Concurrent transverse myelitis and acute inflammatory demyelinating polyneuropathy.

A woman in her 40s presented with thoracic banding dysaesthesia and lower motor neuron weakness. Spinal imaging revealed a short segment of transverse myelitis and neurophysiology was suggestive of concurrent acute inflammatory demyelinating polyneuropathy. The patient improved with consecutive intravenous immunoglobulin and methylprednisolone treatment. Acute inflammatory demyelinating polyneuropathy is a progressive immune-mediated peripheral neuropathy which responds to intravenous immunoglobulin or plasmapheresis, whereas transverse myelitis is a central inflammatory syndrome usually treated with corticosteroid. We highlight differentiating features of the clinical presentation and the utility of investigations such as neurophysiology and MRI along with a review of treatment and the role for corticosteroid therapy.

Dengue-associated longitudinally extensive transverse myelitis.

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

MOG-IgG testing strategies in accordance with the 2023 MOGAD criteria: a clinical-laboratory assessment.

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

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.

Acute Necrotizing Myelitis Associated with COVID-19.

Acute ascending hemorrhagic longitudinally extensive transverse myelitis is a rare inflammatory demyelinating disorder, which invades several vertebral segments and progresses rapidly and manifests severe symptoms. We present a case of acute necrotizing myelitis associated with COVID-19 infection. A 10-year-old female, with no previous medical history and no prior administration of COVID-19 vaccination, contracted COVID-19 in early April 2022. Two weeks later, she suffered from severe posterior neck pain and also presented with motor weakness and numbness in both lower extremities, making it difficult to walk independently and spontaneously void urine. Initial spinal cord MR showed longitudinally segmental extensive T2 hyperintensities. Cerebrospinal fluid (CSF) analysis revealed elevated red blood cell, normal white blood cell, and elevated protein levels and absence of oligoclonal bands. CSF culture and viral polymerase chain reaction were negative. Autoimmune work-up was negative. She was started on intravenous methylprednisolone 1g/day for 5 days and immunoglobulin (Ig) 2 g/kg for 5 days. She was also treated with six courses of therapeutic plasma exchange. Nevertheless, her pain and motor weakness persisted. She eventually developed respiratory failure. Follow-up MR presented a newly noted small hemorrhagic component. She was consequently treated with two additional courses of methylprednisolone and Ig. At 6-months follow-up, neurological examination showed improvement with normal sensory function and motor grade IV function in both upper extremities. We present the case of acute necrotizing myelitis associated with COVID-19 infection. Multiple courses of methylprednisolone and Ig showed mild improvement in motor and sensory function. However, poor prognosis was unavoidable due to rapid progression of the 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.

Transverse myelitis in children and adults.

Transverse myelitis is a noncompressive myelopathy of inflammatory origin. The causes are broad, ranging from infective or toxic to immuno-mediated etiology. They can be manifestations of systemic diseases, such as sarcoidosis and systemic lupus erythematous, or phenotypes of neuroinflammation; in a portion of cases, the etiology remains unknown, leading to the designation idiopathic. The clinical presentation of transverse myelitis depends on the level of spinal cord damage and may include sensorimotor deficits and autonomic dysfunction. The age of onset of the disorder can impact the symptoms and outcomes of affected patients, with differences in manifestation and prognosis between children and adults. Spinal cord magnetic resonance imaging and cerebrospinal fluid examination are the main diagnostic tools that can guide clinicians in the diagnostic process, even though the search for antibodies that target the structural components of the neural tissue (anti-aquaporin4 antibodies and anti-myelin-oligodendrocyte antibodies) helps in the distinction among the immune-mediated phenotypes. Management and outcomes depend on the underlying cause, with different probabilities of relapse according to the phenotypes. Hence, immunosuppression is often recommended for the immune-mediated diseases that may have a higher risk of recurrence. Age at onset has implications for the choice of treatment.

Infectious Myelitis.

Myelitis is an extensive group of pathologies, including inflammatory, demyelinating, and infectious disorders, sometimes mimicking tumors. This article will discuss infectious myelitis, mainly the patterns of spinal cord involvement caused by each infectious agent and the contribution of magnetic resonance imaging as a major tool to establish the specific diagnosis.

A Case of Transverse Myelitis Following Treatment with Atezolizumab for Advanced Hepatocellular Carcinoma.

The results of the IMbrave150 study have led to widespread use of the combination therapy of atezolizumab and bevacizumab as a first-line treatment for unresectable or metastatic hepatocellular carcinoma (HCC). Compared to traditional cytotoxic chemotherapy agents, immune checkpoint inhibitors show a spectrum of side effects ranging from mild side effects such as skin rash to potentially severe systemic effects such as myocarditis. We present a case of transverse myelitis diagnosed during the treatment of HCC with atezolizumab and bevacizumab combination therapy.

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.

Comparison of acute flaccid myelitis and transverse myelitis in children and evaluation of diagnostic criteria.

BACKGROUND AND PURPOSE: Acute flaccid myelitis (AFM) and transverse myelitis (TM) are serious conditions that may be difficult to differentiate, especially at onset of disease. In this study, we compared clinical features of pediatric AFM and TM and evaluated current diagnostic criteria, aiming to improve early and accurate diagnosis. METHODS: Two cohorts of children with enterovirus D68-associated AFM and clinically diagnosed TM were compared regarding presenting clinical features, additional investigations, and outcome. Current diagnostic criteria for AFM and TM were applied to evaluate their specificity. RESULTS: Children with AFM (n = 21) compared to those with TM (n = 36) were younger (median 3 vs. 10 years), more often had a prodromal illness (100% vs. 39%), predominant proximal weakness (69% vs. 17%), and hyporeflexia (100% vs. 44%), and less often had sensory deficits (0% vs. 81%), bowel and/or bladder dysfunction (12% vs. 69%), and hyperreflexia (0% vs. 44%). On magnetic resonance imaging, brainstem involvement was more common in AFM (74% vs. 21%), whereas supratentorial abnormalities were only seen in TM (0% vs. 40%). When omitting the criterion of a sensory level, 11 of 15 (73%) children with AFM fulfilled the diagnostic criteria for TM. Of children with TM, four of 33 (12%) fulfilled the diagnostic criteria for probable/definite AFM. CONCLUSIONS: Although there is considerable overlap between AFM and TM in children, we found important early differentiating clinical and diagnostic features. Meeting diagnostic criteria for AFM in children with TM and vice versa underlines the importance of thorough clinical examination and early and accurate diagnostic studies.

Current perspectives on the diagnosis and management of acute transverse myelitis.

INTRODUCTION: Acute transverse myelitis (ATM) is a term that encompasses a wide range of etiologies from immune-mediated to infectious. Management and prognosis differ for each specific etiology, and thus determining the disease-specific diagnosis of ATM is crucial. AREAS COVERED: The distinguishing clinical, radiologic, serologic, and cerebrospinal fluid features for common etiologies of ATM, such as multiple sclerosis, aquaporin-4-IgG-positive neuromyelitis optica spectrum disorder (AQP4+NMOSD), myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD), and spinal cord sarcoidosis, are covered. Acute flaccid myelitis variant of ATM is also explored. Red flags suggesting ATM mimics are briefly reviewed. Management of ATM in this review mainly focuses on treatment for immune-mediated causes and is divided into acute treatment, preventive treatment for certain etiologies, and supportive management. Although maintenance attack-prevention treatment for immune-mediated ATM is mainly guided by observational studies and expert opinion, clinical trials have been completed in AQP4+NMOSD and are underway in MOGAD to help provide solid evidence for treatment efficacy. EXPERT OPINION: The term ATM should be replaced by a disease-specific diagnosis to direct management. Discovery of disease-associated antibodies has changed the landscape of ATM diagnosis and allowed research on disease mechanisms. Translating our knowledge on pathophysiology into targeted therapy with monoclonal antibodies has provided new treatment options for patients.

Extensive longitudinal acute transverse myelitis complicated by pulseless ventricular tachycardia and recent shingles vaccination.

This case describes a 50-year-old male with a history of psoriatic arthritis who presented to the emergency department with a chief complaint of ascending bilateral lower extremity paresthesia one week following a shingles vaccine. MRI of the patient's spine was significant for longitudinally extensive T2 hyperintensity involving the lower cervical spine with extension into the upper thoracic spine suggestive of acute transverse myelitis (ATM). The patient's hospital course was complicated by a self-limiting episode of pulseless ventricular tachycardia accompanied by a brief loss of consciousness. Initial treatment included IV solumedrol, however due to lack of clinical improvement after a 5-day steroid treatment, plasmapheresis was initiated. The patient's condition improved with plasmapheresis and he was subsequently discharged to a rehab facility with a diagnosis of ATM of unclear etiology. Extensive serology, cardiac and CSF studies failed to determine the cause of this patient's myelitis or pulseless ventricular tachycardia. The following case report explores the potential factors that may have contributed to this patient's symptoms.

Transverse Myelitis as a Rare Neurological Complication of Coronavirus Disease 2019: A Case Report and Literature Review.

The novel Severe Acute Respiratory Syndrome Coronavirus 2 (SARS-CoV-2) is primarily a respiratory virus. However, an increasing number of neurologic complications associated with this virus have been reported, e.g., transverse myelitis (TM). We report a case of a 39-year-old man admitted to Namazi Hospital affiliated with Shiraz University of Medical Sciences, Shiraz, Iran. In December 2020, the patient was infected with Coronavirus Disease 2019 (COVID-19). During hospitalization, the patient suffered from sudden onset of paraplegia, and urinary retention, and had a T6-T7 sensory level. TM was diagnosed and an extensive workup was performed to rule out other etiologies. Eventually, para-infectious TM associated with COVID-19 was concluded. The patient received pulse methylprednisolone therapy of 1 g/day for 10 consecutive days followed by seven sessions of plasma exchange without a favorable response. The patient then underwent regular physical rehabilitation and tapering oral administration of prednisolone 1 mg/Kg. As a result, weakness in the lower extremities improved slightly after six months. Overall, we suspect a correlation between COVID-19 and TM, however, further studies are required to substantiate the association.

Constipation in transverse myelitis.

Transverse myelitis is an extremely rare neuroinflammatory disorder. About half of the affected patients develop paraplegia associated with urinary and bowel dysfunction. The bowel dysfunction is thought to be benign and is usually managed with dietary management and laxatives. We report a case of a man in his 60s presenting with transverse myelitis and the acute disease course complicated with treatment refractory intestinal dysfunction resulting in intestinal perforation, eventually leading to his death. Thus, this case helps us weigh the fact that intestinal dysfunction in the case of transverse myelitis is not always benign but can lead to deadly outcomes as well.

Anti-myelin Oligodendrocyte Glycoprotein Antibody-positive Myelitis after Coronavirus Disease 2019.

We herein report a case of anti-myelin oligodendrocyte glycoprotein (MOG) antibody-related myelitis caused by coronavirus disease (COVID-19) infection in 2021. A 22-year-old man with no history of any related illness contracted COVID-19. Eight days later, he developed bladder problems, paraplegia and sensory disturbances. Cervical spinal cord magnetic resonance imaging revealed extensive hyperintensity at T2 and spinal cord lesions extending from C4 to Th1. The patient was diagnosed with transverse myelitis and started on intravenous methylprednisolone, plasma exchange and intravenous immunoglobulin therapy. The symptoms improved only after intravenous methylprednisolone therapy. Anti-MOG antibodies were found in his serum and cerebrospinal fluid during routine screening. As this observation is unusual and could cause serious health problems, we wonder if COVID-19 triggered this autoimmune response.

Pediatric acute flaccid myelitis: Evaluation of diagnostic criteria and differentiation from other causes of acute flaccid paralysis.

BACKGROUND: Acute flaccid paralysis (AFP) is characterized by rapidly progressive limb weakness with low muscle tone. It has a broad differential diagnosis, which includes acute flaccid myelitis (AFM), a rare polio-like condition that mainly affects young children. Differentiation between AFM and other causes of AFP may be difficult, particularly at onset of disease. Here, we evaluate the diagnostic criteria for AFM and compare AFM to other causes of acute weakness in children, aiming to identify differentiating clinical and diagnostic features. METHODS: The diagnostic criteria for AFM were applied to a cohort of children with acute onset of limb weakness. An initial classification based on positive diagnostic criteria was compared to the final classification, based on application of features suggestive for an alternative diagnosis and discussion with expert neurologists. Cases classified as definite, probable, or possible AFM or uncertain, were compared to cases with an alternative diagnosis. RESULTS: Of 141 patients, seven out of nine patients initially classified as definite AFM, retained this label after further classification. For probable AFM, this was 3/11, for possible AFM 3/14 and for uncertain 11/43. Patients initially classified as probable or possible AFM were most commonly diagnosed with transverse myelitis (16/25). If the initial classification was uncertain, Guillain-Barré syndrome was the most common diagnosis (31/43). Clinical and diagnostic features not included in the diagnostic criteria, were often used for the final classification. CONCLUSION: The current diagnostic criteria for AFM usually perform well, but additional features are sometimes required to distinguish AFM from other conditions.