Optic Neuritis During Lactation: A Case Series

2020 ◽  
pp. 089033442097049
Author(s):  
Johnson Jeslin ◽  
C. Seetharaman ◽  
Virna M. Shah
Keyword(s):  
Optic Neuritis ◽  
Demyelinating Disease ◽  
Case Series ◽  
Rare Condition ◽  
Complete Recovery ◽  
High Dose ◽  
Oral Steroids ◽  
Optic Neuritis Treatment Trial ◽  
Intravenous Steroids ◽  
Sudden Blindness

Background Optic neuritis is a rare condition that can lead to sudden blindness and also could be a precursor to multiple sclerosis. When it occurs postpartum during lactation, it is called lactation optic neuritis. Main issue We present four cases of optic neuritis in lactating mothers, two of which had additional features of demyelinating disease upon neurological imaging. Management All participants were treated with high dose intravenous steroids followed by 11 days of oral steroids, per the optic neuritis treatment trial, which led to complete recovery of vision. Two participants with demyelinating disease on magnetic resonance imaging scans were advised to wean, because of a need for immunosuppressive therapy later. Conclusion Optic neuritis during lactation should be suspected following acute loss of vision. Prompt referral to an ophthalmologist is mandated for early diagnosis and treatment to prevent long-term co-morbidities.

Acute Disseminated Encephalomyelitis followed by Optic Neuritis: A Rare Syndrome of Uncertain Treatment and Prognosis

2020 ◽  
Vol 51 (04) ◽  
pp. 286-291 ◽  
Author(s):  
Maria Serra ◽  
Anna Presicci ◽  
Martina Fucci ◽  
Mariella Margari ◽  
Roberto Palumbi ◽  
...  
Keyword(s):  
Optic Neuritis ◽  
Clinical Laboratory ◽  
Case Reports ◽  
Oral Prednisone ◽  
Clinical History ◽  
Complete Recovery ◽  
Acute Disseminated Encephalomyelitis ◽  
Cochrane Library ◽  
High Dose ◽  
Intravenous Steroid

Abstract Aim Acute Disseminated Encephalomyelitis followed by optic neuritis (ADEM-ON), first described in 2013, is a rare demyelinating syndrome, typical of the pediatric age. We conducted a mini review of the existing literature, focusing on clinical, laboratory, radiological, therapeutic, and prognostic aspects in order to improve the identification of new cases. Methods We searched PubMed and Cochrane Library for studies on ADEM-ON between 2013 and 2018. Results Examination of the reported cases (three case reports and eight observational studies) established the following features. Time between ADEM and ON is highly variable. Almost all patients show antimyelin oligodendrocyte glycoprotein antibody (MOG-abs) seropositivity. High-dose intravenous steroid and plasmapheresis efficacy is reported for the acute phase; oral prednisone and other maintenance drugs may be useful in avoiding relapses. The clinical history may lead to a complete recovery but also to residual deficits. Conclusion MOG-abs detection strongly supports ADEM-ON diagnosis, confirming this entity as part of MOG-abs spectrum disorder. Owing to the very small number of cases so far reported, predicting clinical evolution is very difficult.

scholarly journals Vision improvement in severe acute isolated optic neuritis after plasma exchange treatment in Chinese population: a prospective case series study

2020 ◽  
Vol 13 ◽  
pp. 175628642094797
Author(s):  
Shaoying Tan ◽  
Tsz Kin Ng ◽  
Quangang Xu ◽  
Mo Yang ◽  
Yuan Zhuang ◽  
...  
Keyword(s):  
Optic Neuritis ◽  
Plasma Exchange ◽  
Time Window ◽  
Case Series ◽  
Visual Outcome ◽  
Chinese Patients ◽  
High Dose ◽  
Visual Outcomes ◽  
Double Filtration Plasmapheresis ◽  
Case Series Study

Background: Plasma exchange (PE) is often considered as an effective treatment for neuromyelitis optica spectrum disorder (NMOSD) and several inflammatory demyelinating disorders of the central nervous system. This study aimed to evaluate the visual outcomes of Chinese patients with severe acute isolated optic neuritis (ON) who received PE therapy after high-dose intravenous methylprednisolone (IVMP) treatment. Methods: Thirty-seven acute isolated ON patients experiencing their first attack of severe visual impairment without neurological disability were recruited. All subjects received five cycles of double-filtration plasmapheresis. Visual acuity (VA) was documented at onset, 1 day before PE treatment, at each cycle of PE therapy and at the 1-month follow-up visit. Results: This study included 26 female (70.3%) and 11 male (29.7%) subjects, and 18 subjects (48.6%) had bilateral involvement. The time window between onset and PE treatment was 27.3 ± 12.7 days (range: 6–53 days). Mean VA (logMAR) of the studied eyes at onset, 1-day before PE treatment/after IVMP and after the fifth PE treatment were 3.41 ± 1.50, 2.61 ± 1.64 and 1.66 ± 1.52, respectively ( p < 0.001). Nineteen eyes (51.4%) showed no light perception at the onset, and 17 eyes (45.9%) improved to Snellen VA >20/800 after IVMP and PE treatments, among which five eyes (13.5%) recovered to Snellen VA 20/20 ( p < 0.001). Predictors of good visual outcome included body mass index [odds ratio (OR) = 0.734, p = 0.044], serum AQP4 antibody-positive status (OR = 0.004, p = 0.001), bilaterality (OR = 0.042, p = 0.008) and time window from onset to PE therapy per 1 day (OR = 0.79, p = 0.002). Conclusion: This study revealed that PE treatment effectively improves the visual outcomes of patients experiencing their first attack of severe acute isolated ON after high-dose IVMP treatment. Better visual outcomes can be achieved with early PE treatment.

scholarly journals Mitochondrial Uncoupler Prodrug of 2,4-Dinitrophenol, MP201, Prevents Neuronal Damage and Preserves Vision in Experimental Optic Neuritis

2017 ◽  
Vol 2017 ◽  
pp. 1-10 ◽  
Author(s):  
Reas S. Khan ◽  
Kimberly Dine ◽  
John G. Geisler ◽  
Kenneth S. Shindler
Keyword(s):  
Optic Neuritis ◽  
Demyelinating Disease ◽  
Ganglion Cells ◽  
Neuronal Damage ◽  
Neuroprotective Effect ◽  
High Dose ◽  
Axonal Loss ◽  
Mitochondrial Uncoupling ◽  
The Central Nervous System ◽  
Mitochondrial Uncoupler

The ability of novel mitochondrial uncoupler prodrug of 2,4-dinitrophenol (DNP), MP201, to prevent neuronal damage and preserve visual function in an experimental autoimmune encephalomyelitis (EAE) model of optic neuritis was evaluated. Optic nerve inflammation, demyelination, and axonal loss are prominent features of optic neuritis, an inflammatory optic neuropathy often associated with the central nervous system demyelinating disease multiple sclerosis. Currently, optic neuritis is frequently treated with high-dose corticosteroids, but treatment fails to prevent permanent neuronal damage and associated vision changes that occur as optic neuritis resolves, thus suggesting that additional therapies are required. MP201 administered orally, once per day, attenuated visual dysfunction, preserved retinal ganglion cells (RGCs), and reduced RGC axonal loss and demyelination in the optic nerves of EAE mice, with limited effects on inflammation. The prominent mild mitochondrial uncoupling properties of MP201, with slow elimination of DNP, may contribute to the neuroprotective effect by modulating the entire mitochondria’s physiology directly. Results suggest that MP201 is a potential novel treatment for optic neuritis.

scholarly journals Acute Clinical Worsening after Steroid Administration in Cervical Myelitis May Reveal a Subdural Arteriovenous Fistula

2016 ◽  
Vol 8 (3) ◽  
pp. 234-242 ◽  
Author(s):  
Silvia Rain ◽  
Jan Udding ◽  
Daniel Broere
Keyword(s):  
Arteriovenous Fistula ◽  
Clinical Manifestations ◽  
Rare Condition ◽  
Complete Recovery ◽  
Mri Imaging ◽  
Hyperintense Lesion ◽  
Steroid Administration ◽  
Sensory Deficits ◽  
Intravenous Steroids ◽  
Clinical Worsening

Subdural arteriovenous fistula (SDAVF) is a rare condition characterized by clinical manifestations ranging from mild bilateral sensory deficits to quadriplegia. The diagnosis is often delayed due to unspecific neurological symptoms, initially diagnosed as polyneuropathy or myelopathy. The diagnosis can be delayed for as long as 1–15 years. The following report describes a cervical SDAVF case initially misdiagnosed as myelitis transversa and treated with intravenous steroids. A 56-year-old male presented with sensory deficits and mild leg and right arm weakness. Cervical MRI showed a central medullary hyperintense lesion with contrast enhancement. After metabolic, infectious, and malignant causes were excluded, myelitis transversa was presumed and the patient was treated intravenously with methylprednisolone. Shortly after that, he developed quadriplegia. Cervical MRI imaging showed engorged cervical perimedullary vessels, which were not visible on the initial MRI. The diagnosis was revised and a SDAVF identified. Prompt surgical treatment led to a complete recovery. The effect of intravenous steroids in SDAVF is controversial. Acute clinical worsening after steroid administration is previously reported in several publications; however, due to the paucity of clinical studies on SDAVF, this effect remains mostly overlooked or unknown. The findings in this patient support the causative relation between SDAVF clinical worsening and steroid administration. We propose that acute clinical worsening under steroids in patients initially diagnosed with myelitis should raise suspicion of an SDAVF.

scholarly journals To study visual and neurological outcome after treatment according to ONTT protocol

2022 ◽  
Vol 7 (4) ◽  
pp. 728-730
Author(s):  
I D Chaurasia ◽  
Yogita Chaurasia
Keyword(s):  
Optic Neuritis ◽  
Neurological Outcome ◽  
Visual Recovery ◽  
Treatment Trial ◽  
Neurological Outcomes ◽  
Protein Calorie Malnutrition ◽  
Oral Steroids ◽  
Optic Neuritis Treatment Trial ◽  
Generalized Lymphadenopathy

To evaluate the visual and neurological outcomes of Optic Neuritis Treatment Trial (ONTT). 40 Patients presenting with optic neuritis were enrolled in this study to analyze the Visual and Neurological outcomes after treatment according to ONTT Protocol, with emphasis on signs of anaemia, protein calorie malnutrition, vitamin deficiency, generalized lymphadenopathy, sinusitis, septic foci. Patients were followed up for three subsequent visits to assess the rate of visual recovery.Maximum patient 35(87.5) treated according to ONTT protocol while 5 (12.5%) treated with oral steroids alone. Maximum 33 eyes (70.2%) attain BCVA &#62; 6/36 after 1st follow-up (with in 1 month) while 10(21.3%) eyes attain BCVA of 6/6 after 1st follow-up. 11 eyes (25.6%) attain BCVA 6/6 after II follow-up. General prognosis for recovery of vision was good and was slightly worse in more severely affected cases in the present series. Pallor of the optic disc and defect of vision did not always correspond–3 eyes which showed temporal of the disc at the end of follow up had a final vision of 6/9 or better in each eye.

Efficacy of High-dose Steroids for Visual Acuity Improvement in Methanol-induced Toxic Optic Neuropathy

2019 ◽  
Vol 44 (2) ◽  
pp. 60
Author(s):  
Theresia Yinski ◽  
Syntia Nusanti
Keyword(s):  
Visual Acuity ◽  
Optic Neuropathy ◽  
Alcohol Intoxication ◽  
Age Distribution ◽  
Case Series ◽  
High Dose ◽  
Retrospective Case ◽  
Toxic Optic Neuropathy ◽  
Sudden Blindness

Introduction : Methanol-induced toxic optic neuropathy (TON) is defined as a visual impairment due to optic nerve damage by methanol poisoning. Not only is this disease entity underdiagnosed at times, this sudden blindness is also often diagnosed at a stage where recovery of vision is no longer possible. Materials and Methods : A literature search was conducted using PubMed, ClinicalKey, Google Scholar and ScienceDirect by combining the keywords ‘methanol’ or ‘methyl alcohol’, ‘intoxication’ or ‘poisoning’, ‘toxic optic neuropathy’, and ‘visual acuity’ with ‘high-dose steroid’. Results : The total amount of subjects in each article varied from 2 to 37, with mean age distribution of 26.34 to 55 years old, where most patients were male. The follow up duration varied from 1 week to 1 year. Four articles do not mention high-dose steroids treatment as therapy while the other four mention use of 1000 mg of intravenous methylprednisolone per day with divided doses of either 2x500 mg or 4x250 mg. Improvement percentages show 100% improvement in all studies that used high-dose steroids, while in the non-high-dose steroids studies the improvement percentages range from 33.33% to 90%. Summary : High-dose steroids are showing efficacy in improving visual acuity and reducing the inflammation in methanol-induced TON. The period of how fast the therapy takes effect is inconclusive, as the mean follow-up time differs widely per study. However, because most of the reviewed studies here are retrospective case series, a larger, more comprehensive study is required to acknowledge more of the efficacy profile.

scholarly journals New Treatment Options for Pituitary Granulomatosis With Polyangiitis

2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A594-A594
Author(s):  
Hessa Boharoon ◽  
Majid AlAmeri ◽  
Abdulla Mohamed Alnuaimi ◽  
Nigel Mendoza ◽  
Stephen McAdoo ◽  
...  
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Treatment Guidelines ◽  
Dynamic Assessment ◽  
Treatment Options ◽  
Case Series ◽  
Rare Condition ◽  
High Dose ◽  
Pituitary Mass ◽  
Remission Maintenance ◽  
History Of

Abstract Background: Granulomatosis with polyangiitis (GPA) rarely involves the pituitary gland and has been reported in 1% of all cases of GPA. Most frequently, it presents with pituitary mass effect and diabetes insipidus (DI). To date, there are no treatment guidelines for this rare condition. Case Presentation: Case 1: A 55 year old female with a history of ANCA-positive pulmonary GPA, previously treated with glucocorticoids and immunosuppressants, presented two years later with cranial DI and bitemporal hemianopia. MRI showed a large sellar mass with suprasellar extension. High dose glucocorticoids resulted in good clinical and radiological response. Further treatment consisted in a combination of Cyclophosphamide and Rituximab (RTX). Further doses of RTX are planned aiming for a period of B-cell depletion. Case 2: A 38 year old female, presented with polyuria, recurrent nosebleeds, headaches and a left visual field defect. Pituitary profile revealed ACTH deficiency and MRI showed a heterogenous cystic lesion with peripheral enhancement and stalk thickening. Steroid replacement led to immediate improvement in her symptoms. Cranial DI was confirmed and raised Proteinase 3 (PR3) antibody suggested GPA. A combination of prednisolone and Methotrexate led to significant improvement of MRI appearances and declining PR3 antibody levels. For remission maintenance, two cycles of RTX were given with further radiological and biochemical improvement, and, following dynamic assessment of her HPA axis, she could be fully weaned off steroids. Case 3 is a 47 year old female with a history of childhood asthma. She was found to have cavitating lung lesions. ANCA positivity confirmed GPA and she was commenced on high-dose steroids. During follow-up, she developed headache, polyuria and polydipsia. MRI pituitary showed a suprasellar lesion and pituitary biopsy revealed inflammatory hypophysitis. Cranial DI was confirmed by water deprivation testing. Previous allergic reactions to both RTX and Ofatumumab precluded anti-CD40 monoclonal antibodies and she was commenced on Azathioprine. A further recurrence of pituitary GPA necessitated escalation of the steroid dose and switch of azathioprine to mycophenolate mofetil. She remains in remission and her steroids reduced to a maintenance dose. Conclusion: GPA pituitary has been observed to occur at variable time after diagnosis often in the absence of any other systemic features. A combination of glucocorticoids and RTX has been approved for severe relapsing pulmonary GPA, however, limited data is available for pituitary GPA. In this case series, the response to high dose steroids and RTX for remission maintenance has been encouraging. Experience with ‘conventional’ immunosuppresants remains limited and therapeutic responses remain variable. Further clinical studies are required to establish effective treatment for pituitary GPA.

Therapeutic efficacy of plasma exchange in NMO-IgG-positive patients with neuromyelitis optica

2007 ◽  
Vol 13 (1) ◽  
pp. 128-132 ◽  
Author(s):  
S Watanabe ◽  
I Nakashima ◽  
T Misu ◽  
I Miyazawa ◽  
Y Shiga ◽  
...  
Keyword(s):  
Optic Neuritis ◽  
Plasma Exchange ◽  
Neuromyelitis Optica ◽  
Therapeutic Efficacy ◽  
Demyelinating Disease ◽  
High Risk Group ◽  
Functional Improvement ◽  
High Dose ◽  
Humoral Immune

Neuromyelitis optica (NMO) is an inflammatory demyelinating disease of the central nervous system (CNS) with a poor prognosis in terms of the optic-spinal function. Recently, a serum autoantibody (NMO-IgG) binding to the blood–brain barrier region was detected exclusively in patients with NMO and its high risk group. We treated six NMO-IgG-positive patients (all female; age 21–67 years old, median 41; three with optic neuritis and three with myelitis) who were unresponsive to high-dose intravenous methylprednisolone (HIMP), with plasma exchange (PE) (three to five exchanges, 2–3 L each). Three of the patients(one with optic neuritis and two with myelitis) showed definite functional improvement following PE. The clinical improvement started to appear after one or two exchanges, while there was little or no improvement in the other three patients. Such quick clinical responses to PE suggest a pathogenetic role of humoral immune factors in NMO, although delayed responses to the corticosteroid therapy might have contributed to the therapeutic efficacy, in part. Further clinical and in vitro studies are needed to determine whether the removal of NMO-IgG is directly relevant to the therapeutic efficacy. PE may hasten the functional recovery from corticosteroid-resistant relapses in some NMO-IgG-positive patients with NMO.

Autoimmune Encephalitis in Children: A Case Series at a Tertiary Care Center

2020 ◽  
Vol 35 (9) ◽  
pp. 591-599 ◽  
Author(s):  
Alice Rutatangwa ◽  
Nikita Mittal ◽  
Carla Francisco ◽  
Kendall Nash ◽  
Emmanuelle Waubant
Keyword(s):  
San Francisco ◽  
Care Center ◽  
Tertiary Care ◽  
Case Series ◽  
Autoimmune Encephalitis ◽  
Complete Recovery ◽  
Response To Treatment ◽  
High Dose ◽  
Presenting Symptoms ◽  
Nmdar Encephalitis

Autoimmune encephalitis is the third most common cause of encephalitis in children. We provide a detailed account of presenting symptoms, diagnosis, and response to treatment in pediatric autoimmune encephalitis patients evaluated at University of California San Francisco within a 2.5-year period. Eleven were identified: anti– N-methyl-d-aspartate receptor (NMDAR) encephalitis (n = 4), antibody-negative autoimmune encephalitis (n = 4), steroid-responsive encephalopathy associated with thyroiditis (SREAT) (n = 2), and glial fibrillary acidic protein (GFAP)–associated encephalitis (n = 1). Most common presenting symptoms included seizures and behavior changes (54%). More than 90% of patients showed improvement following first-line immunotherapy (high-dose corticosteroids, intravenous immunoglobulin, and/or plasma exchange). A total of 64% received second-line treatment with rituximab, cyclophosphamide, or mycophenolate mofetil. One patient with NMDAR encephalitis died despite escalating immunotherapy. None of the patients showed complete recovery after median follow-up of 9 months (range 0.5-66). Children with autoimmune encephalitis have a diverse clinical presentation and may lack an identifiable autoantibody. Majority of patients show a good response to immunotherapy; however, recovery can be delayed.

scholarly journals Rheumatoid Meningitis a Rare Extra-Articular Manifestation of Rheumatoid Arthritis: Report of 6 Cases and Literature Review

2020 ◽  
Vol 9 (6) ◽  
pp. 1625
Author(s):  
Mélanie Trabelsi ◽  
Xavier Romand ◽  
Mélanie Gilson ◽  
Mathieu Vaillant ◽  
Pierre-André Guerne ◽  
...  
Keyword(s):  
Rheumatoid Arthritis ◽  
Lumbar Puncture ◽  
Clinical Symptoms ◽  
Complete Recovery ◽  
High Rate ◽  
High Dose ◽  
Biologic Dmards ◽  
Oral Steroids ◽  
Fluid Attenuated Inversion Recovery ◽  
Rheumatoid Meningitis

Objectives. Central neurological manifestations of rheumatoid arthritis (RA) like rheumatoid meningitis (RM) are rare, little known and have a high rate of morbi-mortality. METHODS. We described six cases of RM that were directly related to RA activity after exhaustive assessment. RESULTS. They were mainly women, aged of 50 to 69. All were positive for anti-cyclic citrullinated peptide antibodies and half for rheumatoid factors. RA activity, duration, and treatments were heterogeneous including oral steroids, conventional synthetic disease modifying anti-rheumatic drugs (DMARDs) and biologic DMARDs. Symptoms were various, with acute or progressive beginning; main were: generalized or focal seizure (4/6), fever (3/6), headaches (3/6), and frontal syndrome (2/6). Imaging lesions were four leptomeningitis, one pachymeningitis, and one association of both. MRI usually showed hypersignal in various territories in T2-FLAIR (fluid attenuated inversion recovery) mode, and enhancement in T1-weighted mode after gadolinium injection. All patients had lumbar puncture that found sterile cerebrospinal fluid, no neoplasic cell, elevated cell count in 5/6 cases and elevated proteins concentration in 3/6 cases. Cerebral biopsy was possible for three patients, and definitively confirmed the diagnosis of aseptic lepto- or pachymenintis, excluding vasculitis and lymphoma. Different treatments were used like intravenous high dose steroids, immunoglobulins or biologic DMARDs, with variable clinical and imaging outcome: one death, one complete recovery, and four recoveries with sequelae. Conclusions. Clinical symptoms, imaging, lumbar puncture, and serological studies are often nonspecific, only histologic examination can confirm the diagnosis of RM. Any central neurological manifestation in RA patients, even in quiescent and ancient RA, should warn the physician.

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