A Rare Case of Longitudinally Extensive Transverse Myelitis Following Febrile Illness: A Case Report.

Longitudinally extensive transverse myelitis (LETM) is a rapidly progressing demyelinating disease affecting the spinal cord over three or more vertebral segments. Most causes are idiopathic, while others include infections, autoimmune causes, central nervous system demyelinating diseases, and post vaccination. Here, we report a 37-year-old male who presented with a fever for six days with no source of infection and complained of pain and weakness in the bilateral lower limbs eight hours after admission. Though the neurological examination of the lower limbs was normal at that time, reduced power was detected 16 hours later, with loss of proprioception and sensation of pain with a sensory level at T4 vertebrae. Then, the patient became unable to vocalize, and the chest X-ray showed an elevated left hemidiaphragm. Thirty hours after admission, the patient went into type 2 respiratory arrest and was intubated. The magnetic resonance imaging (MRI) showed a longitudinally extensive transverse myelitis extending from the C2 vertebrae to the conus medullaris. Febrile illness is common in the medical setting in Sri Lanka, but its association with LETM is unusual. Since LETM is very rare and is a rapidly progressive disease, a high degree of clinical suspicion is crucial for early diagnosis and the initiation of treatment. This case underscores the importance of early diagnosis, which would require timely MRI, and prompt treatment with intravenous (IV) glucocorticoids or plasma exchange to reduce morbidity and mortality.

Post-infectious Transverse Myelitis Secondary to Hand, Foot, and Mouth Disease in a Pregnant Daycare Worker.

Transverse myelitis (TM) is a rare inflammatory disorder of the spinal cord that infections, vaccines, and autoimmune processes can cause or may have no discernible cause. About half of the cases are caused by an infection, usually a viral respiratory infection, flu-like illness, or sometimes a gastrointestinal infection. Although coxsackieviruses and enteroviruses are known to cause TM, it is more commonly associated with respiratory symptoms or systemic signs than a rash. In this case, we present a pregnant daycare worker who had a case of longitudinally extensive TM after an episode of hand, foot, and mouth disease (HFMD), which only showed the typical rash without fever or systemic signs.

Transverse Myelitis as a Presenting Symptom of Celiac Disease.

Celiac disease (CD) is associated with several neurological diseases. We report a case of a 25-year-old man with CD that was discovered during hospitalization for acute transverse myelitis. The diagnosis of CD was suspected after positive serological tests and was confirmed with duodenal biopsy. Steroid pulse therapy and plasma exchange stabilized the patient's condition. The patient started a gluten-free diet and rituximab therapy before discharge. Although the association of CD with various neurological diseases is well established, this case report describes a less explored association between CD and transverse myelitis.

Practical approach to the child presenting with acute generalised weakness.

Acute generalised muscle weakness in children is a paediatric emergency with a broad differential diagnosis. A careful history and neurologic examination guides timely investigation and management. We review some of the more common causes of acute generalised muscle weakness in children, highlighting key history and examination findings, along with an approach to lesion localisation to guide differential diagnosis and further investigation.

Concurrent Longitudinal Extensive Transverse Myelitis and Leptomeningitis in West Nile Virus: A Report of a Rare Case.

Here we report a rare case with concurrent longitudinal extensive transverse myelitis (LETM) and leptomeningitis due to West Nile virus infection. A 47-year-old man initially presented with a six-day progressive, intermittent low-grade fever, headache, diplopia, malaise, myalgia, lower back pain, and difficulty walking that developed into progressive asymmetric paralysis. Initial lab work was notable for mild lactic acidosis and hyperCKemia. Brain MRI with contrast demonstrated small foci of leptomeningeal enhancement in the cerebellum, pons, medulla, and right CN VI at the cisternal segment. MRI of the spine was remarkable for edema in the spinal cord extending from T10 to L1 with diffuse enlargement of the cord contour at T11 to L1 and subtle enhancement of nerve roots within the thecal sac and cauda equina regions. The patient responded partially to five-day intravenous immunoglobulin therapy (total dose, 2 g/kg). Electromyography four months after the onset of symptoms also showed chronic reinnervation with active denervating features in thoracolumbar myotomes. Clinically, this case highlights the ill-defined and non-specific nature of the presentation of West Nile neuroinvasive disease. It can pose a diagnostic challenge for clinicians and, if unrecognized, is associated with significant morbidity and mortality in older and compromised individuals.

Severe Spinal Cord Inflammation in a Young Woman Diagnosed With Systemic Lupus Erythematosus: A Case Study.

We describe a case of longitudinally extensive transverse myelitis (LETM), an uncommon and dangerous complication of systemic lupus erythematosus (SLE) that struck a 22-year-old woman with SLE. Chronic autoimmune illness (e.g., SLE) affects the skin, kidneys, joints, blood, and neurological system, among other organs. LETM is a condition where the spinal cord becomes inflamed and damaged, causing neurological problems, such as weakness, sensory loss, and bladder dysfunction. The patient presented with abdominal pain, vomiting, body aches, and fatigue, followed by shock, hypoxia, urinary retention, and constipation. Moreover, she had severe and asymmetric weakness, sensory loss, and areflexia in her limbs. She was diagnosed with LETM based on a nerve conduction study and MRI of the spine, which showed a motor neuron disease pattern and T2 hyperintense signals throughout the spinal cord gray and white matter. She responded well to immunoglobulins, plasma exchange, and high-dose steroids as treatment. Although her prognosis is favorable, there might be some lingering neurological issues or limitations. This instance highlights the significance of treating individuals with SLE as soon as possible after developing LETM.

Clinical Research into Central Nervous System Inflammatory Demyelinating Diseases Related to COVID-19 Vaccines.

Various vaccines have been developed in response to the SARS-CoV-2 pandemic, and the safety of vaccines has become an important issue. COVID-19 vaccine-related central nervous system inflammatory demyelinating diseases (CNS IDDs) have been reported recently. We present one case of AstraZeneca vaccine-related myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease and a literature review of another 78 patients published from January 2020 to October 2022. Patients were divided into three vaccine types (viral vector, mRNA, and inactivated vaccines) for further analyses. Among 79 patients with COVID-19 vaccine-related CNS IDDs, 49 (62%) cases received viral vector vaccines, 20 (25.3%) received mRNA vaccines, and 10 (12.7%) received inactivated vaccines. Twenty-seven cases (34.2%) were confirmed with autoantibodies, including fifteen patients (19%) with anti-MOG, eleven (13.9%) with anti-aquaporin 4 (AQP4), and one (1.3%) with both antibodies. Significantly, more males developed CNS IDDs post viral vector vaccines compared to mRNA and inactivated vaccines. Patients receiving mRNA vaccines were older than those receiving other types. Furthermore, mRNA and inactivated vaccines correlated more with anti-AQP4 antibodies, while viral vector vaccines showed higher MOG positivity. This research suggests potential associations between COVID-19 vaccine-related CNS IDDs and gender, age, and autoantibodies, contingent on vaccine types. Protein sequence analysis implies similarities between the S protein and AQP4/MOG. Further studies may elucidate the mechanisms of CNS IDDs, aiding vaccine selection for specific types.

Functional Recovery and Regenerative Effects of Direct Transcutaneous Electrical Nerve Stimulation in Treatment of Post-COVID-19 Guillain-Barré and Acute Transverse Myelitis Overlap Syndrome: A Clinical Case.

Transcutaneous electrical nerve stimulation (TENS) has proven effective in treating pain in many experimental and clinical studies. In addition to the analgesic effect, direct TENS of peripheral nerves had anti-inflammatory and regenerative effects in the treatment of distal polyneuropathy and spinal cord injury. This work demonstrates the experience of using direct TENS in the treatment of a 52-year-old patient with post-COVID-19 Guillain-Barré (GBS) and acute transverse myelitis (ATM) overlap syndrome. Direct TENS of peripheral nerves showed high efficiency in enhancing the therapeutic effect of combined plasma exchange and pharmacotherapy by 89.5% with a significant reduction in neuropathic pain, motor and sensory deficits, bladder and bowel disorders and regression of neurophysiological changes. We suggest that direct TENS of peripheral nerves can be a promising option for combined therapy of GBS and ATM overlap syndrome and other diseases with the simultaneous development of distal polyneuropathy and spinal cord injury. Further trial studies are required.

Acute Transverse Myelitis as an Unusual Complication of Dengue Fever: A Case Report and Literature Review.

Dengue fever, the most prevalent arbovirus disease, has a broad spectrum of clinical manifestations, ranging from asymptomatic to dengue hemorrhagic fever and dengue shock syndrome. Dengue fever has the potential to involve the nervous system. Acute transverse myelitis is a life-threatening complication of dengue fever, though rarely reported. We report a case of dengue fever-induced transverse myelitis in a 51-year-old male who presented with progressive paraplegia, sensory disturbance, and urinary retention preceded by a febrile illness, vomiting, and retro-orbital pain two weeks before. His serology was positive for immunoglobulin M (IgM) to dengue virus and non-structural protein (NS-1). Magnetic resonance imaging revealed hyperintense signals suggestive of acute transverse myelitis. After ruling out all other possible causes, a possible diagnosis of dengue fever-induced transverse myelitis was made. His condition improved gradually after starting methylprednisolone.

Lumbar decompression and fusion for symptomatic spinal stenosis in a patient with chronic thoracic sensory level from prior transverse myelitis: a case report.

BACKGROUND: Many patients with transverse myelitis suffer from sensory loss below the spinal level of the lesion. This is commonly associated with chronic neuropathic pain. However, the presence of somatic pain below a complete thoracic sensory level after transverse myelitis is exceptionally rare, and it is unclear if surgical decompression is an effective form of treatment for these patients. CASE PRESENTATION: In this report, we describe a 22-year-old Caucasian female who suffered from chronic lumbar back pain despite a complete thoracic sensory level secondary to prior transverse myelitis. Imaging demonstrated multilevel central stenosis below the sensory level, and her pain improved after surgical decompression. To our knowledge, this is the first reported case of symptomatic lumbar stenosis below a sensory level after transverse myelitis successfully treated with surgical decompression. CONCLUSION: This is the first reported case of a patient with symptomatic lumbar stenosis after transverse myelitis whose lower back pain and quality of life improved following surgical decompression and fusion. This case provides evidence that typical lumbago is possible in patients with sensory loss from transverse myelitis, and standard lumbar decompression may provide benefit for these patients.

Acute disseminated encephalomyelitis and transverse myelitis following COVID-19 vaccination - A self-controlled case series analysis.

Acute Disseminated Encephalomyelitis (ADEM) and Transverse Myelitis (TM) are within the group of immune mediated disorders of acquired demyelinating syndromes. Both have been described in temporal association following various vaccinations in case reports and case series and have been evaluated in observational studies. A recent analysis conducted by The Global Vaccine Data Network (GVDN) observed an excess of ADEM and TM cases following the adenoviral vectored ChAdOx1 nCoV-19 (AZD1222) and mRNA-1273 vaccines, compared with historically expected background rates from prior to the pandemic. Further epidemiologic studies were recommended to explore these potential associations. We utilized an Australian vaccine datalink, Vaccine Safety Health-Link (VSHL), to perform a self-controlled case series analysis for this purpose. VSHL was selected for this analysis as while VSHL data are utilised for GVDN association studies, they were not included in the GVDN observed expected analyses. The VSHL dataset contains vaccination records sourced from the Australian Immunisation Register, and hospital admission records from the Victorian Admitted Episodes Dataset for 6.7 million people. These datasets were used to determine the relative incidence (RI) of G040 (ADEM) and G373 (TM) ICD-10-AM coded admissions in the 42-day risk window following COVID-19 vaccinations as compared to control periods either side of the risk window. We observed associations between ChAdOx1 adenovirus vector COVID-19 vaccination and ADEM (all dose RI: 3.74 [95 %CI 1.02,13.70]) and TM (dose 1 RI: 2.49 [95 %CI: 1.07,5.79]) incident admissions. No associations were observed between mRNA COVID-19 vaccines and ADEM or TM. These findings translate to an extremely small absolute risk of ADEM (0.78 per million doses) and TM (1.82 per million doses) following vaccination; any potential risk of ADEM or TM should be weighed against the well-established protective benefits of vaccination against COVID-19 disease and its complications. This study demonstrates the value of the GVDN collaboration leveraging large population sizes to examine important vaccine safety questions regarding rare outcomes, as well as the value of linked population level datasets, such as VSHL, to rapidly explore associations that are identified.

Neurosarcoidosis and Transverse Myelitis: Life-Threatening Manifestations of Sarcoidosis.

Sarcoidosis, a systemic granulomatous disorder, typically involves the lungs, skin, and lymph nodes. Neurological manifestations are diverse and may include longitudinally extensive transverse myelitis (LETM), an uncommon inflammatory disorder of the spinal cord. We present a case of a 62-year-old female with LETM as the initial manifestation of sarcoidosis. The patient exhibited progressive bilateral lower extremity weakness, urinary retention, and sensory disturbances. Diagnostic workup revealed characteristic findings on spinal magnetic resonance imaging (MRI), cerebrospinal fluid analysis, and thoracic biopsy. Treatment with high-dose corticosteroids and subsequent immunomodulatory therapy resulted in significant improvement. Our case highlights the importance of including sarcoidosis in the differentials of LETM, particularly in patients with no respiratory manifestations.

Síndrome de Brown-Séquard como forma de presentación de mielitis transversa idiopática / Brown-Séquard syndrome as a presentation of idiopathic transverse myelitis

El síndrome de Brown-Séquard es el conjunto de signos y síntomas causado por hemisección medular de diversos orígenes. Puede generarse por múltiples causas; las traumáticas son las más frecuentes. Las causas menos frecuentes son patología inflamatoria, isquémica, tumoral o infecciosa. Se presenta un niño de 12 años, con instauración aguda y progresiva de un síndrome de hemisección medular derecho, con parálisis hipo/arrefléctica homolateral y afectación de sensibilidad termoalgésica contralateral. En la resonancia magnética de médula espinal, se observó compromiso inflamatorio en hemimédula derecha a nivel de segunda y tercera vértebras torácicas. Con diagnóstico de mielitis transversa idiopática, inició tratamiento con corticoide intravenoso a altas dosis con evolución clínica favorable y restitución de las funciones neurológicas.

Brown-Séquard syndrome refers to a set of signs and symptoms caused by hemisection of the spinal cord from various sources. It may have multiple causes; traumatic injuries are the most frequent ones. The less common causes include inflammation, ischemia, tumors, or infections. This report is about a 12-year-old boy with an acute and progressive course of right hemisection of the spinal cord, with ipsilateral hypo/areflexic paralysis and contralateral loss of thermalgesic sensation. The MRI of the spinal cord showed inflammation in the right side of the spinal cord at the level of the second and third thoracic vertebrae. The patient was diagnosed with idiopathic transverse myelitis and was started on intravenous high-dose corticosteroids; he showed a favorable clinical course and recovered neurological functions.

Postpartum Hypocupric Myelopathy Masquerading as Acute Transverse Myelitis: A Case Report and Literature Review of a Rare Presentation.

The symptoms of transverse myelitis, an acute demyelinating inflammatory condition of the spinal cord, include motor, sensory, and bowel-bladder dysfunction that can develop suddenly or gradually. Several etiologies, such as bacterial, fungal, or viral infections, cancer, autoimmune diseases, vascular problems, and environmental variables, can cause it. The identification of copper deficiency myelopathy (CDM) as a curable cause of non-compressive inflammatory myelopathy has only occurred recently. Patients frequently present with sensory complaints and a spastic gait. The neurological disease may exist independently of the hematologic signs. Only a few cases of copper myelopathy in peripartum women have been documented. Given that hypocupric myelopathy is a treatable cause of debilitating paraplegia, maintaining clinical vigilance will be crucial in minimizing neurological sequelae, as demonstrated in this case report.

'I Thought It Was My Diabetes': An Acute Presentation of Neuromyelitis Optica Spectrum Disorder.

Neuromyelitis Optica Spectrum Disorder (NMOSD) is an immune-mediated neuroinflammatory disease of the central nervous system. Patients typically present with sensory deficits, weakness, and incontinence. This is a case of a 43-year-old female with diabetes mellitus admitted for acute onset leg weakness and stool incontinence. Spinal MRI imaging revealed transverse myelitis, and her lab work was significant for an anti-aquaporin 4 (AQP4) antibody titer of 1:2,560. Initial treatment consisted of a high-dose steroid taper and plasmapheresis. This unique case illustrates the importance in recognizing delayed presentations of rare neuroinflammatory conditions previously assumed to be a sequela of diabetic neuropathy.

Acute encephalopathy with biphasic seizures and late reduced diffusion with concurrent transverse myelitis.

We describe a patient with acute encephalopathy with biphasic seizures and late reduced diffusion (AESD) with unique features, including concurrent transverse myelitis. A 2-year-old previously healthy girl had clinical findings consistent with AESD, occurring in association with influenza A infection. The posterior brain regions were most severely affected, resulting in cortical blindness. She also developed bilateral limb weakness, and spine MRI revealed transverse myelitis in the cervical region. She was treated acutely with intravenous methylprednisolone. Serum anti-myelin oligodendrocyte glycoprotein and anti-aquaporin-4 antibodies were negative, as was an anti-extractable nuclear antigen panel. Although her clinical presentation was severe, she improved dramatically over the following months, and 6 months following initial presentation, her parents felt she had returned to baseline. This is the first report of AESD occurring in combination with transverse myelitis. The co-occurrence of the two conditions is unlikely to be coincidental, suggesting that there may be a shared or overlapping immunological pathway involved. The patient's recovery was impressive, which could partially relate to the acute treatment with corticosteroids.

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

BACKGROUND: Isolated first episodes of longitudinally extensive transverse myelitis (LETM) have typically been associated with neuromyelitis optica spectrum disorder (NMOSD) or myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD). However, in some cases, serological testing and screening for other aetiologies are negative, a condition referred to as double seronegative longitudinally extensive transverse myelitis (dsLETM). OBJECTIVE: The objective of this study was to evaluate comparative outcomes of dsLETM, MOGAD-LETM and NMOSD-LETM. METHODS: Cohort study of LETM cases seen in the UK NMOSD Highly Specialised Service between January 2008 and March 2022. RESULTS: LETM = 87 cases were identified (median onset age = 46 years (15-85); median follow-up = 46 months (1-144); 47% NMOSD-LETM = 41 (aquaporin-4 antibodies (AQP4-IgG) positive = 36), 20% MOGAD-LETM = 17 and 33% dsLETM = 29). Despite similar Expanded Disability Status Scale (EDSS) at nadir, last EDSS was higher in AQP4-IgG and seronegative NMOSD-LETM (sNMOSD) (p = 0.006). Relapses were less common in dsLETM compared to AQP4-IgG NMOSD-LETM and sNMOSD-LETM (19% vs 60% vs 100%; p = 0.001). Poor prognosis could be predicted by AQP4-IgG (odds ratio (OR) = 38.86 (95% confidence interval (CI) = 1.36-1112.86); p = 0.03) and EDSS 3 months after onset (OR = 65.85 (95% CI = 3.65-1188.60); p = 0.005). CONCLUSION: dsLETM remains clinically challenging and difficult to classify with existing nosological terminology. Despite a similar EDSS at nadir, patients with dsLETM relapsed less and had a better long-term prognosis than NMOSD-LETM.

A Systematic Review of the COVID Vaccine's Impact on the Nervous System.

AIMS & OBJECTIVES: The objective of this study was to conduct a systematic review of research pertaining to the COVID-19 vaccine and its association with neurological complications. METHOD: We performed a comprehensive search of the literature using Google Scholar, PubMed, and NCBI databases from December 2021 to December 2022. For Google Scholar, PubMed, and NCBI databases we used the following key search terms: "neurological adverse effects", "COVID-19 vaccination", "SARS-CoV-2", CNS complications, and CNS adverse effects. Two reviewer authors individually searched and assessed the titles and abstracts of all articles. The third reviewer resolved the disagreement between them. Data were documented regarding title, study location, type of study, type of COVID-19 vaccine, type of neurological complications/adverse effects, and sample size. RESULTS: From our findings, it is confirmed that these neurological complications like GuillainBarre syndrome (23.6%), Neuromyelitis Optica spectrum disorder (5.5%), Neuropathy (6.9%), Transverse Myelitis (8.3%) and Acute disseminated Encephalomyelitis (4.1%) are majorly affected in most of the people. The increase in risks associated with SARS-CoV-2 infection far outweighs any previously reported associations with vaccination. CONCLUSION: We found no safety signal was observed between COVID-19 vaccines and the immune-mediated neurological events. Before assuming a causal relationship, the side effects of the COVID-19 vaccine should first be carefully examined to rule out known associated factors. Symptom onset was within two weeks of vaccination in the majority of cases; as such, this seems to be a high-risk period warranting vigilance.

Severe Mimic of Transverse Myelitis - Spontaneous Non-Traumatic Intramedullary Hematomyelia: A Case Report.

Spinal cord hemorrhage remains an uncommon vascular pathology with no standard approach to treatment. Intramedullary hematomyelia is the rarest subtype and has only been described in case reports, making it a diagnosis easier to miss. A 55-year-old male with history of polysubstance abuse presented for abrupt onset non-traumatic back pain that progressed over several days evolving into paraplegia, sensory loss up to the T3 dermatome, and incontinence of bowel and bladder. His MRI imaging of the cervical and thoracic spine was concerning for a T1 and T2 hyperintense lesion extending from the internal medulla to the upper thoracic spine causing edema. The initial differential diagnosis, based on history and progressive neurologic decline, included a longitudinally extensive transverse mellitus of unclear etiology. He received high dose steroids and plasma exchange without any clinical improvement. An extensive autoimmune, inflammatory, infectious, and demyelinating workup via serum and CSF analysis was unremarkable. He was transferred to a tertiary facility, where repeat MRI spinal imaging included GRE sequences that was significant for increased susceptibility. MRA imaging of the spine was completed showing no vascular malformation. Overall, the diagnosis of hematomyelia was felt to be most likely. Despite aggressive inpatient rehabilitation, the patient remains paraplegic and requires long term management consistent with severe spinal cord injury. This case highlights the importance of recognizing vascular syndromes as a cause of acute myelopathy, understanding the imaging findings of blood breakdown products on MRI, and the desperate need for successful therapeutic strategies to combat a potentially devastating neurologic process.