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1.
Spinal Cord Ser Cases ; 10(1): 30, 2024 Apr 25.
Article in English | MEDLINE | ID: mdl-38664389

ABSTRACT

INTRODUCTION: Acute transverse myelitis (ATM) is a rare neurological complication of Coronavirus disease (COVID-19) vaccines. Various vaccines have been linked to ATM, such as non-replicating viral vectors, ribonucleic acid, and inactivated vaccines. An ATM case is presented here involving the BNT162b2 vaccine leading to asymmetrical incomplete paraplegia and neurogenic bladder. CASE PRESENTATION: A 66-year-old male developed urinary retention one day after his second dose of the BNT162b2 vaccine, followed by rapidly progressing lower limb weakness. Clinical examination showed asymmetrical paraparesis, reduced sensation below the T8 level, including perianal sensation, and loss of ankle and anal reflexes. Laboratory tests were largely unremarkable, while the spine MRI revealed thickened conus medullaris with a mild increase in T2/STIR signal intensity and subtle enhancement post gadolinium. Following treatment with methylprednisolone, plasmapheresis, and immunoglobulin, and a rehabilitation program, the patient achieved good motor and sensory recovery, but the bladder dysfunction persisted. Single-channel cystometry indicated neurogenic detrusor underactivity and reduced bladder sensation, as evidenced by low-pressure and compliant bladder. The urethral sphincter appeared intact or overactive. The post-void residual urine was significant, necessitating prolonged intermittent catheterisation. DISCUSSION: Bladder dysfunction due to the COVID-19 vaccine-associated ATM is not as commonly reported as motor or sensory deficits. To our knowledge, this is the first case to highlight a neurogenic bladder that necessitates prolonged intermittent catheterisation as a consequence of COVID-19 vaccine-associated ATM. This report highlights the rare complication of the neurogenic bladder resulting from the BNT162b2 vaccine. Early detection and treatment are crucial to prevent long-term complications.


Subject(s)
BNT162 Vaccine , COVID-19 Vaccines , Myelitis, Transverse , Urinary Bladder, Neurogenic , Humans , Male , Myelitis, Transverse/etiology , Aged , Urinary Bladder, Neurogenic/etiology , Urinary Bladder, Neurogenic/therapy , BNT162 Vaccine/adverse effects , COVID-19 Vaccines/adverse effects , COVID-19/complications
2.
Mult Scler Relat Disord ; 85: 105526, 2024 May.
Article in English | MEDLINE | ID: mdl-38489945

ABSTRACT

BACKGROUND: Eosinophils in cerebrospinal fluid (CSF) are an uncommon finding most often associated with parasitic infections, but have also been described in some neuroinflammatory disorders. Eosinophilic infiltration is not thought to be a typical feature of myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD). We aim to describe the rate of CSF eosinophil positivity in a cohort of pediatric MOGAD patients. METHODS: Single-center retrospective chart review of pediatric MOGAD patients. Clinical and laboratory data was collected from the electronic medical record and analyzed. RESULTS: Of 46 pediatric patients with positive serum myelin oligodendrocyte glycoprotein antibody (MOG-IgG) identified, 38 patients fulfilling internationally proposed MOGAD diagnostic criteria were included for analysis. 6 patients with MOGAD were excluded as no CSF data was available, and 2 patients with positive MOG-IgG but diagnosis more consistent with MS were excluded. Median age was 7.3 years, and 19/38 (50 %) were female. Acute disseminated encephalomyelitis (ADEM) was the most common presenting phenotype (23/38, 61 %), and other phenotypes included optic neuritis (10/38, 26 %), transverse myelitis (3/38, 8 %), and neuromyelitis optica spectrum disorder (NMOSD) (2/38, 5 %). 12 of 36 (33 %) patients with all lumbar puncture (LP) data available had CSF eosinophils present, with eosinophil mean of 3 % and range from 1 % to 18 % of CSF while blood cells. CONCLUSION: CSF eosinophils were present in one third of pediatric MOGAD patients, which is a higher rate than previously reported in either MOGAD or aquaporin-4 antibody positive NMOSD cohorts. Understanding the CSF composition of pediatric MOGAD patients helps to facilitate more prompt diagnosis and treatment and may shed light onto underlying pathologic mechanisms of disease with the goal to inform future therapeutic targets.


Subject(s)
Autoantibodies , Eosinophils , Myelin-Oligodendrocyte Glycoprotein , Humans , Myelin-Oligodendrocyte Glycoprotein/immunology , Female , Male , Child , Retrospective Studies , Eosinophils/immunology , Child, Preschool , Adolescent , Autoantibodies/cerebrospinal fluid , Autoantibodies/blood , Encephalomyelitis, Acute Disseminated/immunology , Encephalomyelitis, Acute Disseminated/cerebrospinal fluid , Encephalomyelitis, Acute Disseminated/blood , Encephalomyelitis, Acute Disseminated/diagnosis , Neuromyelitis Optica/cerebrospinal fluid , Neuromyelitis Optica/immunology , Neuromyelitis Optica/blood , Infant , Myelitis, Transverse/immunology , Myelitis, Transverse/cerebrospinal fluid , Myelitis, Transverse/blood , Optic Neuritis/immunology , Optic Neuritis/cerebrospinal fluid , Optic Neuritis/blood , Demyelinating Autoimmune Diseases, CNS/cerebrospinal fluid , Demyelinating Autoimmune Diseases, CNS/immunology , Demyelinating Autoimmune Diseases, CNS/blood
3.
BMJ Case Rep ; 17(3)2024 Mar 25.
Article in English | MEDLINE | ID: mdl-38531558

ABSTRACT

We diagnosed a patient with dengue fever who developed acute onset of sensorimotor quadriparesis with bladder involvement, and facial nerve involvement. Despite initial negative results in routine investigations and cerebrospinal fluid analysis, spinal MRI confirmed longitudinally extensive transverse myelitis. The aetiological workup was negative, prompting an investigation into the presence of dengue in the cerebrospinal fluid, which returned positive. This case underscores the importance of considering rare neurological complications in dengue, the value of advanced diagnostic techniques and the potential effectiveness of tailored interventions in challenging cases.


Subject(s)
Dengue , Myelitis, Transverse , Myelitis , Humans , Myelitis, Transverse/diagnosis , Dengue/complications , Magnetic Resonance Imaging/methods , Quadriplegia/complications , Facial Nerve , Myelitis/complications
4.
Spinal Cord Ser Cases ; 10(1): 13, 2024 Mar 15.
Article in English | MEDLINE | ID: mdl-38490982

ABSTRACT

INTRODUCTION: Acute transverse myelitis (ATM) refers to a rare severe acquired spinal cord inflammation, with a challenging diagnostic work-up and treatment. CASE PRESENTATION: We report the case of a 42-year-old patient who presented with loss of temperature and pain sensation beneath the C5 dermatome in her left side and reported a history of a possible respiratory tract illness 10 days ago. Within 2 days, clinical worsening was noted, compatible with Brown-Sequard syndrome. Spinal magnetic resonance imaging revealed a T2 sequence abnormal signal from level C4 to T3 and cerebrospinal fluid (CSF) studies showed only a mild pleocytosis mononuclear type. Extensive CSF and blood tests revealed only high Mycoplasma pneumoniae IgM and IgG titers. Treatment with high-dose intravenous methylprednisolone and oral azithromycin were administrated and the patient recovered completely within two months. DISCUSSION: We would like to highlight the importance for physicians to consider M. pneumoniae in their differential diagnosis as a potential cause when encountering patients with symptoms of ATM and inflammatory Brown-Sequard syndrome.


Subject(s)
Brown-Sequard Syndrome , Myelitis, Transverse , Adult , Female , Humans , Brown-Sequard Syndrome/etiology , Decompression, Surgical , Magnetic Resonance Imaging , Mycoplasma pneumoniae , Myelitis, Transverse/complications
5.
Vaccine ; 42(9): 2212-2219, 2024 Apr 02.
Article in English | MEDLINE | ID: mdl-38350771

ABSTRACT

Acute Disseminated Encephalomyelitis (ADEM) and Transverse Myelitis (TM) are within the group of immune mediated disorders of acquired demyelinating syndromes. Both have been described in temporal association following various vaccinations in case reports and case series and have been evaluated in observational studies. A recent analysis conducted by The Global Vaccine Data Network (GVDN) observed an excess of ADEM and TM cases following the adenoviral vectored ChAdOx1 nCoV-19 (AZD1222) and mRNA-1273 vaccines, compared with historically expected background rates from prior to the pandemic. Further epidemiologic studies were recommended to explore these potential associations. We utilized an Australian vaccine datalink, Vaccine Safety Health-Link (VSHL), to perform a self-controlled case series analysis for this purpose. VSHL was selected for this analysis as while VSHL data are utilised for GVDN association studies, they were not included in the GVDN observed expected analyses. The VSHL dataset contains vaccination records sourced from the Australian Immunisation Register, and hospital admission records from the Victorian Admitted Episodes Dataset for 6.7 million people. These datasets were used to determine the relative incidence (RI) of G040 (ADEM) and G373 (TM) ICD-10-AM coded admissions in the 42-day risk window following COVID-19 vaccinations as compared to control periods either side of the risk window. We observed associations between ChAdOx1 adenovirus vector COVID-19 vaccination and ADEM (all dose RI: 3.74 [95 %CI 1.02,13.70]) and TM (dose 1 RI: 2.49 [95 %CI: 1.07,5.79]) incident admissions. No associations were observed between mRNA COVID-19 vaccines and ADEM or TM. These findings translate to an extremely small absolute risk of ADEM (0.78 per million doses) and TM (1.82 per million doses) following vaccination; any potential risk of ADEM or TM should be weighed against the well-established protective benefits of vaccination against COVID-19 disease and its complications. This study demonstrates the value of the GVDN collaboration leveraging large population sizes to examine important vaccine safety questions regarding rare outcomes, as well as the value of linked population level datasets, such as VSHL, to rapidly explore associations that are identified.


Subject(s)
COVID-19 , Encephalomyelitis, Acute Disseminated , Myelitis, Transverse , Vaccines , Humans , Australia/epidemiology , ChAdOx1 nCoV-19 , COVID-19/epidemiology , COVID-19/prevention & control , COVID-19 Vaccines/adverse effects , Encephalomyelitis, Acute Disseminated/chemically induced , Encephalomyelitis, Acute Disseminated/epidemiology , Myelitis, Transverse/etiology , Myelitis, Transverse/complications , Vaccination/adverse effects
6.
Spinal Cord Ser Cases ; 10(1): 4, 2024 Feb 10.
Article in English | MEDLINE | ID: mdl-38341409

ABSTRACT

INTRODUCTION: Transverse Myelitis is a rare inflammatory disorder of the spinal cord, characterized by the inflammation of the myelin sheath covering nerve fibers. Although rare, Transverse Myelitis holds significant clinical importance due to its potential life-altering consequences. The case report provides insight into the clinical presentation of Transverse Myelitis and the importance of Magnetic Resonance Imaging in confirming Transverse Myelitis. CASE PRESENTATION: A 27-year-old Nigerian female presented to a hospital facility after 2 months onset of paraplegia, urinary, and fecal incontinence. She was diagnosed with Acute Transverse Myelitis with Magnetic Resonance Imaging, a lacking imaging modality in Nigeria. On presentation, it was important to rule out spinal cord compression, a close differential to her presentation. Despite her late arrival at the facility, early diagnosis and prompt initiation of treatment with high-dose intravenous steroids and physiotherapy improved her quality of life. DISCUSSION: This case report reveals the poor health-seeking behavior in developing countries and the need for imaging modalities like Magnetic Resonance Imaging for improved diagnoses of rare neurological conditions such as Transverse Myelitis. The lack of healthcare infrastructure has led to clinical misdiagnosis, patient mismanagement, and underrepresentation of data in the country, underscoring the critical role of diagnostic tools for improved patient care pre-treatment and post-treatment. Additionally, follow-up of these patients is important to prevent the long-term sequelae of Transverse Myelitis like Neuromyelitis Optica or Multiple Sclerosis.


Subject(s)
Myelitis, Transverse , Neuromyelitis Optica , Humans , Female , Adult , Myelitis, Transverse/diagnostic imaging , Quality of Life , Neuromyelitis Optica/diagnosis , Neuromyelitis Optica/drug therapy , Neuromyelitis Optica/pathology , Magnetic Resonance Imaging/methods
7.
J Med Case Rep ; 18(1): 42, 2024 Feb 04.
Article in English | MEDLINE | ID: mdl-38310259

ABSTRACT

BACKGROUND: Many patients with transverse myelitis suffer from sensory loss below the spinal level of the lesion. This is commonly associated with chronic neuropathic pain. However, the presence of somatic pain below a complete thoracic sensory level after transverse myelitis is exceptionally rare, and it is unclear if surgical decompression is an effective form of treatment for these patients. CASE PRESENTATION: In this report, we describe a 22-year-old Caucasian female who suffered from chronic lumbar back pain despite a complete thoracic sensory level secondary to prior transverse myelitis. Imaging demonstrated multilevel central stenosis below the sensory level, and her pain improved after surgical decompression. To our knowledge, this is the first reported case of symptomatic lumbar stenosis below a sensory level after transverse myelitis successfully treated with surgical decompression. CONCLUSION: This is the first reported case of a patient with symptomatic lumbar stenosis after transverse myelitis whose lower back pain and quality of life improved following surgical decompression and fusion. This case provides evidence that typical lumbago is possible in patients with sensory loss from transverse myelitis, and standard lumbar decompression may provide benefit for these patients.


Subject(s)
Low Back Pain , Myelitis, Transverse , Spinal Fusion , Spinal Stenosis , Humans , Female , Young Adult , Adult , Spinal Stenosis/complications , Spinal Stenosis/diagnostic imaging , Spinal Stenosis/surgery , Constriction, Pathologic/complications , Myelitis, Transverse/complications , Myelitis, Transverse/diagnostic imaging , Myelitis, Transverse/surgery , Quality of Life , Back Pain/etiology , Back Pain/surgery , Low Back Pain/etiology , Decompression, Surgical/methods , Lumbar Vertebrae/diagnostic imaging , Lumbar Vertebrae/surgery , Spinal Fusion/methods , Treatment Outcome
8.
J Neurol ; 271(5): 2840-2843, 2024 May.
Article in English | MEDLINE | ID: mdl-38280000

ABSTRACT

BACKGROUND: Live cell-based assay (LCBA) is the gold standard for MOG-IgG detection, and fixed CBA (FCBA) is a widely used commercial alternative. Recent criteria attributed a diagnostic value to MOG-IgG titration with both LCBA and FCBA, with low-titre samples requiring additional supporting features for MOGAD diagnosis. However, FCBA titration is not validated. We aimed to assess the impact of the criteria-based MOG-IgG testing in MOGAD diagnosis. METHODS: Thirty-eight serum samples of LCBA MOG-IgG1-positive MOGAD patients were titred on MOG-IgG LCBA and FCBA, and the presence of supporting features for MOGAD assessed. MOGAD criteria were evaluated in four testing scenarios: (a) FCBA without titration; (b) FCBA with titration; c) LCBA without titration; (d) LCBA with titration. RESULTS: FCBA without titration failed to reach MOGAD diagnosis in 11/38 patients (28.9%, negative results in 5, lack of supporting features in 6). Patients with unconfirmed diagnosis had optic neuritis (ON, n = 8), or transverse myelitis (TM, n = 3). FCBA with titration allowed MOGAD diagnosis in 4 additional patients. Correlation between LCBA and FCBA titres was moderate (Spearman's rho 0.6, p < 0.001). CONCLUSIONS: FCBA yields high rate of misdiagnosis mainly due a lower analytical sensitivity. FCBA titration provides a moderate diagnostic advantage in FCBA positive patients.


Subject(s)
Immunoglobulin G , Myelin-Oligodendrocyte Glycoprotein , Humans , Myelin-Oligodendrocyte Glycoprotein/immunology , Immunoglobulin G/blood , Female , Male , Middle Aged , Adult , Autoantibodies/blood , Optic Neuritis/diagnosis , Optic Neuritis/blood , Optic Neuritis/immunology , Aged , Myelitis, Transverse/diagnosis , Myelitis, Transverse/blood
9.
Mult Scler ; 30(3): 345-356, 2024 Mar.
Article in English | MEDLINE | ID: mdl-38258822

ABSTRACT

BACKGROUND: Isolated first episodes of longitudinally extensive transverse myelitis (LETM) have typically been associated with neuromyelitis optica spectrum disorder (NMOSD) or myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD). However, in some cases, serological testing and screening for other aetiologies are negative, a condition referred to as double seronegative longitudinally extensive transverse myelitis (dsLETM). OBJECTIVE: The objective of this study was to evaluate comparative outcomes of dsLETM, MOGAD-LETM and NMOSD-LETM. METHODS: Cohort study of LETM cases seen in the UK NMOSD Highly Specialised Service between January 2008 and March 2022. RESULTS: LETM = 87 cases were identified (median onset age = 46 years (15-85); median follow-up = 46 months (1-144); 47% NMOSD-LETM = 41 (aquaporin-4 antibodies (AQP4-IgG) positive = 36), 20% MOGAD-LETM = 17 and 33% dsLETM = 29). Despite similar Expanded Disability Status Scale (EDSS) at nadir, last EDSS was higher in AQP4-IgG and seronegative NMOSD-LETM (sNMOSD) (p = 0.006). Relapses were less common in dsLETM compared to AQP4-IgG NMOSD-LETM and sNMOSD-LETM (19% vs 60% vs 100%; p = 0.001). Poor prognosis could be predicted by AQP4-IgG (odds ratio (OR) = 38.86 (95% confidence interval (CI) = 1.36-1112.86); p = 0.03) and EDSS 3 months after onset (OR = 65.85 (95% CI = 3.65-1188.60); p = 0.005). CONCLUSION: dsLETM remains clinically challenging and difficult to classify with existing nosological terminology. Despite a similar EDSS at nadir, patients with dsLETM relapsed less and had a better long-term prognosis than NMOSD-LETM.


Subject(s)
Myelitis, Transverse , Neuromyelitis Optica , Humans , Middle Aged , Cohort Studies , Aquaporin 4 , Neoplasm Recurrence, Local/complications , Prognosis , Autoantibodies , Immunoglobulin G , Myelin-Oligodendrocyte Glycoprotein , Retrospective Studies
11.
Neurol Sci ; 45(3): 1299-1301, 2024 Mar.
Article in English | MEDLINE | ID: mdl-37848777

ABSTRACT

In the COVID-era, other viral pathogens, like influenza B, gain less attention in scientific reporting. However, influenza still is endemic, and rarely affects central nervous system (CNS). Here, we report the case of a 35-year-old male who presented with fever since 1 week, and developed acute ascending flaccid paralysis and urinary retention. The clinical presentation of paraparesis in combination with the inflammation proven by the lumbar puncture, and the MRI full spine, fulfilled the diagnostic criteria of longitudinally extensive transverse myelitis (LETM). In this case, it is most likely based on a post-viral Influenza type B. Additionally, the brain MRI showed a necrotizing encephalopathy bilaterally in the thalamus. Both locations of inflammatory disease were part of one auto-immune-mediated, monophasic CNS disorder: influenza-induced ADEM which is very unique, fortunately with favorable outcome.


Subject(s)
Encephalomyelitis, Acute Disseminated , Influenza, Human , Myelitis, Transverse , Male , Humans , Adult , Myelitis, Transverse/diagnostic imaging , Myelitis, Transverse/etiology , Encephalomyelitis, Acute Disseminated/complications , Encephalomyelitis, Acute Disseminated/diagnostic imaging , Influenza, Human/complications , Influenza, Human/diagnostic imaging , Central Nervous System , Spinal Puncture
12.
Childs Nerv Syst ; 40(1): 227-232, 2024 Jan.
Article in English | MEDLINE | ID: mdl-37737897

ABSTRACT

We present the case of a previously healthy 13-year-old boy who was admitted to the emergency department with acute flaccid paralysis. Magnetic resonance imaging revealed radiological evidence of longitudinally extensive transverse myelitis. Additionally, homogeneous T2 signal increase was observed in the pons and medulla oblongata, initially indicating brainstem encephalitis. Subsequent evaluations confirmed a coexistence of diffuse midline glioma (DMG) in the brain stem alongside acute transverse myelitis (ATM). Children with ATM generally have a more favorable prognosis than adults. However, despite the implementation of advanced treatment methods, the patient's quadriplegia did not improve and resulted in spinal cord sequela atrophy. DMG exhibits an aggressive growth pattern and lacks a known curative treatment. This case represents an exceedingly rare synchronous occurrence of aggressive conditions, underscoring the importance of raising awareness among physicians. Furthermore, we aim to discuss the radiologic differential diagnosis, as this is the first documented instance in the literature.


Subject(s)
Encephalitis , Glioma , Myelitis, Transverse , Male , Adult , Child , Humans , Adolescent , Myelitis, Transverse/complications , Myelitis, Transverse/diagnostic imaging , Brain Stem/diagnostic imaging , Brain Stem/pathology , Glioma/complications , Glioma/diagnostic imaging , Glioma/pathology , Magnetic Resonance Imaging
14.
Ther Apher Dial ; 28(1): 119-124, 2024 Feb.
Article in English | MEDLINE | ID: mdl-37646345

ABSTRACT

INTRODUCTION: Acute disseminated encephalomyelitis is a rare acute demyelinating disease of the central nervous system (CNS). The pathogenesis remains unclear but is suspected to be autoimmune. High doses of methylprednisolone (HDMP) are currently considered standard of treatment. Plasmapheresis (PE) is typically given in steroid refractory cases. There is currently limited evidence supporting its use in ADEM. MATERIALS AND METHODS: We report a 16-year-old girl with ADEM who improved rapidly after initiating PE. RESULTS: The patient presented with acute onset of multifocal CNS symptoms, including encephalopathy, requiring intensive care unit management. Despite HDMP administration, her clinical condition continued to deteriorate. PE was therefore initiated on the same day as HDMP. Her clinical condition improved significantly following the first session. She was extubated and discharged from the intensive care unit the following day. CONCLUSION: HDMP combined with PE may be an effective first-line treatment in patients with fulminant ADEM.


Subject(s)
Encephalomyelitis, Acute Disseminated , Myelitis, Transverse , Humans , Female , Adolescent , Encephalomyelitis, Acute Disseminated/therapy , Encephalomyelitis, Acute Disseminated/diagnosis , Encephalomyelitis, Acute Disseminated/etiology , Myelitis, Transverse/therapy , Myelitis, Transverse/complications , Plasmapheresis , Methylprednisolone/therapeutic use , Intensive Care Units , Magnetic Resonance Imaging
15.
Pediatr Neurol ; 150: 63-64, 2024 Jan.
Article in English | MEDLINE | ID: mdl-37981445

ABSTRACT

Myelitis is a rare inflammatory myelopathy, and known associated etiologies only account for a small number of causes. A significant percentage of cases have an unknown etiology and are considered idiopathic. With 64% to 68% of cases fitting into the idiopathic category, helminth infections, and specifically pinworm parainfections, should be considered in cases that would otherwise be classified as idiopathic. This case report outlines a pediatric patient diagnosed with myelitis given her progressive weakness, fussiness, refusal to bear weight as well as magnetic resonance imaging (MRI) demonstrating T2-hyperintense signal and/or T1 gadolinium enhancement, and/or positive cerebrospinal fluid (CSF) inflammatory markers. This patient had a negative evaluation for typical known etiologies for myelitis including no signs of multiple sclerosis and neuromyelitis optica spectrum disorder on brain MRI, oligoclonal banding and aquaporin-4 autoantibodies, and no evidence of bacterial or viral meningitis given normal cell counts and cultures in CSF. She was found to have a pinworm infection, suggesting a parasitic parainfectious etiology of her myelitis. This case outlines the first case noting the correlation between myelitis and pinworm infection in a pediatric patient.


Subject(s)
Enterobiasis , Myelitis, Transverse , Myelitis , Neuromyelitis Optica , Female , Animals , Humans , Child , Myelitis, Transverse/diagnostic imaging , Myelitis, Transverse/etiology , Enterobius , Enterobiasis/complications , Contrast Media , Gadolinium , Myelitis/complications , Myelitis/diagnostic imaging , Magnetic Resonance Imaging/methods , Autoantibodies/cerebrospinal fluid , Aquaporin 4
16.
Mult Scler Relat Disord ; 81: 105130, 2024 Jan.
Article in English | MEDLINE | ID: mdl-37979410

ABSTRACT

BACKGROUND: Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) is a newly described clinical entity comprised of isolated or recurrent attacks of optic neuritis, transverse myelitis, acute disseminated encephalomyelitis (ADEM), encephalitis, or seronegative NMOSD. Prior studies report that 30-80 % of children and adults with MOGAD go on to have relapses though there are no reliable predictors. The objectives of this study were to (1) describe the demographic, clinical, and radiographic patterns of MOGAD at our center and (2) identify possible predictors of relapsing disease. METHODS: Single-center retrospective cohort study of pediatric and adult subjects with MOGAD evaluated at least once at our center between January 1, 2017 and September 30, 2022. Eligible subjects had a history of positive MOG-IgG and consistent clinical syndrome comprised of an initial attack of optic neuritis (ON), transverse myelitis (TM), ADEM, cerebral cortical encephalitis, seronegative neuromyelitis optica (simultaneous ON and TM), isolated brainstem or cerebellar syndrome, or other (not fitting into another group). Relapsing subjects or those remaining monophasic at 12 months were included in the analyses of predictors of relapsing disease. Covariates included age, sex, race/ethnicity, and index event phenotype. Unadjusted and adjusted risk ratios were calculated for pediatric and adult subjects. RESULTS: We describe the demographic, clinical, and radiographic characteristics of 58 subjects with MOGAD. Covariates from 48 subjects were analyzed for predictors of relapsing disease. In adults, Hispanics and non-White non-Hispanics were at increased risk of relapsing disease compared to non-Hispanic Whites [Adjusted RR 1.52 (95 % CI: 1.01, 2.30)]. There were no significant associations in the pediatric group. CONCLUSION: This study is the first to describe a cohort of MOGAD in the Pacific Northwest. Our findings highlight racial and ethnic differences in risk of relapsing MOGAD in adults. Further studies on racial and ethnic differences in MOGAD are needed to confirm these findings.


Subject(s)
Encephalitis , Myelitis, Transverse , Neuromyelitis Optica , Optic Neuritis , Adult , Humans , Child , Myelin-Oligodendrocyte Glycoprotein , Retrospective Studies , Myelitis, Transverse/diagnostic imaging , Myelitis, Transverse/epidemiology , Neoplasm Recurrence, Local , Optic Neuritis/diagnostic imaging , Optic Neuritis/epidemiology , Northwestern United States , Autoantibodies , Aquaporin 4
17.
Curr Rheumatol Rev ; 20(2): 208-212, 2024.
Article in English | MEDLINE | ID: mdl-37861018

ABSTRACT

BACKGROUND: Transverse myelitis is considered one of the cardinal features of neuromyelitis optica spectrum disorder (NMOSD), an immune-mediated inflammatory condition of the CNS characterized by severe, immune-mediated demyelination and axonal damage predominantly targeting optic nerves and spinal cord. We describe a case in which a diagnosis of NMOSD was established, associated with West Nile Virus (WNV) infection. CASE PRESENTATION: A healthy 18-year-old female presented with intractable hiccups and rapidly progressing paraparesis. MRI demonstrated T2 edema extending from the medulla to the conus, consistent with longitudinally extensive transverse myelitis. Serum and CSF Aquaporin-4 IgG (AQP4) were both positive with high titers. In conjunction with antiviral therapy, immunomodulatory treatment was initiated using pulse methylprednisolone, plasmapheresis and Rituximab. A month and a half after admission, the patient was fully ambulatory with no residual symptoms. On her rheumatology follow-up visit, West Nile Virus-specific IgM in CSF was found to be positive from the patient's initial presentation. CONCLUSION: We propose that West Nile Virus may have been the autoimmune trigger to the patient's development of NMOSD, highlighting the importance of evaluating viral triggers in autoimmune diseases.


Subject(s)
Myelitis, Transverse , Neuromyelitis Optica , Female , Humans , Adolescent , Myelitis, Transverse/complications , Autoantibodies , Neuromyelitis Optica/complications , Aquaporin 4 , Rituximab
18.
JAAPA ; 37(1): 1-3, 2024 Jan 01.
Article in English | MEDLINE | ID: mdl-38128141

ABSTRACT

ABSTRACT: Acute transverse myelitis is a rare condition that can follow a viral infection. At least 43 cases of COVID-19 associated with acute transverse myelitis have been presented in the literature. This case review highlights one such case in a young man. Although rare, acute transverse myelitis following COVID-19 can cause significant disability for patients. Clinicians must be equipped and encouraged to report and study cases of neurologic complications following COVID-19 infection to develop further treatment and cure.


Subject(s)
COVID-19 , Myelitis, Transverse , Humans , Male , COVID-19/complications , Myelitis, Transverse/diagnosis , Myelitis, Transverse/etiology , Adult
19.
Am J Case Rep ; 24: e941808, 2023 Nov 07.
Article in English | MEDLINE | ID: mdl-37933098

ABSTRACT

BACKGROUND Neuromyelitis optica spectrum disorder (NMOSD) is a demyelinating disease of the central nervous system that includes the triad of transverse myelitis, optic neuritis, and area postrema syndrome (APS), characterized by intractable nausea and vomiting. NMOSD can be part of a paraneoplastic syndrome and is associated with seropositivity to aquaporin-4 (AQP-4). We present a patient with uncontrollable nausea and vomiting who developed herpes zoster and acute myelitis and was finally diagnosed with paraneoplastic NMOSD due to breast cancer. CASE REPORT A 51-year-old woman was hospitalized due to 2 weeks of intractable nausea and vomiting. Although contrast-enhanced thoracoabdominal computed tomography (CT) on day 4 suggested breast cancer in her left breast, the etiology of her symptoms remained unknown. On day 13, she developed herpes zoster, followed by acute myelitis on day 25. Magnetic resonance imaging (MRI) showing longitudinal extensive transverse myelitis and an elevated serum AQP-4 antibody level led to the diagnosis of NMOSD. Brain MRI detected a small lesion in the dorsal medulla oblongata, which explained the preceding APS. After starting intravenous methylprednisolone pulse therapy, her nausea and vomiting rapidly subsided. Breast cancer was resected on day 63, and immunohistochemical staining revealed overexpression of AQP-4 in the tumor cells, suggesting paraneoplastic NMOSD. CONCLUSIONS This report has highlighted the presentation and diagnosis of NMOSD and supports the possibility that this can present as part of a paraneoplastic syndrome. In addition, diagnosis of NMOSD preceded by APS requires meticulous history taking and careful interpretation of MRI in the dorsal medulla oblongata.


Subject(s)
Breast Neoplasms , Herpes Zoster , Myelitis, Transverse , Neuromyelitis Optica , Female , Humans , Middle Aged , Neuromyelitis Optica/diagnosis , Breast Neoplasms/complications , Aquaporin 4 , Nausea/etiology , Vomiting/etiology , Autoantibodies
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