 cases. Fat-suppressed FSE imaging of the optic nerve improves anatomical definition and increases lesion detection in optic neuritis.

Adult↗

The epidemiology of optic neuritis in Finland.

Evidence has been presented that optic neuritis partially reflects benign cases of MS which are lost in the epidemiological investigation of the disease. As part of a large epidemiological investigation of MS, 221 patients with pure optic neuritis were identified during the period from January 1, 1967 to December 31, 1971. The mean annual incidence for the whole of Finland was 0.94 per 100,000 population. The female to male ratio was 1.7. The mean age at onset was 31.2 years. The distribution of optic neuritis by counties showed the highest mean annual incidence in the southwestern county of Turku and Pori (1.69) and in the western county of Vaasa (1.68). The prevalence data for MS were highest in these counties. A highly significant deviation from a random distribution according to place at onset and place of birth was obtained. Even the geographical distribution by smaller units, i.e. the combined clerical districts, revealed a firm accumulation to the western districts in the county of Vaasa and to the southwestern districts in the county of Turku and Pori. Thus, optic neuritis showed a similar geographical distribution of Jalasjärvi with several familial cases of MS did not increase the familial percentage when both conditions were considered as a single group. The risk of getting optic neuritis seems to depend on the influence of factors present during childhood. The epidemiological data point to a common factor in the aetiology of optic neuritis and MS.

Adolescent↗

Optic neuritis in children with poor recovery of vision.

We reviewed the records of 10 children with optic neuritis in whom recovery of vision was poor or incomplete. Our cases were otherwise similar to those described in previous studies in that they were always bilateral, often accompanied by a viral prodrome (seven of 10), and usually associated with disc oedema (seven of 10). Seven of twenty eyes had a final visual acuity of 6/60 or worse and only one patient regained 6/6 vision in either eye. In three patients the best vision in either eye was 6/60 or worse. Recovery of vision was often slow, taking up to six years. Five of 10 patients have developed multiple sclerosis (MS), and one child had acute disseminated encephalomyelitis (ADEM) with optic neuritis. Optic neuritis in children does not always carry a good prognosis for recovery of vision; however, the failure of vision recovery in a short period of time does not necessarily indicate a poor outcome. Some children with optic neuritis develop MS, which can develop even when optic neuritis follows a viral illness.

Adolescent↗

Evolving management of optic neuritis and multiple sclerosis.

PURPOSE: To review the relation of optic neuritis to multiple sclerosis (MS) and the indications, modalities, and results of therapy for optic neuritis, for both visual and general neurologic function. DESIGN: Literature review and author's experience. METHODS: Analysis of both laboratory and clinical evidence supporting the use of corticosteroids, immunomodulation agents, and other modalities in the treatment of optic neuritis and MS. RESULTS: Although treatment of optic neuritis with corticosteroids may hasten visual recovery to a minor degree, it has no long-term beneficial effect on visual outcome. Optic neuritis is frequently the initial manifestation of multiple sclerosis. The risk of later development of clinically definite MS (CDMS) correlates with white matter demyelinative lesions on magnetic resonance imaging (MRI). The role of corticosteroid therapy alone in reducing the risk of subsequent MS is unclear, but recent studies suggest that the combination of immunomodulation agents (IMAs) and corticosteroids significantly reduces the later development of MS. Current research indicates that, contrary to previous doctrine, axonal damage is an early finding in MS. CONCLUSIONS: The risk of MS after optic neuritis may be predicted. The use of corticosteroids and IMAs, particularly in those at substantial risk, reduces the frequency and severity of developing CDMS. Earlier, more aggressive therapy in optic neuritis may be proven to reduce permanent axonal injury and progressive disability in MS.

Adjuvants, Immunologic↗

Long time echo stir sequence magnetic resonance imaging of optic nerves in optic neuritis.

Magnetic resonance imaging of optic nerves was obtained in 13 patients with acute optic neuritis and in 13 patients with a previous history of optic neuritis (ON), assessed by clinical, visual fields and visual evoked potentials evaluations. Results of the conventional short tau inversion recovery (STIR) sequence obtained with short time echo (STE-STIR: 22 ms) were compared with long time echo (LTE-STIR: 80 ms) sequence. The conventional STE-STIR sequence revealed lesions in 78.5% of acute ON and in 58.8% of optic nerves affected by previous ON. The LTE-STIR sequence was diagnostic in 92.8% of acutely symptomatic nerves, in 94.1% of nerves with previous ON. The calculated length of optic nerve lesions was significantly longer in imaging obtained with the LTE-STIR sequence than with the conventional STE-STIR sequences, both in acute and previous ON.

Adult↗

Computed tomography of chiasmal optic neuritis.

Three cases of chiasmal optic neuritis are described with bitemporal visual field loss and enlargement of the optic chiasm as demonstrated by computed tomography (CT). Exploratory craniotomies were performed in two of the patients, but no tumor was found. After corticosteroid treatment, all three patients experienced virtually complete restoration of normal vision. Although the CT presentation in these patients was indistinguishable from that of a chiasmal glioma, the clinical presentation of acute visual loss in an older child or adult should suggest chiasmal optic neuritis. A period of observation, with or without a trial of corticosteroids, might permit the latter diagnosis and obviate exploratory craniotomy.

Adolescent↗