[Optical coherence tomography angiography in the diagnosis of multiple sclerosis]. / Opticheskaya kogerentnaya tomografiya-angiografiya v diagnostike rasseyannogo skleroza.

PURPOSE: This study analyzes the main changes in retinal microcirculation in patients with multiple sclerosis (MS) and their relationship with the type of disease course. MATERIAL AND METHODS: 159 patients (318 eyes) were examined. The groups were formed according to the type of course and duration of MS: group 1 - 37 patients (74 eyes; 23.27%) with relapsing-remitting MS (RRMS) less than 1 year; group 2 - 47 patients (94 eyes; 29.56%) with RRMS from 1 year to 10 years; group 3 - 44 patients (86 eyes; 27.05%) with RRMS >10 years; group 4 - 32 patients (64 eyes; 20.12%) with secondary progressive MS (SPMS). Subgroups A and B were allocated within each group depending on the absence or presence of optic neuritis (ON). Patients underwent standard ophthalmological examination, including optical coherence tomography angiography (OCTA). RESULTS: A decrease in the vessel density (wiVD) and perfusion density (wiPD) in the macular and peripapillary regions was revealed, progressing with the duration of the disease and with its transition to the progressive type. The minimum values were observed in patients with SPMS (group 4), with the most pronounced in the subgroup with ON (wiVD = 16.06±3.65 mm/mm2, wiPD = 39.38±9.46%, ppwiPD = 44.06±3.09%, ppwiF = 0.41±0.05). CONCLUSION: OCTA provides the ability to detect subclinical vascular changes and can be considered a comprehensive, reliable method for early diagnosis and monitoring of MS progression.

Optic neuritis and mydriasis after vaccination: a case report.

BACKGROUND: Optic neuritis (ON) is an inflammatory demyelinating condition of the optic nerve, with various causes. Its incidence is higher in children and young adults than in older adults of both genders, but is more common in women than in men. ON is rarely associated with mydriasis, and it is seldom triggered by vaccines against tetanus and diphtheria. CASE REPORT: A 36-year-old Caucasian woman presented with bilateral ON that had started 18 days after administration of a booster dose of the double adult vaccine (dT) against diphtheria and tetanus. Bilateral mydriasis persisted after treatment and clinical resolution of the ON. She experienced severe headache, blurred vision, decreased visual acuity in the right eye and bilateral mydriasis, a diagnosis confirmed by imaging tests. Treatment with oral corticosteroids resulted in rapid resolution of the neuritis; however, mydriasis persisted for several months. CONCLUSION: This study describes a very unusual case of bilateral ON associated with prolonged mydriasis after vaccination against tetanus and diphtheria that regressed after treatment with oral corticosteroids. Prolonged mydriasis was the manifestation that differed from the other cases previously described.

[Leber's hereditary optic neuropathy].

Leber's hereditary optic atrophy (LHON) is a genetic optic neuropathy that is more prevalent in young males but can occur from childhood to old age. The primary cause is mitochondrial genetic mutations, which are associated with dysfunction of mitochondrial electron transport chain complex I. It manifests as acute to subacute visual impairment, often starting unilaterally but progressing to involve both eyes within weeks to months. Visual loss is severe, with many patients having corrected visual acuity below 0.1. The differential diagnosis of optic neuritis is essential, and assessments such as pupillary light reflex, fluorescein fundus angiography, and magnetic resonance imaging can be useful for differentiation. LHON should be considered as one of the differential diagnoses for optic neuritis, and collaboration between neurologists and ophthalmologists is crucial for accurate diagnosis and appropriate treatment.

Ocular Manifestations of Multiple Sclerosis: A Population-Based Study.

BACKGROUND: To evaluate the population-based frequency and severity of multiple sclerosis (MS)-related ocular diseases. METHODS: Retrospective, population-based study examining patients with MS between January 1, 1998 and December 31, 2011. Patients were identified using the Rochester Epidemiology Project, which is a record-linkage system of medical records for all patient-physician encounters among Olmsted County, Minnesota residents. Diagnosis of MS was confirmed based on neuroimaging, cerebrospinal fluid studies, and serum studies for each patient according to the 2017 McDonald criteria. Patient data were obtained using the medical records and followed through April 1, 2018. RESULTS: Of the 116 patients with MS, 66% were female and the median age of onset was 36 years (interquartile range 27.5-43.5 years). About half (61/116, 53%) had MS-related neuro-ophthalmic manifestations during their disease course, and about one-fourth (33/116, 28%) had visual symptoms as their presenting symptom of MS, most commonly as optic neuritis (26/116, 22%). Optic neuritis was the leading MS-related ocular condition (37%), followed by internuclear ophthalmoplegia (16%) and nystagmus (13%). Optic neuritis was mostly unilateral (40/43, 93%), with 16% (6/43) having a visual acuity of 20/200 or worse at nadir but ultimately 95% (35/37) improving to a visual acuity of 20/40 or better. CONCLUSIONS: This study provides the population-based frequency of MS-related ocular disease, which demonstrates a high frequency of ocular manifestations in MS both at disease onset and during the disease course, emphasizing the utility of neuro-ophthalmologists, or collaboration between neurologists and ophthalmologists, in the care of patients with MS.

Optic neuritis secondary to syphilis.

Optic neuritis is a rare manifestation of syphilis, and the involvement of the central nervous system should be considered synonymous with neurosyphilis. This infectious disease, well known as the great imitator, can affect any structure and produce multiple clinical symptoms. Here, we report a case of a 62- year-old male patient who presented to our service with decreased vision and myodesopsias in right eye. The posterior segment showed a hyperemic nerve with peripapillary hemorrhages and retinal pigment epithellium hyperplasia. The patient was recently diagnosed with HIV. Serology for syphilis was positive with posterior decreased levels of nontreponemal test following treatment with ceftriaxone. Optic neuritis can occur at any stage of syphilis and must always be considered a differential diagnosis.

An atypical case of bilateral optic neuritis after strabismus surgery under general anesthesia.

INTRODUCTION: Strabismus surgery under general anesthesia is a common procedure with rare complications in the form of hemorrhage, infection, slipped muscle, lost muscle, scleral perforation, and anterior segment ischemia. We report a unique case of bilateral optic neuritis following squint surgery under general anesthesia in a 15-year-old girl. METHODS: A 15-year-old girl presented with accommodative esotropia with V pattern. She underwent uneventful bilateral inferior oblique recession surgery under general anesthesia with Propofol 60 mg, Atracurium 30 mg, and Fentanyl 70 mcg. On the first post-operative day, the patient had an acute onset of temporal headache which was non-radiating. She responded to supportive treatment and was discharged. However, on the 7th postoperative day, she presented with a constant severe headache in the bitemporal region (left > right) for 3 days. She also experienced a painless diminution of vision for 2 days. There was no vomiting, fever, loose stools, diplopia, difficulty in breathing, peripheral sensation loss, generalized weakness, or bowel/bladder incontinence. RESULTS: The best corrected visual acuity was 6/9 in right eye, and 6/9p in left eye with a relative afferent pupillary defect (RAPD) in the left eye. Both optic discs appeared hyperemic with blurred margins. Magnetic resonance imaging (MRI) of the brain and orbit showed hyperintensity along the intraorbital and intracanalicular parts of bilateral optic nerves on T2 weighted image suggesting bilateral optic neuritis. She received intravenous methylprednisolone pulse therapy followed by oral steroids and responded to the medical treatment with improvement in vision but developed steroid-induced glaucoma requiring medical management over several weeks. DISCUSSION: Neuro-ophthalmic complication in the form of non-arteritic ischemic optic neuropathy has been reported after various ophthalmic surgeries, but bilateral optic neuritis has not been reported to date. This possibility should be kept in mind if any patient presents with similar symptoms. This report also highlights IOP monitoring in pediatric patients receiving systemic steroids to prevent loss of vision due to steroid-induced glaucoma.

Relationship between pattern reversal visual evoked potential P100 wave latency and dysfunctional HDL in patients with multiple sclerosis subjected to an optic neuritis attack: A case-control study.

Optic neuritis frequently occurs during the clinical course of multiple sclerosis (MS). In this condition, demyelination of the optic nerve occurs, which electrophysiologically causes a delay in P100 wave latency. Sensitive cholesterol homeostasis is critical for the formation of the myelin sheath and for myelin to become functionally mature. High-density lipoprotein (HDL) becomes dysfunctional under oxidative stress and plays an important role in the pathogenesis of MS. In this study, HDL levels of MS patients suffering from optic neuritis were compared with those of healthy individuals, and the relationship between pattern reversal visual evoked potential (PRVEP) P100 wave latency and HDL levels in patients with optic neuritis attacks was analyzed. PRVEP studies were performed in patients with MS who had an episode of optic neuritis, and P100 wave latencies were measured. Peripheral blood samples were collected from healthy participants and patients. Lipid levels and myeloperoxidase (MPO) and paraoxonase (PON) activities were measured, and the MPO/PON ratio was then calculated. The lipid profiles and dysfunctional HDL levels in the healthy and patient groups were compared. Finally, the relationship between these parameters and the PRVEP-P100 wave latency was examined. Total cholesterol and low-density lipoprotein (LDL) levels were significantly higher in the patient group (P = .044; P = .038, respectively). There was no statistically significant difference in HDL levels between groups (P = .659). The distribution of MPO values was similar between groups (P = .452). PON values were significantly lower, whereas the MPO/PON ratios were significantly higher in the patient group than in the control group (P = .025; P = .028, respectively). A statistically significant positive correlation was found between the elevated MPO/PON ratio, representing dysfunctional HDL, and both the mean and maximum PRVEP-P100 wave latencies (P < .001, R = 0.690; P < .001, R = 0.815, respectively). A dysfunctional form of HDL may lead to poor deactivation of remyelination-limiting factors and may ultimately be associated with poor outcomes in optic neuritis.

Myelin oligodendrocyte glycoprotein (MOG) associated optic neuritis in a patient with idiopathic intracranial hypertension (IIH) and compressive optic neuropathy case report.

BACKGROUND: Myelin oligodendrocyte glycoprotein-associated disease (MOGAD) has a wide phenotypic expression and should be considered in a differential diagnosis of patients with optic disc edema and increased intracranial pressure because MOGAD can mimic IIH and compressive optic neuropathy. CASE PRESENTATION: A 53-year-old woman with a history of presumed idiopathic intracranial hypertension ("IIH") presented with new headache and visual loss. She had a BMI of 35.44 kg/m2 and a past medical history significant for depression, hepatitis C, hyperlipidemia, and uterine cancer post-hysterectomy. She had undergone multiple lumboperitoneal shunts for presumed IIH and had a prior pituitary adenoma resection. Her visual acuity was no light perception OD and counting fingers OS. After neuro-ophthalmic consultation, a repeat cranial MRI showed symmetric thin peripheral optic nerve sheath enhancement of the intra-orbital optic nerves OU. Serum MOG antibody was positive at 1:100 and she was treated with intravenous steroids followed by plasma exchange and rituximab. CONCLUSIONS: This case highlights the importance of considering MOGAD in the differential diagnosis of optic neuropathy. Although likely multifactorial, we believe that the lack of improvement in our case from presumed IIH and despite adequate neurosurgical decompression of a pituitary adenoma with compression of the optic apparatus reflected underlying unrecognized MOGAD. Clinicians should consider repeat imaging of the orbit (in addition to the head) in cases of atypical IIH or compressive optic neuropathy especially when the clinical course or response to therapy is poor or progressive.

Rare Combination of Abducens Nerve Palsy and Optic Neuritis on the Same Side: Case Report and Review of 8 Patients in Literature.

The concurrent development of abducens nerve palsy and optic neuritis on the same side is rare. Here we presented an 82-year-old man who developed the combination of abducens nerve palsy and optic neuritis on the left side 2 months after the sixth inoculation of COVID-19 mRNA vaccine. In past history at 45 years old, he experienced subarachnoid hemorrhage and underwent surgery for the clipping of intracranial aneurysm. The patient had no systemic symptoms, such as general fatigue, fever, arthralgia, and skin rashes. Physical and neurological examinations were also unremarkable. Since the aneurysmal metal clip used at that time was not compatible with magnetic resonance imaging, he underwent computed tomographic (CT) scan of the head and showed no space-occupying lesion in the orbit, paranasal sinuses, and brain. As an old lesion, the anterior temporal lobe on the left side had low-density area with metallic artifact on the left side of the skull base, indicative of metal clipping. In 4 weeks of observation from the initial visit, he showed complete recovery of visual acuity and became capable of abducting the left eye in full degrees. We also reviewed 8 patients with the combination of abducens nerve palsy and optic neuritis in the literature to reveal that the combination of signs did occur in mild meningitis with rare infectious diseases and in association with preceding herpes zoster in the first branch of the trigeminal nerve. The course of the present patient suggested that the combination of signs might be vaccine-associated.

Optic Nerve MRI T2-Hyperintensity: A Nonspecific Marker of Optic Nerve Damage.

BACKGROUND: MRI abnormalities are common in optic neuropathies, especially on dedicated orbital imaging. In acute optic neuritis, optic nerve T2-hyperintensity associated with optic nerve contrast enhancement is the typical imaging finding. In chronic optic neuropathies, optic nerve T2-hyperintensity and atrophy are regularly seen. Isolated optic nerve T2-hyperintensity is often erroneously presumed to reflect optic neuritis, frequently prompting unnecessary investigations and neuro-ophthalmology consultations. Our goal was to determine the significance of optic nerve/chiasm T2-hyperintensity and/or atrophy on MRI. METHODS: Retrospective study of consecutive patients who underwent brain/orbital MRI with/without contrast at our institution between July 1, 2019, and June 6, 2022. Patients with optic nerve/chiasm T2-hyperintensity and/or atrophy were included. Medical records were reviewed to determine the etiology of the T2-hyperintensity and/or atrophy. RESULTS: Four hundred seventy-seven patients (698 eyes) were included [mean age 52 years (SD ±18 years); 57% women]. Of the 364 of 698 eyes with optic nerve/chiasm T2-hyperintensity without atrophy, the causes were compressive (104), inflammatory (103), multifactorial (49), glaucoma (21), normal (19), and other (68); of the 219 of 698 eyes with optic nerve/chiasm T2-hyperintensity and atrophy, the causes were compressive (57), multifactorial (40), inflammatory (38), glaucoma (33), normal (7), and other (44); of the 115 of 698 eyes with optic nerve/chiasm atrophy without T2-hyperintensity, the causes were glaucoma (34), multifactorial (21), inflammatory (13), compressive (11), normal (10), and other (26). Thirty-six eyes with optic nerve/chiasm T2-hyperintensity or atrophy did not have evidence of optic neuropathy or retinopathy on ophthalmologic examination, and 17 eyes had clinical evidence of severe retinopathy without primary optic neuropathy. CONCLUSIONS: Optic nerve T2-hyperintensity or atrophy can be found with any cause of optic neuropathy and with severe chronic retinopathy. These MRI findings should not automatically prompt optic neuritis diagnosis, workup, and treatment, and caution is advised regarding their use in the diagnostic criteria for multiple sclerosis. Cases of incidentally found MRI optic nerve T2-hyperintensity and/or atrophy without a known underlying optic neuropathy or severe retinopathy are rare. Such patients should receive an ophthalmologic examination before further investigations.

Prevalence of serum MOG antibody and AQP4 antibody in optic neuritis after SARS-CoV-2 infection.

Purpose: To evaluate the prevalence of serum myelin oligodendrocyte glycoprotein antibody (MOG-Ab) and aquaporin-4 antibody (AQP4-Ab) in optic neuritis (ON) patients after severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection by cell-based indirect immunofluorescence assay (CBA). Methods: In this prospective case series study, 35 patients clinically diagnosed as ON and laboratory-confirmed SARS-CoV-2 infection from 8 December 2022 to 8 February 2023 were included. All patients' clinical and laboratory data were collected and analyzed. Results: The mean age of the 35 patients (46 eyes) was 38.2 years (ranging from 6 to 69 years), and 17 cases were female patients. Thirty-three and two cases showed positive SARS-CoV-2 RNA test results before or shortly after ON onset, respectively. ON occurred unilaterally in 24 cases and bilaterally in 11 cases. Ophthalmic examination revealed swollen optic disc in 37 eyes, normal optic disc in 6 eyes, and temporally or wholly paled optic disc in 3 eyes. CBA revealed seropositive MOG-Ab in 10 cases and AQP4-Ab in 2 cases, respectively, of which 2 AQP4-Ab-seropositive cases and 1 MOG-Ab-seropositive case had a past medical history of ON. Most ON patients showed a rapid and dramatic response to pulse steroid therapy. The median of BCVA at the onset and at the last follow-up was 20/500 (ranging from light perception to 20/20) and 20/67 (ranging from counting fingers to 20/20), respectively. Conclusion: Serum MOG-Ab and AQP4-Ab were detected in 28.6% (10/35) and 5.7% (2/35) ON cases after SARS-CoV-2 infection. SARS-CoV-2 infection may trigger an onset or a relapse of ON, as well as the production of MOG-Ab.

Beyond Myelin Oligodendrocyte Glycoprotein and Aquaporin-4 Antibodies: Alternative Causes of Optic Neuritis.

Optic neuritis (ON) is the most common cause of vision loss in young adults. It manifests as acute or subacute vision loss, often accompanied by retrobulbar discomfort or pain during eye movements. Typical ON is associated with Multiple Sclerosis (MS) and is generally mild and steroid-responsive. Atypical forms are characterized by unusual features, such as prominent optic disc edema, poor treatment response, and bilateral involvement, and they are often associated with autoantibodies against aquaporin-4 (AQP4) or Myelin Oligodendrocyte Glycoprotein (MOG). However, in some cases, AQP4 and MOG antibodies will return as negative, plunging the clinician into a diagnostic conundrum. AQP4- and MOG-seronegative ON warrants a broad differential diagnosis, including autoantibody-associated, granulomatous, and systemic disorders. These rare forms need to be identified promptly, as their management and prognosis are greatly different. The aim of this review is to describe the possible rarer etiologies of non-MS-related and AQP4- and MOG-IgG-seronegative inflammatory ON and discuss their diagnoses and treatments.

SARS-CoV-2 Parainfectious Optic Neuropathy: 3 Case Reports and a Review of the Literature.

BACKGROUND: Parainfectious optic neuritis is an inflammatory reaction that occurs shortly after an infection without direct invasion by a pathogen. The clinical profile depends on the infectious organism. Cases of SARS-CoV-2 parainfectious optic neuritis have been reported in the literature, but there are no reviews that have applied strict inclusion criteria to more definitively establish the clinical profile associated with SARS-CoV-2. METHODS: We present 3 new cases of SARS-CoV-2 parainfectious optic neuritis. We also review the literature for definite cases by selecting only those with unambiguous clinical features and MRI findings of optic neuritis, positive SARS-CoV-2 polymerase chain reaction or serology, and the absence of myelin oligodendrocyte-glycoprotein or aquaporin-4 antibodies or other diseases associated with optic neuritis. RESULTS: We report 2 cases of monophasic, unilateral SARS-CoV-2 parainfectious optic neuritis with optic disc edema and nadir visual acuities of finger counting. We report 1 case of mild SARS-CoV-2 parainfectious optic neuritis that featured cotton wool spots, peripapillary wrinkles and hemorrhages, and recurrence after an initial steroid taper. We identified 6 cases of unambiguous SARS-CoV-2 parainfectious optic neuritis from the literature. Combining our case series with the case reports in the literature, the average age was 42.8 years, 3/9 had bilateral disease, 6/8 had optic disc edema, 8/9 had nadir visual acuity of finger counting or worse, and all recovered visual acuity to 20/40 or better after therapy with steroids. CONCLUSIONS: SARS-CoV-2 parainfectious optic neuritis has a clinical profile that is atypical for idiopathic optic neuritis but fairly typical of parainfectious forms of optic neuritis with a severely reduced nadir visual acuity, high likelihood of bilaterality, high incidence of optic disc edema, and prompt and significant response to corticosteroids. Further study with long-term follow-up and epidemiologic investigation will be needed to further characterize this clinical entity.

Myelin oligodendrocyte glycoprotein antibody-associated optic neuritis in a post-COVID-19 infection patient.

PURPOSE: SARS-CoV-2 viral infection affects multiple systems including the respiratory, gastrointestinal, neurological, cardiac, and ophthalmic systems. We report a case of myelin oligodendrocyte glycoprotein (MOG) related optic neuritis in a SARS-CoV-2 (COVID-19) patient. METHODS: Case report. RESULTS: A 36-year-old Malay gentleman with underlying hypertension presented with the first episode of bilateral progressively worsening blurred vision for 1 week associated with retrobulbar pain. There were no other neurological symptoms. He had fever a week before the eye symptoms and tested positive for COVID-19. He received COVID-19 booster vaccine a month before the disease onset. On examination, his vision was hand motion on right eye and 6/18 on left eye. Relative afferent pupillary defect (RAPD) was positive on right eye with abnormal optic nerve function tests. Anterior segments were unremarkable. Fundus examination showed bilateral optic disc swelling. MRI revealed multifocal hyperintense subcortical white matter lesions. Optic nerves appeared normal with no enhancement seen. Blood investigation showed a positive serum MOG antibody. Intravenous methylprednisolone was commenced followed by oral prednisolone after which his vision and ocular symptoms markedly improved. The oral prednisolone was tapered alongside addition of azathioprine. At 1 month, the disease was stable with no recurrence. CONCLUSION: While optic neuritis has been associated with both COVID-19 infection and vaccination, MOG IgG antibody-mediated optic neuritis is also a possible manifestation. This type of optic neuritis associated with COVID-19 infection does not show a similar pattern of frequent recurrences as seen in non-COVID-19 related optic neuritis.

[Combination of post-infectious optic neuritis and Guillain-Barré syndrome]. / Sochetanie postinfektsionnogo nevrita zritel'nykh nervov i sindroma Giiena­Barre.

The coexistence of optic neuritis and Guillain-Barré syndrome is a rare combination of neurological diseases. The trigger of an autoimmune inflammatory process is often a respiratory mycoplasma infection. Ignorance of such combination can lead to diagnostic and therapy mistakes. This article describes the case of a rare combination of overlapping optic neuritis and Guillain-Barré syndrome, associated with Mycoplasma pneumoniae and provides the short literature review. Further studies are required to identify common pathogenetic mechanisms of combined inflammatory lesions of the optic nerves and peripheral nervous system.

An update on optic neuritis.

Optic neuritis (ON) is the most common cause of subacute optic neuropathy in young adults. Although most cases of optic neuritis (ON) are classified as typical, meaning idiopathic or associated with multiple sclerosis, there is a growing understanding of atypical forms of optic neuritis such as antibody mediated aquaporin-4 (AQP4)-IgG neuromyelitis optica spectrum disorder (NMOSD) and the recently described entity, myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease (MOGAD). Differentiating typical ON from atypical ON is important because they have different prognoses and treatments. Findings of atypical ON, including severe vision loss with poor recovery with steroids or steroid dependence, prominent optic disc edema, bilateral vision loss, and childhood or late adult onset, should prompt serologic testing for AQP4-IgG and MOG-IgG. Although the traditional division of typical and atypical ON can be helpful, it should be noted that there can be severe presentations of otherwise typical ON and mild presentations of atypical ON that blur these traditional lines. Rare causes of autoimmune optic neuropathies, such as glial fibrillary acidic protein (GFAP) and collapsin response-mediator protein 5 (CRMP5) autoimmunity also should be considered in patients with bilateral painless optic neuropathy associated with optic disc edema, especially if there are other accompanying suggestive neurologic symptoms/signs. Typical ON usually recovers well without treatment, though recovery may be expedited by steroids. Atypical ON is usually treated with intravenous steroids, and some forms, such as NMOSD, often require plasma exchange for acute attacks and long-term immunosuppressive therapy to prevent relapses. Since treatment is tailored to the cause of the ON, elucidating the etiology of the ON is of the utmost importance.

[Hereditary optic neuropathy associated with demyelinating diseases of the central nervous system]. / Nasledstvennaya opticheskaya neiropatiya v sochetanii s demieliniziruyushchimi zabolevaniyami tsentral'noi nervnoi sistemy.

Demyelinating optic neuritis and hereditary optic neuropathy (HON) take a leading place among the diseases, the leading clinical syndrome of which is bilateral optic neuropathy with a simultaneous or sequential significant decrease in visual acuity. Optic neuritis can occur at the onset or be one of the syndromes within multiple sclerosis (MS), neuromyelitis optica spectrum disorders (NMOSD), and myelin oligodendrocyte glycoprotein (MOG) antibody disease (MOGAD). HON are a group of neurodegenerative diseases, among which the most common variants are Leber's hereditary optic neuropathy (LHON), associated with mitochondrial DNA (mtDNA) mutations, and autosomal recessive optic neuropathy (ARON), caused by nuclear DNA (nDNA) mutations in DNAJC30. There are phenotypes of LHON «plus¼, one of which is the association of HON and CNS demyelination in the same patient. In such cases, the diagnosis of each of these diseases causes significant difficulties, due to the fact that in some cases there are clinical and radiological coincidences between demyelinating and hereditary mitochondrial diseases.

MOG-specific T cell response amplified in para- and post-SAR-CoV-2 infection in myelin oligodendrocyte glycoprotein antibody-associated disease.

We describe clinical characteristics and deep immunophenotypes in two patients with myelin-oligodendrocyte-glycoprotein (MOG)-antibody-associated-disease after COVID-19. The para-COVID case was a 74-year-old man who developed optic neuritis two days after COVID-19. Immunological assays revealed reduced absolute CD8+ T- and B-cell counts with increased frequency of NK cells. Post-COVID case was a 63-year-old man with optic neuritis six months after COVID-19, a frequency of CD8+ T-cells was elevated with a relatively low fraction of naïve and a high fraction of effector memory CD8+ T-cells. There was increased frequency of CD8+CD38+HLA-DR+ T-cells in the para-COVID case; interestingly, CD4+CD38+HLA-DR+ T cell frequency was increased in the post-COVID case. Both had increased SARS-CoV-2-specific and MOG-specific T-cell responses.

Acute optic neuritis: What are the clues to the aetiological diagnosis in real life?

BACKGROUND: Previous cross-sectional studies have reported distinct clinical and radiological features among the different acute optic neuritis (ON) aetiologies. Nevertheless, these reports often included the same number of patients in each group, not taking into account the disparity in frequencies of ON aetiologies in a real-life setting and thus, it remains unclear what are the truly useful features for distinguishing the different ON causes. To determine whether clinical evaluation, ophthalmological assessment including the optical coherence tomography (OCT), CSF analysis, and MRI imaging may help to discriminate the different causes of acute ON in a real-life cohort. METHODS: In this prospective monocentric study, adult patients with recent acute ON (<1 month) underwent evaluation at baseline and 1 and 12 months, including, high- and low-contrast visual acuity, visual field assessment and OCT measurements, baseline CSF analysis and MRI. RESULTS: Among 108 patients, 71 (65.7%) had multiple sclerosis (MS), 19 (17.6%) had idiopathic ON, 13 (12.0%) and 5 (4.6%) had myelin oligodendrocyte glycoprotein and aquaporin-4 antibodies, at last follow up respectively.At baseline, the distribution of bilateral ON, CSF-restricted oligoclonal bands, optic perineuritis, optic nerve length lesions and positive dissemination in space and dissemination in time criteria on MRI were significantly different between the four groups (p <0.001). No significant difference in visual acuity nor inner retinal layer thickness was found between the different ON aetiologies. CONCLUSIONS: In this large prospective study, bilateral visual involvement, CSF and MRI results are the most useful clues in distinguishing the different aetiologies of acute ON, whereas ophthalmological assessments including OCT measurements revealed no significant difference between the aetiologies.