The role of plasmapheresis in severe acute disseminated encephalomyelitis with clinical findings of transverse myelitis.

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

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.

COVID-19-associated transverse myelitis treated by therapeutic plasma exchange: A case report.

BACKGROUND: Transverse myelitis (TM) is a very uncommon condition in children which can be associated with viral infections. Acute TM cases have been reported after Coronavirus disease 2019 (COVID-19) infection during the pandemic. CASE REPORT: We report a child with TM related to severe acute respiratory syndrome coronavirus 2, who was successfully treated with therapeutic plasma exchange (TPE). Inability to walk and urinary retention were the central nervous system symptom. Spinal magnetic resonance imaging revealed signal changes in the spinal cord. Her neurological symptoms worsened despite receiving IVIG and high-dose steroids for the first 3 d. We performed 10 TPE sessions with 5% albumin replacement and the neurological symptoms rapidly improved. CONCLUSION: We demonstrated that a child diagnosed with acute TM related to COVID-19 infection, was successfully treated with TPE.

A case report of longitudinal extensive transverse myelitis: immunotherapy related adverse effect vs. COVID-19 related immunization complications.

Background: Transverse myelitis (TM) is a rare, acquired neuro-immunological spinal cord disorder that occurs with rapid onset of motor weakness, sensory deficits with bowel and bladder dysfunction. Patients being treated with immune checkpoint inhibitors (ICIs) for advanced malignancy have a known higher propensity of developing neuro immune complications. With the advent of COVID-19 pandemic there have been reported cases of TM with COVID-19 immunization. The reported infrequency of TM with both of the aforementioned causes makes delineation of the etiology challenging.Methods: We present a patient with metastatic small cell lung cancer (SCLC) on maintenance Atezolizumab immunotherapy who developed longitudinal extensive transverse myelitis (LETM) after administration of second dose of COVID-19 mRNA vaccine one day prior to presenting symptoms of acute paralysis of the lower extremity, sensory loss from chest down with overflow incontinence. A clinical diagnosis of myelopathy was supported by MRI of the spine illustrating enhancing lesions from C7-T7 concerning for LETM.Results: A 5-day course of pulsed methylprednisolone followed by therapeutic plasma exchange for 3 days resulted in only minimal improvement in the neurologic exam with increased strength in his lower extremities while the sensory level remained unchanged.Conclusions: This case demonstrates the complication and symptomatology of TM in the setting of anti-PD-L1 monoclonal antibody with coincidental COVID-19 mRNA vaccine administration. The causal relationship between the vaccine and LETM is difficult to establish. However, the presence of a known inciting factor hints at a possible exaggeration of the existing neuro-inflammatory process.

Rapid Onset of Relapsing Longitudinally Extensive Transverse Myelitis After Lumbar Spinal Surgery.

A 58-year-old woman experienced relapsing acute longitudinally extensive transverse myelitis that developed rapidly in 3 days after lumbar surgery. The patient had a history of systemic lupus erythematosus with acute transverse myelitis and had undergone plasmapheresis 16 years ago. New neurologic deficits including paraplegia of the lower limbs, sensory alterations, and bowel incontinence presented 3 days postoperatively. Magnetic resonance imaging revealed a long-segment hyperintense signal over the thoracic spine on T2-weighted imaging. Intravenous pulse therapy with high-dose corticosteroid was first used for 5 days but was ineffective. Plasmapheresis after pulse therapy resulted in improved neurologic deficit. The patient then underwent 6 months of rehabilitation therapy but was partially wheelchair bound. She no longer had bladder and bowel incontinence.

COVID-19 vaccine associated transverse myelitis-Evusheld as an option when vaccination is not recommended due to severe adverse events.

Individuals who experience severe COVID-19-vaccine-related adverse reactions such as transverse myelitis may be precluded from receiving further vaccination to protect from SARS-CoV-2 infection. Although the mechanism of autoimmune spinal cord inflammation resulting in transverse myelitis is unclear, it may be safe to administer antibody therapy for preventing COVID-19. Recently, Evusheld, tixagevimab with cilgavimab, two spike-protein directed monoclonal antibodies were authorized by the U.S. FDA and U.K. MHRA for administration to individuals when vaccination is not recommended. We report the safe administration of Evusheld to a patient who experienced transverse myelitis 11 months previously as a result of receiving the Moderna mRNA vaccine. This patient has experienced no adverse events to Evusheld. Additional experience and data collection are warranted to determine the safety of this prophylactic therapy.

Acute demyelinating encephalomyelitis and transverse myelitis in a child with COVID-19.

BACKGROUND: Corona virus disease 2019 (COVID-19) includes a wide range of diseases with varying pathophysiology in children and adults. Although the disease mainly affects the respiratory tract, neurological involvement is also reported in the literature. The most common neurological complaints due to COVID-19 are headache, dizziness and anosmia. Acute necrotizing myelitis, acute demyelinating encephalomyelitis (ADEM), acute axonal neuropathy, acute transverse myelitis, and Guillian-Barre syndrome have been reported as neurological dysfunctions associated with COVID-19. CASE: A ten-year-old male patient presented with complaints of fever, headache and generalized muscle pain. The patient developed inability to walk and significant muscle weakness during the disease course, and he was diagnosed with ADEM and transverse myelitis on magnetic resonance imaging (MRI). As the etiological agent, COVID-19 was detected in both the respiratory panel sample and the cerebrospinal fluid (CSF) sample by the polymerase chain reaction (PCR) technique. Pulse steroid, IVIG, and plasmapheresis treatment were administered. He started to stand with support during follow-up. CONCLUSION: We presented a case of COVID-19 related ADEM and transverse myelitis who responded to pulse steroid, IVIG, and plasmapheresis.

Longitudinal extensive transverse myelitis after chemoradiation therapy with durvalumab, a rare complication: case report.

BACKGROUND: Longitudinal extensive transverse myelitis is a rare and potentially life-threatening complication of chemoradiation. Certain chemotherapy agents have been proposed to increased neurotoxicity with chemoradiation therapy. One such agent is durvalumab, a human IgG1 monoclonal antibody that blocks programmed death ligand 1, allowing T-cells to recognize and kill tumor cells. Durvalumab and other immune checkpoint inhibitors may also cause transverse myelitis without concomitant treatment with radiation. Durvalumab is a standard therapy for non-small cell lung carcinoma. Here we present a case of a 68-year-old male who presented after chemoradiation and durvalumab therapy with transverse myelitis extending outside the irradiation site. CASE PRESENTATION: A 68-year-old male presented to the emergency department with pain and weakness in his feet and hesitancy of urination. Medical history is significant for non-small cell lung cancer treated with chemoradiotherapy and consolidation therapy with durvalumab for one year. His last radiation treatment was 15 months prior, and his last infusion of durvalumab was 3 months prior. Exam revealed severe weakness of bilateral legs with absent vibration sensation. MRI showed central longitudinal extensive transverse myelitis extending from C4-T11. CSF studies showed 8 WBC with 63% lymphocyte predominance and a protein of 48. Oligoclonal bands and angiotensin-converting enzyme were negative. Serum Neuromyelitis Optica antibody (AQP4-IgG) and Myelin oligodendrocyte glycoprotein antibody (MOG-IgG) were negative. Infectious workup came back negative. The patient was treated with steroids and plasma exchange with mild improvement. Etiology remained unknown, but longitudinal extensive transverse myelitis following durvalumab chemoradiotherapy was thought to be the likely cause. He was discharged on a high-dose prednisone taper with outpatient follow-up. His condition worsened near the end of the steroid taper. High-dose prednisone and cyclophosphamide infusions were started with mild improvement and stabilization of the patient's condition. He transitioned to methotrexate after completion of six cyclophosphamide infusions. The patient expired due to complications from his cancer. CONCLUSION: Longitudinal extensive transverse myelitis is a rare and potentially life-threatening complication of durvalumab therapy. As durvalumab has become a standard treatment for non-small cell lung cancer, it is important to be able to identify and treat side effects.

Physiotherapy management of acute transverse myelitis in a pediatric patient in a Nigerian hospital: a case report.

BACKGROUND: Transverse myelitis is a rare neurological disorder of the spinal cord, caused by inflammation and damage of the myelin sheath of the neurons of the spinal cord across one or more spinal segments. This causes a disruption in the passage of nervous signals leading to motor, sensory, and autonomic dysfunction. This affects the physical and psychological health, as well as the functional status of the patient. This case report presents the physiotherapy evaluation and management of acute transverse myelitis in a pediatric patient. CASE PRESENTATION: A 17-year-old Nigerian male diagnosed with acute transverse myelitis was referred to the physiotherapy team for expert management. The patient presented with severe muscle spasms and frequent jerking movements, shocking sensations, hypertonicity, and spasticity (modified Ashworth scale: 1+ on the right, > 2 on the right), and muscle strength of the lower limbs (Oxford muscle grading: 3/5 on the left, 1/5 on the left) with impaired functional status (Functional Independence Measure: 70/126).The patient tolerated and participated in the physiotherapy interventions (cryotherapy, soft tissue mobilization, splinting) and exercises (free active, resistance and functional exercises) in the ward and outpatient clinic, as well as subsequent home programmes (free active, resistance and functional exercises). The patient also received other medical and pharmacological interventions in the ward. After 23 days of therapy, the patient improved in all clinical outcomes, including muscle spasm and hypertonicity, spasticity (modified Ashworth scale: 0 bilaterally), sensation, and muscle strength (Oxford muscle grading: 5/5 bilaterally). The patient's overall functional status also improved (Functional Independence Measure: 117/126). CONCLUSIONS: Physiotherapy improved the symptoms of acute transverse myelitis in this patient. Randomized controlled trials are required to replicate these findings.

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.

Approach to Myelopathy and Myelitis.

Myelopathy can present acutely or more insidiously and has a broad differential diagnosis. In addition to the clinical history and neurologic examination, diagnostic testing, including MRI and cerebrospinal fluid analysis, as well as thorough review of patient comorbidities, risk factors, and potential toxic exposures, can help neurohospitalists distinguish between various causes and potentially start appropriate empiric therapy while awaiting definitive testing. This article focuses on how imaging can help in determining the most likely cause of myelopathy and highlights a range of causes, including compressive, vascular, metabolic and toxic, infectious, autoimmune, neoplastic, and paraneoplastic causes of spinal cord dysfunction.

Longitudinally extensive transverse myelitis in childhood: Clinical features, treatment approaches, and long-term neurological outcomes.

AIM: Longitudinally extensive transvers myelitis (LETM) is a rare and disabling condition in childhood. The aim of the present study was to share experiences from our center regarding the treatment features and clinical and radiologic course in our LETM patients in light of the literature data. MATERIAL AND METHOD: The study was designed as cross-sectional and included children who followed for LETM at our pediatric neurology clinic between 2010 and 2019. ATM was diagnosed according to the diagnostic criteria report from the Transverse Myelitis Consortium Working Group. LETM was defined as the presence of spinal cord lesions spanning a length of 3 or more consecutive vertebral segments. The patients' medical records were examined in terms of demographic characteristics, presenting symptoms, history of infection prior to and during LETM, prodromal history, neurological examination, laboratory and radiological findings, clinical course, and treatment. The Barthel Index was used to assess the physical independence in activities of daily living of patients with LETM who were followed for at least one year. RESULTS: A total of 15 (8 girl) patients were included in the study. The patients were between 1 and 17 years of age. Presenting symptoms included inability to walk in 12 patients, incontinence in 9 patients, low back pain in 4 patients, abdominal pain in 2 patients, and inability to use the arms in 2 patients. In Barthel Index assessment of physical independence in activities of daily living, 8 patients were evaluated as completely independent, 3 patients as moderately dependent, and 2 patients as slightly dependent. When the 4 patients with motor area impairment and moderate dependency according to the Barthel Index were examined, it was noted that all of them had been admitted 4 days after the onset of symptoms and that 2 (13.3%) had cervicothoracic involvement and 2 (13.3%) had involvement of the entire cord. CONCLUSION: Shorter delay from symptom onset to initiation of immunomodulatory therapy as well as effective rehabilitation resulted in favorable outcomes, with the most noticeable improvement in the areas of motor function and incontinence.

Bladder management in children with transverse myelitis.

BACKGROUND: Patients with transverse myelitis (TM) often present with urinary retention. While many recover their bladder function, some have persistent voiding dysfunction, and both intermediate and long-term outcomes are variable. OBJECTIVE: In patients who develop urinary retention requiring clean intermittent catheterization (CIC) at onset of TM, we sought to assess factors associated with improved voiding function and the risk of requiring persistent CIC over time. STUDY DESIGN: We reviewed children evaluated at our institution for TM from April 1998 to October 2018. Patients were included if they required CIC at initial presentation of TM. Demographics, initial and follow up neurologic exams were evaluated for their association with a return to baseline volitional voiding after requiring catheterization upon diagnosis of TM, with or without medical therapy. RESULTS: Among the 78 patients who presented with TM during the study period, 43 patients required CIC, with median follow up of 2.7 years. When evaluating for demographic or sensorimotor features associated with improvement to baseline voiding function in patients who initially required CIC, preserved lower extremity reflexes at presentation was the only significant prognostic factor (p < 0.05). Additionally, having complete lower motor neurologic recovery was associated with volitional voiding (p < 0.05). Among the 43 patients who were initially catheterizing, 27/43 (62%) were volitionally voiding at median follow up of 7 months from initial presentation, while the remaining 16/43 remained on CIC for a median follow up of 3.6 years. The cumulative risk of remaining on CIC was 60%, 47%, and 42% at 1, 5, and 10 year follow up, respectively, though there was not a significant difference in the rate of bladder recovery if patients had preserved reflexes. DISCUSSION: In children with TM who initially developed urinary retention, intact reflexes at presentation were associated with urologic recovery. Additionally, complete neurologic recovery was associated with volitional voiding. While 62% were volitionally voiding at most recent follow-up, the cumulative incidence of dependence on CIC within the first year of diagnosis was 60%, with a relatively few patients regaining volitional voiding by 10 years of follow-up. CONCLUSION: Among those initially evaluated for urinary retention in the setting of transverse myelitis, intact lower extremity reflexes on physical exam was associated with improved voiding function at most recent follow-up. However, more than half the patients on CIC at initial presentation required CIC at one year of follow-up. Careful, long-term monitoring of voiding status in patients with TM is recommended, even with improvement of neurological status.

Spinal cord stimulator for neuropathic pain in a patient with severe disability due to transverse myelitis.

Transverse myelitis is an inflammatory disease of the central nervous system that disrupts nerve signals' conduction. The illness is characterised by weakness in the lower limbs accompanied by paresthesia and urinary and bowel incontinence. The most disabling sequel is the onset of chronic neuropathic pain, which can severely limit the patient's independence and negatively affect her quality of life. We present the case of a patient who received a spinal neurostimulator after a failure of conventional medical treatment. Masking pain through paresthesia, a mechanism provided by the device significantly reduces pain perception. The treatment success in our patient represents an advance in pain therapy.

Acute transverse myelitis after SARS-CoV-2 infection: a rare complicated case of rapid onset paraplegia.

A 63-year-old Caucasian male, known case of controlled type 2 diabetes, chronic renal failure, and ischemic heart disease, was presented with weakness and loss of movement in lower limbs, an absent sensation from the chest below, constipation, and urinary retention. About 4 days before these symptoms, he experienced a flu-like syndrome. Suspicious for COVID-19, his nasopharyngeal specimen's reverse transcription-polymerase chain reaction (RT-PCR) resulted positive. Chest X-ray and HRCT demonstrated severe pulmonary involvement. Immediately, he was admitted to the emergency ward, and the treatment was started according to the national COVID-19 treatment protocol. Subsequently, diagnostic measures were taken to investigate the patient's non-heterogeneous peripheral (spinal) neuromuscular manifestations. Brain CT scan and MRI were normal, but spinal MRI with gadolinium contrast showed extensive increased T2 signal involving central gray matter and dorsal columns, extended from C7 to T12 with linear enhancement in the sagittal plane, posteriorly within the mid and lower thoracic cord. The CSF specimen demonstrated pleocytosis, positive RT-PCR for SARS-CoV-2, and elevated IgG index. Clinical presentation, MRI, CSF, and laboratory findings prioritized the acute transverse myelitis (ATM) as a probable complication of COVID-19 infection over other differential diagnoses. Intravenous methylprednisolone and, subsequently, IV human immunoglobulin were added to the treatment regimen. In the end, the complete resolution of dysesthesia, urinary retention, and constipation were achieved. After continuous and extended respiratory and motor rehabilitation programs, he was discharged asymptomatic.

Myelitis and Other Autoimmune Myelopathies.

PURPOSE OF REVIEW: This article provides an update on the clinical diagnosis and management of immune-mediated myelopathies, including the relevance of imaging, ancillary testing with an emphasis on autoantibody biomarkers, recognition of myelitis mimics, and therapeutic approach. RECENT FINDINGS: The imaging characterization of immune-mediated myelopathies and the discovery of neural autoantibodies have been crucial in improving our ability to accurately diagnose myelitis. The identification of autoantibodies directed against specific central nervous system targets has led to major improvements in our understanding of the mechanisms underlying inflammation in myelitis. It has also allowed distinction of these myelopathy etiologies from noninflammatory etiologies of myelopathy and from multiple sclerosis and provided insight into their risk of recurrence, treatment response, and long-term clinical outcomes. Prompt recognition and appropriate testing in the setting of acute and subacute myelopathies is critical as timely administration of immunotherapy can help improve symptoms and prevent permanent neurologic disability. A patient should not be classified as having "idiopathic transverse myelitis" without a comprehensive evaluation for a more specific etiology. Achieving the correct diagnosis and learning to recognize noninflammatory myelitis mimics is crucial as they have therapeutic and prognostic implications. SUMMARY: Identifying the clinical and radiographic features of immune-mediated myelitis and recognizing mimics and pitfalls will help clinicians treat confirmed autoimmune myelitis appropriately.

COVID-19, HHV6 and MOG antibody: A perfect storm.

BACKGROUND: Serious neurological complications of SARS-CoV-2 are increasingly being recognized. CASE: We report a novel case of HHV6 myelitis with parainfectious MOG-IgG in the setting of COVID-19-induced lymphopenia and hypogammaglobulinemia. The patient experienced complete neurological recovery with gancyclovir, high dose corticosteroids, and plasma exchange. To our knowledge, this is the first case of HHV6 reactivation in the central nervous system in the setting of COVID19 infection and the first case of MOG-IgG myelitis in the setting of SARS-CoV-2 and HHV6 coinfection. CONCLUSION: Patients with neurological manifestations in the setting of COVID19-related immunodeficiency should be tested for opportunistic infections including HHV6. Viral infection is a known trigger for MOG-IgG and therefore this antibody should be checked in patients with SARS-CoV-2 associated demyelination.