Multifocal malignant optic glioma of adulthood presenting as acute anterior optic neuropathy

We report a 63-year-old, previously healthy female patient with glioblastoma multiforme of the optic nerve and chiasm presenting as acute anterior optic neuropathy. She presented with a 3-week history of progressively increasing headaches, retrobulbar pain, rapidly progressive visual loss in the right eye and blurred vision in the left eye. Early clinical examination revealed right optic disc swelling and she was initially diagnosed with demyelinating optic neuritis. Her clinical course deteriorated with total visual loss in the right eye and progressive visual loss in the left eye despite treatment with intravenous (IV) methylprednisone and IV immunoglobulins. MRI revealed enhancement of the right optic nerve and optic chiasm, with multiple periventricular hyperintense foci. Six weeks later, the patient presented with left facial palsy and left hemiparesis. Follow-up MRI showed multiple enhancing lesions in addition to the previous lesions involving right lentiform and right thalamic nucleus, right cerebral peduncle, right temporal and parietal lobes. Although the optic nerve biopsy was inconclusive, the brain biopsy revealed glioblastoma multiforme. This report demonstrated that malignant glioma of adulthood may be multicentric and may mimic optic neuritis clinically, which might help explain the difficulties in diagnosis.     

Acute zonal occult outer retinopathy and multiple sclerosis

The case of a 30-year-old woman who had two episodes of photopsia along with sudden-onset monocular visual field defects, developing into bilateral tunnel vision within 4 years, is reported. She also had episodes of a right hemiparesis and right-sided hypoaesthesia, accompanied by severe fatigue. This patient fulfilled the criteria for both clinically definite multiple sclerosis and acute zonal occult outer retinopathy (AZOOR). AZOOR can have an onset with monocular visual field loss, and can be distinguished from optic neuritis. In addition, some observations suggest common neuropathological and inflammatory mechanisms between multiple sclerosis and AZOOR.     

Stem Cell Ophthalmology Treatment Study(SCOTS) for retinal and optic nerve diseases: a case report of improvement in relapsing auto-immune optic neuropathy

We present the results from a patient with relapsing optic neuropathy treated within the Stem Cell Ophthalmology Treatment Study(SCOTS). SCOTS is an Institutional Review Board approved clinical trial and has become the largest ophthalmology stem cell study registered at the National Institutes of Health to date(www.clinicaltrials.gov Identifier NCT 01920867). SCOTS utilizes autologous bone marrow-derived stem cells(BMSCs) for treatment of retinal and optic nerve diseases. Pre-treatment and post-treatment comprehensive eye exams of a 54 year old female patient were performed both at the Florida Study Center, USA and at The Eye Center of Columbus, USA. As a consequence of a relapsing optic neuritis, the patient's previously normal visual acuity decreased to between 20/350 and 20/400 in the right eye and to 20/70 in the left eye. Significant visual field loss developed bilaterally. The patient underwent a right eye vitrectomy with injection of BMSCs into the optic nerve of the right eyeand retrobulbar, subtenon and intravitreal injection of BMSCs in the left eye. At 15 months after SCOTS treatment, the patient's visual acuity had improved to 20/150 in the right eye and 20/20 in the left eye. Bilateral visual fields improved markedly. Both macular thickness and fast retinal nerve fiber layer thickness were maximally improved at 3 and 6 months after SCOTS treatment. The patient also reduced her mycophenylate dose from 1,500 mg per day to 500 mg per day and required no steroid pulse therapy during the 15-month follow up.     

Retrobulbar optic neuritis in a two-year-old boy

We report a 2-year-4-month-old boy with retrobulbar optic neuritis. He had a sudden onset of impaired vision, which progressed to total blindness within a day. The visual evoked potential (VEP) showed no activity, but the electroretinogram was normal. Computed tomography (CT) and magnetic resonance imaging (MRI) showed no abnormal findings in the visual tract. The cerebrospinal fluid (CSF) myelin basic protein (MBP) level was elevated and serum anti-myelin antibody was positive. These findings suggested that optic neuritis in our patient was induced by retrobulbar demyelination, perhaps as a result of an autoimmune process. His visual impairment recovered gradually, but not completely, following oral prednisolone therapy. We have followed him for one year since discharge and have found neither recurrence of optic neuritis nor any other neurological disorders. Optic neuritis in children is rare and, to our knowledge, this patient is one of the youngest to be reported. This case suggests that autoimmune mechanisms may induce optic neuritis even in early childhood. In addition to VEP and MRI studies, the CSF MBP and serum anti-myelin antibody can be useful in the diagnosis and follow-up the patients with optic neuritis.     

Do visual evoked potentials give relevant information to the neuro-ophthalmological examination in optic nerve lesions?

The visual evoked potentials (VEPs) and neuro-ophthalmological examinations of 134 patients were compared. The VEPs were abnormal in 95 % of the eyes with optic neuritis. Defective color vision was found in 99 %, visual field defects in 88 %, decreased vision in 66 % and an afferent pupillary defect in 55 %. 29 patients with optic neuritis were followed up with repeated tests. VEPs and color vision recovered more slowly than visual acuity and visual field.
Abnormal VEPs were observed in 68 % of 50 MS patients. An analysis of symptomatic and asymptomatic eyes showed that testing of color vision, visual field and red-free ophthalmoscopy were equally as useful diagnostic tools as VEPs. 4 (8 %) of the MS patients had abnormal VEPs despite a normal neuro-ophthalmological examination; 94 % of MS patients with symptoms and 47 % of MS patients without visual symptoms had abnormal VEPs.
VEPs were pathological in 59 % of 24 patients with traumatic or compressive optic nerve diseases or optic atrophies of unknown etiology. The neuro-ophthalmological examination was more sensitive than VEPs in the diagnosis of these disorders. A neuro-ophthalmological examination is in most cases sufficient to diagnose optic nerve lesions. VEPs are of diagnostic aid especially in mild optic nerve lesions.     

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.     

Charles Bonnet syndrome after herpes simplex encephalitis

Visual impairment associated with Charles Bonnet syndrome is rarely reported in childhood. We describe a child who presented with visual hallucinations and postinfectious bilateral retrobulbar optic neuritis. The patient had undergone acyclovir therapy for 3 weeks because of herpes encephalitis. Four days after therapy was completed, he experienced visual impairment in both eyes. He manifested a bilateral decrease in visual acuity, with normal funduscopic findings. The patient experienced visual hallucinations for about 1 week, and then experienced total loss of vision. During his hallucinations, the patient did not exhibit behavioral changes or cognitive impairment. The visual hallucinations included unfamiliar children hiding under his bed, and he spoke to someone whom he did not know. Magnetic resonance imaging indicated bilateral optic nerve hyperintensity on T(2)-weighted and contrast-enhanced images. The patient received corticosteroid therapy for his retrobulbar optic neuritis, and his vision returned to normal after 1 month. Although rare, visual impairment can be associated with complex visual hallucinations indicative of Charles Bonnet syndrome.     

Optic neuritis caused by varicella infection in an immunocompetent child

Chickenpox may lead to several neurologic complications, but optic neuritis has rarely been described. We report on a 6-year-old immunocompetent patient who presented with unilateral optic neuritis and severe visual loss because of varicella infection. A week after varicella eruption, the child experienced blurred vision. An examination revealed decreased visual acuity of his right eye, a right pupil poorly reactive to light, and almost no color vision in his right eye. A history of chickenpox, the fundus examination, and the measurement of visual-evoked potentials allowed us to make a diagnosis of optic neuritis caused by varicella infection. The patient received only symptomatic relief with antipyretics. Three months later, his visual acuity improved to 20/40 in the right eye.     

The electroretinogram in multiple sclerosis and demyelinating optic neuritis

The electroretinogram (ERG) to flashes of white light presented under photopic conditions and the pattern reversal visual evoked potentials (PR-VEPs) from both eyes were recorded from 14 patients with multiple sclerosis (MS) with monocular demyelinating optic neuritis (DON) and from 11 patients soon after presenting with monocular demyelinating optic neuritis alone. Fifteen and 10 normal subjects, matched for age and sex, were used as controls for each group of patients respectively. In the DON group of patients and controls the flicker following ERG (FF-ERG) to white flashes of light at 40 Hz was also recorded. Skin electrodes and averaging procedures were used for all the recordings. The PR-VEP elicited with stimulation of the affected eye was absent or abnormally delayed, and the amplitude of the 'b' wave of ERG of the affected eye was diminished in all patients. The 'b' wave latency, however, was similar in both affected and non-affected eyes and the controls. There was no difference in 'a' wave amplitude and latency between eyes of patients and normal subjects. The FF-ERG in 8 out of 10 patients with satisfactory recordings was diminished in the affected eye. These results provide neurophysiological evidence that retinal damage is not due to loss of myelin but is an early feature of demyelinating optic neuritis. This damage preferentially affects the retinal elements associated with the generation of the 'b' wave of the ERG, probably the glial cells of Müller.     

Asymmetric papilledema and visual loss in pseudotumour cerebri

We report the case of a 26 year old obese woman who presented with intermittent headaches and blurred vision in her left eye (OS) and on clinical examination had an enlarged visual field blind spot OS with OS disc edema. After an extensive neurologic work up including two nondiagnostic lumbar punctures, a clinical diagnosis of OS anterior ischemic optic neuropathy was made. Gradual progression of visual field loss OS prompted reassessment of the diagnosis and intracranial pressure was confirmed to be markedly elevated by usage of a subarachnoid monitoring bolt, thus establishing the diagnosis of pseudotumour cerebri. An optic nerve sheath fenestration was performed OS with subsequent reversal of the progressive visual field loss.     

Results of static perimetry in subclinical optic neuritis in multiple sclerosis

Static perimetry of the central visual field was performed using Harms apparatus and Friedmann's analyser in 20 M.S. patients with subclinical optic neuritis at least for one eye. Sub-clinical optic neuritis was defined as delayed or absent pattern reversal visual evoked potentials (VEPs) associated with normal routine ophthalmological examination (visual acuity, optic fundi, colour vision, kinetic perimetry). Results in patients were compared to data obtained in 22 control subjects, matched for age. A highly significant loss of mean retinal sensitivity was observed in the 20 central degrees of the visual field of eyes with abnormal VEPs. Central relative scotomata were disclosed in 18 of the 33 eyes with abnormal VEPs. Static perimetry was found to be abnormal, for at least one eye, in 15 in the 20 M.S. patients. Thus abnormal VEPs in M.S. are often associated to a lowered capacity to detect a stationary stimulus in the central visual field. The Friedmann's visual field analyser allows a quick and reliable evaluation of the loss of retinal sensitivity in M.S. patients.     

Optic nerve MRI enhancement in posterior ischaemic optic neuropathy due to internal carotid artery dissection

Posterior ischaemic neuropathy (PION) is characterized by infarction in the retrobulbar optic nerve. A 73-year-old man suddenly experienced blurred vision in his left eye and intermittent weakness in his right hand. He had visual defects of superior lateral quarter and inferior medial quarter areas in the left eye. Gadolinium-enhanced magnetic resonance imaging (MRI) revealed segmental enhancement in the left optic nerve. A cerebral angiogram showed a left internal carotid dissection (ICD). He did not have fever, and his laboratory and cerebrospinal fluid tests were normal. These findings were suggestive of PION associated with ICD. No reports of PION caused by ICD has been reported and his is the first case in which MRI showed optic nerve enhancement due to ICD.     

A case of Cogan's syndrome associated with multiple cranial neuropathy

A 52-year-old woman was admitted to our hospital because of disturbance of right visual acuity and double vision. At 38-year-old she became deaf bilaterally and experienced many vertigo attacks. She was diagnosed as Ménière disease. At 45-year-old vertigo attacks disappeared. At 47-year-old right peripheral facial nerve palsy developed transiently with interstitial keratitis and episcleritis of the both eyes. Oral adrenocorticosteroid therapy produced an improvement of interstitial keratitis and episcleritis. On admission, ophthalmological examination revealed bilateral interstitial keratitis and episcleritis, right retrobulbar optic neuritis and she was proven to have bilateral sensorineural deafness by otologist. Neurological examination revealed right abducens nerve paresis. Laboratory examinations revealed slightly increased erythrocyte sedimentation rate. CRP was positive. Serological tests for syphilis were negative. CSF showed mildly elevated protein level. Orbital CT scans revealed the swelling of right optic nerve. Cerebral MRI showed multiple high spotty areas in left thalamus, bilateral basal ganglia and deep white matter in T2 weighted images. After treatment with adrenocorticosteroid, right optic neuritis and abducens nerve paresis improved together with bilateral interstitial keratitis and episcleritis. Multiple cranial neuropathy may develop with Cogan's syndrome.     

Short-latency sinusoidal wavelets to bright flashed stimuli: studies with corneal lens, nasopharyngeal, retrobulbar and scalp recordings

Short-latency flash visual evoked potentials (VEPs) and electroretinograms (ERGs) were recorded in 30 healthy volunteers and 14 patients (7 with retrobulbar neuritis and 7 with retinitis pigmentosa). Simultaneous recordings were performed by corneal, scalp, nasopharyngeal and retrobulbar (5 patients) electrodes. In 18 out of 30 healthy controls a brief sequence of oscillating wavelets was recorded between 15 and 40 ms on the scalp sites behind the vertex. In retrobulbar neuritis (RBN) patients normal responses were recorded by lens, retrobulbar, nasopharyngeal and frontal scalp electrodes. On the contrary none of these patients displayed short-latency activity behind the Cz scalp position. In 5 out of the 7 patients with retinitis pigmentosa, corneal, nasopharyngeal and scalp electrodes failed to detect any reliable waveform time-locked to the flash onset. In the remaining 2, a small lens ERG was recorded, while all other electrodes recorded a sequence of low-volted wavelets initiating 30 ms after the stimulus onset. In these patients an occipital VEP reduced in amplitude and with prolonged latency was also recorded. It is concluded that in presence of a normal corneal ERG because of the presence of volume spread oscillating retinal activity, it is hard to define while part of the scalp recorded, short latency, oscillating potentials is generated in subcortical visual structures.     

Bilateral non-arteritic anterior ischemic optic neuropathy following second-trimester spontaneous abortion-related haemorrhage

Bilateral anterior ischemic optic neuropathy is a rare complication of massive haemorrhage and related hypotension and anaemia in young individuals. We report a 34-year-old woman with bilateral non-arteritic ischemic optic neuropathy (NAION) after a massive spontaneous abortion-related haemorrhage who presented with sudden painless visual loss in her left eye. Visual acuity was 20/20 in the right eye with only hand motion discernible in the left eye. There was a left relative afferent papillary defect (RAPD). Fundus examination revealed bilateral swollen, hyperaemic optic discs and nerve fiber layer haemorrhages. Brain MRI and magnetic resonance venography were normal. The diagnosis of bilateral NAION was made and intravenous pulse corticosteroid therapy (1000 mg/day) was administered for three days. On the sixth day, optic disc oedema regressed bilaterally and on the third week, the visual acuity improved to 20/80 in the left eye. The visual field showed only a small spared area in the nasal region, and persistent RAPD was present. After two months, fundus examination showed a small and crowded optic disc on the right and a pale optic disc on the left. Severe acute haemorrhage is an important risk factor for NAION in healthy young individuals. In addition to correction of hypotension and anaemia, intravenous high dose corticosteroid might be beneficial for treatment.     

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.     

Sphenoid sinus mucocele with recurrent visual disturbance.

We present a case of sphenoid sinus mucocele with recurrent visual disturbance on the same side. A 22-year-old female showed two episodes of visual disturbance in the left eye for 3 months, and acute retrobulbar optic neuritis was diagnosed. With corticosteroid, visual disturbance improved in 1 week. MRI and CT scans showed mucocele in the left sphenoid sinus, and left optic nerve swelling with high intensity was observed in T2-weighted MRI. No destruction of the optic canal was found. The contiguous inflammation in the optic nerve rather than compression was considered as pathogenesis.     

Recovery from optic neuritis is associated with a change in the distribution of cerebral response to visual stimulation: a functional magnetic resonance imaging study

OBJECTIVES: Recovery to normal or near normal visual acuity is usual after acute demyelinating optic neuritis, despite the frequent persistence of conduction abnormalities as evidenced by the visual evoked potential (VEP). This raises the possibility that cortical adaptation to a persistently abnormal input contributes to the recovery process. The objective of this study was to investigate the pattern of cerebral response to a simple visual stimulus in recovered patients in comparison to normal subjects. METHODS: Functional magnetic resonance imaging (fMRI) was used to study the brain activation pattern induced by a periodic monocular 8Hz photic stimulus in seven patients who had recovered from a single episode of acute unilateral optic neuritis, and in seven normal controls. VEPs and structural optic nerve MRI were performed on patients. RESULTS: Stimulation of either eye in controls activated only the occipital visual cortex. However, in patients, stimulation of the recovered eye also induced extensive activation in other areas including the insula-claustrum, lateral temporal and posterior parietal cortices, and thalamus; stimulation of the clinically unaffected eye activated visual cortex and right insula-claustrum only. The volume of extraoccipital activation in patients was strongly correlated with VEP latency (r = 0.71, p = 0.005). CONCLUSIONS: The extraoccipital areas that were activated in patients all have extensive visual connections, and some have been proposed as sites of multimodal sensory integration. The results indicate a functional reorganisation of the cerebral response to simple visual stimuli after optic neuritis that may represent an adaptive response to a persistently abnormal input. Whether this is a necessary part of the recovery process remains to be determined.     

Symptomatology of infrequent visual disturbances in multiple sclerosis patients

Two cases of clinically certain multiple sclerosis were observed in whom rather infrequently occurring symptoms of damage to the visual pathways were noted. In one case during right-sided optic neuritis cyanopsia developed, followed after five months by left-sided alternatively darkening and lightening of vision. In another case with bilateral retrobulbar optic neuritis attacks of fading-out vision appeared when the patient fixed an object. Two years later the patient had episodes of chloropsia.