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Optic neuritis and myelopathy in systemic lupus erythematosus.

The optic neuritis of systemic lupus erythematosus (S.L.E.) more frequently results in the persistence of a central scotoma or complete blindness after a single attack than demyelinating optic neuritis, although the initial clinical presentations may be identical. A significant number of patients, however, recover normal vision. Optic neuritis may be the presenting symptom of S.L.E. and as myelopathy may also occur in the course of the disease, confusion with multiple sclerosis may result, especially if there are no arthritic, cutaneous nor visceral manifestations. We report a case of lupus optic neuritis associated with anticardiolipin antibodies and a circulating lupus anticoagulant and suggest these may be a marker for vasculitic optic neuritis and play a role in its aetiology.

Antibodies↗

Continuing optic nerve atrophy following optic neuritis: a serial MRI study.

To investigate optic neuritis as a model for atrophy in multiple sclerosis (MS) lesions we performed serial magnetic resonance imaging (MRI) on 10 patients with a history of optic neuritis using a fat saturated short-echo fast fluid-attenuated inversion recovery (sTE fFLAIR) sequence. The first study was performed a median of 19.5 months after the onset of optic neuritis and the second 1 year later. Using a computer-assisted contouring technique, a blinded observer calculated the mean area of the intro-orbital optic nerves. The mean area of affected optic nerves decreased over 1 year by 0.9 mm2 from 11.1 to 10.2 mm2 (p = 0.01). Poor visual acuity and decreased visual-evoked potential (VEP) amplitude were associated with atrophy. These findings suggest that atrophy is a feature of focal demyelinating lesions, it may evolve over several years, and may have functional significance. Optic neuritis provides a model to study the effect of inflammatory demyelination through the ability to accurately measure visual function and to visualize and measure the optic nerves using magnetic resonance imaging.

Adult↗

Measurement of the retinal nerve fibre layer with scanning laser polarimetry in patients with previous demyelinating optic neuritis.

OBJECTIVES: Subjective visual deficits are common after demyelinating optic neuritis despite the frequent return of normal visual acuity. Visual and electrodiagnostic tests have demonstrated evidence of these persisting functional abnormalities, which are thought to be secondary to demyelination and variable axonal loss in the optic nerve. Scanning laser polarimetry (SLP) is a new image analysis technique which uses the polarising properties of the retinal nerve fibre layer (RNFL) to produce a quantitative measure of its thickness. This study was carried out to assess the prevalence, extent, and pattern of RNFL loss after demyelinating optic neuritis using SLP. METHODS: Twenty four patients with a history of previous demyelinating optic neuritis were re-examined. Examination included measurement of logmar visual acuity, Pelli-Robson contrast sensitivity, and the presence of a relative afferent pupil defect and optic atrophy. SLP was performed and a mean RNFL profile from a series of three images from each eye was constructed. This was compared with normative data from 20 age matched normal subjects. The lower 99.9% confidence limit of the normal data was calculated and used as the cut off criterion for abnormality. RESULTS: There were a total of 31 eyes with a history of demyelinating optic neuritis and SLP disclosed an abnormality in 29 (94%) of these. Twenty three eyes recovered an acuity of 0.0 or better, 21 of which had evidence of RNFL loss on polarimetry. Scanning laser polarimetry was the only abnormality found in nine of the 31 eyes (29%). The pattern and extent of RNFL loss was very variable and there was no significant difference in these indices between patients with multiple sclerosis compared with those with isolated demyelinating optic neuritis. CONCLUSION: Scanning laser polarimetry can provide a quantitative measure of RNFL loss after demyelinating optic neuritis, demonstrating its occurrence in a high percentage of patients recovering normal visual acuity.

Adult↗

Central nervous system involvement in optic neuritis.

Thirty patients with clinically uncomplicated optic neuritis were subjected to a battery of electrophysiological tests, including visual evoked response (VER), auditory brainstem evoked response (ABER) and somatosensory evoked response (SSER). Blink reflex, electronystagmography (ENG), and computed tomography examinations were also carried out on all patients. These non-invasive tests indicated that in 11 of the 30 patients (37%), the optic neuritis was a symptom of subclinical multiple sclerosis, and that another five patients showed signs of mild central nervous system (CNS) involvement which may develop into multiple sclerosis later. This was confirmed by cerebrospinal fluid (CSF) analysis, using isoelectric focusing, showing oligoclonal extra bands in 11 out of these sixteen. A new classification of optic neuritis is proposed on the basis of these findings.

Adult↗

Optic neuritis after anthrax vaccination.

OBJECTIVE: To report the occurrence of optic neuritis after anthrax vaccination in two patients. DESIGN: Observational case reports, review of literature. METHODS: Description of clinical history, examination, neuroimaging, and further studies in two patients experiencing optic neuritis in temporal association with anthrax vaccination. MAIN OUTCOME MEASURES: Visual acuity, visual fields. RESULTS: Two patients, 39 and 23 years of age, were seen with acute optic neuritis 1 month and 2 weeks, respectively, after anthrax booster vaccination and successfully treated with intravenous methylprednisolone. The first patient had a typical presentation and course of unilateral retrobulbar optic neuritis with excellent visual recovery. The second patient had a bilateral anterior optic neuritis and has required chronic immunosuppression to maintain his vision. Retinal and optic nerve autoantibodies were present in the second patient. No cross-reactive epitopes between anthrax vaccine and retina/optic nerve were identified. CONCLUSION: Optic neuritis is a potential adverse reaction of anthrax vaccination.

Acute Disease↗

Magnetic resonance imaging, evoked responses and cerebrospinal fluid findings in a follow-up study of children with optic neuritis.

Fourteen patients with previous optic neuritis (ON) in childhood were examined by magnetic resonance (MR) imaging from two months to 14 years after the onset of symptoms. Five patients had a single monosymptomatic course of ON but 7 developed multiple sclerosis (MS); 2 had another demyelinating disease. Eight patients had high signal intensity areas in the T2 weighted images compatible with MS plaques in MR scan; 2 with monosymptomatic ON, 5 with MS and one with another demyelinating disease. The plaques were periventricular or in the optic radiation. The plaques could already be seen during the first symptoms of ON. All 7 MS patients had abnormal visual evoked response, 3/4 abnormal somatosensory evoked response and 5/6 intrathecal immunoglobulin production, when examined at onset of optic neuritis or at follow-up. All patients except one, with lesions in MR, had either oligoclonal CSF antibodies or Dr2 HLA antigen. We suggest that MR is a very sensitive test showing MR abnormalities in children with ON. It is an important tool in the early assessment of MS.

Adolescent↗

Role of vegetarianism, smoking, and hydroxocobalamin in optic neuritis.

Only 20 patients with optic neuritis of unknown aetiology could be collected in Bombay over a period of two and a half years. Only six of them were smokers and only three (one a non-smoker) had bilateral centrocaecal scotomata similar to those found in tobacco amblyopia. In 11 untreated patients the serum vitamin B(12) and plasma thiocyanate levels were estimated and found to show no significant differences from those in normal control subjects. Treatment with hydroxocobalamin in the majority of cases and with cyanocobalamin, corticotrophin, or prednisolone in the rest showed equally good results; spontaneous improvement was seen in one case.No significant role could be assigned to smoking (cyanide) or to vegetarianism in the production of optic neuritis in these patients; nor was there any evidence of depletion of total (cyanide-extracted) B(12) or of an increase in the proportion of non-cyanide-extracted B(12) in the serum.

Adolescent↗

Putative role of calpain in the pathophysiology of experimental optic neuritis.

Since myelin proteins are degraded in autoimmune demyelinating diseases such as optic neuritis, proteinases are believed to participate in myelinolysis. Calpain (calcium activated neutral proteinase) degrades myelin proteins at physiological pH and is found in glial and inflammatory cells involved in demyelination. To examine the putative role of calpain in myelinolysis, the activity and expression (translational and transcriptional) of this enzyme and endogenous inhibitor, calpastatin were examined in optic nerves of Lewis rats with experimental allergic encephalomyelitis (EAE), an animal model of optic neuritis. Calpain activity was examined via Western blotting by measuring the extent of myelin protein degradation and calpain-specific fodrin proteolysis in optic nerves from controls versus rats with experimental optic neuritis. RT-PCR studies demonstrated no significant change in millicalpain, microcalpain, or calpastatin expression at the mRNA level in optic nerves from animals with experimental optic neuritis compared to controls. However, myelin associated glycoprotein (MAG) levels were decreased by 25.5% while calpain translational expression and calpain-autolyzed fodrin levels were increased by 72.1% and 462.8% respectively, in experimental optic neuritis compared to controls. Translational expression of calpastatin isoforms (80, 68 and 55 KD) was not significantly different in rats with experimental optic neuritis compared to controls. Thus, increased activity and translational expression of calpain in experimental optic neuritis suggests this proteinase may participate in the degradation of myelin and cytoskeletal proteins in demyelinating diseases such as optic neuritis.

Animals↗

Ehrlichiosis optic neuritis.

PURPOSE: To describe a case of ehrlichiosis optic neuritis. DESIGN: Single observational case report. METHODS: A 41-year-old woman with symptoms and clinical and imaging signs consistent with optic neuritis presented to a tertiary care academic center for comprehensive neuro-ophthalmic evaluation. Main outcome measures included preoptic and postoptic neuritis polyvalent ehrlichiosis titers and magnetic resonance imaging (MRI) of orbits with gadolinium. RESULTS: Ehrlichiosis titers drawn 11 days before onset of eye symptoms were negative. Titers drawn 7 days after symptoms began were positive. The optic nerve enhanced with gadolinium on MRI. CONCLUSIONS: Ehrlichiosis can cause optic neuritis and should be considered in patients with optic neuritis after a febrile, flu-like illness in an endemic area.

Adult↗

Optic neuritis in multiple sclerosis.

PURPOSE: To review the clinical features, natural history, potential pathogenic mechanisms, differential diagnosis, and management of optic neuritis in multiple sclerosis. METHODS: Relevant literature regarding optic neuritis in multiple sclerosis from 1970 to the present was reviewed. RESULTS: Optic neuritis is an acute inflammatory optic neuropathy. It is the most common type of optic neuropathy causing acute visual loss in young adults (peak age at 30-40 years), especially among women. Patients usually present with an acute reduction of visual acuity, orbital pain exacerbated by eye movements, dyschromatopsia, and an afferent papillary defect, with or without swelling of the optic nerve head. Visual field testing most often reveals central defects, but others, such as centrocecal, can also occur. Magnetic resonance image (MRI) scanning of the brain should be undertaken in all cases of acute optic neuritis for diagnostic and prognostic purposes. The brain lesions of multiple sclerosis are commonly seen as T2 ovoid high-signal white matter lesions on MRI scans of the brain located in perivenular regions perpendicular to ventricles with variable enhancement. For atypical presentations of optic neuritis, additional laboratory tests, such as cerebrospinal fluid analysis, serologic tests, and visual evoked potentials, prove to be useful in the diagnosis and subsequent management of the patient. The recommended treatment for optic neuritis is intravenous steroids, as shown in the Optic Neuritis Treatment Trial (ONTT). CONCLUSION: Optic neuritis is often the initial presentation of multiple sclerosis. Recent advances in the understanding of the immune basis for multiple sclerosis has led to earlier and more effective treatment of this disease.

Brain↗

Optic neuritis: evaluation with orbital Doppler sonography.

PURPOSE: To evaluate orbital blood flow velocities with Doppler sonography in patients with acute unilateral optic neuritis. MATERIALS AND METHODS: Orbital Doppler sonography was performed in 20 patients with acute unilateral optic neuritis. Optic neuritis was diagnosed by a neurologist on the basis of clinical presentation, presence of decreased visual acuity, and assessment of visual evoked potentials. The peak systolic and end diastolic velocities and the resistive index were measured in the ophthalmic and central retinal arteries of both orbits. The values obtained from affected and unaffected orbits were compared by using the paired t test. RESULTS: The peak systolic and end diastolic velocities in the ophthalmic artery were significantly increased in the affected orbits (for peak systolic velocity P <.001, for end diastolic velocity P <.05). Resistive indexes in the ophthalmic arteries did not differ (P >.05). The difference between the peak systolic and end diastolic velocities and resistive indexes in the central retinal arteries of affected and normal eyes was not statistically significant (P >.05). CONCLUSION: Peak systolic and end diastolic velocities in the ophthalmic artery are increased in patients with acute optic neuritis.

Acute Disease↗

Management of optic neuritis.

To improve understanding and effectiveness of therapy in optic nerve disease, various causes of so-called optic neuritis should be identified when possible. The clinical characteristics of demyelinating optic neuropathy can be contrasted with those of ischemic optic neuropathy, nutritional optic neuropathy, true optic nerve inflammation (e.g., luetic), optic nerve infiltration with tumor, and compression neuropathy caused by adjacent tumor. Radiologic studies and other means of investigating patients with optic neuritis are reviewed. Arguments in favor of, and against, treatment of presumed demyelinating optic neuritis are presented along with representative corticosteroid treatment regimens. The natural tendency toward spontaneous improvement of optic neuritis makes the effect of treatment difficult to assess.

Adrenocorticotropic Hormone↗

Steady-state PVECP is superior to transient PVECP in the diagnosis of optic neuritis.

PURPOSE: To compare the diagnostic value for optic neuritis between transient pattern visual evoked potentials (PVECP) and steady-state PVECP. SUBJECTS & METHODS: We retrospectively studied 86 eyes of 69 patients with optic neuritis who visited our clinic. PVECPs to 3 rev/sec stimulation (transient) and 12 rev/sec stimulation (steady-state), were recorded in all patients. RESULTS: All of the cases with non-recordable transient PVECP showed non recordable steady-state PVECP Conversely, the cases with non-recordable steady-state PVECP often showed measurable transient PVECP where the latency of the P100 component was delayed. In 51 cases with unilateral optic neuritis, the amplitude ratio of the P100; affected eye/non-affected eye was significantly smaller in the steady-state PVECP than in the transient PVECP CONCLUSION: To detect visual dysfunction in optic neuritis, a steady-state PVECP was more sensitive than a transient PVECP.

Adolescent↗