Left Optic Neuritis Research Articles

CD19-Directed CAR T-Cells in a Patient With Refractory MOGAD: Clinical and Immunologic Follow-Up for 1 Year.

In MOG antibody-associated disease (MOGAD), relapse prevention and the treatment approach to refractory symptoms are unknown. We report a patient with refractory MOGAD treated with CD19-directed CAR T-cells. CD19-directed CAR T-cells (ARI-0001) were produced in-house by lentiviral transduction of autologous fresh leukapheresis and infused after a conventional lymphodepleting regimen. A 18-year-old man developed 2 episodes of myelitis associated with serum MOG-IgG, which were followed by 6 episodes of left optic neuritis (ON) and sustained the presence of MOG-IgG over 6 years despite multiple immunotherapies. After the sixth episode of ON, accompanied by severe residual visual deficits, CAR T-cell treatment was provided without complications. Follow-up of cell counts showed complete depletion of CD19+ B cells at day +7; reconstituted B cells at day +141 showing a naïve B-cell phenotype, and low or absent memory B cells and plasmablasts for 1 year. MOG-IgG titers have remained undetectable since CAR T-cell infusion. The patient had an early episode of left ON at day +29, when MOG-IgG was already negative, and since then he has remained free of relapses without immunotherapy for 1 year. This clinical case shows that CD19-directed CAR T-cell therapy is well-tolerated and is a potential treatment for patients with refractory MOGAD. This provides Class IV evidence. It is a single observational study without controls.

A case report of pediatric-onset MS associated uveitis

BackgroundTo report a case of Pediatric-onset MS associated uveitis managed with local and systemic medications.Case presentationAn 11-year-old boy who was diagnosed with Pediatric-onset MS (POMS) with the first presentation of left optic neuritis in another center, was referred to our clinic with the complaint of non-improved vision in the left eye despite receiving IV 5gr methylprednisolone. After the ophthalmologic examinations, the patient was diagnosed as bilateral POMS-associated intermediate uveitis, and local treatment with corticosteroid was administered to both eyes. He was continued on systemic therapy such as Rituximab and five sessions of plasmapheresis. After four months, the patient's vision improved from FC at 50cm to 9/10 in the left eye. The intensity of intraocular inflammation decreased in both eyes. In fluorescein angiography findings, the optic disc, as well as vascular leakage, subsided bilaterally.ConclusionDespite its rarity, POMS-associated uveitis presents a considerable challenge that necessitates the collaborative efforts of neurologists and ophthalmologists to achieve the most effective treatment outcomes.

A Case of Retinal Edema in Myelin Oligodendrocyte Glycoprotein Antibody-Associated Optic Neuritis

Purpose: To report a case of retinal edema and retinal hemorrhage in myelin oligodendrocyte glycoprotein (MOG)-immunoglobulin G (IgG) positive optic neuritis.Case summary: A 49-year-old male visited the clinic due to decreased vision and eye pain in the left eye after 2 times of right eye and 1 time of left eye optic neuritis. The patient presented with eyelid swelling and retinal hemorrhage as well as optic disc swelling of the left eye. Optical coherence tomography (OCT) showed parafoveal intraretinal fluid. The patient received the intravenous high-dose steroids and the immune globulin therapy. He was then put on maintenance oral steroids and immunosuppressive agents. After the treatment, the best corrected visual acuity of left eye was improved to 0.2. Retinal hemorrhage and retinal edema of the left eye decreased, and optic nerve pale in both eyes was observed. OCT showed overall atrophy of the retinal nerve fiber layer in both eyes.Conclusions: Some patient with MOG-IgG positive optic neuritis will have eyelid swelling or retinal edema that is necessary to be differentiated from eyelid inflammatory disorder or macular disease such as diabetic retinopathy. Thus, clinician should consider MOG-IgG positive optic neuritis in patients with retinal edema when accompanied by eye pain with severe vision loss or repeated disc swelling. When diagnosed with MOG-IgG positive optic neuritis, a combined treatment of relatively long-term steroid treatment and immunosuppressants may be required.

Unveiling the Enigma: A Challenging Diagnostic Journey to Oculocutaneous Sarcoidosis

Introduction Sarcoidosis is a complex, multisystem disorder characterized by the formation of non-caseating granulomas in various organs, most commonly the lungs and lymph nodes. Ocular involvement is frequent, occurring in up to 78% of patients, while skin manifestations are seen in approximately 25–35% of patients. Here we present a case of a 13-year-old boy with generalized body pain that later evolved to oculocutaneous sarcoidosis. Case Report A 13-year-old Sri Lankan boy presented in 2021 with severe generalized body pain, bilateral cervical lymph node swelling, occasional fever, and elevated inflammatory markers. Initial investigations for infections, malignancies, and autoimmune diseases were inconclusive. Lymph node biopsy suggested toxoplasmosis, but serological tests were normal. He later developed left eye visual blurring and periorbital swelling, with MRI showing left optic neuritis and orbital soft tissue oedema. Treatment with IV Methylprednisolone and oral prednisolone resolved his symptoms but was lost to follow-up. A year later, he presented with transient self-resolving subcutaneous nodules over trunk and limbs. due to its transient nature. ANA, RF, ACE level, serum Calcium, serum protein electrophoresis and IgG4 levels were negative. He was started on steroids, Methotrexate, and Infliximab assuming the possibility of autoinflammatory syndrome or sarcoidosis. In 2024 he again developed bilateral submandibular and cervical lymphadenopathy with acute right side visual blurring. MRI showed right optic neuritis and lacrimal gland swelling. (Figure 1) Lymph node Biopsy confirmed non-caseating granulomas. (Figure 2). Based on the findings, a diagnosis of oculocutaneous sarcoidosis was made. Treatment with steroids, methotrexate and Adalimumab was initiated, resulting in a remarkable response. Conclusion Sarcoidosis should be considered as a differential diagnosis in cases of optic neuritis with lymphadenopathy and cutaneous manifestations. Diagnosis of oculocutaneous sarcoidosis is challenging and often requires a multidisciplinary approach involving dermatologists, ophthalmologists, and rheumatologists.

NCMP-21. AQP4-MOG DOUBLE-POSITIVE PARANEOPLASTIC NEUROLOGICAL SYNDROME IN A PATIENT WITH TRIPLE NEGATIVE BREAST CANCER

Abstract We report a rare case of paraneoplastic neurological syndrome (PNS) with dual seropositivity of anti-aquaporin-4 (AQP-4) and myelin oligodendrocyte glycoprotein (MOG) antibodies in a 40 year-old lady with metastatic triple negative breast cancer with involvement of mediastinal, right axillary, and supraclavicular lymph nodes. She received multiple lines of anti-neoplastic treatment including immunotherapy with pembrolizumab as well as chemotherapy with taxol, carboplatin, gemcitabine, capecitabine, cytoxan, sacituzumab and nevalbine. Paraneoplastic meningoencephalomyelitis developed 2 years after diagnosis of breast cancer and 1 year after discontinuation of immunotherapy with pembrolizumab. She first developed longitudinally extending transverse myelitis extending from T11-12 that symptomatically improved with high dose intravenous methylprednisolone. One month later she developed left optic neuritis and meningoencephalitis with new enhancing lesions in the brain and spinal leptomeninges. Cerebrospinal fluid (CSF) analysis during both episodes revealed normal glucose, protein, elevated white blood cell count. Cytology was negative for malignancy. CSF was positive for neuromyelitis optica (NMO) IgG antibody APQ-4 and autoimmune myelopathy panel was positive for MOG antibody. Patient had significant clinical and radiographic improvement after completion of plasmapheresis and intravenous immunoglobulin. There was resolution of pleocytosis on repeat CSF testing. She did not have recurrence of paraneoplastic syndrome with maintenance rituximab every six months and daily low dose prednisone. She succumbed to progressive systemic metastatic disease 4.5 years after her breast cancer diagnosis. This case demonstrates that these antibodies can occur concurrently and cause clinical features like both neuromyelitis optica spectrum disorder (NMOSD) and myelin oligodendrocyte glycoprotein antibody disease (MOGAD) in a patient with a singular type of cancer. We highlight the importance of testing for paraneoplastic etiology in cancer patients with radiographic menigoencephalomyelitis or meningitis with atypical symptoms of meningeal carcinomatosis and/or CSF profile negative for leptomeningeal carcinomatosis.

Anti-aquaporin-4 immunoglobulin G/anti-myelin oligodendrocyte glycoprotein immunoglobulin G double-positive paraneoplastic neurological syndrome in a patient with triple-negative breast cancer.

We report a rare case of paraneoplastic neurological syndrome with dual seropositivity of anti-aquaporin-4 and myelin oligodendrocyte glycoprotein antibodies in a 40 year-old woman with metastatic triple-negative breast cancer. She received multiple lines of anti-neoplastic treatment, including immunotherapy with pembrolizumab, as well as cytotoxic chemotherapy. Paraneoplastic meningoencephalomyelitis developed 2 years after diagnosis of breast cancer and 1 year after discontinuation of immunotherapy with pembrolizumab. She first developed longitudinally extending transverse myelitis followed by left optic neuritis and meningoencephalitis with new enhancing lesions in the brain and spinal leptomeninges. Cerebrospinal fluid analysis during both episodes showed normal glucose and protein, and elevated white blood cell count. Cytology was negative for malignancy. Cerebrospinal fluid was positive for neuromyelitis optica immunoglobulin G antibody anti-aquaporin-4, and autoimmune myelopathy panel was positive for myelin oligodendrocyte glycoprotein antibody. The patient had significant clinical and radiographic improvement after completion of five cycles of plasmapheresis followed by intravenous immunoglobulin. She did not have recurrence of paraneoplastic syndrome with maintenance rituximab every 6 months and daily low-dose prednisone. She succumbed to progressive systemic metastatic disease 4.5 years after her breast cancer diagnosis. This case shows that these antibodies can occur concurrently and cause clinical features, such as both neuromyelitis optica spectrum disorder and myelin oligodendrocyte glycoprotein antibody disease, in a patient with a singular type of cancer. We highlight the importance of testing for paraneoplastic etiology in cancer patients with radiographic menigoencephalomyelitis or meningitis with atypical symptoms of meningeal carcinomatosis and/or cerebrospinal fluid profile negative for leptomeningeal carcinomatosis.

MOGAD following anti-NMDAR encephalitis: A case report

Anti-N-methyl-D-aspartate receptor antibody and myelin oligodendrocyte glycoprotein antibody can coexist. Some patients have NMDAR encephalitis and MOG antibody disease successively. We report a rare case of MOGAD following anti-NMDAR encephalitis. Three years ago, a female of 44 years, our patient developed headache, mental disorder, and epilepsy. Cerebrospinal fluid was positive for NMDAR antibodies, and the patient’s symptoms improved after immunomodulatory treatment. Three months ago, the patient had a sudden loss of vision in the left eye. Orbital magnetic resonance imaging was supportive of left optic neuritis. Cerebrospinal fluid was positive for NMDAR and MOG antibodies. She was then diagnosed with MOGAD with anti-NMDAR encephalitis. In conclusion, when patients with anti-NMDAR encephalitis have demyelinating symptoms such as decreased vision, numbness or weakness of the limbs, it is necessary to consider whether they are combined with MOGAD.

A case report of neuromyelitis optica spectrum disorder induced by pembrolizumab treatment for lung adenocarcinoma: a clinical and immunohistochemical study

BackgroundWe report a case of neuromyelitis optica spectrum disorders (NMOSD), who developed after the pembrolizumab treatment, an immune checkpoint inhibitor, against lung adenocarcinoma. The present case is discussed with the lung adenocarcinoma specimen which was stained by aquaporin-4 (AQP4) and with literature review of NMOSD linked to immune checkpoint inhibitors.Case presentationA 62-year-old Japanese man presented with acute diencephalic syndrome, left optic neuritis, and myelitis 5 months after initiation of pembrolizumab treatment for lung adenocarcinoma. He was diagnosed with NMOSD based on serum anti-aquaporin-4 (AQP4) antibody positivity. Immunohistochemistry of lung biopsy samples showed AQP4 expression on CD68+ cells. This is the fifth reported case of AQP4+ NMOSD triggered by an immune checkpoint inhibitor and the first with a brain lesion. Four out of five NMOSD cases, including the present case and one case with lung metastasis, had lung cancer.ConclusionsImmune checkpoint inhibitors may trigger AQP4+ NMOSD owing to their molecular similarity to AQP4 expressed in lung and glial tissues. Prompt brain/spinal cord imaging and anti-AQP4 antibody testing may facilitate early diagnosis of immune-mediated adverse event in central nervous system associated with immune checkpoint inhibitors.

Myelin oligodendrocyte glycoprotein antibody-associated optic neuritis and myelitis in COVID-19: a case report and a review of the literature

BackgroundOur case explored the spectrum of autoimmune and infectious neurological complications of Coronavirus Disease 2019. In addition, we also reviewed and discussed clinical features, neuroimaging, CSF findings, and outcomes in patients with COVID-19-associated Myelin Oligodendrocyte Glycoprotein Antibody Disorder (MOGAD) CNS inflammatory disorder.Case presentationHere we presented a case of post-Coronavirus Disease 2019 infection Myelin Oligodendrocyte Glycoprotein Antibody Disorder in a 41-year-old male who presented with gait instability, urinary retention, and confusion. Workup done in hospital showed transverse myelitis in cervical spine region and left optic neuritis. Laboratory findings showed Myelin Oligodendrocyte Glycoprotein-IgG antibodies were positive in serum (1:100), suggestive of post-COVID Myelin Oligodendrocyte Glycoprotein Antibody Disorder.ConclusionTo our knowledge, this is the first comprehensive case report and the literature review that includes the clinical features, neuroimaging, CSF findings, and outcomes in COVID-19-associated Myelin Oligodendrocyte Glycoprotein Antibody Disorder.

326. Radiologic Findings of COVID-19 Associated Mucormycosis (CAM) from India

BackgroundThe unique feature of the second wave of the COVID -19 pandemic in India has been the alarming surge of acute invasive fungal infection among COVID -19 patients. The increased incidence of rhino-orbito-cerebral mucormycosis is a matter of concern, as this fulminant infection has high morbidity and mortality. Hence, it is imperative to understand it’s imaging features, for early diagnosis, staging and treatment.MethodsWe systematically reviewed 32 COVID-19 cases with imaging diagnosis of acute invasive fungal rhino-sinusitis or rhino-orbital-cerebral disease between March to May 2021. These patients underwent contrast MRI of the paranasal sinus, orbit and brain. Contrast enhanced CT chest and paranasal sinuses were done as needed.ResultsThe age group ranged between 30 to 71 yrs with male preponderance. The most common predisposing factors were intravenous steroid therapy and supplemental oxygen. All cases were confirmed by fungal culture and most common was Mucor. The rhino-orbito-cerebral mucormycosis was staged as belowStageNo. of cases1 (Limited to nasal cavity)22 (Involving Paranasal sinuses) 143 (Involvement of orbit)84 (Involvement of CNS)8In our study we found that the most common site in the nasal cavity was the middle turbinate /meatus and the earliest sign was non-enhancing / “black” turbinate. Premaxillary and retroantral fat necrosis was the earliest sign of soft tissue invasion. Spread via the sphenopalatine foramen and pterygopalatine fossa was more common than bony erosions. Orbital cellulitis and optic neuritis were the most common among stage 3 cases. Of patients with CNS involvement, the most common were cavernous sinus thrombosis and trigeminal neuritis. Two patients with pulmonary mucormycosis showed large necrotic cavitary lesions, giving the characteristic “bird’s nest” appearance.Figure 1. Black turbinate Contrast enhanced coronal T1 FS images of paranasal sinuses shows necrotic non-enhancing right superior and middle turbinates (*)Figure 2: Axial contrast enhanced T1 FS image showing necrotic non enhancing premaxillary (arrowhead) and retroantral fat (straight arrow) walled off by thin enhancing rim. Figure 3: Contrast enhanced axial T1 FS images of paranasal sinuses shows necrotic non-enhancing left middle meatus spreading along sphenopalatine foramen in to pterygopalatine fossa (arrow head) ConclusionThe mortality rate was 20% in our study. In our short term follow up, 30 % of recovered patients had relapse on imaging due to incomplete clearance and partial antifungal treatment. High clinical suspicion and low imaging threshold are vital for early Mucormycosis detection in COVID-19 patients. Familiarity with early imaging signs is critical to prevent associated morbidity /mortality.Figure 4: Contrast enhanced coronal T1 FS and diffusion weighted images shows necrotic non-enhancing left middle meatus with left orbital cellulitis (*) and optic neuritis (white arrow) Figure 5. Bird’s nest Axial CT chest image in lung window shows necrotic right upper lobe cavity with internal septations and debris on a background of surrounding COVID-19 changes.Disclosures All Authors: No reported disclosures

Patients as Educators What We Can Learn from the Patient- The Story of a Multiple Sclerosis Patient in Taiwan

Narrative medicine is intended to present patients' illness stories regarding their sufferings. It can be helpful in portraying complete and authentic pictures of illness, and can also enhance the resilience of patients and physicians. Purpose: Based on this theory, this study sought to chronicle the story of a multiple sclerosis patient in Taiwan who initially presented with acute left optic neuritis 29 years ago. Methods: The audio of an in-depth interview was recorded after obtaining the patient's written consent, and was then transcribed verbatim. Immersion and crystallization was used to develop the themes of the interview. Results: The themes that emerged via the immersion and crystallization were as follows: ＂how Taiwanese culture resulted in the decision to delay treatment for a total loss of left eye vision lasting five days,＂ ＂the rationales underlying the differing opinions of relatives,＂ ＂the real impacts of the disease,＂ ＂how resilience is built in patients and physicians,＂ ＂why traditional Chinese acupuncture is popular in Taiwan,＂ and ＂how trust was built in this patient's relationship with her doctor.＂ Through the patient's storytelling, we learned not only about her physical suffering but also about the psycho-social impacts of her illness. Her narrative also revealed how traditional Taiwanese culture has shaped her mental status and even helped to build her resilience. Conclusions: Moreover, developing narrative competency might assist us in our daily practice and also help clinicians and patients to be resilient when facing suffering and fighting off illnesses together.

Bullous pemphigoid as an injection site reaction of glatiramer acetate

Glatiramer acetate (GA) is one of the well-tolerated disease-modifying therapeutic options, which is commonly administered subcutaneously in patients with multiple sclerosis (MS). The current study aimed at defining a bullous pemphigoid (BP) skin reaction in a patient with MS receiving treatment with GA. A 29-year-old women with MS, receiving GA treatment within the past nine months, November 11, 2018, was admitted to our MS clinic due to itching skin eruptions at the site of injection. Her disease was started at February 12, 2017 with left optic neuritis; and because of six periventricular and eleven juxtacortical brain magnetic resonance imaging (MRI) lesions without any enhancement, lumbar puncture was done for her. Due to positive cerebrospinal fluid (CSF) oligoclonal band, MS was diagnosed for her. At that time, she refused to start disease modifying treatment. GA was started 9 months before admission for her. After dermatologic consultation, the dermatologist defined the lesions as fluid-filled and blistering at the site of injection without mucosal involvement (Figure 1). Figure 1. Large, fluid-filled blisters Timeline: Skin lesions appeared exactly at the GA injection site.

Neuro‐retinal alteration in dizygotic twins with multiple sclerosis

AbstractPurposeThe aim was to evaluate the retina and the optic nerve in two dizygotic twins suffering from multiple sclerosis (MS).MethodThe neuroretinal structures were analysed at diagnosis from only the right eyes of both two (A and B) dizygotic twins by the optical coherence tomography (OCT) device Heidelberguffered in her left eye. The protocol used was Posterior Pole (with an area of examin Spectralis®, in order to avoid bias due to a previous optic neuritis that the twin A sation 30° × 25°) that measured the thickness in microns (μm) of the whole retina, the ganglion cell layer (GCL) by segmentation in the macular area and the peripapilar retinal nerve fibre layer (RNFL). The functional situation of the disease was evaluated with the Expanded Disability Status Scale (EDSS) by neurologist.ResultsThe onset of the disease of twin A was with an episode of left optic neuritis in 2017, whereas twin B with a cerebellar syndrome in 2013. Both twins were diagnosed of MS in 2018. At that moment, the twin B showed worse systemic functional status than twin A (EDSS: 3.5 versus 0 respectively), lower thickness in the peripapilar RNFL at temporal (54 versus 96 μm) and inferior temporal (109 versus 180 μm) sectors and also in GCL specifically at the inferior (31 versus 45 μm) and temporal (30 versus 40 μm) sectors.ConclusionsThe analysis of the posterior pole retinal thickness with segmentation could be a useful tool for the management of MS. The GCL showed an early and more intense damage than RNFL at the earliest years of the disease without previous episodes of optic neuritis in this case of two dizygotic twins.

A case of paraneoplastic neuromyelitis optica associated with small cell lung carcinoma

Neuromyelitis optic spectrum disorders are demyelinating conditions that are typically idiopathic, though various case reports have demonstrated an association with malignancy.We present the case of a 64year old woman with NMOSD in the setting of small cell lung cancer. She had longitudinally extensive transverse myelitis and left eye optic neuritis; aquaporin-4 antibodies were elevated. Biopsy of mediastinal adenopathy was positive for SCLC.Malignancy should be considered in any patient with an atypical presentation of NMOSD, or who does not respond to traditional therapies.

Severe aquaporin 4-IgG-positive neuromyelitis optica with disseminated herpes zoster in a pregnant woman successfully treated with intravenous immunoglobulin.

A 26-year-old, 17-week pregnant woman developed aquaporin-4-IgG-positive severe longitudinally extensive transverse myelitis during the course of disseminated herpes zoster and became quadriparetic. She was unresponsive to high-dose intravenous methylprednisolone but became able to walk without assistance after intravenous immunoglobulin. One and a half months later, left optic neuritis developed but her vision improved with intravenous immunoglobulin. The only sequela was left T5 girdle sensation, and she delivered a healthy baby. Intravenous immunoglobulin may be a rescue therapy in aquaporin-4-IgG-positive neuromyelitis optica attacks in pregnant women, especially those with severe infections.

PO130 A case of neuromyelitis optica spectrum disease, necrotizing myositis and breast cancer

We present the case of a 46-year-old Caucasian woman, who developed seronegative necrotizing myositis (with elevated creatine kinase [CK, 54 000]) whilst on tamoxifen treatment following remission of breast carcinoma (Grade II mucinous, solid papillary and ductal breast carcinoma), which was treated six months previously with surgery and adjuvant radiotherapy. The necrotizing myositis was initially treated successfully with prednisolone. However, on prednisolone cessation, she represented with myositis, which required steroid therapy again. Within two weeks, patient developed acute loss of vision in the left eye, which deteriorated to counting fingers over days, leading to the diagnosis of severe left optic neuritis. Serum positivity for Aquaporin-4 antibodies confirmed a diagnosis of neuromyelitis optica spectrum disease (NMOSD). The patient had no other relevant past medical history or co-morbidities, namely other organ-specific or systemic autoimmune manifestations. She was treated with intravenous steroids and plasma exchange, but she did not recover her vision. She continues on immune therapy and her three pathologies remain stable. Previous case reports have noted possible associations of NMOSD with myositis and others with malignancies, including breast cancer. Our case illustrates that NMOSD may be, very rarely, associated with, or preceded by, necrotizing myositis and may also be a paraneoplastic presentation.

Longitudinally extensive myelitis in MS mimicking neuromyelitis optica.

A 23-year-old Caucasian woman developed simultaneous onset longitudinally extensive transverse myelitis (LETM; figure, A.a) and left optic neuritis. She had no significant neurologic history, but her brother had MS. At nadir, she had Medical Research Council grade 2/5 power in the lower limbs and a sensory level at T4. MRI of the brain identified lesions within the right middle cerebellar peduncle (figure, A.b) and adjacent to the posterior horn of the left lateral ventricle (figure, A.c). Oligoclonal bands were present in the CSF, and serum autoantibodies to aquaporin-4 and myelin oligodendrocyte glycoprotein were absent on repeated testing 2 months apart. Visual evoked potentials were abnormal only on the left. Seronegative neuromyelitis optica spectrum disorder (NMOSD) was diagnosed1 and treated with corticosteroids, but she relapsed 2 months later. Azathioprine was added to steroids but she relapsed again 4 months later. Repeat MRI showed fragmentation of the LETM into multiple short cord lesions (figure, B.a) and 2 new juxtaventricular lesions in the brain (figure, B.b and c), more in keeping with MS than NMOSD. She was initiated on natalizumab and has remained without new clinical events for the last 12 months. MRI was repeated 8 months into treatment with natalizumab and confirmed that there were no new lesions. Acknowledgment: The UK NMO Service is funded by the highly specialized commissioning group of NHS England.

Acute optic neuritis: a clinical paradigm for evaluation of neuroprotective and restorative strategies?

What makes the acute optic neuritis model unique in an era of trials for neuroprotective and myelin repair agents? Acute optic neuritis (AON) is a common, and often the earliest manifestation of central nervous system (CNS) inflammatory demyelinating disorders like multiple sclerosis (MS) and neuromyelitis optica (NMO). It affects at least half the patients with MS and is the presenting feature in 15–20% of patients. Vision loss in AON secondary to NMO tends to be more severe with less potential for recovery and greater axonal degeneration, as measured by optical coherence tomography (OCT) (Bennett et al., 2014). Several unique features of the afferent visual pathway (AVP) make the AON model an ideal system to study disease pathogenesis and evaluate potential neuroprotective and myelin repair strategies.

P057 - Remarkable response of seropositive neuromyelitis optica to rituximab therapy – First report from kingdom of Bahrain

Background Neuromyelitis optica (NMO) is an inflammatory demyelinating disease of the central nervous system that causes severe optic neuritis and myelitis attacks. Humoral immunity seems to have a prominent role in the pathogenesis of the disease. Recently the nature and understanding of NMO have been revolutionized by two factors (1) the identification of both a NMO IgG as a sensitive and specific diagnostic marker and (2) the identification of both specific radiological and pathologic features of the disease. Also with the limited treatment options, recently positive results reported with the use of the anti-CD20 monoclonal antibody rituximab are promising. Objectives We present 2 cases, first to be reported in Kingdom of Bahrain, of NMO with positive NMO IgG and favorable response to Rituximab. Methods and results Case # 1: 56 year old female patient. She had on 2008 left optic neuritis with residual visual loss. On September 2011 she had right optic neuritis. For both she received Methylprednisolone plus therapy for 4 days with partial response and residual visual loss. On October 2011, she developed severe myelitis with severe spastic paraplegia (grade 0–1) and urinary retention. The patient became bed bound. Her initial MRI of brain showed few scattered lesions for which the patient was diagnosed as multiple sclerosis and maintained on Betaferon injection as a disease modifying therapy. The repeated MRI showed long segment lesion of the spinal cord and her blood test was positive to NMO IgG. The patient did not respond to steroid or IVIg therapy. However, she responded remarkably well to Rituximab injection. In 3 months she is fully independent in her movements with almost normal power and continent. Case # 2: 43 year old female patient. On 2009, she developed a right optic neuritis that responded well to steroid therapy. 2 years later she developed spastic Paraparesis due to myelitis, she responded partially to steroid therapy and was put on Avonex injection as her MRI brain showed changes was diagnosed as MS in private hospital. However, one year later, she developed another sever myelitis that made her wheelchair bound with bladder involvement. Her MRI showed fairly long segment involvement of spinal cord. Her serum test showed positive NMO IgG. I gave her Rituximab injection therapy on which she showed remarkable improvement. In 3 months she is able to walk by herself and she independent in her ADL and bladder continent. Conclusion The remarkable recovery of these 2 patients from their severe disability in this case report support other reports of the effectiveness of Rituximab in the treatment of NMO and the humoral immunity nature of the illness. These are the first cases of NMO with positive IgG to be reported from Bahrain. NMO is not often seen in Bahrain despite high prevalence of MS.

P010 - Neuromyelitis optica: Case reports from Oman

Neuromyelitis optica (NMO), a multiple sclerosis mimic, is a rare disorder with a combination of optic neuritis and transverse myelitis. Only few cases were reported in Oman and were considered a diagnostic challenge. Most of them had an unusual presentation and were confused with multiple sclerosis. The neuromyelitis optica IgG test helped confirming the diagnosis. We present 4 cases of neuromyelitis optica from a tertiary referral hospital, Royal Hospital, Oman. The criterion of Wingerchuk et al. was used for the diagnosis. All patients had MRI brain and spine and neuromyelitis optica IgG test. First patient , a 29 years old female, was referred to outpatient clinic with a history of an attack of left optic neuritis. Subsequently had 3 recurrent attacks in Rt eye. After each attack she received IV methylprednisolone followed by a tapering dose of oral prednisolone. She had a partial recovery after each attack. Initial CSF oligoclonal bands and NMO IgG were −ve. MRI brain showed non-specific white matter changes. After the 4th attack of optic neuritis she was started on a monthly dose of cyclophosphamide. After the 7th dose she was changed to oral mycophenolate. After 9 months she had a new attack of optic neuritis and same treatment was continued. After 2 years free of relapses mycophenolate was stopped. After 4 months she developed acute paraplegia. MRI spine showed focal T2 hyperintensities from T3 to T5. Repeat NMO IgG was +ve. At this point a diagnosis of neuromyelitis optica was made and patient was planned for Rituximab therapy. Second patient is a 35 years old female, presented to neurology clinic with history of recurrent optic neuritis for which she was followed up by ophthalmologist. After 16 months she was admitted with acute transverse myelitis and optic neuritis. Initial NMO IgG was −ve. MRI brain showed nonspecific T2 hyperintensities. MRI spine showed diffuse T2 hyperintensties from C5 through T10. She was treated with IV methyl prednisolone, and because the response was not favorable, IV immunoglubulins was given, and maintained on prednisolone (tapering dose) and mycophenolate. On out patient follow up she continued to have mild residual weakness. After a year she developed another attack of paraplegia and optic neuritis; and was started on interferon. A repeat NMO IgG was +ve. After 2 months she developed another attack of paraplegia. And after 4 months it also recurred. She was offered treatment with Mitoxantrone but refused. Third patient is a 24 years old female, transferred to our hospital with 2 months H/O acute transverse myelitis and optic neuritis for which she was admitted to a local hospital and received IV methyl prednisolon. MRI brain showed multiple white matter T2 hyperintensities. MRI spine showed diffuse T2 hyperintensities C2 to T1 and T7–T12. Patient refused lumbar puncture. She was seen in neurology clinic after 2 months with 3 weeks H/O Lt optic neuritis and received IV methyl prednisolone. NMO IgG was +ve and was started on interferon. Seen again after 4 months. Doing well, ambulant without support. Patient lost to follow up. She subsequently deteriorated and was started on Rituximab. Fourth patient is a 46 years old female, admitted with urinary retention, generalized weakness, and altered sensorium. MRI brain : non-specific T2 white matter changes. MRI spine : extensive T2 hyperintensities from C2 to T1 and from T8 to T10 with contrast enhancement. Treated with methyl prednisolone. Had gradual progressive recovery. After 4 months had another attack of transverse myelitis. MRI spine : T2 hyperintensities from lower medulla to T10. Received IV methyl prednisolone. CSF oligoclonal bands was +ve. NMO IgG was +ve. ANCA-PR3 A6 was +ve. Cyclophosphamaide treatment was attempted but was not tolerated by patient and was stopped. Neuromyelitis optica should be suspected in any patient presenting with optic neuritis, transverse myelitis or both with an MRI brain not suggestive of multiple sclerosis. Neither a positive oligo clonal band in CSF, nor an initial negative NMO IgG test can exclude the diagnosis of NMO, as the former may be present in 35% of patients with NOM and the last can turn positive later on in the disease coarse. The diagnosis of NMO warrants early initiation of a disease specific therapy to prevent relapses.