Longitudinally Extensive Transverse Myelitis Research Articles

Neurological Involvement in a Portuguese Cohort of IgG4-Related Disease.

Neurological involvement in immunoglobulin G4-related disease (IgG4-RD) is increasingly recognized. Its diagnosis can be challenging due to clinical mimics and difficulty in obtaining nervous system biopsies. The aim of this study was to describe a cohort of neurological IgG4-RD patients. Patients were recruited from a neuroimmunology tertiary center. Clinical, laboratory, neuroimaging and histological data were reviewed. Fifteen patients (60% women), with a median age of 53 years (48.5 - 65.0) were included: 13 (86.7%) classified as possible IgG4-RD, one (6.7%) as probable and one (6.7%) as definitive. The most common neurological phenotypes were meningoencephalitis (26.7%), orbital pseudotumor (13.3%), cranial neuropathies (13.3%), peripheral neuropathy (13.3%), and longitudinally extensive transverse myelitis (LTEM) (13.3%). Median serum IgG4 concentration was 191.5 (145.0 - 212.0) mg/dL. Seven in 14 patients had CSF pleocytosis (50.0%) and oligoclonal bands restricted to the intrathecal compartment, while most cases presented elevated CSF proteins (64.3%). Magnetic resonance imaging abnormalities included white matter lesions in four (26.7%), hypertrophic pachymeningitis in two (13.3%), and LETM in two (13.3%). Two patients had biopsy-proven IgG4-RD in extra-neurological sites. This study highlights the phenotypical variability of the neurological IgG4-RD. Biopsy inaccessibility reinforces the importance of new criteria for the diagnosis of this subset of patients.

Parainfectious Hemorrhagic Longitudinally-extensive Transverse Myelitis Secondary to COVID-19 (P1-13.010)

Parainfectious Hemorrhagic Longitudinally-extensive Transverse Myelitis Secondary to COVID-19 (P1-13.010)

Myelitis associated with COVID-19: clinical, radiological, and laboratory characteristics

Aim: The current study aimed to describe various types of myelitis associated with a novel coronavirus infection [coronavirus disease 2019 (COVID-19)] as well as to analyze cytokine profiles and cerebrospinal fluid (CSF) parameters in affected patients and to compare them to patients with other immune-mediated disorders—multiple sclerosis (MS), in order to identify possible common pathogenetic pathways and consequently treatment targets. Methods: Clinical, radiological, and laboratory characteristics were studied based on patients’ history. CSF from patients with myelitis associated with COVID-19 (11 patients) was compared with CSF of healthy controls (HC) (7 patients) and patients with MS (37 patients) from the non-COVID era. CSF cytological examination, protein levels and oligoclonal bands (OCBs) evaluation, severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) virus detection and cytokine profiling using Bio-Plex Pro Human Inflammation Panel 1, 37-Plex were performed. Results: In total 11 patients with different types of myelitis developed up to 3 months after COVID-19 were enrolled in the study. Radiological findings were diverse: short transverse myelitis (lesion of fewer than 3 segments) (n = 6), longitudinal extensive transverse myelitis (LETM) (n = 2), multifocal spinal cord lesions (n = 1), and myelitis involving dorsal and lateral columns (n = 2). The most pronounced response to treatment was observed in patients with partial transverse myelitis and patients with anti-myelin oligodendrocyte glycoprotein (MOG) antibodies (MOG Abs). Multiple comparisons have demonstrated decreased levels of interleukin-10 (IL-10), interferon-α2 (IFN-α2), IFN-β, and thymic stromal lymphopoietin (TSLP), and increased IL-19 and B cell activating factor (BAFF) in patients with COVID-19 myelitis (CM) compared to the MS group. The highest BAFF and a proliferation-inducing ligand (APRIL) concentrations were found in patients with the most profound neurological disability. Conclusions: Myelitis associated with COVID-19 is clinically and radiologically heterogeneous. Evaluation of cytokine profiles in patients with myelitis associated with COVID-19 revealed their relative similarity with ones of MS patients, except for a few cytokines. BAFF/APRIL system as well as IL-10 is well-known for the role in the development and progression of autoimmune diseases, however, their links with COVID-19 and effects on the development of immune-mediated central nervous system (CNS) disorders after SARS-CoV-2 remain to be further studied.

Neuromyelitis Optica Spectrum Disorder (NMOSD) or Chronic-Relapsing Inflammatory Optic Neuropathy (CRION)?: A Challenge in Diagnosis and Treatment

Abstract
 Introduction : Neuromyelitis optica spectrum disorder (NMOSD) is a chronic inflammatory, immune-mediated central nervous system disease targeting the AQP4-water channel membrane protein of the optic nerve and the spinal cord. This case report aims to present a case of seroconverting NMOSD which was initially diagnosed as chronic relapsing inflammatory optic neuropathy (CRION).
 Case Illustration : 15-year-old female with recurrent, steroid-dependent optic neuritis of the LE. During the first attack, brain MRI showed optic nerve inflammation with normal brain findings. Initial test for AQP4-IgG, MOG-Ab and ANA profile were negative. The diagnosis of CRION was made. Treatment with corticosteroid and immunosuppressant agent was maintained for one year. After treatment cessation, however, LE optic neuritis recurred with bilateral lower limb weakness. Spinal MRI showed signs of longitudinal extensive transverse myelitis. Re-testing for AQP4-IgG showed positive result. Diagnosis of NMOSD was made, and the patient was treated with high-dose intravenous corticosteroid followed by oral dose, and immunosuppressant. Final visual acuity of the LE was 1.0, with minimal residual visual field defect and good motor function.
 Discussion : During the initial attack, the patient did not meet the 2015 NMOSD international consensus diagnostic criteria. The AQP4-IgG was negative and no other corresponding signs and symptoms. The criteria was met one year after: optic neuritis, LETM and positive seroconversion of AQP4-IgG.
 Conclusion : Diagnosing NMOSD is at times challenging especially during initial attack. Retesting of the AQP4- IgG is at times necessary in seronegative patients, especially in children showing ADEM-like event with optic neuritis and LETM to achieve diagnosis and proper treatment strategy.

COVID-19 Associated Longitudinal Extensive Transverse Myelitis with Cerebral Spinal Fluid SARS-CoV-2 Detection

There have been multiple reports of COVID-19 associated neurological symp-toms, including several cases of para-infectious myelitis.We report a case of an 84-year-old male patient presenting with acute paraparesis coinciding with active mild COVID-19 pneumonia. Spinal cord magnetic resonance imaging (MRI) showed longitudinally extensive transverse myelitis (LETM) affecting the entire cervical cord to the tenth thoracic level, and cerebral spinal fluid (CSF) was positive for SARS-CoV-2. The patient was evaluated for other causes of LETM, which were convincingly excluded. He was treated with steroid pulses and plasmapheresis, with significant imaging improvement. The patient has since made a good recovery, being now able to walk unassisted. Although several instances of COVID-19 associated myelitis have been reported, positive identification of the virus in CSF is rare. The identification of viral material may support the hypothesis of direct spinal cord infection in co-occurrence with a para-infectious inflammatory reaction.

The absence of antibodies in longitudinally extensive transverse myelitis may predict a more favourable prognosis

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.

Longitudinally extensive transverse myelitis, a disabling disorder with a good prognosis: a case series from Nepal.

Longitudinally extensive transverse myelitis (LETM) is a rare spinal cord disorder with variable etiologies and presentations. It can present solely or as an association with other neurological disorders. It was a series of cases of LETM in a tertiary care hospital. The initial three cases presented with bilateral lower extremity weakness and were diagnosed as transverse myelitis while, the fourth case, already diagnosed as LETM presented with seizure followed by loss of consciousness. All four cases had a good prognosis to date with continued physiotherapy. The early diagnosis of the disease helps to guide the optimal management and decide the potential need for physiotherapy.

Aquaporin-4-IgG positive neuromyelitis optica spectrum disorder in a paraneoplastic context

ObjectiveThe association between aquaporin-4-immunoglobulin-G-positive neuromyelitis optica spectrum disorder (AQP4-IgG-NMOSD) and cancer via a plausible immunological response has been reported. Here, we investigated the frequency of cancer in a large cohort of patients with AQP4-IgG-NMOSD. MethodsBetween May 2005 and January 2023, patients with AQP4-IgG-NMOSD and a history of cancer were included by searching for diagnostic codes of both NMOSD and cancer in the electronic medical records and/or reviewing the database of the National Cancer Center registry of inflammatory diseases of the central nervous system. Probable paraneoplastic AQP4-IgG-NMOSD was defined according to the 2021 Criteria for Paraneoplastic Neurological Syndrome. ResultsOf 371 patients with AQP4-IgG-NMOSD, 23 (6.2%) had a history of cancer and four (1.1%) experienced NMOSD in a paraneoplastic context. Among the four patients with probable paraneoplastic AQP4-IgG-NMOSD, the types of cancer were lung (1 adenocarcinoma, 1 squamous cell carcinoma) and colorectal (2 adenocarcinomas). In three patients, the first NMOSD symptoms developed after a cancer diagnosis (median, 8 months [range, 4–23]), and one patient's symptoms preceded the cancer diagnosis (6 months). Compared to the 367 non-paraneoplastic patients, those in the paraneoplastic context had an older age at onset (median: 59.5 vs. 37 years, p = 0.012) and a higher proportion of longitudinally extensive transverse myelitis (LETM) as an initial manifestation (4/4[100%] vs. 130/367[35.4%], p = 0.017). ConclusionsIn a large cohort of patients with AQP4-IgG-NMOSD, the frequency of cancer was low. Older age, LETM features at onset, and adenocarcinoma as the histological type were usually observed in patients with AQP4-IgG-NMOSD in a paraneoplastic context.

Multisystem inflammatory syndrome in adult with longitudinally extensive transverse myelitis following SARS-CoV-2 vaccination: A case report

Multisystem inflammatory syndrome in adults (MIS-A) is a known complication arising after severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection or vaccination. However, longitudinal extensive transverse myelitis (LETM), which is a neurological manifestation of MIS-A, is rarely reported among individuals after SARS-CoV-2 infection or vaccination. Here, we present a case of a 38-year-old female, with a history of SARS-CoV-2 infection in May 2021 and subsequent SARS-CoV-2 vaccination (on November 1, 2021; COVISHIELD™, ChAdOx1 nCoV-19 Corona Virus Vaccine), who presented with acute flaccid quadriparesis with bowel and bladder involvement since December 26, 2021. While being treated in the hospital, she developed fever, rash, shock, myocarditis, breathlessness, jaundice, and acute kidney injury. Magnetic resonance Imaging revealed LETM. She was negative for SARS-CoV-2 infection and showed raised in SARS-CoV-2 antibody titer. Thus, she was diagnosed as having MIS-A with LETM and managed with Intravenous Immunoglobulin, antibiotics, hemodialysis, and steroids with improvement.

Post Varicella Neurological Complication: A Case of Transverse Myelitis

Longitudinal extensive transverse myelitis is defined as a spinal cord lesion that extends over three or more spinal segments. Varicella zoster virus is an exclusively human neurotropic virus that reactivates following the weaning of cell-mediated immunity as age advances, resulting in herpes zoster. We report the case of a 61-year lady presenting with dorsal cord longitudinally extending transverse myelitis following herpes zoster infection that resolved with immunomodulation and antivirals.

Clinical profile of longitudinally extensive transverse myelitis: A study in a tertiary care hospital in Western Rajasthan

As there are wide differentials for longitudinally extensive transverse myelitis (LETM), its aetiology should be found to optimise therapy.Clinical profile of LETM.ambispective observational study.73 patients with acute to subacute myelitis involving 3 or more vertebral segments were properly investigated.Student t test and Mann-whitney test used, and comparison of data done using SPSS version 22.0The etiology found were in form of neuromyelitis optica spectrum disorder (NMO-SD) in 18(24.6%), para-infectious in 13(17.8%), idiopathic in 17(23.2%), infectious in 9(12.3%), and rest were acute disseminated encephalomyelitis (ADEM), subacute combined degeneration (SACD), infarct, radiation myelitis etc.It is important to find etiology of LETM to optimise the therapy as the in inflammatory myelitis other than NMOSD, the line of management differs. Relapsing nature of some myelitides is an important aspect in management

The Coexisting Neuromyelitis Optica Spectrum Disorder and Systemic Lupus Erythematosus: A Therapeutic Challenge.

Neuromyelitis Optica (NMO), or Devic's disease, is an immune-mediated, usually relapsing, central nervous system (CNS) demyelination disorder associated with optic neuritis and transverse myelitis. It is characterised by the presence of longitudinally extensive transverse myelitis (LETM) and antibodies against water channel aquaporin-4 (AQP4-immunoglobulin G [IgG]). The term NMO spectrum disorder (NMOSD) includes patients with limited forms of NMO who are at risk of recurrence. Often patients with NMO or NMOSD have an associated systemic autoimmune disease, most commonly systemic lupus erythematosus (SLE) or Sjogren syndrome (SS) or a related profile of non-organ-specific autoantibodies. The intriguing aspect of coexisting NMOSD and SLE is whether they are independent diseases that can coexist with each other or the serological findings specific to both diseases in a patient is a non-specific finding of no prognostic or therapeutic concern. We have presented two cases of NMOSD coexisting with SLE and based upon the existing evidence in the literature we present that the two conditions are independent of each other, and, at times, it can throw a therapeutic challenge to any clinician.

The temporal relationship of paraneoplastic aquaporin-4-IgG seropositive neuromyelitis optica spectrum disorder (NMOSD) and breast cancer: a systematic review and meta-analysis.

Neuromyelitis optica spectrum disorder (NMOSD) is an autoimmune astrocytopathy with evidence of neuroinflammation and demyelination that affects the central nervous system and is mediated by aquaporin-4 (AQP4) immunoglobulin (IgG). AQP4-IgG may also be present in paraneoplastic syndromes secondary to malignancy such as breast cancer. A systematic review and meta-analysis of the literature were completed using PubMed, Scopus, and ScienceDirect databases (CRD42022352109). A total of 12 publications, which included 19 cases, met the inclusion criteria and were assessed in both the qualitative and quantitative synthesis. The mean age was 51.26 years (SD: 13.12, SEM: 3.01), and 100% of the cases were reported in women. Speech abnormalities and symptoms of myelopathy were the most observed neurological manifestations. MRI often revealed longitudinally extensive transverse myelitis (LETM) involving the cervical spine. Three of 19 (15.9%) cases were diagnosed with NMOSD and breast cancer within the same month. Five of 19 (26.1%) cases had a diagnosis of breast cancer preceding that of NMOSD. Eight of 19 (42.1%) cases were diagnosed with breast cancer after NMOSD. The median time of breast cancer diagnosis was 1.0 months (range 216 months) after NMOSD. The diagnosis of breast cancer most often occurs after the onset of the paraneoplastic NMOSD symptoms. However, a wide time range for the diagnosis of breast cancer was observed both before and after the onset of neurological symptoms. Older women with a new diagnosis of NMOSD should be considered for frequent breast cancer screening.

Cervical myelitis: a practical approach to its differential diagnosis on MR imaging.

Differential diagnosis of non-compressive cervical myelopathy encompasses a broad spectrum of inflammatory, infectious, vascular, neoplastic, neurodegenerative, and metabolic etiologies. Although the speed of symptom onset and clinical course seem to be specific for certain neurological diseases, lesion pattern on MR imaging is a key player to confirm diagnostic considerations. The differentiation between acute complete transverse myelitis and acute partial transverse myelitis makes it possible to distinguish between certain entities, with the latter often being the onset of multiple sclerosis. Typical medullary MRI lesion patterns include a) longitudinal extensive transverse myelitis, b) short-range ovoid and peripheral lesions, c) polio-like appearance with involvement of the anterior horns, and d) granulomatous nodular enhancement prototypes. Cerebrospinal fluid analysis, blood culture tests, and autoimmune antibody testing are crucial for the correct interpretation of imaging findings. The combination of neuroradiological features and neurological and laboratory findings including cerebrospinal fluid analysis improves diagnostic accuracy. · The differentiation of medullary lesion patterns, i. e., longitudinal extensive transverse, short ovoid and peripheral, polio-like, and granulomatous nodular, facilitates the diagnosis of myelitis.. · Discrimination of acute complete and acute partial transverse myelitis makes it possible to categorize different entities, with the latter frequently being the overture of multiple sclerosis (MS).. · Neuromyelitis optica spectrum disorders (NMOSD) may start as short transverse myelitis and should not be mistaken for MS.. · The combination of imaging features and neurological and laboratory findings including cerebrospinal fluid analysis improves diagnostic accuracy.. · Additional brain imaging is mandatory in suspected demyelinating, systemic autoimmune, infectious, paraneoplastic, and metabolic diseases..

Fahr's syndrome as a manifestation of autoimmune polyendocrine syndrome-1 and its unusual association with neuromyelitis optica spectrum disorder.

Fahr's syndrome, also known as bilateral striopallidodendate calcinosis, is a rare inherited neurodegenerative illness characterized by abnormal calcium deposition in several areas of the brain, resulting in a wide range of neuropsychological symptoms. Fahr's syndrome, secondary to autoimmune polyendocrine syndrome type 1, which includes adrenal insufficiency and mucocutaneous candidiasis in addition to hypoparathyroidism, is exceedingly rare. No case report has been documented to date to show the co-occurrence of Fahr's syndrome and neuromyelitis optica spectrum disorder. Here, we discuss the case of a 30-year-old man with a previous history of seizures and symptoms of ectodermal dystrophy presented with seizures, left-sided hemiparesis, dysarthria, and other characteristics indicative of severe hypocalcemia. The neuroimaging findings strongly suggested Fahr's syndrome, with radiographic evidence of Neuromyelitis optica spectrum disorder as longitudinal extensive transverse myelitis in the cervical spinal cord, high titers of serum aquaporin-4 antibodies, and demyelinating neuropathy on nerve conduction studies. This distinct neuropsychological presentation and neuroimaging findings led to the diagnosis of Fahr's syndrome as a result of hypoparathyroidism caused by autoimmune polyendocrine syndrome type 1 with cooccurrence of neuromyelitis optica spectrum disorder. The patient's clinical symptoms improved considerably after he was treated based on a provisional diagnosis. The clinical importance of our case is significant for both neuropsychiatrists and endocrinologists, as autoimmune polyendocrine syndrome should be considered as the etiology of Fahr's syndrome. This case report also aims to report this unusual association of Neuromyelitis optica spectrum disorder with Fahr's syndrome to give the future prospective to know whether this association is incidental or there is a missing link between these two different disorders.

Rhombencephalitis with longitudinal extensive transverse myelitis in lupus: an overlap syndrome?

A 47-year-old woman presented to the emergency department of our hospital in August, 2021, with a 2-day history of intractable nausea and vomiting (reminiscent of an area postrema syndrome) accompanied by fever and dizziness. She had previously been diagnosed with systemic lupus erythematosus (SLE) and was taking 400 mg hydroxychloroquine per day. Her last flare was an episode of class IV nephritis more than 10 years ago. On that occasion, she received pulsed methylprednisolone (1 g intravenously for 3 days) and mycophenolate mofetil (a maintenance dose of 2 g per day for 5 years).

A score that predicts aquaporin-4 IgG positivity in patients with longitudinally extensive transverse myelitis.

Longitudinally extensive transverse myelitis (LETM) associated with aquaporin-4 autoantibodies (AQP4-IgG) can cause severe disability. Early diagnosis and prompt treatment are critical to prevent relapses. A novel score is described based on clinical and neuroimaging characteristics that predicts AQP4-IgG positivity in patients with LETM. Patients were enrolled both retrospectively and prospectively from multiple Italian centers. Clinical and neuroimaging characteristics of AQP4-IgG positive and negative patients were compared through univariate and multivariate analysis. Sixty-six patients were included. Twenty-seven (41%) were AQP4-IgG positive and median age at onset was 45.5 years (range 19-81, interquartile range 24). Female sex (odds ratio [OR] 17.9, 95% confidence interval [CI] 2.6-381.9; p = 0.014), tonic spasms (OR 45.6, 95% CI 3.1-2197; p = 0.017) and lesion hypointensity on T1-weighted images (OR 52.9, 95% CI 6.8-1375; p = 0.002) were independently associated with AQP4-IgG positivity. The AQP4-IgG positivity in myelitis (AIM) score predicted AQP4-IgG positivity with 85% sensitivity and 95% specificity. Positive and negative likelihood ratios were 16.6 and 0.2 respectively. The inter-rater and intra-rater agreement in the score application were both excellent. The AIM score predicts AQP4-IgG positivity with good sensitivity and specificity in patients with a first episode of LETM. The score may assist clinicians in early diagnosis and treatment of AQP4-IgG positive LETM.

The absence of antibodies in longitudinally extensive transverse myelitis and the impact on prognosis (P9-5.029)

<h3>Objective:</h3> Evaluation of clinical outcomes in LETM. <h3>Background:</h3> Longitudinally extensive transverse myelitis (LETM) is typically defined as spinal cord inflammation that spans 3 or more vertebral segments on MRI. It is a prototypical manifestation of neuromyelitis optica spectrum disorder (NMOSD) but is also seen in myelin oligodendrocyte glycoprotein antibody disease (MOGAD) and much less commonly multiple sclerosis. In some cases, despite extensive investigation, a definitive diagnosis cannot be reached, a condition referred to as double seronegative LETM (dsLETM). <h3>Design/Methods:</h3> Cohort study of LETM cases reviewed in NMO UK Highly Specialised Service at the Walton Centre NHS Foundation Trust between Jan 2008-March 2022. Clinical presentation, radiological characteristics, aquaporin-4 antibodies (AQP4-IgG) and MOG antibodies, relapses, treatment, and EDSS at follow-up were extracted from case records. <h3>Results:</h3> LETM=93 cases were identified; NMOSD=41 (AQP4-IgG positive; 88%), dsLETM=29, MOGAD=17, others=6. The median onset age was 46 (15–85) yrs with a median follow-up of 46 (1–144) months. EDSS at nadir was similar between subgroups (NMOSD; 7, MOGAD; 7.5, dsLETM; 6.7, p&gt;0.05) but patients with dsLETM were less likely to relapse (dsLETM; 21% vs. NMOSD; 56% vs. MOGAD; 29%, p=0.008). Long term immunosuppression was used in NMOSD (100%), MOGAD (76%) and dsLETM (34%) and was associated with a reduction in median annualised relapse rate (NMOSD; 1.65 to 0.09, MOGAD; 0.75 to 0.07, and dsLETM; 1 to 0.11). Although EDSS scores at last review were higher in NMOSD than MOGAD (6 vs. 4; p=0.018) and dsLETM (6 vs 4.5; p=0.04), dsLETM patients still reached a significant disability (EDSS&gt;6) in 35% of cases. <h3>Conclusions:</h3> Patients with dsLETM relapsed less as compared to NMOSD and MOGAD and immunosuppression reduced the risk further. Although last EDSS was lower in dsLETM as compared to NMOSD, a third of patients still suffered from significant disability. <b>Disclosure:</b> Dr. Rocchi has nothing to disclose. Dr. Forcadela has nothing to disclose. Dr. Jacob has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for ALEXION. Dr. Hamid has nothing to disclose. Dr. Huda has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Guidepoint.

Rare Case of Neurosarcoidosis presenting as Isolated Longitudinal Extensive Transverse Myelitis (LETM). (P9-5.006)

<h3>Objective:</h3> NA <h3>Background:</h3> Neurosarcoidosis is seen in only 5–16% of the patients with sarcoidosis. Incidence of longitudinal extensive transverse myelitis (LETM) in neurosarcoidosis is even rarer. <h3>Design/Methods:</h3> 37-year-old male with history of recurrent LETM and seizure disorder was admitted for worsening lower extremity weakness and unresponsiveness. History was significant for transverse myelitis diagnosed 8 months ago after presenting with lower extremity weakness and urinary incontinence. MRI of the spine at that time revealed focus of abnormal T2 signal in upper thoracic spinal cord extending from the mid T3 level inferiorly through the T5–6 level, with associated cord expansion. <h3>Results:</h3> Repeat MRI of the spine and brain showed progression of disease in the spine with nodular leptomeningeal enhancement most prominent within the posterior fossa. Extensive workup including neuromyeltitis optica, paraneoplastic panel, anti-aquaporin Ab and Anti-MOG Ab testing was negative. Patient was treated with steroids and plasmapheresis treatment (PLEX). PLEX treatments were held due to concern for infection. He was switched to steroid taper and biweekly Rituximab infusion. Rituximab infusions were stopped due to side effects and patient was trialed on mycophenolate mofetil. However, patient was transitioned back to steroid taper and rituximab infusion after worsening of symptoms. Patient eventually underwent meningeal biopsy which showed granulomatous leptomeningitis and parenchymal microglial activation. With strong suspicion for Neurosarcoidosis now based on accumulated evidence the patient was restarted on IV methylprednisolone and subsequent steroid taper. Improvement of his symptoms with partial recovery of strength was noted. Patient was subsequently transitioned to Leflunomide, infliximab infusions and steroid taper. Follow up MRI of the brain and spinal cord showing improvement in areas of enhancement. <h3>Conclusions:</h3> Our patient presented with neurosarcoidosis manifesting as LETM with no prior diagnosis of sarcoidosis or evidence of system involvement on further thorough workup. The diagnosis was challenging confirmed after a neural biopsy given absence of non-neurological manifestations. <b>Disclosure:</b> Dr. Khosa has nothing to disclose. Dr. Anandaram has nothing to disclose. Dr. Khosa has nothing to disclose. Dr. Buttar has nothing to disclose.

Adult-Onset Acute Flaccid Myelitis with White Matter-predominant Longitudinally Extensive Transverse Myelitis Following Coxsackie B5 Enterovirus Infection: a case report (P8-10.001)

<h3>Objective:</h3> To describe an adult-onset case of transverse myelitis associated with enterovirus (Coxsackie B5) infection. <h3>Background:</h3> Both Acute Flaccid Myelitis (AFM) and longitudinally-extensive transverse myelitis (LETM) are more commonly associated with pediatric enteroviral infections than adult infections. However, we present an adult case of AFM due to Coxsackie B5 virus with an atypical pattern on spinal imaging. <h3>Design/Methods:</h3> N/A <h3>Results:</h3> A 55-year-old male presented with acute urinary retention and lower extremity flaccid paraplegia two weeks after gastrointestinal illness. Neurologic exam revealed flaccid paralysis of bilateral lower extremities with absent lower extremity reflexes, and a sensory level to pinprick at T5. Vibration was diminished up to the ankle and proprioception was absent at the toes bilaterally. MRI thoracic spine showed T2 hyperintensity of ventral and bilateral lateral columns spanning T4-T10 levels, with notable sparing of central gray matter. MRI brain revealed four periventricular and subcortical T2/FLAIR hyperintense lesions as well as one cortical lesion. Cerebrospinal Fluid (CSF) studies demonstrated 16 WBC, 63 Glucose, and 56 Protein with one unique oligoclonal band. CSF PCR panel was positive for Enterovirus Coxsackie B5. Of note spinal angiogram, NMO, MOG, other rheumatologic and infectious usual myelitis workup was negative. The patient was treated with two days of IV steroids, five days of IV immunoglobulin therapy (IVIG), and five PLEX sessions with minimal improvement. <h3>Conclusions:</h3> We describe an adult patient presenting with Coxsackie B5-associated AFM with LETM. This patient demonstrated atypical MRI findings of T2 hyperintensity of the ventral and bilateral lateral columns spanning T4-T10 with notable sparing of central gray matter along with abnormal EMG suggesting anterior horn cell involvement. This case report highlights the importance of consideration of enterovirus as an etiology of atypical AFM with LETM in the adult population. <b>Disclosure:</b> Ms. Manouvakhova has nothing to disclose. Dr. Moghavem has nothing to disclose. Dr. Pulst-Korenberg has nothing to disclose.