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1.  Timing of Corticosteroid Therapy Is Critical to Prevent Retinal Ganglion Cell Loss in Experimental Optic Neuritis 
Corticosteroids currently used to treat optic neuritis can speed visual recovery but do not improve the final visual outcome that correlates with loss of retinal ganglion cells. These studies demonstrate that corticosteroids have the ability to prevent neuronal damage if treatment is initiated early in experimental optic neuritis, suggesting that immunosuppressive treatment protocols may be developed to prevent permanent vision loss after recurrent episodes of optic neuritis.
Purpose.
Acute vision loss from optic neuritis typically resolves; however, recovery is often not complete. Permanent vision loss from retinal ganglion cell (RGC) death occurs in 40% to 60% of patients. Current therapy (high-dose corticosteroids) speeds recovery but does not change final visual outcomes. Here the authors examined whether corticosteroids administered early in the disease course can prevent RGC loss in experimental optic neuritis.
Methods.
RGCs were retrogradely labeled with fluorogold in SJL/J mice. Experimental autoimmune encephalomyelitis (EAE) was induced by immunization with proteolipid protein peptide. Optic neuritis began 9 days after immunization. Mice were treated daily with dexamethasone, methylprednisolone, or PBS from days 0 to 14 or days 10 to 14 and then were killed on day 14, 18, or 22.
Results.
Corticosteroid treatment initiated before optic neuritis onset (days 0–14) suppressed EAE and reduced optic neuritis incidence through day 14. In the few eyes that developed optic neuritis, inflammation was mild, and RGC loss was attenuated. After treatment was stopped on day 14, mice rapidly developed EAE and optic neuritis by day 18, but RGC loss was still reduced. By day 22, RGC loss increased to levels similar to those of untreated optic neuritis eyes. Corticosteroid treatment after optic neuritis onset (days 10–14) slowed EAE progression and showed a trend toward suppression of optic neuritis and RGC loss on day 14 that was lost by day 18.
Conclusions.
Corticosteroids can suppress optic neuritis and prevent RGC loss if treatment is initiated before optic nerve inflammation onset. Treatment is less effective after inflammation begins. Results suggest that chronic immunomodulation may prevent recurrent optic neuritis and RGC damage.
doi:10.1167/iovs.09-4009
PMCID: PMC2868414  PMID: 19892867
2.  Idiopathic hypertrophic pachymeningitis presenting with a superficial soft tissue mass 
Idiopathic hypertrophic pachymeningitis (IHP) is a chronic progressive diffuse inflammatory fibrosis of the dura-mater, leading to its diffuse enlargement. The following describes a case of IHP presenting with a superficial soft tissue mass. A 40-year-old female came to hospital with a subcutaneous lump over the left face and frontal headache for 6 months. An excision biopsy revealed chronic inflammation. Magnetic resonance imaging (MRI) of the brain showed left mastoiditis and early dural inflammation of the left temporal region. A few months later, she developed diplopia, complex partial seizures, and retrobulbar neuritis of the left optic nerve. Repeat MRI brain demonstrated meningeal thickening on both sides of the tentorium cerebelli extending to the left tempero-parietal meninges. The meningeal biopsy revealed markedly thickened fibro-connective dural tissue with infiltration of chronic inflammatory cells. There was no evidence of bacterial, fungal, tuberculous or neoplastic infiltration. IHP was diagnosed and steroid therapy initiated. Within weeks, she showed marked clinical improvement. IHP is a diagnosis of exclusion. The absence of underlying infective, neoplastic, or systemic autoimmune disease favors IHP. The above patient had headache, neuro-ophthalmic signs, seizures, which are features of IHP. However, superficial soft tissue involvement is rare.
doi:10.4103/0976-3147.98240
PMCID: PMC3409998  PMID: 22865979
Idiopathic hypertrophic pachymeningitis; tumefactive fibro-inflammatory lesion; tolosa-hunt syndrome
3.  Orbital high resolution magnetic resonance imaging with fast spin echo in the acute stage of Leber's hereditary optic neuropathy 
Some evidence suggests that the primary locus of the lesion in Leber's hereditary optic neuropathy (LHON) may be intraocular rather than retrobulbar. To clarify this issue, the condition of the retrobulbar portion of the optic nerve was evaluated in patients with the acute stage of LHON. High resolution MRI with fast spin echo sequences of the optic nerve complex in the orbit was carried out. Five patients with acute stage LHON were compared with seven patients with acute stage optic neuritis. On T2 weighted fast spin echo MRI, signal changes did not appear in the retrobulbar optic nerve complex in acute stage LHON. By comparison, patients with optic neuritis showed pronounced high signals in the optic nerve. Subsequent orbital MRI in the atrophic stages of the same patients with LHON showed an increase in signal intensity in the optic nerve toward the orbital apex in both eyes. The present results support the hypothesis that a primary lesion in LHON may be intraocular.


PMCID: PMC2169910  PMID: 9436742
4.  Baseline Magnetic Resonance Imaging of the Optic Nerve Provides Limited Predictive Information on Short-Term Recovery after Acute Optic Neuritis 
PLoS ONE  2015;10(1):e0113961.
Background
In acute optic neuritis, magnetic resonance imaging (MRI) may help to confirm the diagnosis as well as to exclude alternative diagnoses. Yet, little is known on the value of optic nerve imaging for predicting clinical symptoms or therapeutic outcome.
Purpose
To evaluate the benefit of optic nerve MRI for predicting response to appropriate therapy and recovery of visual acuity.
Methods
Clinical data as well as visual evoked potentials (VEP) and MRI results of 104 patients, who were treated at the Department of Neurology with clinically definite optic neuritis between December 2010 and September 2012 were retrospectively reviewed including a follow up within 14 days.
Results
Both length of the Gd enhancing lesion (r = -0.38; p = 0.001) and the T2 lesion (r = -0.25; p = 0.03) of the optic nerve in acute optic neuritis showed a medium correlation with visual acuity after treatment. Although visual acuity pre-treatment was little but nonsignificantly lower if Gd enhancement of the optic nerve was detected via orbital MRI, improvement of visual acuity after adequate therapy was significantly better (0.40 vs. 0.24; p = 0.04). Intraorbitally located Gd enhancing lesions were associated with worse visual improvement compared to canalicular, intracranial and chiasmal lesions (0.35 vs. 0.54; p = 0.02).
Conclusion
Orbital MRI is a broadly available, valuable tool for predicting the improvement of visual function. While the accurate individual prediction of long-term outcomes after appropriate therapy still remains difficult, lesion length of Gd enhancement and T2 lesion contribute to its prediction and a better short-term visual outcome may be associated with detection and localization of Gd enhancement along the optic nerve.
doi:10.1371/journal.pone.0113961
PMCID: PMC4312052  PMID: 25635863
5.  Clinical profile and short-term outcomes of optic neuritis patients in India 
Indian Journal of Ophthalmology  2014;62(3):265-267.
Purpose:
To evaluate the clinical profile and short-term visual outcome of optic neuritis (ON) patients in India.
Materials and Methods:
In this prospective study carried out over a period of 3 years, 99 eyes of 83 ON patients were examined and followed up for 10.8 ± 8.2 months for type of presentation, recurrence rate, and visual outcome.
Results:
Mean age was 27.6 ± 8.8 years. Female preponderance was seen (70% of cases). Papillitis (53.5% of eyes) was more common than retrobulbar neuritis (46.5% of eyes). Bilateral presentation was seen in 19.3% cases. Baseline median logMAR visual acuity (VA) was 1.6 ± 0.8, which improved to 0.2 ± 0.6, with approximately 64% of eyes retaining VA of 20/40 or more. Two patients had previous diagnosis of multiple sclerosis (MS). MS was newly diagnosed in two patients. Recurrence was seen in 16% of eyes and was more common in cases of retrobulbar neuritis.
Conclusion:
The clinical profile of ON in Indian patients is different from that in the Western population. Unlike reported in the Western literature, papillitis is frequent in the Indian setup, with lower recurrence rates but poorer outcomes.
doi:10.4103/0301-4738.121131
PMCID: PMC4061659  PMID: 24722269
Clinical profile; Indian population; optic neuritis treatment trial; optic neuritis
6.  Relationship between Optical Coherence Tomography and Electrophysiology of the Visual Pathway in Non-Optic Neuritis Eyes of Multiple Sclerosis Patients 
PLoS ONE  2014;9(8):e102546.
Purpose
Loss of retinal ganglion cells in in non-optic neuritis eyes of Multiple Sclerosis patients (MS-NON) has recently been demonstrated. However, the pathological basis of this loss at present is not clear. Therefore, the aim of the current study was to investigate associations of clinical (high and low contrast visual acuity) and electrophysiological (electroretinogram and multifocal Visual Evoked Potentials) measures of the visual pathway with neuronal and axonal loss of RGC in order to better understand the nature of this loss.
Methods
Sixty-two patients with relapsing remitting multiple sclerosis with no previous history of optic neuritis in at least one eye were enrolled. All patients underwent a detailed ophthalmological examination in addition to low contrast visual acuity, Optical Coherence Tomography, full field electroretinogram (ERG) and multifocal visual evoked potentials (mfVEP).
Results
There was significant reduction of ganglion cell layer thickness, and total and temporal retinal nerve fibre layer (RNFL) thickness (p<0.0001, 0.002 and 0.0002 respectively). Multifocal VEP also demonstrated significant amplitude reduction and latency delay (p<0.0001 for both). Ganglion cell layer thickness, total and temporal RNFL thickness inversely correlated with mfVEP latency (r = −0.48, p<0.0001 respectively; r = −0.53, p<0.0001 and r = −0.59, p<0.0001 respectively). Ganglion cell layer thickness, total and temporal RNFL thickness also inversely correlated with the photopic b-wave latency (r = −0.35, p = 0.01; r = −0.33, p = 0.025; r = −0.36, p = 0.008 respectively). Multivariate linear regression model demonstrated that while both factors were significantly associated with RGC axonal and neuronal loss, the estimated predictive power of the posterior visual pathway damage was considerably larger compare to retinal dysfunction.
Conclusion
The results of our study demonstrated significant association of RGC axonal and neuronal loss in NON-eyes of MS patients with both retinal dysfunction and post-chiasmal damage of the visual pathway.
doi:10.1371/journal.pone.0102546
PMCID: PMC4148263  PMID: 25166273
7.  Unilateral Measles-Associated Retrobulbar Optic Neuritis without Encephalitis: A Case Report and Literature Review 
Case Reports in Neurology  2010;2(3):128-132.
Optic neuritis (ON) is a rare neurological complication of measles infection. Little is known about measles-associated retrobulbar ON. Here, we report a distinct patient with unilateral retrobulbar ON due to measles infection. A 26-year-old woman developed maculopapular rash and Koplik spots. On the following 3 days, she noticed blurred vision in the left eye. A Goldmann visual field test showed inferior nasal quadrantanopsia in the left eye. Visual acuity was 20/20 in OD and 20/100 in OS. Pupillary size was 2.0 mm in the right eye and 4.0 mm in the left eye. Light reflexes were slightly sluggish in the left eye. Ophthalmoscopy showed mild pallor of the left optic disc. Central critical flicker fusion frequency (CFF) was 40.7 Hz in the right eye and 10.1 Hz in the left eye (normal =29.0). Visual evoked potentials showed that P100 latencies were delayed on the left side (133 ms). Brain and spinal cord MRI was normal. Orbital MRI displayed abnormal enhancement in the left optic nerve. Serum and cerebrospinal fluid IgG titers of anti-measles antibodies were increased. Left measles-associated ON was diagnosed. Methylprednisolone pulse therapy followed by oral administration of prednisolone ameliorated visual acuity, visual field and CFF. The neuroophthalmic profile of our patient indicated that measles infection triggered isolated retrobulbar ON, leading to unusual visual deficits. Thus, physicians should pay more attention to variable patterns of measles-associated ON.
doi:10.1159/000322143
PMCID: PMC2988846  PMID: 21113282
Measles virus; Optic nerve MRI; Quadrantanopsia; Retrobulbar optic neuritis
8.  Early Onset Optic Neuritis Following Measles-Rubella Vaccination 
Purpose
To report two cases of optic neuritis with onset less than 24 hours following measles-rubella (MR) vaccination.
Case Report
Two teenage patients developed acute optic neuritis 6 to 7 hours after MR booster vaccination. The first patient demonstrated bilateral papillitis and severe visual loss but improved significantly with pulse intravenous steroid therapy with methylprednisolone 500 mg/day. The second patient had unilateral retrobulbar optic neuritis and demonstrated excellent visual recovery without intervention.
Conclusion
Acute optic neuritis is a rare complication of MR vaccination and may occur early after immunization.
PMCID: PMC3589229  PMID: 23479534
9.  Delay in diagnosis of optic nerve and chiasmal compression presenting with unilateral failing vision. 
British Medical Journal  1975;1(5948):22-25.
Out of 29 patients who presented with failing vision in one eye due to optic nerve or chiasmal compression, compression was initially diagnosed in only five. The errors in diagnosis and lack of ophthalmological follow-up led to delays of up to many years with serious deterioration in acuity before referral for intracranial investigation. The chief causes of error were lack of charting of the visual fields, too ready acceptance of the diagnosis of the neuritis in the absence of essential features, and the infrequent use of skull radiographs.
PMCID: PMC1671928  PMID: 1120222
10.  Hyperacute Corticosteroid Treatment of Optic Neuritis at the Onset of Pain May Prevent Visual Loss: A Case Series 
Aim. To show that high-dose corticosteroids may prevent visual loss in patients with optic neuritis (ON) treated at the prodromal, hyperacute, phase of retrobulbar pain. Method. Prospective case series: patients were recruited with a history of ON associated with pain. The patients were advised to report immediately to the investigators should the pain recur in either eye. Where possible, orbital magnetic resonance imaging (MRI) was performed to confirm a recurrence of ON and treatment with high-dose corticosteroids was commenced. Visual function and the patient's subjective account were monitored. Results. Eight patients (including cases of MS, CRION and NMO) presented in the hyperacute phase. MRI confirmed optic nerve inflammation in 5/5. Treatment was commenced immediately, and, in all cases, no visual loss ensued. Conclusion. MRI can be used to confirm acute optic neuritis prior to visual loss in the hyperacute phase. We suggest that treatment with high-dose corticosteroids may abort the attack and prevent loss of vision in patients with ON who are treated at the onset of pain. This has potential implications for the management of acute ON and also for our understanding of the pathogenesis and potential therapeutic targets in the neuroinflammatory conditions associated with ON.
doi:10.1155/2011/815068
PMCID: PMC3198616  PMID: 22096643
11.  Subretinal angiostrongyliasis-induced optic neuritis 
A 27-year-old Thai male presented with progressive visual loss and a membrane-like floater in the right eye that had persisted for 1 month. He had a history of eating raw foods, including snails. His initial visual acuity was counting fingers at 1 ft and he had a relative afferent pupillary defect. A movable larva with subretinal tracks was found in the subretinal space near a normal optic disc. Visually evoked potentials showed delayed latency, which indicated secondary retrobulbar optic neuritis. A diode laser was directly applied to the motile worm. The patient was subsequently prescribed oral prednisolone and albendazole. After treatment, his visual acuity was slightly improved at 2/60. Ocular manifestation is a very rare event resulting from parasitic infection. In only 1.1% of angiostrongyliasis cases is an Angiostrongylus cantonensis larva identified in the eye. Ocular angiostrongyliasis with optic neuritis may be secondary to mechanical injury and/or inflammatory reactions. Steroid treatment is recommended, although most patients have only slight visual improvement after treatment.
doi:10.2147/OPTH.S43565
PMCID: PMC3693582  PMID: 23818750
Angiostrongylus cantonensis; intraocular; ocular angiostrongyliasis; parasitic infection
12.  Treatmenf of optic neuritis by retrobulbar injection of triamcinolone. 
British Medical Journal  1977;1(6075):1495-1497.
In a single-blind controlled clinical trial patients with optic neuritis caused by demyelination were given a single retrobulbar injection of triamcinolone. Though the treated group showed a trend towards more rapid recovery of vision than the controls, there was no significant difference in visual acuity, colour vision, or visual fields during the first six months after treatment. We conclude that routine use of corticosteroids is not justified in unilateral optic neuritis when vision in the other eye is good. Shortening the period of visual disability in bilateral disease or unilateral disease when vision in the other eye is poor, however, may be justifiable.
PMCID: PMC1607274  PMID: 326332
13.  Primary Sjögren's syndrome related optic neuritis 
AIM
To determine the clinical features, diagnosis and treatment of the primary Sjögren syndrome (SS) related optic neuritis.
METHODS
The clinical data of 8 patients (12 eyes) with primary SS related optic neuritis were analyzed retrospectively.
RESULTS
Eight of 128 consecutive patients with optic neuritis resulted from varied causes fulfilled the diagnostic criteria for the primary SS. They presented initially with the signs and symptoms of non-specific optic neuritis, and 5 patients presenting without dryness showed a chronic inflammation of submandibular gland or parotid gland, and lymphocyte infiltration was demonstrated by labial gland biopsy in 2 patients. There were serum positive titers for anti-Sjögren syndrome A (SSA) in 7 patients and anti-Sjögren syndrome B (SSB) in 8 patients. Anti-aquaporin-4 (AQP4) antibody was negative in all the 8 patients. Both glucocorticoids and immunosuppressive agent were administered, and visual acuity elevated in 8 eyes (66.7%), 3 patients (37.5%) recurred in the follow-up.
CONCLUSION
Primary SS related optic neuritis is less common and easily misdiagnosed. The conventional therapies for optic neuritis could not control the recurrence.
doi:10.3980/j.issn.2222-3959.2013.06.26
PMCID: PMC3874534  PMID: 24392343
optic neuritis; primary Sjögren's syndrome; anti-aquaporin-4
14.  The achiasmia spectrum: congenitally reduced chiasmal decussation 
The British Journal of Ophthalmology  2005;89(10):1311-1317.
Aim: To describe the clinical spectrum of achiasmia, a congenital disorder of reduced relative decussation at the optic chiasm.
Methods: A retrospective case note and patient review of nine children (four boys). Achiasmia was defined by the combination of a characteristic asymmetry of the monocular visual evoked potential (VEP) response to flash and neuroimaging showing reduced chiasmal size.
Results: Three of the children had an associated skull base encephalocele with agenesis of the corpus callosum. In two patients achiasmia was associated with septo-optic dysplasia. Three patients had no neuroimaging abnormalities other than reduced chiasmal size and have no known pituitary dysfunction. One child had multiple physical deformities but the only brain imaging abnormality was reduced chiasmal size.
Conclusions: Some children with disorders of midline central nervous system development, including septo-optic dysplasia and skull base encephaloceles, have congenitally reduced chiasmal decussation. Reduced relative decussation may co-exist with overall chiasmal hypoplasia. Children with an apparently isolated chiasmal decussation deficit may have other subtle neurological findings, but our clinical impression is that most of these children function well.
doi:10.1136/bjo.2005.068171
PMCID: PMC1772901  PMID: 16170123
chiasm; achiasmia; non-decussating retinal fugal fibre syndrome; septo-optic dysplasia; children
15.  A Congruous Superior Quadrantanopsia Following a Junctional Scotoma Induced by Asperogillosis 
A 69-year old man presented to us with decreased vision in his right eye and a relative afferent pupillary defect. Under the presumption that he was suffering from retrobulbar optic neuritis or ischemic optic neuropathy, visual field tests were performed, revealing the presence of a junctional scotoma. Imaging studies revealed tumorous lesions extending from the sphenoid sinus at the right superior orbital fissure, with erosion of the right medial orbital wall and optic canal. Right optic nerve decompression was performed via an endoscopic sphenoidectomy, and histopathologic examination confirmed the presence of aspergillosis. The patient did not receive any postoperative antifungal treatment; however, his vision improved to 20 / 40, and his visual field developed a left congruous superior quadrantanopsia 18 months postoperatively. A junctional scotoma can be caused by aspergillosis, demonstrating the importance of examining the asymptomatic eye when a patient is experiencing a loss of vision in one eye. Furthermore, damage to the distal optic nerve adjacent to the proximal optic chiasm can induce unusual congruous superior quadrantanopsia.
doi:10.3341/kjo.2011.25.4.294
PMCID: PMC3149145  PMID: 21860581
Aspergillus; Hemianopsia; Optic nerve; Scotoma
16.  Case of acute zonal occult outer retinopathy with abnormal pattern visual evoked potentials 
Electrophysiological and morphological findings were studied in a case of acute zonal occult outer retinopathy (AZOOR) showing abnormal pattern visual evoked potentials (VEPs) at the onset and significant functional recovery in the natural course. A 21-year-old woman presented with acute onset of photopsia and a large scotoma in the right eye of 2 weeks duration. Her visual acuity was 20/20 in both eyes with no ophthalmoscopic and fluorescein angiographic abnormalities. However, a relative afferent pupillary defect and an enlarged blind spot were found in the right eye. The pattern VEPs were severely reduced when the right eye was stimulated. The amplitudes of both rod and cone full-field electroretinographics (ERGs) were reduced in the right eye. The amplitudes of the multifocal ERGs were reduced in the area of the enlarged blind spot. Irregularities in the inner segment/outer segment (IS/OS) line of the photoreceptors were observed over the nasal fovea by optical coherence tomography (OCT). The patient was followed without treatment. The enlarged blind spot disappeared in 3 months after the onset. At 5 months, reappearance of the IS/OS line was detected by OCT. At 6 months, the P100 recovered to normal values. At 1 year, the reduced full-field ERGs were almost normal size and the multifocal ERGs in the area corresponding to the enlarged blind spot were also improved. ERG findings are crucial for differentiating AZOOR from retrobulbar neuritis, especially in patients with abnormal pattern VEPs. The pattern VEPs, full-field ERGs, multifocal ERGs, and OCT images can be abnormal in the early phase of AZOOR, but they can all improve during the natural course.
doi:10.2147/OPTH.S23194
PMCID: PMC3180490  PMID: 21966193
AZOOR; pattern VEP; full-field ERG; multifocal ERG; OCT
17.  Clinically Isolated Syndromes Suggestive of Multiple Sclerosis: An Optical Coherence Tomography Study 
PLoS ONE  2012;7(3):e33907.
Background
Optical coherence tomography (OCT) is a simple, high-resolution technique to quantify the thickness of retinal nerve fiber layer (RNFL), which provides an indirect measurement of axonal damage in multiple sclerosis (MS). This study aimed to evaluate RNFL thickness in patients at presentation with clinically isolated syndromes (CIS) suggestive of MS.
Methodology
This was a cross-sectional study. Twenty-four patients with CIS suggestive of MS (8 optic neuritis [ON], 6 spinal cord syndromes, 5 brainstem symptoms and 5 with sensory and other syndromes) were prospectively studied. The main outcome evaluated was RNFL thickness at CIS onset. Secondary objectives were to study the relationship between RNFL thickness and MRI criteria for disease dissemination in space (DIS) as well as the presence of oligoclonal bands in the cerebrospinal fluid.
Principal Findings
Thirteen patients had decreased RNFL thickness in at least one quadrant. Mean RNFL thickness was 101.67±10.72 µm in retrobulbar ON eyes and 96.93±10.54 in unaffected eyes. Three of the 6 patients with myelitis had at least one abnormal quadrant in one of the two eyes. Eight CIS patients fulfilled DIS MRI criteria. The presence of at least one quadrant of an optic nerve with a RNFL thickness at a P<5% cut-off value had a sensitivity of 75% and a specificity of 56% for predicting DIS MRI.
Conclusions
The findings from this study show that axonal damage measured by OCT is present in any type of CIS; even in myelitis forms, not only in ON as seen up to now. OCT can detect axonal damage in very early stages of disease and seems to have high sensitivity and moderate specificity for predicting DIS MRI. Studies with prospective long-term follow-up would be needed to establish the prognostic value of baseline OCT findings.
doi:10.1371/journal.pone.0033907
PMCID: PMC3309007  PMID: 22448279
18.  A case report of adalimumab-associated optic neuritis 
Purpose
To describe a case of retrobulbar optic neuritis that presented within 3 weeks of adalimumab treatment initiation.
Methods
This index case was evaluated with visual field testing, brain magnetic resonance imaging, lumbar puncture, and laboratory evaluation, and treated with intravenous methylprednisolone followed by a steroid taper.
Results
Our patient made a full visual recovery, but was found to have extensive T2/FLAIR foci of hyperintensities that enhanced and had restricted diffusion on magnetic resonance imaging (MRI). Six months later, these demyelinating lesions still persisted and our patient was initiated on immunomodulatory treatment.
Conclusion
With the extensive burden of disease at presentation and persistence of lesions on follow-up MRI, this unusual case seems to suggest an unmasking of an underlying demyelinating process by adalimumab. The clinician should be mindful of this association and monitor for any manifestations and treat appropriately.
doi:10.1007/s12348-011-0058-2
PMCID: PMC3438296  PMID: 22271346
Tumor necrosis factor; Optic neuritis; Multiple sclerosis; Adalimumab
19.  The primary vascular dysregulation syndrome: implications for eye diseases 
The EPMA Journal  2013;4(1):14.
Vascular dysregulation refers to the regulation of blood flow that is not adapted to the needs of the respective tissue. We distinguish primary vascular dysregulation (PVD, formerly called vasospastic syndrome) and secondary vascular dysregulation (SVD). Subjects with PVD tend to have cold extremities, low blood pressure, reduced feeling of thirst, altered drug sensitivity, increased pain sensitivity, prolonged sleep onset time, altered gene expression in the lymphocytes, signs of oxidative stress, slightly increased endothelin-1 plasma level, low body mass index and often diffuse and fluctuating visual field defects. Coldness, emotional or mechanical stress and starving can provoke symptoms. Virtually all organs, particularly the eye, can be involved. In subjects with PVD, retinal vessels are stiffer and more irregular, and both neurovascular coupling and autoregulation capacity are reduced while retinal venous pressure is often increased. Subjects with PVD have increased risk for normal-tension glaucoma, optic nerve compartment syndrome, central serous choroidopathy, Susac syndrome, retinal artery and vein occlusions and anterior ischaemic neuropathy without atherosclerosis. Further characteristics are their weaker blood–brain and blood-retinal barriers and the higher prevalence of optic disc haemorrhages and activated astrocytes. Subjects with PVD tend to suffer more often from tinnitus, muscle cramps, migraine with aura and silent myocardial ischaemic and are at greater risk for altitude sickness. While the main cause of vascular dysregulation is vascular endotheliopathy, dysfunction of the autonomic nervous system is also involved. In contrast, SVD occurs in the context of other diseases such as multiple sclerosis, retrobulbar neuritis, rheumatoid arthritis, fibromyalgia and giant cell arteritis. Taking into consideration the high prevalence of PVD in the population and potentially linked pathologies, in the current article, the authors provide recommendations on how to effectively promote the field in order to create innovative diagnostic tools to predict the pathology and develop more efficient treatment approaches tailored to the person.
doi:10.1186/1878-5085-4-14
PMCID: PMC3693953  PMID: 23742177
Primary vascular dysregulation; Endothelial dysfunction; Vasospasm; Glaucoma; Retinal venous pressure; Risk factors; Molecular targets; Predictive diagnostics; Targeted prevention; Integrative medical approach
20.  Three Cases with Visual Hallucinations following Combined Ocular and Occipital Damage 
Case Reports in Medicine  2013;2013:450725.
Charles Bonnet syndrome is an underrecognized disease that involves visual hallucinations in visually impaired patients. We present the cases of three patients who experienced complex visual hallucinations following various pathomechanisms. In two cases, diagnosis showed coexistence of occipital lobe damage with ocular damage, while in the third case it showed occipital lobe damage with retrobulbar optic neuritis. Theories of pathogenesis and the neuroanatomical basis of complex visual hallucinations are discussed and supported by literature review.
doi:10.1155/2013/450725
PMCID: PMC3860123  PMID: 24376461
21.  Experimental mouse model of optic neuritis with inflammatory demyelination produced by passive transfer of neuromyelitis optica-immunoglobulin G 
Background
Although optic neuritis (ON) is a defining feature of neuromyelitis optica (NMO), appropriate animal models of NMO ON are lacking. Most NMO patients are seropositive for immunoglobulin G autoantibodies (NMO-IgG) against the astrocyte water channel aquaporin-4 (AQP4).
Methods
Several approaches were tested to develop a robust, passive-transfer mouse model of NMO ON, including NMO-IgG and complement delivery by: (i) retrobulbar infusion; (ii) intravitreal injection; (iii) a single intracranial injection near the optic chiasm; and (iv) 3-days continuous intracranial infusion near the optic chiasm.
Results
Little ON or retinal pathology was seen using approaches (i) to (iii). Using approach (iv), however, optic nerves showed characteristic NMO pathology, with loss of AQP4 and glial fibrillary acidic protein immunoreactivity, granulocyte and macrophage infiltration, deposition of activated complement, demyelination and axonal injury. Even more extensive pathology was created in mice lacking complement inhibitor protein CD59, or using a genetically modified NMO-IgG with enhanced complement effector function, including significant loss of retinal ganglion cells. In control studies, optic nerve pathology was absent in treated AQP4-deficient mice, or in wild-type mice receiving control (non-NMO) IgG and complement.
Conclusion
Passive transfer of NMO-IgG and complement by continuous infusion near the optic chiasm in mice is sufficient to produce ON with characteristic NMO pathology. The mouse model of NMO ON should be useful in further studies of NMO pathogenesis mechanisms and therapeutics.
doi:10.1186/1742-2094-11-16
PMCID: PMC3909205  PMID: 24468108
NMO; Neuroinflammation; Mouse models; Aquaporin; Astrocyte
22.  Infectious and Noninfectious Granulomatosis in Patient with Multiple Sclerosis: Diagnostic Dilemmas and Followup 
Case Reports in Immunology  2014;2014:876525.
Patient was followed up over the course of 30 years. In 1978, after severe systemic infection followed by fever, pulmonary edema, and numerous neurological manifestations, patient was differentially diagnosed with apoplectic form of multiple sclerosis (MS), which was confirmed a year later via neurological and MRI findings. Approximately 20 years following the initial attack, sarcoidosis was diagnosed during the regular preoperative procedures required for cataract surgery. As consequence of lower immune system, infectious granulomatosis in form of pulmonary tuberculosis developed. Ophthalmological findings revealed bilateral retrobulbar neuritis (RBN) approximately six years after initial attack. This developed into total uveitis with retinal periphlebitis and anterior granulomatous uveitis—all of which are clinically similar in both MS and sarcoidosis.
doi:10.1155/2014/876525
PMCID: PMC4207459  PMID: 25379311
23.  Bilateral neuro-retinitis following chick embryo cell anti-rabies vaccination – a case report 
BMC Ophthalmology  2005;5:20.
Background
The Optic nerve is rarely involved after sheep brain anti-rabies vaccination in the form of retrobulbar neuritis or papillitis. Bilateral neuroretinitis after chick embryo cell antirabies vaccination has not been reported.
Case presentation
We report the case of a 56 year old male who developed bilateral neuro-retinitis following three injections of antirabies vaccine prepared from the chick embryo.
Conclusion
The chick embryo cell antirabies vaccine can cause bilateral neuroretinits which has not been reported previously.
doi:10.1186/1471-2415-5-20
PMCID: PMC1208903  PMID: 16105182
24.  Toxic ocular effects of ethambutol. 
Ethambutol hydrochloride has been used in the treatment of tuberculosis for 25 years. Its only important adverse effect, retrobulbar neuritis, is thought to be a minor concern with conventional dosages. The author presents four cases of serious visual impairment due to ethambutol therapy. Three of the patients were receiving a maintenance dosage of 15 mg/kg per day. The fourth patient was inadvertently given 25 mg/kg per day for 4 months. After therapy with the drug was stopped, improvement was slow, with complete recovery in two cases and only minor residual changes in a third. The patient who received the higher dosage of ethambutol suffered permanent, marked impairment. Ethambutol appears to contribute little to modern short-course antituberculous regimens that include more potent agents such as isoniazid and rifampin. In view of this and the potential for serious visual impairment, alternative antituberculous agents should be considered.
PMCID: PMC1492367  PMID: 3607664
25.  Survival pattern and cause of death in patients with multiple sclerosis: results from an epidemiological survey in north east Scotland. 
The mean survival period in a series of 216 multiple sclerosis deaths, which formed part of a large prevalence sample observed in the Grampian region of Scotland, was 24.5 years, with an insignificant difference between females (25.7 years) and males (23.5 years). A third of the patients survived for over 30 years after onset. The age at death ranged between 25-80 years, with majority of the deaths occurring in the seventh decade (37%). On comparing life expectancy with the Scottish general population using life tables, only a slight reduction in the short-term (less than 10 years from onset) survival was noted in all age groups, with the exception of those with onset over the age of 50 years. The long-term life expectancy was however markedly reduced in all age groups compared with the controls. The survival period could be accurately predicted from the degree of disability at a point in time, and could be correlated with a number of clinical features, the most important of which was the age at onset. Eighty five per cent of those with onset of multiple sclerosis over the age of 50 years died within 20 years. Patients with a cerebellar disturbance at onset survived the shortest, and those with a brainstem lesion or retrobulbar neuritis the longest; those with a pyramidal dysfunction had an intermediate prognosis. Other parameters which could be correlated with the survival were: the timing and frequency of occurrence of psychiatric and urinary symptoms, interval between onset and first relapse and the course of the disease. As expected, most patients (89%) were significantly disabled (unable to walk) prior to death, only a minority, however, had become so within 10 years of the onset (10%). Sixty two per cent of the patients died of complications of multiple sclerosis. No unusual excess of any disease was noted amongst other causes. As expected, the majority of patients (55%) had bronchopneumonia as the terminal event, 11% had septicaemia, 15% had myocardial infarction and 4% had documented pulmonary embolism. This is the largest series of its kind where prognosis, judged by survival period, has been assessed amongst all multiple sclerosis patients derived from a prevalence sample and observed till death.
PMCID: PMC1031962  PMID: 3495637

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