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Primary Sjögren's syndrome initially manifested by optic neuritis: MRI findings.

We herein describe the MRI findings in a patient clinically diagnosed with primary Sjögren's syndrome (SjS) initially manifested by retrobulbar optic neuritis. A 63-year-old woman suddenly had left ocular pain and progressive visual disturbance. MR T2-weighted images revealed hyperintensity in the left optic nerve, with swelling. Contrast-enhanced T1-weighted images showed no abnormal enhancement. Follow-up MRI 6 months after admission revealed no significant changes in the affected optic nerve. To our knowledge, optic neuritis as a complication of SjS has been reported in ten patients [1, 2, 3, 4, 5, 6] and MRI findings in only one of them [6]. We thought MR images were useful for visualizing optic nerve involvement in SjS and observing its course.

Female↗

Chronic inflammatory demyelinating polyradiculoneuropathy with histologically proven optic neuritis.

Although patient series of clinical, electrophysiological, or magnetic resonance imaging evidence for involvement of the central nervous system in patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) have been published, the histological proof has never been reported. We present the case of a 46-year-old male patient who developed CIDP in his early 20s and who died of relapsing severe pneumonia. In late stages of the disease the patient presented visual loss and bilateral atrophy of the optic nerve. Neuropathological examination revealed severe peripheral neuropathy consistent with CIDP and central involvement with bilateral optic neuritis. This is the first case reporting CIDP and histologically proven optic neuritis.

Axons↗

Visual recovery following acute optic neuritis--a clinical, electrophysiological and magnetic resonance imaging study.

This study reports the prospective follow-up of a cohort of patients with acute optic neuritis examined with serial visual tests, visual evoked potentials (VEPs), conventional and triple-dose gadolinium (Gd)-enhanced magnetic resonance imaging (MRI) to examine which factors are important in visual recovery. Thirty-three patients were recruited with acute unilateral optic neuritis. A clinical and VEP assessment was performed on each. Optic nerve MRI was performed using fast spin echo (FSE) (on all) and triple-dose Gd-enhanced T1-weighted sequences (n = 28). Optic nerve lesion lengths were measured. Serial assessments were performed on 22 of the patients up to one-year. Serial Gd-enhanced optic nerve imaging was performed on 15 of the patients until enhancement ceased. The final 30-2 Humphrey visual field mean deviation (MD) was 2.55 dB higher in patients in the lowest quartile of initial Gd-enhanced lesion length compared with the other quartiles (p < 0.01) but recovery was not related to the duration of enhancement. The initial recovery of Humphrey MD was 4.60 dB units per day in patients with good eventual recoveries (MD > -6.0 dB) and 0.99 dB per day in poor-recovery patients (p = 0.02).Good-recovery patients had mean central field VEP amplitudes 2.29 microV higher during recovery than poor-recovery patients (p = 0.047). The results suggest that factors which are associated with a better prognosis are: having a short acute lesion on triple-dose gadolinium enhanced imaging, higher VEP amplitudes during recovery and a steep gradient of the initial improvement in vision.

Adult↗

Long-term recovery and fellow eye deterioration after optic neuritis, determined by serial visual evoked potentials.

Twelve optic neuritis patients (part of a larger group in whom the effects of intravenous methylprednisolone treatment were previously reported), were followed-up 3 years from the onset of symptoms with visual evoked potentials (VEPs), contrast sensitivity and visual field examination. Findings from the previously "unaffected" eyes, none of which had had symptomatic optic neuritis, were also assessed. Between 6 months and 3 years after the onset of symptoms the VEPs of the affected eyes showed a significant shortening of mean latency (whole field, 131-123 ms; central field, 136-125 ms). Conversely, the responses of the previously unaffected eyes showed a contemporaneous latency prolongation (significant for the whole field, 110-113 ms) which exceeded the expected effect of aging. Contrast sensitivity tests showed no significant change in the affected eyes but a mild deterioration in the unaffected eyes, while the visual fields showed no overall pattern of improvement or deterioration. If the strong tendency for VEP latencies to shorten is due to ongoing remyelination, the lack of significant improvement in visual function may be because the visual deficit at 6 months is due to irreversible axonal loss rather than demyelination. The absence of functional deterioration in the affected eye, while VEPs and contrast sensitivity deteriorated in the unaffected eye, suggests that long-term remyelination may for a while counteract the effects of insidious demyelination and axonal degeneration which affect the visual pathway during clinical remission.

Adult↗

Isolated bilateral anterior optic neuritis following chickenpox in an immunocompetent adult.

Chickenpox may lead to several different neurological complications, but optic neuritis has rarely been described; in particular, only one case of isolated bilateral anterior optic neuritis (AON) in an immune-competent adult has so far been reported. We describe a second case of this type and consider similarities and differences between our patient and all other cases of AON following chickenpox. Then, we discuss the therapeutic role of steroids and advance the hypothesis of different pathogenetic pathways in immune-competent and immune-compromised subjects.

Adult↗

Optic neuritis complicating measles, mumps, and rubella vaccination.

A 6-year-old boy developed bilateral optic neuritis with decreasing visual acuity 18 days after administration of live attenuated trivalent measles, mumps, and rubella vaccine. The patient was treated with oral corticosteroids. The optic neuritis resolved within several weeks and normal vision returned. An afferent pupillary defect persistent in the more severely involved eye for 14 months following vaccination.

Adult↗

Suppression of visual perception by transcranial magnetic stimulation--experimental findings in healthy subjects and patients with optic neuritis.

The influence of noninvasive magnetic brain stimulation by a magnetic coil (MC) placed over the occiput on perception and correct reporting of a briefly presented set of 3 letters of the alphabet was examined in 15 patients with prolonged VEP latencies due to neuritis of the optic nerve. The results derived from observing these patients were compared to the results obtained from an age-matched control group of 20 healthy voluntary subjects examined under the same experimental conditions. In both groups it was possible to demonstrate that transcranial magnetic stimulation is able to suppress recognition of the letters if applied with a certain delay time after a brief presentation of the visual stimulus. The groups were compared to each other with regard to the delay with which it was possible to demonstrate the most effective suppression. In the healthy subjects, this delay was found between 60 and 100 msec. In the patients, it was prolonged to 80-140 msec. This prolongation was closely related to the VEP latency (P100). Furthermore, visual suppression and the influence on it by different parameters were studied in detail in healthy subjects; the visual suppression depends on visual (e.g., brightness, duration) and magnetic (e.g., intensity) stimulus conditions. The method described seems to be of considerable value in the investigation of basic mechanisms of visual perception. This includes pathophysiological changes caused by optic neuritis and possibly other disorders affecting the visual system.

Adult↗

Subarachnoid block and enlargement of the spinal canal in hypertrophic neuritis.

A case of Dejerine-Sottas hypertrophic neuritis is reported. The patient, a 45-year-old male, suffered from chronic hypertrophic polyneuropathy, abnormal pupils, fasciculations, tremor, back pain, impotence, sphincter disorders, cramps, and lightning pains in the lower extremities. Besides extensive subarachnoid block, there was X-ray evidence of enlargement of the bony spinal canal with scalloping of the lumbar vertebrae. Surgical exploration showed these abnormalities to be due to extreme hypertrophy of the cauda equina. Histologic findings in peripheral nerve and lumbar root biopsies were typical of hypertrophic neuropathy of the onion bulb type. Vertebral changes secondary to hypertrophied nerve roots appear not to have been described before in hypertrophic neuritis; however, knowledge of their possible occurrence may be of practical importance in the management of similar future cases. A simple way of visualizing enlarged peripheral nerves is briefly described.

Cauda Equina↗

Retrobulbar optic neuritis in a patient with acute type B hepatitis.

A case is described of retrobulbar optic neuritis in a 33-year-old female with acute type B hepatitis. The ocular distress, principally affecting the left eye, followed the normalisation of liver function tests and subsided after steroid therapy. At the onset of ocular symptomatology, complement activation, involving both classic and alternative pathways, and high levels of circulating immune complexes were present. HBsAg was positive. No relapse was observed during follow-up for 3 years. An association between HBV-infection, optic neuritis and an immune complexes-mediated neurotoxic activity are hypothetized.

Adult↗

Visual evoked responses and ophthalmological examination in optic neuritis. A follow-up study.

Ophthalmological examination and visual evoked responses (VERs) were repeated at 6-120 (mean 46) months after the first attack of acute optic neuritis in 80 patients who had abnormal VERs in 98 symptomatic eyes at the initial examination. The wide field VER returned to within the normal range in 19/98 (19%) symptomatic eyes. Fifteen percent of patients had completely normal VERs at follow-up. The yield of VER abnormalities was increased by the use of central field in addition to wide field stimulation. One or more components of the ophthalmological examination were abnormal in 91% of symptomatic eyes at final review. Although the ophthalmological examination was a more sensitive index than the VER of past optic neuritis in symptomatic eyes, the reverse was the case in asymptomatic eyes.

Acute Disease↗

Experimental neuritis induced by a mixture of neural antigens and influenza vaccines. A possible model for Guillain-Barré syndrome.

We describe the development in rats of a possible model for Guillain-Barré syndrome (GBS): experimental neuritis (EN). The clinical symptoms, histopathology and the presence of antibody to nervous tissue are features that EN has in common with both GBS and experimental allergic neuritis (EAN), another GBS model. However, EN may be a more appropriate model than EAN for studying the role of autoimmune reactions in diseases such as GBS, which are triggered by various viruses or antigens, since EN depends on such agents being administered concomitantly with the syngeneic tissue.

Animals↗

The pattern electroretinogram: N95 amplitudes in normal subjects and optic neuritis patients.

Recent work has suggested that the N95 peak of the transient pattern electroretinogram (PERG) may be a more sensitive indicator of the late stages of retinal function prior to optic nerve activation than the P50 peak. In this report, we show that a new measure of N95 amplitude, based on digital filtering methods to identify a non-linear baseline before measurement, greatly reduced the amplitude variation in a population of 50 normal subjects when compared with two other plausible measures. We then used that new measure to follow the time course of N95 amplitudes in 12 optic neuritis patients. It was found that maintenance of a normal N95 amplitude at 6 months after onset of optic neuritis was always associated with excellent clinical recovery as measured by visual fields, acuity, presence or absence of an afferent pupil and optic atrophy, and contrast sensitivity (CS). Loss of N95 amplitude to below laboratory limits of normal was associated with abnormalities in these indicators of visual function. This study supports the idea that the N95 peak represents retinal ganglion cell function.

Adolescent↗

The dynamics of the vestibulo-ocular reflex in patients with vestibular neuritis.

The dynamics of the vestibulo-ocular reflex (VOR) were studied in 14 patients at the onset of vestibular neuritis, and at follow-up 1 year later. A velocity step stimulus of 150 degrees/s was used to investigate the VOR time constant and gain, and the results were related to the caloric response. In the acute, vertiginous phase of the disease, the VOR time constant was reduced but was almost normalized 1 year later, both among patients who regained normal caloric side-difference and among those who did not. However, the increase in VOR time constant was greater among those who regained normal caloric excitability, and regression analysis showed a correlation between the prolongation of the VOR time constant and the recovery of caloric excitability. These findings suggest that VOR dynamics are modulated during the acute phase of vestibular neuritis, and that there is recovery with vestibular compensation. Furthermore, the recovery of the VOR time constant is not solely dependent on the recovery of normal caloric excitability. This implies that central storage of velocity information may be involved in the VOR, even in cases of asymmetric vestibular input after vestibular compensation.

Adolescent↗

The 'flight of colours' test in multiple sclerosis, retrobulbar neuritis and healthy controls.

The results of the test for afterimages ('flight of colours'; FOC) in healthy controls, MS patients and persons with a history of retrobulbar neuritis are presented. The FOC proved to be a reliable test of involvement of the visual system in these diseases. This concerns the duration of the afterimages as well as the palette of colours. The results were in accordance with those of the VEP, provided patients with a retrobulbar neuritis more than three years ago were omitted. In these persons the FOC proved to be more sensitive than the flash-VEP in detecting old lesions. It was concluded that these methods measure different aspects of the visual system.

Adult↗

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↗

Optic neuritis due to acquired toxoplasmosis.

A pediatric patient with well documented acquired toxoplasmic optic neuritis is presented. Use of immunoglobulin M immunofluorescent antibody studies allow easier identification of recently acquired toxoplasmosis. Toxoplasmic optic neuritis and retinitis should be considered along with toxoplasmic meningitis or encephalitis as complications of acquired toxoplasmosis. Therapy is available which may decrease the duration of visual symptomatology and reduce residual visual impairment.

Adolescent↗

Optic neuritis with silent cerebral lesions: availability of FLAIR sequences.

A 10-year-old male suffering from acute optic neuritis is reported with multiple silent cerebral lesions, which remained visible only with fluid-attenuated inversion recovery sequences at 3-week intervals. Conventional sequences with magnetic resonance imaging did not visualize the lesions. Fluid-attenuated inversion recovery sequences are highly sensitive for the detection of silent lesions in acute optic neuritis.

Brain↗

Simvastatin treatment does not protect retinal ganglion cells from degeneration in a rat model of autoimmune optic neuritis.

In patients with multiple sclerosis (MS), non-remitting deficits are mainly caused by axonal and neuronal damage. We demonstrated previously that myelin oligodendrocyte glycoprotein (MOG)-induced experimental autoimmune encephalomyelitis in rats provokes severe axonal and neuronal injury even before clinical manifestation of the disease. In our present study, we investigated effects of simvastatin treatment on degeneration of retinal ganglion cell (RGC) bodies as well as their axons during MOG-induced optic neuritis. Electrophysiological functions of optic nerves and RGCs were analyzed in vivo. Although neuroprotective effects of simvastatin have been demonstrated before in other experimental settings, we did not observe an increase in RGC survival nor an improvement of visual functions. As we could not reproduce the anti-inflammatory effects that were observed under statin therapy in other EAE models, we hypothesize that patients suffering from optic neuritis might not take advantage of simvastatin applications.

Animals↗