Influenza B-induced longitudinally extensive transverse myelitis and bithalamic acute disseminated encephalomyelitis.

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.

An atypical case of acute transverse myelitis following COVID-19 infection.

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.

Transverse Myelitis Following SARS-CoV-2 Vaccination: A Pharmacoepidemiological Study in the World Health Organization's Database.

BACKGROUND: Transverse myelitis (TM) has recently been associated by health authorities with Ad26.COV2.S (Janssen/Johnson & Johnson), one of the 5 US Food and Drug Administration (FDA) or European Medicines Agency (EMA) labeled severe acute respiratory syndrome-coronavirus 2 (SARS-CoV-2) vaccines. It is unknown whether a similar association exists for the other FDA or EMA labeled SARS-CoV-2 vaccines (BNT162b2 [Pfizer/BioNTech], mRNA-1273 [Moderna], ChAdOx1nCov-19 [Oxford-AstraZeneca], and NVX-CoV2373 [Novavax]). This study aimed to evaluate the association between SARS-CoV-2 vaccine class and TM. METHODS: This observational, cross-sectional, pharmacovigilance cohort study examined individual case safety reports from VigiBase, the World Health Organization's pharmacovigilance database. We first conducted a disproportionality analysis with the information component (IC) using the reports of TM that occurred within 28 days following exposure to the FDA or EMA labeled SARS-CoV-2 vaccines, from December 1, 2020 (first adverse event related to a SARS-CoV-2 vaccine) to March 27, 2022. Second, we analyzed the clinical features of SARS-CoV-2 vaccine-associated TM cases reported in VigiBase. RESULTS: TM was significantly associated both with the messenger ribonucleic acid (mRNA)-based (n = 364; IC025  = 0.62) and vector-based (n = 136; IC025  = 0.52) SARS-CoV-2 vaccines that are authorized by the FDA or the EMA. CONCLUSIONS: Findings from this observational, cross-sectional pharmacovigilance study showed that mRNA-based and vector-based FDA/EMA labeled SARS-CoV-2 vaccines can be associated with TM. However, because TM remains a rare event, with a previously reported rate of 0.28 cases per 1 million vaccine doses, the risk-benefit ratio in favor of vaccination against SARS-CoV-2 virus remains unchallenged. Rather, this study suggests that clinicians should consider the diagnosis of TM in patients presenting with early signs of spinal cord dysfunction after SARS-CoV-2 vaccination. ANN NEUROL 2022;92:1080-1089.

Transverse myelitis associated with systemic lupus erythematosus (SLE-TM): A review article.

Systemic lupus erythematosus-related transverse myelitis (SLE-TM) is a rare but serious complication of SLE, which may result in significant morbidity. Its incidence is estimated between 0.5% and 1% of all SLE patients but may be the presenting feature in 30%-60% of these patients. Unfortunately, due to lack of high-quality studies, data regarding this condition remains limited. Its pathogenesis remains largely unknown and clinical presentation is variable. There are still no set guidelines regarding diagnosis, management, or monitoring and the role of autoantibodies remains controversial. In this review, we aim to summarize the available data regarding the epidemiology, pathogenesis, clinical features, management, and prognosis of this rare disease.

Primary intramedullary spinal cord lymphoma misdiagnosed as longitudinally extensive transverse myelitis: a case report and literature review.

BACKGROUND: Primary intramedullary spinal cord lymphoma (PISCL) is rare and easily misdiagnosed with the lack of typical clinical features and non-specific imaging manifestations. CASE PRESENTATION: A 49-year-old man was admitted to our hospital because of persistent limbs numbness, pinprick-like pain in the posterior neck and unsteady gaits. He has brisk tendon reflexes and positive Babinski's sign. Magnetic resonance imaging (MRI) of the cervical spine showed an abnormal signal with aberrant reinforcement at medulla oblongata and the level of C1-C7. He was clinically diagnosed as longitudinally extensive transverse myelitis (antibody-negative). Steroid pulse therapy was administered and resulted in reduced symptoms. One month later, his situation was exacerbated compared to the onset. We launched a new cascade of steroid pulse therapy. But it did not improve his symptoms. Finally, the biopsy pathology confirmed PISCL. Chemotherapy, radiotherapy and zanubrutinib were administered and until now about 3 years into treatment the patient is still survival. CONCLUSIONS: Based on our case and literature review, we recommend that spinal onset patients react ineffectively to standard immunoglobulins or hormonal treatments or experience a relapse after a short time relief should take PISCL into consideration.

Bagel sign in neuro Behçet's disease myelitis: neuroimages.

Neuro-Behçet's disease (NBD) is a chronic heterogenous autoimmune disorder. It may involve central or pripheral nervous system but rarely shows spinal cord involvement (SCI). Bagel Sign is a unique sign of SCI due to NBD. It is a central T2W hyperintense lesion with a hypointense core on axial magnetic resonance imaging (MRI). This sign may be a complete or an incomplete ring with or without post-contrast enhancement. Here we report a patient with NBD whose primary presentaion was transverse myelitis. A 14-year-old patient was admitted due to triparesis and urinary retention. He had T2W hyperintensities from the left basal ganglion down to the T10 level. A similar anteromedial spot has been described in anterior spinal cord infarction which favors an ischemic pathogenesis for NBD. To our knowledge this is the first report of Bagel Sign and longitudinally extensive transverse myelitis with gray matter involvement.

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

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.

Early SARS-CoV-2-associated acute transverse myelitis: A case for neurotropism?

There are increasing reports of immune-mediated and para-infectious syndromes beyond the well-known respiratory manifestations of severe-acute-respiratory syndrome coronavirus 2 (SARS-CoV-2) infection. However, the spectrum of severe neurological sequelae of SARS-CoV-2 remains undefined. We present the case of a 66-year-old female with rapidly progressive lower limb neurology 3 days post SARS-CoV-2 infection. Clinical and radiological findings were in keeping with transverse myelitis and treatment success was achieved with methylprednisolone and remdesivir. This report will discuss the associations between SARS-CoV-2 and acute transverse myelitis. We believe this is one of few described cases of early SARS-CoV-2-associated transverse myelitis secondary to neurotropism and the first successfully treated with the inclusion of remdesivir in the therapeutic regimen.

Non-invasive near-infrared spectroscopy assessment of the spinal neurovascular response in a patient with transverse myelitis: a case report.

BACKGROUND: Transverse myelitis (TM) is characterized by acute development of motor, sensory and autonomic dysfunctions due to horizontally diffused inflammation in one or more segments of the spinal cord in the absence of a compressive lesion. The not well-known inflammation process induces demyelination resulting in neurological dysfunction. CASE PRESENTATION: In this case report we used a functional Near-Infrared Spectroscopy (fNIRS) technique to evaluate changes in the peri-spinal vascular response induced by a peripheral median nerve electrical stimulation in a patient with chronic transverse myelitis (TM). fNIRS showed drastically reduced signal amplitude in the peri-spinal vascular response, compared to that obtained from a healthy control group throughout most of the C7-T1 and T10-L2 spinal cord segments. CONCLUSION: The potential use of this relatively non-invasive fNIRS technology support the potential clinical application of this method for functional test of the spinal cord through the assessment of the spinal neurovascular response.

Severe disease exacerbation after mRNA COVID-19 vaccination unmasks suspected multiple sclerosis as neuromyelitis optica spectrum disorder: a case report.

BACKGROUND: Since the beginning of the COVID-19 pandemic and development of new vaccines, the issue of post-vaccination exacerbation or manifestation of demyelinating central nervous system (CNS) disorders has gained increasing attention. CASE PRESENTATION: We present a case of a 68-year-old woman previously diagnosed with multiple sclerosis (MS) since the 1980s who suffered a rapidly progressive severe sensorimotor paraparesis with loss of bladder and bowel control due to an acute longitudinal extensive transverse myelitis (LETM) after immunization with the mRNA Pfizer-BioNTech COVID-19 vaccine. Detection of Aquaporin-4-antibodies (AQP4) in both serum and CSF led to diagnosis of AQP4-antibody positive neuromyelitis optica spectrum disorder (NMOSD). Treatment with intravenous corticosteroids and plasmapheresis led to a slight improvement of the patient's symptoms. CONCLUSIONS: Pathogenic mechanisms of post-vaccination occurrence of NMOSD are still unknown. However, cases like this should make aware of rare neurological disorders manifesting after vaccination and potentially contribute to improvement of management of vaccinating patients with inflammatory CNS disorders in the future. So far two cases of AQP4-antibody positive NMOSD have been reported in association with viral vector COVID-19 vaccines. To our knowledge, we report the first case of AQP4-antibody positive NMOSD after immunization with an mRNA COVID-19-vaccine.

Acute transverse myelitis in an adult-patient with underlying ulcerative colitis: a case report.

BACKGROUND: Ulcerative colitis (UC) is an idiopathic inflammatory bowel disease that limits to colon mucosa, which characterised by relapsing and remitting abdominal pain and diarrhea. Neurological complications in UC patients are usually underestimated. The most frequently reported neurological disorders associated with UC are peripheral neuropathy, cerebrovascular disease and demyelinating disease. However, acute transverse myelitis (TM) is rarely reported in UC patients. CASE PRESENTATION: We report a case of a 39-year-old man presented with fatigue, muscle weakness, numbness in the lower limbs and fingers with underlying UC. Laboratory results revealed elevated neutrophil count, high-sensitivity C-reactive protein and erythrocyte sedimentation rate. Strip-shaped high signal intensity was identified in the cervical and thoracic spinal cord on T2-weighted magnetic resonance imaging. Acute TM was diagnosed. Significant improvements after intravenous high-dose methylprednisolone were observed. CONCLUSION: We speculate that acute TM may be the extraintestinal manifestation of UC, which may be related to the abnormalities of cell-mediated and humoral immunity rather than the side effect of mesalazine.

Acute transverse myelitis progressing to permanent quadriplegia following COVID-19 infection.

As of January 2022, there have been over 350 million confirmed cases of COVID-19 in the world. The most common symptoms in those infected are fever, cough, malaise, and myalgia, however pulmonary, hematologic, gastrointestinal, renal, and neurologic complications have also been reported. Acute transverse myelitis (ATM) is an uncommon neurological syndrome characterized by acute or subacute spinal cord dysfunction that can lead to paresthesias, sensory and autonomic impairment, and even paralysis. Etiologies are often unclear; however, potential causes include infection, neoplastic, drug or toxin induced, autoimmune, and acquired. Treatment for ATM primarily consists of steroids and plasmapheresis, which often reverses any neurologic symptoms. ATM has rarely been reported as a complication of COVID-19 infections. A 43-year-old female presented to the emergency department for evaluation of progressive numbness and tingling in her legs ten days after developing upper respiratory symptoms from a COVID-19 infection. Physical examination and magnetic resonance imaging confirmed a diagnosis of ATM. During her hospital course, she experienced rapid progression of her paresthesias and developed complete loss of motor function in her upper and lower extremities. Within 48 hours after emergency department arrival, she required intubation due to worsening diaphragmatic and chest wall paralysis. Her treatment included a long-term steroid regimen and plasmapheresis, and unfortunately, she did not have any neurologic recovery. We present a very rare case of ATM progressing to complete quadriplegia following COVID-19 infection.

Transverse myelitis associated with primary biliary cirrhosis: clinical, laboratory, and neuroradiological features.

PURPOSE: Only five patients diagnosed with transverse myelitis (TM) associated with primary biliary cirrhosis (PBC) have been reported in the literature to date. We report two additional patients with TM associated with PBC at our hospital and review all seven cases. MATERIALS AND METHODS: An association between neuromyelitis optic spectrum disease (NMOSD) and PBC is reported for the first time in one of our patients. The second patient was diagnosed with TM associated with PBC without Sjögren's syndrome (SS). A literature review was performed using the PubMed database. RESULTS: All patients diagnosed with TM associated with PBC were female with a median age of 53 years. TM was associated with SS in 71.4% of the patients. Complete TM and incomplete TM were diagnosed in 71.4% and 28.6% of the patients. The erythrocyte sedimentation rate was increased in 83.3% of patients. All patients were positive for anti-mitochondrial antibodies. Other autoantibodies, including anti-nuclear antibodies, rheumatoid factor, anti-SSA antibody, were detected in some patients. Cerebrospinal fluid analysis was abnormal in 83.3% of patients. The spinal cord lesions involved more than three vertebral segments in 85.7% of patients. Glucocorticoids were administered in 85.7% of patients, and good responses were observed. CONCLUSIONS: The association between TM and PBC may be missed by neurologists. More attention should be paid to the association between NMOSD and PBC. Most patients show SS and may experience relapse, and there is a good rationale for early commencement of immunosuppressive therapy.

Late-onset neuromyelitis optica spectrum disorder: A case series from Iran.

INTRODUCTION: Neuromyelitis optica spectrum disorder (NMOSD) is a disabling autoimmune disease of the central nervous system that can start at ages of 50 or more, when it is called late-onset NMOSD (LO-NMOSD). Data on this disorder are sparse. In this cross-sectional study, patient characteristics of the disease were studied. LO-NMOSD patients of a tertiary center in Tehran were studied from 2016 to 2020. CASE REPORTS: Eight patients were identified, half of whom were men. The diagnostic delay was from no time-lapse to three years (mean: 0.62, SD: 1.06), which was significantly shorter than in early-onset patients. Seven patients (87.5%) tested positive for AQP4-IgG which was significantly higher compared to early-onset patients (p-value=0.01). Four patients (50%) had both transverse myelitis and optic neuritis as presenting symptoms, while three (38%) had just myelitis and only one (12%) had optic neuritis. CONCLUSION: There is discrepancy regarding different aspects of LO-NMOSD. Further studies are needed to clarify the subject in order to enhance diagnosis and treatment.

A case report of transverse myelitis in a patient with human immunodeficiency virus.

Acute or subacute transverse myelitis is a rare disease caused by inflammation of the spinal cord. The extensive differential diagnosis of this disease require physician to timely decide the necessary investigations and plan for effective treatment. We report a case of anomalous multifactorial transverse myelitis in a patient with human immunodeficiency virus (HIV) who responded well with multimodal therapy. The patient was given immunosuppressive agent and plasma exchange for the demyelinating disease as well as antibiotics for neurosyphilis, antiviral for cytomegalovirus (CMV) neurologic disease, and highly active antiretroviral therapy (HAART). Three months after his first presentation, patient showed full neurology recovery and suppressed HIV viral load.

Atypical myelitis in patients with multiple sclerosis: Characterization and comparison with typical multiple sclerosis and neuromyelitis optica spectrum disorders.

BACKGROUND: Atypical myelitis in multiple sclerosis (MS) is characterized by extensive myelitis in the longitudinal (longitudinally extensive transverse myelitis) or axial plane (transverse myelitis). OBJECTIVE: To characterize a cohort of MS patients with atypical myelitis. METHODS: Atypical myelitis was extracted from the French and Luxembourg MS databases and compared to two cohorts of MS patients with typical myelitis and neuromyelitis optica spectrum disorders (NMOSDs) patients with myelitis. RESULTS: We enrolled 28 MS patients with atypical myelitis, 68 MS patients with typical myelitis and 119 NMOSD patients with a first episode of myelitis. MS patients with atypical myelitis were characterized by a mean age of 34.0 (±10.7) years and 64.3% were women. In 82.1% of the patients, atypical myelitis was the first episode of MS. Mean Expanded Disability Status Scale (EDSS) scores at nadir and 3-6 months after onset were 4.1 ± 2.1 and 3.3 ± 2, respectively. Differences between groups revealed a predominance of cervicothoracic myelitis and a higher level of disability in NMOSD patients. Disability in MS patients with atypical myelitis was more severe than in the MS patients with typical myelitis; 28% had already converted to progressive MS within our mean follow-up of 39.6 (±30.4) months. CONCLUSION: Atypical myelitis may be the first presentation of MS and is associated with poorer prognosis.

Neurobrucellosis with ischemic stroke and spinal cord involvement: a case report.

BACKGROUND: Brucellosis is a common zoonotic disease that may have a variety of clinical manifestations when it affects the nervous system. Ischemic stroke is a rare clinical symptom, but if it is not diagnosed and treated early, it may cause more severe consequences. CASE PRESENTATION: We report a 38-year-old man presenting with hearing impairment for four years and sudden weakness of the right limb for two years, recurrent aphasia, and gradual weakness of bilateral lower limbs for nine months. He had bilateral positive Babinski's sign. Cerebrospinal fluid (CSF) showed raised protein and pleocytosis. Magnetic resonance imaging (MRI) showed ischemic infarcts in the pons and extensive enhancement of spinal meninges combined with spinal cord atrophy and ischemia. The tests revealed Brucella Rose Bengal positive in serum and CSF. Brucella culture in CSF was also positive. Next-generation sequencing (NGS) of CSF revealed positive for Brucella with 105 species were detected. He showed significant improvement with antibiotics at five months follow-up. CONCLUSIONS: Neurobrucellosis may mimic stroke and transverse myelitis like syndromes. NB is a treatable infectious condition and should always be considered in the differentials, especially if there are risk factors, as in our case.

Neurological and Neuropsychiatric Impacts of COVID-19 Pandemic.

BACKGROUND: Albeit primarily a disease of respiratory tract, the 2019 coronavirus infectious disease (COVID-19) has been found to have causal association with a plethora of neurological, neuropsychiatric and psychological effects. This review aims to analyze them with a discussion of evolving therapeutic recommendations. METHODS: PubMed and Google Scholar were searched from 1 January 2020 to 30 May 2020 with the following key terms: "COVID-19", "SARS-CoV-2", "pandemic", "neuro-COVID", "stroke-COVID", "epilepsy-COVID", "COVID-encephalopathy", "SARS-CoV-2-encephalitis", "SARS-CoV-2-rhabdomyolysis", "COVID-demyelinating disease", "neurological manifestations", "psychosocial manifestations", "treatment recommendations", "COVID-19 and therapeutic changes", "psychiatry", "marginalised", "telemedicine", "mental health", "quarantine", "infodemic" and "social media". A few newspaper reports related to COVID-19 and psychosocial impacts have also been added as per context. RESULTS: Neurological and neuropsychiatric manifestations of COVID-19 are abundant. Clinical features of both central and peripheral nervous system involvement are evident. These have been categorically analyzed briefly with literature support. Most of the psychological effects are secondary to pandemic-associated regulatory, socioeconomic and psychosocial changes. CONCLUSION: Neurological and neuropsychiatric manifestations of this disease are only beginning to unravel. This demands a wide index of suspicion for prompt diagnosis of SARS-CoV-2 to prevent further complications and mortality.

Les impacts neurologiques et neuropsychiatriques d'une infection à la COVID-19. CONTEXTE: Bien qu'il s'agisse principalement d'une maladie des voies respiratoires, la maladie infectieuse à coronavirus apparue en 2019 (COVID-19) s'est avérée avoir un lien de causalité avec une pléthore d'impacts d'ordre neurologique, neuropsychiatrique et psychologique. Cette étude entend donc analyser ces impacts tout en discutant l'évolution des recommandations thérapeutiques se rapportant à cette maladie. MÉTHODES: Les bases de données PubMed et Google Scholar ont été interrogées entre les 1er janvier et 30 mai 2020. Les termes clés suivants ont été utilisés : « COVID-19 ¼, « SRAS ­ CoV-2 ¼, « Pandémie ¼, « Neuro ­ COVID ¼, « AVC ­ COVID ¼, « Épilepsie ­ COVID ¼, « COVID ­ encéphalopathie ¼, « SRAS ­ CoV-2 ­ encéphalite ¼, « SRAS ­ CoV-2 ­ rhabdomyolyse ¼, « COVID ­ maladie démyélinisante ¼, « Manifestations neurologiques ¼, « Manifestations psychosociales ¼, « Recommandations thérapeutiques ¼, « COVID-19 et changement thérapeutiques ¼, « Psychiatrie ¼, « Marginalisés ¼, « Télémédecine ¼, « Santé mentale ¼, « Quarantaine ¼, « Infodémique ¼ et « Médias sociaux ¼. De plus, quelques articles de journaux relatifs à la pandémie de COVID-19 et à ses impacts psychosociaux ont également été ajoutés en fonction du contexte. RÉSULTATS: Il appert que les manifestations neurologiques et neuropsychiatriques des infections à la COVID-19 sont nombreuses. Les caractéristiques cliniques d'une implication des systèmes nerveux central et périphérique sautent désormais aux yeux. Ces caractéristiques ont fait l'objet d'une brève analyse systématique à l'aide de publications scientifiques. En outre, la plupart des impacts d'ordre psychologique de cette pandémie se sont révélés moins apparents que les changements réglementaires, socioéconomiques et psychosociaux. CONCLUSION: Les manifestations neurologiques et neuropsychiatriques de cette maladie ne font que commencer à être élucidées. Cela exige donc une capacité accrue de vigilance en vue d'un diagnostic rapide, et ce, afin de prévenir des complications additionnelles et une mortalité accrue.

ACUTE TRANSVERSE MYELITIS AS A NEUROLOGICAL COMPLICATION OF COVID-19: A CASE REPORT.

We aim to report a COVID-19-related case of acute myelitis that has not been associated with any other viral infections. A 23-year-old student was admitted to the hospital within a month from the time of loss of smell and taste with features of acute-onset non-compressive myelitis with paresthesia on both sides from the Th9 level. Complex neurological, clinical, laboratory, and neuroimaging examination was performed within 24 hours of admission. MRI of the spine showed a segment of increased T2 signal in the center of the spinal cord at Th11-Th12. Elevated protein level and lymphocytic pleocytosis were detected in the cerebrospinal fluid. A serologic blood test for SARS-CoV-2 showed recent infection. PCR for other viral infections was negative. The patient was treated with injectable steroids and showed full recovery. Specific neurological features of acute myelitis associated with COVID-19 were reported, described, and analyzed. Patient was treated and recovered.