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PubMed1998

Acute optic neuritis: combined immunological markers and magnetic resonance imaging predict subsequent development of multiple sclerosis. The Optic Neuritis Study Group.

The diagnostic significance of intrathecally synthesized IgG and virus-specific antibodies to measles, rubella and varicella-zoster (MRZ) in cerebrospinal fluid (CSF) remains controversial in cases of acute optic neuritis (AON). This study evaluates the prognostic value of baseline CSF and serum markers in AON, and correlates them with magnetic resonance imaging (MRI) and progression to multiple sclerosis (MS). Paired CSF and serum samples from 36 AON patients, 26 MS patients and 22 controls were analyzed for albumin, IgG, oligoclonal IgG (OI), MRZ antibodies, and blood-CSF barrier function; baseline MRI scanning of the head was also performed. The most sensitive parameter for detection of intrathecal inflammation in AON was OI (75%). Baseline MRI scans revealed abnormalities in 46% of the 28 patients with AON. Fifty percent of AON patients developed MS over the following 4 years. Ninety four percent of patients progressing to MS were positive for either OI, MRI or both. Of the AON patients initially positive for MRI and intrathecally-produced MRZ antibodies, 86% developed MS after 4 years. Only 17% of AON patients with negative results for OI and MRI developed MS. Six patients with abnormal OI but normal MRI progressed to MS. CSF and serum analyses, together with MRI, are the methods of choice for prognostic evaluation of patients with AON.

Acute Disease↗
PubMed1997

Cytomegalovirus optic neuritis.

Cytomegalovirus (CMV) optic neuritis is a rapidly blinding disease. Untreated eyes have a final visual acuity of no light perception. Treated eyes maintain visual acuity of approximately 20/100. Relapse occurs often in subjects maintained on single-dose ganciclovir and is controlled by double-dose ganciclovir. Central visual field testing is indicated to detect indolent cases of optic neuritis, because relapse can be silent with stable visual acuity and fundus appearance. Survival of patients with CMV optic neuritis is comparable to that of a matched group having CMV retinitis alone. Early recognition and therapy of CMV papillitis protect against irreversible visual loss. CMV papillitis does not carry a worse prognosis for survival than CMV retinitis alone.

Antiviral Agents↗
PubMed2003

Combination of high-dose intravenous immunoglobulins and itraconozole in treating chronic mycotic demyelinating optic neuritis.

Mycotic demyelinating optic neuritis is a neurological disorder of the visual system caused by mycotoxins released by indoor toxic molds. Although the health effect of indoor toxic mold on the population worldwide is now one of the "emerging diseases", its involvement in chronic demyelinating optic neuritis has not been reported. Most of the neurological and immunologic abnormalities associated with toxic mold mycotoxins are very difficult to treat successfully, especially neural demyelination of the central and peripheral nervous systems. This paper presents the case of a 42-year-old white female, in whom chronic demyelinating optic neuritis with persistent visual defects due to chronic exposure to toxic molds was diagnosed at the age of 34 years. In spite of all the therapeutic services given to her for over 8 years, her illness persisted and was difficult to treat. However, we successfully treated her with a combination of intravenous immune globulin (IVIG) and itraconozole (Sporanox) when all other treatment modalities failed. This is probably the first report where persistent toxic mold-induced neurological and immunologic disorders were successfully treated with a combination of itraconozole and IVIG.

Adult↗
PubMed1997

Diameter of the optic nerve in idiopathic optic neuritis and in anterior ischemic optic neuropathy.

PURPOSE: There is considerable overlap in the clinical profile of patients with idiopathic optic neuritis (ON) and anterior ischemic optic neuropathy (AION). We tested the hypothesis that the retrobulbar diameter of the optic nerve may be a criterion for the differential diagnosis between ON and AION. METHODS: The diameter of the optic nerve was measured by B-scan ultrasonography with the eye in an abducted position. Only patients with a unilateral optic neuropathy were included, 16 ON patients (mean age 24 years, 5 with and 11 without disc swelling) and 9 patients with AION (mean age 72 years). As controls for the ON patients 10 young normal subjects (mean age 25 years) and as controls for the AION patients 10 elderly subjects with eye problems not related to the optic nerve (mean age 76 years) were examined. RESULTS: In the ON patients with disc swelling the diameter of the optic nerve was 5.4 +/- 0.5 mm in the affected and 3.0 +/- 0.3 mm in the unaffected side. This difference was significant (Wilcoxon-test, p = 0.043). In the ON patients without disc swelling the diameter of the optic nerve was 4.4 +/- 0.4 mm in the affected and 3.0 +/- 0.3 mm in the unaffected side. This difference was significant (Wilcoxon-test, p = 0.003). In the AION patients the diameter of the optic nerve was 3.0 +/- 0.3 mm on the affected and 2.8 +/- 0.4 mm on the unaffected side. This difference was not significant (Wilcoxon-test, p = 0.093). Comparing the optic nerves with ON and AION to those of the controls, the diameter was significantly enlarged in the nerves with ON and normal in the nerves with AION (one factor repeated ANOVA). CONCLUSION: The diameter of the optic nerve is increased in ON without disc swelling and even more so in ON with disc swelling. The enlargement is probably due to edema of the nerve itself, not the surrounding subarachnoidal space. In AION, the diameter of the optic nerve is normal. Measuring the diameter of the optic nerve by B-scan ultrasonography is particularly useful in the differential diagnosis between ON with disc swelling and AION.

Adolescent↗
PubMed2002

Use of magnetic resonance imaging to differentiate optic neuritis and nonarteritic anterior ischemic optic neuropathy.

OBJECTIVE: To determine if magnetic resonance imaging (MRI) of the optic nerves obtained during the acute phase can distinguish patients with optic neuritis (ON) from those with nonarteritic anterior ischemic optic neuropathy (NAION). DESIGN: Retrospective, neuroradiologic, observational study. PARTICIPANTS: Sixty-four patients diagnosed as having either ON or NAION who were diagnosed by clinical criteria and imaged by MRI. METHODS: Demographic information on the MRI scans was masked and the patients were presented randomly and in a blinded fashion to a neuroradiologist (JDR) for determination of abnormalities. Reproducibility was assessed by presenting 10 of the scans a second time to the same neuroradiologist. MAIN OUTCOME MEASURES: The presence or absence and location of abnormal MRI signals of the optic nerve. RESULTS: Evaluation of reproducibility revealed identical interpretations of the ten scans submitted a second time. The optic nerve was abnormal in the clinically affected eye in 31 of the 32 ON patients but in only 5 of the 32 NAION patients. Thirty of the 31 ON patients who received gadolinium had enhancement, and 27 of the 32 ON patients had increased short T(1) inversion recovery signal in the clinically affected optic nerve. The five NAION patients with abnormal scans in the clinically affected eye had increased short T(1) inversion recovery signal, and in two of these, there also was enhancement of the optic nerve. For the ON patients, enhancement involved the entire length of the intraorbital optic nerve in 18 cases and the intracranial segment of the optic nerve in 19 cases. CONCLUSIONS: Our study shows that MRI scanning of the optic nerve shows significantly different results between patients clinically diagnosed with either ON or NAION.

Acute Disease↗
PubMed1997

MRI of optic nerve enlargement in optic neuritis.

We report two cases of optic neuritis with optic nerve enlargement on MRI. Both had a clinical course typical for optic neuritis but also had a neuroradiologic finding most commonly seen with optic nerve glioma or meningioma, which initially led to an incorrect diagnosis. We review the six previous reported cases of optic nerve enlargement in optic neuritis. Including our cases, six of eight reported cases were in children with severe initial visual loss. Optic nerve enlargement is a rare finding in optic neuritis that might be a subtype of optic neuritis.

Child↗
PubMed2006

Imaging of the optic disc and retinal nerve fiber layer in acute optic neuritis.

PURPOSE: To demonstrate whether optical coherence tomography (OCT-3) and scanning laser ophthalmoscopy (HRT-2) can be used to measure changes of the optic disc and peripapillary retinal nerve fiber layer (RNFL) in eyes with acute retrobulbar optic neuritis that have no clinically apparent optic disc swelling. To correlate these findings with presentation magnetic resonance imaging (MRI) of the affected optic nerve. METHODS: Eight consecutive patients with acute retrobulbar optic neuritis, who had no prior optic neuritis in either eye, were prospectively investigated at presentation and at between 1 and 3 months with clinical examination, OCT-3, HRT-2. At presentation, MRI of the optic nerves were performed in 7/8 patients. RESULTS: Compared to unaffected eyes, affected eyes without clinically seen optic disc swelling at baseline, there was a non-significant trend to increased thickness in the total RNFL, superior and nasal measurements. Baseline HRT in affected eyes showed smaller mean cup to disc ratio (p=0.003) and a smaller cup area (p=0.002) compared with the unaffected eye. The MRI-demonstrated optic nerve lesion did not correlate with OCT RNFL thickening or HRT decrease of the physiological cup. Follow-up imaging of the affected eyes showed normalization of HRT cup size parameters and OCT RNFL thickness (p<0.04). At follow-up, the temporal RNFL had thinning in 7/8 affected eyes (46.8 mum, p=0.021) compared with fellow unaffected eyes (57.8 mum), which did not change. CONCLUSION: OCT-3 and HRT demonstrate mild RNFL thickening or optic disc swelling in acute optic neuritis, even when swelling is not seen clinically. OCT-3 appears to reveal measurable RNFL thinning in the temporal quadrant after retrobulbar optic neuritis, even though vision improves. RNFL imaging may be useful in future studies of residual injury after optic neuritis.

Acute Disease↗
PubMed1980

A simple psychophysical technique provides early diagnosis in optic neuritis.

Patients with optic neuritis describe image fading which is particularly evident at bright light levels. The effect is dependent on adaptation level, and high adaptation levels serve as a provocative test. Kinetic and static perimetry and interferometric acuity tests repeated in time reveal this loss in sensitivity. The latter test, using a large field and a high luminance level, is particularly sensitive to both the residua of pathology and very early phases of disease, often before subjective manifestations of anomalies are present. These effects are large in patients with demyelinating diseases. Here, we show that such changes may be revealed in optic neuritis due to other causes as well. Note, the same form of response may be detected in more central lesions, but usually in more circumscribed areas of field.

Adult↗
PubMed1995

Optic neuritis and demyelination.

The Optic Neuritis Treatment Trial continues to generate information and controversy on the visual and neurologic outcome and treatment of optic neuritis. At the same time, other researchers explored cerebrospinal fluid parameters in multiple sclerosis, treatment of experimental optic neuritis, corticosteroid treatment of multiple sclerosis, and variations and mimickers of optic neuritis.

Demyelinating Diseases↗
PubMed1992

[Short-term effect of megadose steroid therapy in optic neuritis].

15 patients with unilateral optic neuritis and 2 patients with bilateral optic neuritis were treated with 1000 mg methylprednisolone i.v. per day for 5 days. In the cases of unilateral optic neuritis, visual acuity was reduced to < or = 0.1, in those with bilateral optic neuritis to < or = 0.6 in the better eye. The treatment was started one to 70 days after the onset of the neuritis. We examined whether vision recovered rapidly during the treatment. As a rapid recovery we defined a fourfold improvement on a logarithmic scale during the 5 days of methylprednisolone medication. Such a rapid recovery was found in 11 of the 15 patients with unilateral and in 1 of the 2 patients with bilateral optic neuritis. A similar recovery was not found before and after the treatment interval. Although we did not have a control group, the correlation in time between the therapy and the rapid recovery suggests that the megadose steroids were effective in our patients. This interpretation is compatible with the results of the randomized controlled multicenter trial of Beck et al. (New Engl. J. Med. 326:81, 1992): However, the beneficial effect was seen up to 6 months only; one year after treatment, visual functions did no longer differ between the megadose and the placebo groups. Low-dose oral steroids did not improve visual function at any time and carried a higher risk for new episodes of neuritis, compared to placebo. Therefore, the "traditional" low-dose steroid therapy for optic neuritis has become obsolete.

Adolescent↗
PubMed1992

Acute optic neuritis associated with immunization with the CNS myelin proteolipid protein.

Optic nerve tissue for SJL/J mice immunized with the central nervous system (CNS) myelin-specific proteolipid protein (PLP) was examined for histopathologic evidence of optic neuritis. Optic nerves isolated 17 d after immunization with PLP revealed an interstitial and submeningeal inflammatory infiltrate consisting of neutrophils and monocytes. In all cases, histologic evidence of optic nerve involvement correlated serologically with the presence of circulating anti-PLP antibodies. Control animals had no histopathologic evidence of disease or anti-PLP antibody. In many respects, the observed histopathologic profile of PLP-induced optic neuritis is similar to that associated with human inflammatory demyelinating diseases such as multiple sclerosis (MS). Because optic neuritis frequently is associated with some of the earliest clinical symptoms of MS, the acute nature of optic nerve involvement in this animal model suggests that immune recognition of the myelin PLP may play a significant role in the pathophysiology of optic nerve damage associated with sensitization to CNS-specific antigens.

Acute Disease↗
PubMed1990

"Optic neuritis": a masquerading disease.

Optic neuritis is a clinical entity with specific characteristics. Many disease can be associated with the symptomatology. We retrospectively selected the files of 16 persons out of 60 optic neuritis patients. Selection was based on the detection of a non-common etiology. As underlying etiology for "optic neuritis" we found e.g.: histiocytosis, intracerebral aneurysm, spongioblastoma.

Diagnosis, Differential↗
PubMed1998

Effects of intravenous methylprednisolone on outcome in MRI-based prognostic subgroups in acute optic neuritis.

Treatment of acute optic neuritis with steroids has been shown to hasten visual recovery without affecting the final degree of recovery. However, MRI-clinical studies indicate that patients with long optic nerve lesions, particularly those that involve the nerve within the optic canal, may have a worse prognosis for recovery of vision. Partly because such lesions could lead to swelling and subsequent ischemic optic nerve damage, steroids could have a selective beneficial effect on this subgroup of patients. The present randomized trial was designed to test this possibility. Sixty-six patients with acute optic neuritis received IV saline or IV methylprednisolone. The clinical, psychophysical, electrophysiologic, and MRI outcomes were assessed after 6 months. Patients with short lesions presented earlier than those with long lesions (involving three or more 5-mm-thick slices of any part of the optic nerve, as well as its intracanalicular portion), and lesion length was significantly less in patients presenting within a week of onset of symptoms. Lesions also tended to lengthen during follow-up in individual patients. Treatment did not limit lesion length in either the long or short lesion subgroup and had no significant effect on final visual outcome. We conclude that steroids do not improve visual outcome or lesion length in patients with acute optic neuritis.

Acute Disease↗
PubMed2005

Controversies in optic neuritis pain diagnosis.

Optic neuritis (ON) refers to any inflammatory disorder of the optic nerve. In clinical practice ON is mainly diagnosed by ophthalmologists and less frequently by neurologists. ON diagnostic criteria are included in different classification systems both in neurologic and ophthalmologic fields. Diagnosis of ON is still very unsatisfactory. Indeed diagnostic criteria are not uniform and therefore the diagnosis is still mainly formulated according to the clinical experience only. A consensus on practice guidelines for ON diagnosis might be useful. Ocular pain is a milestone in ON diagnosis, but it is too often mistreated by both the patient and the clinician. The International Headache Society (IHS) Classification of Headache Disorders provides in its 1988 and 2004 versions the diagnostic criteria for ON. These criteria are not spread and followed by the large majority of neurologists, but they are mainly applied by the experts in headache disorders. On the other hand, ON is a disorder widely encountered by neurologists and ophthalmologists. The latest IHS version defines the criteria of the pain features more precisely, but it is still unsatisfactory. In a future revision, the pain should be further detailed. Further studies aimed at validation of the diagnostic criteria of ON are strongly needed.

Diagnosis, Differential↗
PubMed2002

Functional magnetic resonance imaging of the cortical response to photic stimulation in humans following optic neuritis recovery.

Recovery from optic neuritis has been shown to be associated with an abnormal functional MRI (fMRI) response following exposure of the eye to an epoch based (ON-OFF design) flickering photic stimulus. Visual cortex activation is reduced during photic stimulation, whilst extra-occipital areas are extensively activated with a peak blood oxygen level dependent response during the OFF phase of the stimulus paradigm. We performed a further fMRI experiment to determine whether the abnormal extra-occipital response is a phase-specific phenomenon or whether it results from a delayed haemodynamic response. A cohort of patients that recovered from optic neuritis was studied, this time using a longer photic stimulation epoch (40 s). The extra-occipital response again peaked during the baseline condition, indicating that the phenomenon is phase dependent. Our results also reinforce the important findings of extra-occipital activation following optic neuritis which may represent an adaptive reorganization of the cerebral response.

Adult↗
PubMed1996

Long echo time STIR sequence MRI of optic nerves in optic neuritis.

MRI of the optic nerves was obtained in 13 patients with acute optic neuritis and 13 with a previous optic neuritis (ON), assessed by clinical features, visual fields and visual evoked potentials. Results of the conventional short tau inversion recovery (STIR) sequence obtained with a short echo time (STE-STIR; 22 ms) were compared with those of a long echo time (LTE-STIR: 80 ms) sequence. The conventional STE-STIR sequence revealed lesions in the optic nerves in 78.5% of acute and 58.8% of previous ON. The LTE-STIR sequence showed abnormalities in 92.8% of acutely symptomatic nerves and 94.1% of nerves with previous ON. The optic nerve lesions appeared significantly longer with the LTE-STIR sequence than with the conventional STE-STIR sequences, in both acute and previous ON.

Adult↗