Showing papers on "Myelitis published in 2023"

Monkeypox encephalitis with transverse myelitis in a female patient

Abstract: The 2022 monkeypox outbreak has affected 110 countries worldwide, outside of classic endemic areas (ie, west Africa and central Africa). On July 23, 2022, the outbreak was classified by WHO as a public health emergency of international concern. Clinical presentation varies from mild to life-changing symptoms; neurological complications are relatively uncommon and there are few therapeutic interventions for monkeypox disease. In this Grand Round, we present a case of monkeypox with encephalitis complicated by transverse myelitis in a previously healthy woman aged 35 years who made an almost complete recovery from her neurological symptoms after treatment with tecovirimat, cidofovir, steroids, and plasma exchange. We describe neurological complications associated with orthopoxvirus infections and laboratory diagnosis, the radiological features in this case, and discuss treatment options.

Neurological consequences of SARS-CoV-2 infections in the pediatric population

Abstract: COVID-19 in the pediatric population is mostly asymptomatic. However, 1 out of 5 children presents non-specific neurologic symptoms such as headache, weakness, or myalgia. Furthermore, rarer forms of neurological diseases are increasingly being described in association to a SARS-CoV-2 infection. Encephalitis, stroke, cranial nerves impairment, Guillain-Barré syndrome or acute transverse myelitis have been reported and account for around 1% of pediatric COVID-19 cases. Some of these pathologies may occur during or after the SARS-CoV-2 infection. The pathophysiological mechanisms range from direct invasion of the central nervous system (CNS) by SARS-CoV-2 itself to postinfectious immune-mediated CNS inflammation. In most cases, patients presenting neurological pathologies related to SARS-CoV-2 infection are at greater risk of life-threatening complications and should be closely monitored. Further studies are needed to acknowledge the potential long-term neurodevelopmental consequences of the infection.

Transverse myelitis caused by herpes zoster following COVID-19 vaccination: A case report

Abstract: BACKGROUND Transverse myelitis (TM) is characterized by sudden lower extremity progressive weakness and sensory impairment, and most patients have a history of advanced viral infection symptoms. A variety of disorders can cause TM in association with viral or nonviral infection, vascular, neoplasia, collagen vascular, and iatrogenic, such as vaccination. Vaccination has become common through the global implementation against coronavirus disease 2019 (COVID-19) and reported complications like herpes zoster (HZ) activation has increased. CASE SUMMARY This is a 68-year-old woman who developed multiple pustules and scabs at the T6-T9 dermatome site 1 wk after vaccination with the COVID-19 vaccine (Oxford/AstraZeneca ([ChAdOx1S{recombinant}]). The patient had a paraplegia aggravation 3 wk after HZ symptoms started. Spinal magnetic resonance imaging (MRI) showed transverse myelitis at the T6–T9 Level. Treatment was acyclovir with steroids combined with physical therapy. Her neurological function was slowly restored by Day 17. CONCLUSION HZ developed after COVID-19 vaccination, which may lead to more severe complications. Therefore, HZ treatment itself should not be delayed. If neurological complications worsen after appropriate management, an immediate diagnostic procedure, such as magnetic resonance imaging and laboratory tests, will start and should treat the neurological complications.

Case report: A case of acute disseminated encephalomyelitis after SARS-CoV-2 infection in pediatric patients

Abstract: Introduction Since the beginning, there has been enough evidence about the multi-systematic involvement of the coronavirus disease 2019 (COVID-19), which is caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). Recent observations have revealed that, together with others, typical neurological manifestations are also associated with COVID-19 infection. In the first 2 years, children accounted for a few percent of cases, but with the emergence of the Omicron variant, the number of cases in the pediatric population has increased. It has been described that ~5% of the affected population suffered from severe neurological complications, such as seizure, coma, encephalitis, demyelinating disorders, and aseptic meningitis. Acute disseminated encephalomyelitis (ADEM) is an inflammatory demyelinating disease of the central nervous system. Typically, it presents in childhood and occurs 1 or 2 weeks after infection or vaccination. Case presentation We present the case of a 12-year-old boy who developed ADEM, 10 days after an asymptomatic SARS-CoV-2 infection. Neurological symptoms began with headache, fever, irritability, paraplegia, and loss of sensitivity from the T1 level. The diagnosis of ADEM was confirmed by the typical signs found on brain MRI, whereas spinal cord MRI showed signs of transverse myelitis. The cerebrospinal fluid (CSF) testing excluded infections and did not reveal oligoclonal antibody bands (anti-MOG-negative and anti-AQP-negative). High-dose steroids (30 mg/kg/day) and IVIG (2 g/kg) were administered to the patient without any clinical improvement. The patient received a cycle of plasma exchange therapy, followed by rituximab infusion, with partial improvement. After 3 months, the magnetic resonance imaging (MRI) results demonstrated radiological improvement in accordance with the ADEM diagnosis. Conclusion This clinical case confirms that SARS-CoV-2 infections are increasingly implicated in severe neurological consequences in both adult and pediatric patients. While the most frequent complications that were reported in children included headache, altered mental status, and encephalopathy, ~5% of the individuals suffered from severe neurological complications, leading to lifelong sequelae. All physicians must be aware of these data and detect neurological signs of severe (or not) complications that require a specific follow-up and treatment.

A Case of Transverse Myelitis After Moderna Severe Acute Respiratory Syndrome Coronavirus Vaccination

Abstract: Transverse myelitis (TM) is an inflammatory syndrome of the spinal cord that presents with acute-to-subacute neurological deficits. The differential for TM is broad and includes demyelinating, infectious, neoplastic and paraneoplastic, autoimmune, and metabolic/toxic etiologies. With the novel severe acute respiratory syndrome coronavirus pandemic, more commonly referred to as the coronavirus infectious disease of 2019 (COVID-19), there have been increasing reports of neurological complications. In this case report, we describe a novel case of longitudinally-extensive TM associated with the Moderna vaccination.

Anti-Argonaute antibodies as a potential biomarker in NMOSD

Abstract: Background and objectives Neuromyelitis optica spectrum disorders (NMOSDs) are a group of diseases mainly characterised by recurrent optic neuritis and/or myelitis. Most cases are associated with a pathogenic antibody against aquaporin-4 (AQP4-Ab), while some patients display autoantibodies targeting the myelin oligodendrocyte glycoprotein (myelin oligodendrocyte glycoprotein antibodies (MOG-Abs)). Anti-Argonaute antibodies (Ago-Abs) were first described in patients with rheumatological conditions and were recently reported as a potential biomarker in patients with neurological disorders. The aims of the study were to investigate if Ago-Abs can be detected in NMOSD and to evaluate its clinical usefulness. Methods Sera from patients prospectively referred to our centre with suspected NMOSD were tested for AQP4-Abs, MOG-Abs and Ago-Abs with cell-based assays. Results The cohort included 104 prospective patients: 43 AQP4-Abs-positive cases, 34 MOG-Abs positive cases and 27 double-negative patients. Ago-Abs were detected in 7 of 104 patients (6.7%). Clinical data were available for six of seven patients. The median age at onset of patients with Ago-Abs was 37.5 [IQR 28.8–50.8]; five of six patients tested positive also for AQP4-Abs. Clinical presentation at onset was transverse myelitis in five patients, while one presented with diencephalic syndrome and experienced a transverse myelitis during follow-up. One case presented a concomitant polyradiculopathy. Median EDSS score at onset was 7.5 [IQR 4.8–8.4]; median follow-up was 40.3 months [IQR 8.3–64.7], and median EDSS score at last evaluation was 4.25 [IQR 1.9–5.5]. Conclusion Ago-Abs are present in a subset of patients with NMOSD and, in some cases, represent the only biomarker of an autoimmune process. Their presence is associated with a myelitis phenotype and a severe disease course.

Familial Mediterranean Fever and Transverse Myelitis: A Causal Relation?

Abstract: Familial Mediterranean fever (FMF) is a rare autoinflammatory disorder characterized mainly by recurrent self-limited episodes of fever and polyserositis. FMF-related neurologic complication is an old debate, and the correlation between FMF and demyelinating disorders has been a matter of dispute for a long time. Few reports demonstrated a relationship between FMF and multiple sclerosis; however, the existence of a causal relationship between FMF and demyelinating disorders is still a puzzle. This report presents the first case of transverse myelitis following FMF attacks in which neurologic manifestations were resolved using colchicine treatment. Due to relapses of FMF, which were accompanied by transverse myelitis, rituximab was administered, which resulted in stabilizing disease activity. Accordingly, in the case of colchicine-resistant FMF and FMF-related demyelinating conditions, rituximab could be considered as a potential therapeutic option to alleviate both polyserositis and demyelinating manifestations.

A Case of Seronegative NMOSD-Related Myelitis Masquerading as VZV-Related Myelitis (P13-5.013)

Abstract:

Objective:

NA

Background:

Longitudinally extensive transverse myelitis (LETM) is caused by a myriad of etiologies. We present a case of Neuromyelitis Optica Spectrum Disorder (NMOSD)-related myelitis, initially seronegative, masquerading as VZV-related myelitis.

Design/Methods:

A case report.

Results:

A woman in her sixties reported a one-month history of bilateral lower extremity paresthesias and weakness. Days prior, she felt tingling on her back with eruption of a painful rash, and was diagnosed with varicella zoster (VZV). She had transient improvement, before worsening 2 months later with involvement of the arms and signs of myelopathy on exam. MR imaging revealed a long segment T2 hyperintense cord expansion extending from the medulla throughout the thoracic spine with additional hyperintensities in the conus medullaris with patchy enhancement. Work up was significant for lymphocytic-predominant CSF pleocytosis with elevated protein and IgG index. Serum VZV IgG was positive with negative IgM. CSF VZV PCR was negative. Aquaporin-4 antibody was negative. Testing for other causes of myelopathy was unrevealing. She was treated with IV Acyclovir, IV pulse steroids, and subsequently plasma exchange. She improved without further immunomodulation. Three months later, she had another attack and imaging showed new occipital enhancing lesion consistent with demyelination. At this visit, aquaporin-4 antibodies returned positive with a titer of 1:100. She received IV steroids and started rituximab. The patient was diagnosed with LETM from NMOSD. Due to symptom severity, she underwent plasma exchange despite a potential alternative etiology. VZV myelitis and her history of shingles were likely a red herring in the setting of her positive NMO testing. This emphasizes the importance of rechecking antibodies for high clinical suspicion, as the results can drastically influence management and outcome. In the presence of unconfirmed alternative etiologies, neurologists should have low threshold for aggressively treating inflammatory myelitis, using intravenous immunoglobulin or plasma exchange if indicated.

Conclusions:

NA Disclosure: Dr. Alshaer has nothing to disclose. Dr. Cavanagh has nothing to disclose. Dr. Antzoulatos has nothing to disclose.

Case Report Of Transverse Myelitis: Rare Neurological Complications Of Herpes Zoster Virus

Abstract: Introduction: Transverse myelitis is a rare disease that accured by incident 0,3%-1% Though it rare happened transverse myelitis deserve to diccus because the variant presentation that make clinical challenge to dianosis to pre-observation for upgrade’s prognosis. Case Report: we reported case of transverse myelitis that was neurology complication to rare herpes zoster virus. Woman 27 years old came by early complaint of tingling fever, pain, shiver continue on foot and the right hand. After got well, patient cameback to her disease bacause of the worst condition that cold not mover her left foot. Diagnosis transverse myelitis could stand through clinical sympton and magnetic resonance imaging (MRI) patient that came by her skin of hipopigment have to always considere the happening of transverse myelitis and lost control of defecate on neurologi observation being got paraplegi, monoparese extremitas superior, and hipoanesthesia dermatom C5 on low grade. We got observation of MRI to the level cervicothoracal that show long segment hipeintensity introdural intra medullary to the level craniovertebral junction so C5 and T2 so on that involved 2/3 spinal cord that strength described transverse myelitis. Discussion: Varicella zoster virus cou caused neurologis complication, the of transverse myelitis, of it is sign and sympton like pain, sencoric trouble, weakness on arm and foot, and the problem bladder and intestines. This symptom often occured unilaterally , Ipsilateral toward rash then become bilaterally. Intervention should the customize with pattient complaint: The implement combination asiklovir and high close steroid’s inject significantly relieve the pattient complaining but still occured paraplegi. Keywords: Transverse myelitis, HZV, neurological complication

Atypical course of schistosomal myelitis: a diagnostic challenge

Abstract: Acute myelitis is a severe neurological complication of schistosomiasis which clinical features present as lower limb weakness, sensory disturbance and intestinal or bladder dysfunction. A 60-year-old male farmer presented with low back pain and progressive paresis of right lower limb that worsened to plegia over ten months. MRI demonstrated hypersignal lesions in the T2/FLAIR (T2- weighted-Fluid-Attenuated Inversion Recovery) sequence in the ninth dorsal segment level. A Kato-Katz examination was negative for Schistosoma mansoni and a rectal biopsy confirmed an active infection. The patient received methylprednisolone (MTP) and a single oral dose of praziquantel (50 mg/kg), with partially improvement, being able to walk with assistance. Six months later, after coronavirus disease 2019 vaccine (Pfizer), he reported a worsening of neurological symptoms. New magnetic resonance imaging revealed no change compared to previous one. Aquaporin-4 antibodies were negative in serum, cerebrospinal fluid analysis showed 15 leukocytes (93% mononuclear), protein and glucose within the normal range. Autoimmune profile, serology for syphilis, hepatitis B, Hepatitis C, and human immunodeficiency virus were negative. The patient received a second pulse of MTP and repeat course of praziquantel, after which he improved his left lower limb weakness. Oral corticosteroid therapy was weaned. Nine months later, he experienced new exacerbation of crural paraparesis. Repeat cerebrospinal fluid analysis showed a normal pattern and new spine magnetic resonance imaging had no changes, but stools testing was positive for hookworm eggs, taenia sp and entamoeba coli in three different occasions. Therefore, he also received treatment with ivermectin and albendazole. The delay in diagnosis and treatment likely contributes to poor outcomes. As praziquantel is effective only on the mature adult worms but not on the larval form, a second dose should be considered in recurrent myelitis related to spinal neuroschistosomiasis.

Stealth invaders: unraveling the mystery of neurotropic viruses and their elusive presence in cerebrospinal fluid - a comprehensive review

Abstract: Neurotropic viruses are a threat to human populations due to ongoing zoonosis. A wide array of neurological manifestations can occur most often including parkinsonism, encephalitis/encephalopathy, flaccid myelitis, and Guillain-Barré syndrome. Neuroinvasion occurs through: transneural transmission, blood brain barrier (BBB) dysfunction, and 'trojan horse' mechanism or infected immune cell trafficking into the central nervous system (CNS). Transneural transmission occurs through virus mediated hijacking of intracellular transport proteins allowing retrograde viral transport. BBB dysfunction occurs through cytokine storm increasing membrane permissibility. Increased chemokine expression allows leukocyte trafficking to the BBB. Virally infected leukocytes may successfully pass through the BBB allowing the pathogen to infect microglia and other CNS cell types. We define cerebrospinal fluid (CSF) nondetection as a virus' ability to evade direct CSF detection but still causing significant neurological symptoms and disease. Mechanisms of CSF nondetection include: transneuronal propagation through trans-synaptic transmission, and synaptic microfusion, as well as intrathecal antibody synthesis and virus neutralization. Direct virus detection in CSF is associated with an increased neurological disease burden. However, the lack of CSF detection does not exclude CNS involvement due to possible neuroevasive mechanisms.

P163 Grey matter myelitis is a severe phenotype of myelitis in systemic lupus erythematosus: experience from a tertiary care hospital in south India over a decade

Abstract: Myelitis is a severe yet rare manifestation of systemic lupus erythematosus (SLE). Literature on lupus myelitis is limited to case reports and series. Birnbaum et al. reported the largest series (n = 22) with two distinct phenotypes based on clinical and imaging findings with mechanistic and therapeutic implications. Further validation of these two subtypes is not reported to date. The objective of the study was to identify distinct phenotypes in lupus myelitis and compare activity and outcomes with the formulation of a prediction model for survival. This is a retrospective study. Data of 35 lupus myelitis patients with follow-up were extracted from the lupus registry (n = 1550) in the last 15 years (2007-2022). Grey matter myelitis (GMM) (i.e., flaccidity, hyporeflexia) and white matter myelitis (WMM) (i.e., spasticity, hyperreflexia) were two subtypes. Disease activity was assessed by SLEDAI-2K and outcome by death/recurrence/disability - modified Rankin score (MRS). MRS at discharge and SLEDAI at admission were used as predictors for survival outcomes among subtypes. Median age at presentation was 24.5 years (12.5-36.5); female-male ratio 32:3. 24 patients presented with GMM and 11 with WMM. GMM had more CNS events (p-0.02), high SLEDAI (p-0.021) and extra CNS involvement (p > 0.05). Anticardiolipin antibodies were increased in WMM (p-0.03). Weibull survival probability plot showed that 25%, 50% & 75% of GMM patients had probability of death within 13, 85, and 376 months from the myelitis episode respectively. The same for WMM was 96, 256, 556 months respectively, implying that GMM has a worse survival outcome. Using linear regression, MRS at discharge and SLEDAI can predict survival status in GMM (R = 0.64, p = 0.04) but not in WMM (R = 0.42, p = 0.46). Lupus myelitis has two distinct subtypes. GMM is associated with more active disease, poor survival, and significant disability, and its survival status can be predicted by MRS and SLEDAI scores. Disclosure R. Baisya: None. K. Yerram: None. G. Murthy: None. P.K. Devarasetti: None. L. Rajasekhar: None.

Navigating Through the Recent Diagnostic Criteria for MOGAD

Abstract: International criteria for the diagnosis of myelin oligodendrocyte glycoprotein–associated disease (MOGAD) were published in The Lancet Neurology ® on January 24, 2023.1 The criteria recommend that patients with a clinical event typically associated with MOG antibody (Ab) (optic neuritis, myelitis, acute disseminated encephalomyelitis, cerebral monofocal or polyfocal deficits, brainstem or cerebellar deficits, cortical encephalitis often with seizures) and clear positive serum MOG-Ab results can be diagnosed with MOGAD. A clear positive test is defined as MOG-Ab measured by fixed cell-based assay (CBA) with a titer ≥1:100 or live CBA with a standardized method (that is a clear positive according to the individual assay cutoffs). Patients with low positive serum MOG-Ab titers can be diagnosed with MOGAD if they possess at least 1 supporting clinical or MRI feature. In cases of optic neuritis and myelitis, the supporting features include bilateral simultaneous optic neuritis, longitudinally extensive spinal cord, or optic nerve involvement or a conus lesion. Supporting features can also be applied to patients with positive MOG-Ab results without reported titers and patients with negative serum but positive CSF MOG-Ab. These diagnostic criteria require the exclusion of alternative diagnoses, including multiple sclerosis (MS).

Imaging Spectrum of HTLV-1–Related Neurologic Disease

Abstract: Purpose of Review Human T-cell lymphotropic virus type 1 (HTLV-1)–associated myelopathy (HAM) is a well-recognized neurologic complication of HTLV-1. Beyond HAM, several other neurologic manifestations are increasingly recognized, including acute myelopathy, encephalopathy, and myositis. The clinical and imaging features of these presentations are less well understood and potentially underdiagnosed. In this study, we summarize the imaging features of HTLV-1–related neurologic disease, providing both a pictorial review and pooled series of the less well-recognized presentations. Recent Findings 35 cases of acute/subacute HAM and 12 cases of HTLV-1–related encephalopathy were found. In subacute HAM, cervical and upper thoracic longitudinally extensive tranverse myelitis was noted, while in HTLV-1–related encephalopathy, confluent lesions in the frontoparietal white matter and along the corticospinal tracts were the most prevalent finding. Summary There are varied clinical and imaging presentations of HTLV-1–related neurologic disease. Recognition of these features aids early diagnosis where therapy may have the greatest benefit.

A case of NMO presenting with hyperthyroidism

Abstract: Neuromyelitis optica (NMO) is an autoimmune disease of central nervous system with extensive clinical presentations but based on the international consensus diagnostic criteria 2015. The horizon has broadened to a larger entity called NMOSD or NMO spectrum disorders. Nonneurological systemic autoimmune diseases especially thyroid disorders are commonly associated with NMOSD, among which Hashimoto’s thyroiditis is common, whereas not much literature is available regarding the association of Graves’ disease with NMOSD. A 38-year-old male presented with acute onset upper motor neuron quadriparesis along with features of hyperthyroidism with a prior history of sudden onset bilateral decreased vision of both eyes 3 years back. Magnetic resonance imaging (MRI) showed T2 hyperintensity involving cervicodorsal cord from C4 to D6 indicating a longitudinally extensive transverse myelitis (LETM) but brain MRI was normal. Serum antiaquaporin 4 antibody in the cell-based assay was positive. Serum thyroid stimulating hormone was very low with high free T4 and positive thyroid stimulating hormone receptor antibody and anti-thyroid peroxidase Ab. With optic neuritis, transverse myelitis (LETM), and positive antiaquaporin 4 antibody, he was diagnosed with NMO with Graves’ disease. Autoimmune thyroid diseases are associated with NMO in many studies but precise clinical and serological quantification of thyroid disorders especially Graves’ disease is still lacking. We are presenting a case of NMO with Graves’ disease who presented with symptoms of hyperthyroidism before myelitis. Our patient developed myelitis almost 3 years after having optic neuritis, but before myelitis, he was having symptoms of hyperthyroidism. Hence, patients of suspected NMO must be monitored for other nonneurological autoimmune diseases both clinically and serologically even in early stage when they are not fulfilling the diagnostic criteria.

Central nervous system mycobacteriosis caused by Mycobacterium genavense in degus (Octodon degus).

Abstract: Degus (Octodon degus) that were kept at a breeding facility presented with neurological or respiratory symptoms and died. Necropsies were performed on 9 individuals, and no significant gross lesions were found. Histologically, spinal cord necrosis was observed in all 9 cases and granulomatous myelitis in 5 of the 9 cases. Locally extensive necrosis of the brain and encephalitis were observed in 7 of the 9 cases. Acid-fast bacteria were found in the spinal cords, brains, and lungs from all 9 cases. Immunohistochemically, Mycobacterium tuberculosis antigen was observed in the spinal cords, brains, and lungs from all 9 cases. Double-labeling immunofluorescence revealed M. tuberculosis antigen in IBA1- and myeloperoxidase-immunopositive cells. Extracted genomic DNA from 8 of the 9 cases was successfully amplified with the primers for Mycobacterium genavense ITS1 and hypothetical 21 kDa protein genes, and the polymerase chain reaction products were identified as M. genavense by DNA sequencing. This report highlights the susceptibility of degus to M. genavense infection in the central nervous system.

Development of Acute Transverse Myelitis following COVID-19 Infection: A Review on the Potential Pathways

Abstract: Background: Acute transverse myelitis (ATM) is a rare neurological disorder in adults characterized by localized inflammation of gray and white matter in one or more contiguous spinal cord segments in the absence of a compressive injury. Several reports have connected the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) to the pathophysiology of ATM. Summary: Direct invasion of the spinal cord, cytokine storm, or an autoimmune response are the possible pathways by which the SARS-CoV-2 virus can affect the spinal cord and lead to ATM. Direct invasion is facilitated by the presence of angiotensin-converting enzyme 2 (ACE2) receptors on the membranes of the spinal cord neurons. Cytokine storm syndrome could be derived from elevated levels of several immunological factors following severe involvement with coronavirus disease 2019 (COVID-19). Finally, autoimmune responses can cause post-infectious ATM through several hypothesized processes, including molecular mimicry, epitope spreading, bystander activation, and polyclonal B-cell activation. Key Messages: COVID-19-induced ATM is mostly a longitudinally-extensive ATM (LEATM), in which more spinal cord segments are damaged, which results in a worse sequel compared to short-segment ATM. Therefore, it is suggested that COVID-19 patients, particularly severe cases, be followed up for a probable incidence of ATM, even long after recovery from the disease and elimination of the virus from the host, because an early diagnosis and effective therapy may stop the spread of inflammation to adjacent segments.

Neuromyelitis Optica Revealed by Headache in a Child: A Case Report

Abstract: Devic’s neuromyelitis optica (NMO) is a rare inflammatory disease of the central nervous system that results in optic neuropathy and myelitis. Optic neuritis represents the mode of entry into the disease in more than two thirds of cases. It is a rare entity in children. There is no effective treatment at present, but some molecules can be used, such as corticosteroids, immunosuppressants and plasma exchange. The prognosis in children is generally favorable. Devic’s neuromyelitis is a condition of unknown etiopathogeny which is functionally critical and requires early and appropriate treatment. We report the case of a 12-year-old girl who presented to emergency with a headache and decreased visual acuity, whose investigations led to the diagnosis of Devic’s neuromyelitis optica.

Long-segment myelitis after traumatic paraplegia in COVID-19 positive patient: An unusual case report

Abstract: Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) has devastated the mankind globally, and countless lives have been lost all around the world. This disease has been linked to various extrapulmonary symptoms and consequences in addition to typical respiratory illness. This case highlights a probable neurological complication of SARS-CoV-2 infection. A 28-year-old healthy man, sustained wedge compression of D12 vertebra following road traffic accident, presented with paraplegia. One week following admission, the patient had a progressive neurological deterioration and developed high grade fever with weakness in both upper limbs. The patient developed quadriplegia 10 days after admission. Magnetic resonance imaging (MRI) brain and spine were done. MRI brain was normal, whereas MRI spine showed D11-D12 anterolisthesis with cord compression with T2 hyperintensity of cervical cord. His SARS-CoV-2 reverse transcriptase polymerase chain reaction turned out to be positive. We hereby report a case of posttraumatic long-segment myelitis with coronavirus disease 2019 as a probable etiology.

Concomitant transverse myelitis and Guillain-Barré syndrome following varicella-zoster virus infection

Abstract: To the Editor: Currently known syndromes of acute post-infectious or inflammatory demyelination include acute disseminated encephalomyelitis, encephalitis, transverse myelitis (TM), and Guillain–Barré syndrome (GBS). Demyelination is frequently limited to the central or peripheral nervous systems. The coexistence of myelitis and demyelinating peripheral neuropathy has only been reported in a few children[1,2] and young adults.[3] Also, concomitant TM and GBS in elderly immunocompetent patients has been rarely described. Varicella-zoster virus (VZV) is a well-known pathogenic human herpes virus that causes chickenpox or herpes zoster. It can also cause diverse neurological dysfunction, including encephalitis, myelopathy, peripheral neuropathy, neuralgia, and stroke.[4] Both GBS and acute TM are unusual complications caused by VZV infection.[5] Most VZV patients with GBS or TM achieve excellent outcomes at 1 year (able to walk) after treatment,[6] while the coexistence of GBS and TM caused by VZV infection is very rare, and its treatment strategy and prognosis are not clear. Herein, we reported a case of an immunocompetent man who developed TM and GBS after a VZV infection, the consent form was obtained from the patient. A 58-year-old man presented with neck pain, progressive numbness, and weakness in all limbs, which lasted for 4 weeks. The symptoms progressed rapidly; and soon, he could not sit or stand. The strength in the proximal and distal muscles of the limbs was Medical Research Council (MRC) grade 3/5 and 0/5, respectively. Tendon reflexes were absent in all limbs. Bilateral Babinski's signs were positive. Bilateral pinprick sensation below the C4 level was impaired. The muscles of the limbs were atrophied. He also had defecation dysfunction and urine retention. One month before onset, he developed herpes zoster on the right upper limb and was not treated with acyclovir. Serum and cerebrospinal fluid (CSF) tests for anti-ganglioside, aquaporin 4 (AQP4), myelin oligodendrocyte glycoprotein (MOG), and myelin basic protein (MBP) antibodies were negative. CSF analysis showed a normal white blood cell (WBC) count (4 cells/μL), high protein (92.6 mg/dL), normal glucose, normal immunoglobulin G (IgG) index, and negative CSF oligoclonal bands. VZV antibodies (IgG) were positive in serum and CSF. Polymerase chain reaction (PCR) tests of the CSF for VZV DNA were positive. The MRI of his spine revealed extensive patchy and linear intramedullary lesions with high signal change on T2-weighted MRI from C3 to T2 [Figure 1]. Enhanced cervical MRI showed no significant enhancement of the cervical spinal cord and nerve roots. Nerve conduction studies revealed significantly decreased nerve conduction velocity and reduced compound muscle action potential amplitudes [Supplementary Table 1, https://links.lww.com/CM9/B511]. Bilateral sural sensory, median, and ulnar motor responses were absent. In addition, there was no F response in the bilateral common peroneal nerves and tibial nerves.Figure 1: Cervical spinal magnetic resonance imaging (24 days after onset). (A) High signal intensity on sagittal T2-weighted images within the spinal cord from C3 to T2. (B) An axial T2-weighted image.On the second day in the hospital, he was treated with intravenous acyclovir 500 mg every 8 h for 7 days, immunoglobulin at 0.4 g∙kg-1∙day-1 for 5 days, and intravenous administration of methylprednisolone at 500 mg/day for 6 days. One month later, the strength in his proximal and distal muscles of the extremities was MRC grade 4/5 and 2/5, respectively. The pinprick sensory level decreased to the T4 level. After 6 months, there was very little improvement in his limb power and sensory abnormalities. At 1 year, this patient was still not able to walk independently. Based on the clinical performance, MRI evidence, neuroelectrophysiological findings, and pathogen data, the final diagnosis was VZV-induced TM and acute motor and sensory axonal neuropathy (AMSAN). The pathogenesis of neurological complications related to VZV infection is unclear. Pathologic and virologic analyses of the spinal cord from fatal cases have shown invasion of VZV in the parenchyma; in some cases, invasion of the adjacent nerve roots was also involved.[7] Therefore, the direct invasion of VZV may be one of the reasons for neuropathy caused by VZV. Until now, the pathophysiology of VZV GBS was usually considered to be an immune-mediated post-infectious mechanism. In this patient, we detected positive VZV antibodies and VZV DNA in the CSF. Although anti-ganglioside antibodies were negative, we suggest that the pathogenesis of TM and GBS is related to the direct invasion of the virus and the autoimmune mechanism secondary to infection. Although a standard treatment policy for VZV-induced neurological dysfunction has not yet been established, early diagnosis and aggressive treatment with sufficient acyclovir have been considered helpful in some cases.[8] We also believe that early antiviral therapy is not enough to avoid or mitigate the post-infective autoimmune response. It is well established that TM responds to steroids or immunoglobulin, and GBS responds to immunoglobulin or plasma exchange. Therefore, combined intravenous immunoglobulin and corticosteroid administration is recommended for patients with concomitant TM and GBS.[2,3] Accordingly, this patient was treated with a high dose of intravenous acyclovir, immunoglobulin, and methylprednisolone. Although his muscle strength slightly improved and the level of sensory disturbance significantly decreased, the patient could not walk independently. This patient might have residual or permanent neurological dysfunctions even after the appropriate treatment. There were two possible reasons for this result. First, he began treatment 4 weeks after the onset of the disease; delayed treatment might be the first reason for his poor prognosis. Second, immunoglobulin and steroids might not be effective enough for AMSAN. Therefore, we suggest that early medical intervention may be helpful for better and earlier recovery. Of course, this suggestion has not been supported by strong evidence and still needs more clinical trials to discuss. This report presents a rare microbiologically confirmed case of concurrent TM and AMSAN following VZV infection in an immunocompetent patient. Our data suggest using the VZV antibody and DNA in the CSF to confirm this disease. In addition, we suggest that early diagnosis and early administration of acyclovir, corticosteroids, immunoglobulin, or plasma exchange may be helpful for the rehabilitation of this disease. Funding This study was supported by a grant from the Shanghai Municipal Science and Technology Major Project (No. 2021SHZDZX0100) and the Fundamental Research Funds for the Central Universities. Conflicts of interest None.

COVID-19 Infection Linked to MOG Antibody-Associated Disease (MOGAD): Case Report (P10-5.023)

Abstract:

Objective:

To describe a case of MOG associated Transverse Myelitis after COVID-19 infection in a young adult.

Background:

Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) is an inflammatory disorder of the central nervous system characterized by attacks of immune-mediated demyelination predominantly targeting the optic nerves, brain, and spinal cord. In some cases of MOG associated Acute Transverse Myelitis (ATM), there is an antecedent history of infection or vaccination.

Design/Methods:

We report the case of a patient with different neurological manifestations of CNS demyelination presenting within six weeks of moderate COVID-19 illness.

Results:

A 28-year-old man with hypertension presented with a three-week history of rapidly progressive paraparesis, sensory disturbances, saddle anesthesia, constipation and urinary retention, starting six weeks after moderate COVID-19 illness. MRI revealed non-enhancing T2 and FLAIR hyperintensities in the periventricular and subcortical white matter of both cerebral hemispheres, corpus callosum, inferior aspect of medulla, and a long segment, non-enhancing T2 signal intensity involving the cervical and thoracic spinal cord. Laboratory testing was remarkable for normal CSF analysis, positive serum COVID-19 IGG/IGM antibodies, and MOG antibodies titer at >1:160. Multiple courses of systemic glucocorticoids provided mild to moderate improvement, so subsequent plasma exchange therapy was started with significant resolution of patient’s symptoms. Follow-up MRIs at 2 and 8 months showed interval resolution of cervical spinal cord edema and near-complete resolution of focal hyperintense T2/STIR cord lesions.

Conclusions:

MOGAD is typically steroid-responsive with favorable long-term recovery. Although the present case involved a rare condition of MOGAD associated ATM after COVID-19 infection, detailed studies of additional clinical cases are necessary to determine the causation and the risk of developing this condition. We hypothesized the possibility of an immune mediated reaction triggered by COVID-19, inducing demyelination and autoimmune encephalomyelitis mediated by MOG-antibodies. Disclosure: Dr. Graciano has nothing to disclose. Dr. Ghatali has nothing to disclose.

Six- and 12-month functional outcomes among patients with confirmed acute flaccid myelitis (AFM) with onset in 2018, United States.

Abstract: PURPOSE Acute flaccid myelitis (AFM), an uncommon but serious neurologic condition, primarily affects children, and can progress quickly to paralysis and respiratory failure. Data on long-term outcomes of patients with AFM are limited. This study reports on functional status through 12 months for AFM patients who became ill in 2018 in the United States. METHODS Health departments collected information on outcomes at 6 and 12 months after onset of AFM using a standardized form that asked patients or their parents/guardians about functional status. Analyses were restricted to confirmed cases. RESULTS Of the 238 confirmed AFM cases reported to CDC in 2018, 90 (38%) had assessments at 6 months, 82 (34%) at 12 months, and 49 (21%) at both 6 and 12 months. Among the 49 patients with data at both time points, the proportion of patients reporting significant or severe impairment at 6 months ranged from 2% to 59% depending on the outcome. Although proportions decreased by 12 months and ranged from 2% to 51%, most patients had some impairment at 12 months. No deaths were reported. CONCLUSION Six- and 12-month outcomes in patients with onset of AFM in 2018 span a wide range of functionality, particularly of upper and lower extremities. Importantly, improvement appears to occur over time in some patients.

Emerging trends and insights in acute flaccid myelitis: a comprehensive review of neurologic manifestations.

Abstract: Acute Flaccid Myelitis (AFM) is a neurological condition in the anterior portion of the spinal cord and can be characterised as paraplegia (paralysis of the lower limbs), and cranial nerve dysfunction. These lesions are caused by the infection due to Enterovirus 68 (EV-D68); a member of the Enterovirus (EV) family belongs to the Enterovirus species within the Picornavirus family and a Polio-like virus. In many cases, the facial, axial, bulbar, respiratory, and extraocular muscles were affected, hence reducing the overall quality of the patient's life. Moreover, severe pathological conditions demand hospitalisation and can cause mortality in a few cases. The data from previous case studies and literature suggest that the prevalence is high in paediatric patients, but careful clinical assessment and management can decrease the risk of mortality and paraplegia. Moreover, the clinical and laboratory diagnosis can be performed by Magnetic resonance imaging (MRI) of the spinal cord followed by Reverse transcription polymerase chain reaction (rRT-PCR) and VP1 seminested PCR assay of the cerebrospinal fluid (CSF), stool, and serum samples can reveal the disease condition to an extent. The primary measure to control the outbreak is social distancing as advised by public health administrations, but more effective ways are yet to discover. Nonetheless, vaccines in the form of the whole virus, live attenuated, sub-viral particles, and DNA vaccines can be an excellent choice to treat these conditions. The review discusses a variety of topics, such as epidemiology, pathophysiology, diagnosis/clinical features, hospitalisation/mortality, management/treatment, and potential future developments.

A comparative analysis of demographic, clinical and imaging features of myelin oligodendrocyte glycoprotein antibody positive, aquaporin 4 antibody positive, and double seronegative demyelinating disorders – An Indian tertiary care center prospective study

Abstract: Objectives: The aim of the study was to study the demographical, clinical, radiological features, and outcome of anti-myelin oligodendrocyte glycoprotein (MOG) antibody spectrum disorder and compare these features with patients negative for anti-MOG antibody. MOG antibody-associated disease (MOGAD) and aquaporin-4 (AQP4) antibody-related diseases are immunologically distinct pathologies. Our aim was to compare the clinical and radiological features of MOG antibody-related diseases with AQP4 antibody-related diseases and seronegative demyelinating diseases (Non-multiple sclerosis). Materials and Methods: This was a prospective and cohort study conducted at an apex tertiary care institute in the northern part of India from Jan 2019 to May 2021. We compared clinical, laboratory, and radiological findings of patients with MOGAD, AQP4 antibody-related diseases, and seronegative demyelinating disease. Results: There were a total of 103 patients – 41 patients of MOGAD, 37 patients of AQP4 antibody-related diseases and 25 seronegative demyelinating disease. Bilateral optic neuritis was the most frequent phenotype in patients with MOGAD (18/41) whereas myelitis was the most common phenotype in the AQP4 (30/37) and seronegative groups (13/25). Cortical, juxtacortical lesions, anterior segment optic neuritis, optic sheath enhancement, and conus involvement in myelitis were radiological findings that separated MOGAD from AQP4 related diseases. Nadir Expanded Disability Status Scale (EDSS) and visual acuity were similar across the groups. Last follow-up EDSS was significantly better in the MOG antibody group as compared to AQP4 antibody group (1 [0–8] vs. 3.5 [0–8]; P = 0.03). Encephalitis, myelitis, and seizures were more common in the younger population (<18 vs. >18 years) in MOGAD (9 vs. 2, P = 0.001; 9 vs. 7, P = 0.03; 6 vs. 0, P = 0.001). Conclusion: We identified several clinical and radiological features that can help physicians to distinguish MOGAD from AQP4-immunoglobulin G+neuromyelitis optica spectrum disorder. Differentiation is vital as treatment response might vary among both groups.