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.

The critical role of magnetic resonance imaging in the diagnosis of transverse myelitis: a case report.

INTRODUCTION: Transverse Myelitis is a rare inflammatory disorder of the spinal cord, characterized by the inflammation of the myelin sheath covering nerve fibers. Although rare, Transverse Myelitis holds significant clinical importance due to its potential life-altering consequences. The case report provides insight into the clinical presentation of Transverse Myelitis and the importance of Magnetic Resonance Imaging in confirming Transverse Myelitis. CASE PRESENTATION: A 27-year-old Nigerian female presented to a hospital facility after 2 months onset of paraplegia, urinary, and fecal incontinence. She was diagnosed with Acute Transverse Myelitis with Magnetic Resonance Imaging, a lacking imaging modality in Nigeria. On presentation, it was important to rule out spinal cord compression, a close differential to her presentation. Despite her late arrival at the facility, early diagnosis and prompt initiation of treatment with high-dose intravenous steroids and physiotherapy improved her quality of life. DISCUSSION: This case report reveals the poor health-seeking behavior in developing countries and the need for imaging modalities like Magnetic Resonance Imaging for improved diagnoses of rare neurological conditions such as Transverse Myelitis. The lack of healthcare infrastructure has led to clinical misdiagnosis, patient mismanagement, and underrepresentation of data in the country, underscoring the critical role of diagnostic tools for improved patient care pre-treatment and post-treatment. Additionally, follow-up of these patients is important to prevent the long-term sequelae of Transverse Myelitis like Neuromyelitis Optica or Multiple Sclerosis.

Transverse Myelitis in the Setting of Enterobius vermicularis (Pinworm) Infection: Case Report.

Myelitis is a rare inflammatory myelopathy, and known associated etiologies only account for a small number of causes. A significant percentage of cases have an unknown etiology and are considered idiopathic. With 64% to 68% of cases fitting into the idiopathic category, helminth infections, and specifically pinworm parainfections, should be considered in cases that would otherwise be classified as idiopathic. This case report outlines a pediatric patient diagnosed with myelitis given her progressive weakness, fussiness, refusal to bear weight as well as magnetic resonance imaging (MRI) demonstrating T2-hyperintense signal and/or T1 gadolinium enhancement, and/or positive cerebrospinal fluid (CSF) inflammatory markers. This patient had a negative evaluation for typical known etiologies for myelitis including no signs of multiple sclerosis and neuromyelitis optica spectrum disorder on brain MRI, oligoclonal banding and aquaporin-4 autoantibodies, and no evidence of bacterial or viral meningitis given normal cell counts and cultures in CSF. She was found to have a pinworm infection, suggesting a parasitic parainfectious etiology of her myelitis. This case outlines the first case noting the correlation between myelitis and pinworm infection in a pediatric patient.

Clinical and radiographic features of a cohort of adult and pediatric subjects in the Pacific Northwest with myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD).

BACKGROUND: Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) is a newly described clinical entity comprised of isolated or recurrent attacks of optic neuritis, transverse myelitis, acute disseminated encephalomyelitis (ADEM), encephalitis, or seronegative NMOSD. Prior studies report that 30-80 % of children and adults with MOGAD go on to have relapses though there are no reliable predictors. The objectives of this study were to (1) describe the demographic, clinical, and radiographic patterns of MOGAD at our center and (2) identify possible predictors of relapsing disease. METHODS: Single-center retrospective cohort study of pediatric and adult subjects with MOGAD evaluated at least once at our center between January 1, 2017 and September 30, 2022. Eligible subjects had a history of positive MOG-IgG and consistent clinical syndrome comprised of an initial attack of optic neuritis (ON), transverse myelitis (TM), ADEM, cerebral cortical encephalitis, seronegative neuromyelitis optica (simultaneous ON and TM), isolated brainstem or cerebellar syndrome, or other (not fitting into another group). Relapsing subjects or those remaining monophasic at 12 months were included in the analyses of predictors of relapsing disease. Covariates included age, sex, race/ethnicity, and index event phenotype. Unadjusted and adjusted risk ratios were calculated for pediatric and adult subjects. RESULTS: We describe the demographic, clinical, and radiographic characteristics of 58 subjects with MOGAD. Covariates from 48 subjects were analyzed for predictors of relapsing disease. In adults, Hispanics and non-White non-Hispanics were at increased risk of relapsing disease compared to non-Hispanic Whites [Adjusted RR 1.52 (95 % CI: 1.01, 2.30)]. There were no significant associations in the pediatric group. CONCLUSION: This study is the first to describe a cohort of MOGAD in the Pacific Northwest. Our findings highlight racial and ethnic differences in risk of relapsing MOGAD in adults. Further studies on racial and ethnic differences in MOGAD are needed to confirm these findings.

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.

Coexistence of longitudinally extensive transverse myelitis and diffuse midline glioma in the brainstem in an adolescent boy with acute flaccid paralysis.

We present the case of a previously healthy 13-year-old boy who was admitted to the emergency department with acute flaccid paralysis. Magnetic resonance imaging revealed radiological evidence of longitudinally extensive transverse myelitis. Additionally, homogeneous T2 signal increase was observed in the pons and medulla oblongata, initially indicating brainstem encephalitis. Subsequent evaluations confirmed a coexistence of diffuse midline glioma (DMG) in the brain stem alongside acute transverse myelitis (ATM). Children with ATM generally have a more favorable prognosis than adults. However, despite the implementation of advanced treatment methods, the patient's quadriplegia did not improve and resulted in spinal cord sequela atrophy. DMG exhibits an aggressive growth pattern and lacks a known curative treatment. This case represents an exceedingly rare synchronous occurrence of aggressive conditions, underscoring the importance of raising awareness among physicians. Furthermore, we aim to discuss the radiologic differential diagnosis, as this is the first documented instance in the literature.

The value of 3D T2-weighted SPACE sequence in the differential diagnosis of spinal arteriovenous fistula and acute transverse myelitis.

OBJECTIVE: Spinal arteriovenous fistulas (SAVF) was often neglected and misdiagnosed as acute transverse myelitis (ATM) due to its insidious onset and non-specific clinical symptoms. This study aims to investigate the differential diagnostic value of high-resolution T2-weighted volumetric sequence (3D sampling perfection with application-optimized contrasts using different flip-angle evolutions [SPACE]) in patients with SAVF and ATM. METHODS: Retrospectively analyzed the clinical and radiological findings of 32 SDAVF patients and 32 ATM patients treated at our institutions from May 2018 to January 2023. They all underwent conventional spinal MRI and T2-SPACE examination, compared their performance in identifying lesions, to estimate the value of T2 SPACE sequence in the diagnosis of SAVF and ATM patients. RESULTS: The clue of cauda equina area change (CEAC) in conventional MRI and T2-SPACE sequences is specific for the diagnosis of SAVF. The diagnostic model composed of perimedullary flow voids (PFV) and CEAC has good diagnostic performance (AUCMRI = 0.95; AUCSPACE = 0.935). Compared with conventional MRI, the T2-SPACE sequence has a higher detection rate, sensitivity, and negative predictive value for PFV and CEAC in SAVF patients, but lower specificity and positive predictive value. In T2-SPACE images, there are significant differences in the distribution range, quadrant, and maximum diameter of PFV vessels between SAVF and ATM patients. Moreover, T2-SPACE sequence can determine the site of fistula in most SAVF patients preferably, and the inter-rater agreement was good in the assessment of the fistula. CONCLUSION: The CEAC is a new and useful clue for the diagnosis of thoracolumbar SAVF. And T2-SPACE sequence can more intuitively observe the lesions of SAVF, has good differential diagnostic value for SAVF and ATM patients.

Transverse myelitis associated with Mpox infection.

Neurologic manifestations of mpox (monkeypox) infection are common. Rarely, transverse myelitis has been associated with mpox infection. We describe a case of longitudinally extensive transverse myelitis in a patient with recently diagnosed mpox, presenting as acute flaccid paraplegia. The patient underwent an extensive work-up that included serological and cerebrospinal fluid (CSF) testing and magnetic resonance imaging (MRI). They were treated with tecoviromat, high dose steroids, and intravenous immunoglobulin, followed by plasma exchange. Despite these interventions, there was minimal neurologic improvement. This case underscores the importance of instituting measures designed to prevent mpox infection, including public education initiatives.

Cervical myelitis: a practical approach to its differential diagnosis on MR imaging.

BACKGROUND: Differential diagnosis of non-compressive cervical myelopathy encompasses a broad spectrum of inflammatory, infectious, vascular, neoplastic, neurodegenerative, and metabolic etiologies. Although the speed of symptom onset and clinical course seem to be specific for certain neurological diseases, lesion pattern on MR imaging is a key player to confirm diagnostic considerations. METHODS: The differentiation between acute complete transverse myelitis and acute partial transverse myelitis makes it possible to distinguish between certain entities, with the latter often being the onset of multiple sclerosis. Typical medullary MRI lesion patterns include a) longitudinal extensive transverse myelitis, b) short-range ovoid and peripheral lesions, c) polio-like appearance with involvement of the anterior horns, and d) granulomatous nodular enhancement prototypes. RESULTS AND CONCLUSION: Cerebrospinal fluid analysis, blood culture tests, and autoimmune antibody testing are crucial for the correct interpretation of imaging findings. The combination of neuroradiological features and neurological and laboratory findings including cerebrospinal fluid analysis improves diagnostic accuracy. KEY POINTS: · The differentiation of medullary lesion patterns, i. e., longitudinal extensive transverse, short ovoid and peripheral, polio-like, and granulomatous nodular, facilitates the diagnosis of myelitis.. · Discrimination of acute complete and acute partial transverse myelitis makes it possible to categorize different entities, with the latter frequently being the overture of multiple sclerosis (MS).. · Neuromyelitis optica spectrum disorders (NMOSD) may start as short transverse myelitis and should not be mistaken for MS.. · The combination of imaging features and neurological and laboratory findings including cerebrospinal fluid analysis improves diagnostic accuracy.. · Additional brain imaging is mandatory in suspected demyelinating, systemic autoimmune, infectious, paraneoplastic, and metabolic diseases..

A score that predicts aquaporin-4 IgG positivity in patients with longitudinally extensive transverse myelitis.

BACKGROUND AND PURPOSE: Longitudinally extensive transverse myelitis (LETM) associated with aquaporin-4 autoantibodies (AQP4-IgG) can cause severe disability. Early diagnosis and prompt treatment are critical to prevent relapses. A novel score is described based on clinical and neuroimaging characteristics that predicts AQP4-IgG positivity in patients with LETM. METHODS: Patients were enrolled both retrospectively and prospectively from multiple Italian centers. Clinical and neuroimaging characteristics of AQP4-IgG positive and negative patients were compared through univariate and multivariate analysis. RESULTS: Sixty-six patients were included. Twenty-seven (41%) were AQP4-IgG positive and median age at onset was 45.5 years (range 19-81, interquartile range 24). Female sex (odds ratio [OR] 17.9, 95% confidence interval [CI] 2.6-381.9; p = 0.014), tonic spasms (OR 45.6, 95% CI 3.1-2197; p = 0.017) and lesion hypointensity on T1-weighted images (OR 52.9, 95% CI 6.8-1375; p = 0.002) were independently associated with AQP4-IgG positivity. The AQP4-IgG positivity in myelitis (AIM) score predicted AQP4-IgG positivity with 85% sensitivity and 95% specificity. Positive and negative likelihood ratios were 16.6 and 0.2 respectively. The inter-rater and intra-rater agreement in the score application were both excellent. CONCLUSIONS: The AIM score predicts AQP4-IgG positivity with good sensitivity and specificity in patients with a first episode of LETM. The score may assist clinicians in early diagnosis and treatment of AQP4-IgG positive LETM.

Marked central canal T2-hyperintensity in MOGAD myelitis and comparison to NMOSD and MS.

OBJECTIVE: To assess marked central canal T2-hyperintensity in patients with myelin-oligodendrocyte glycoprotein antibody-associated disease (MOGAD) myelitis compared to myelitis patients with aquaporin-4-antibody-positive neuromyelitis optica spectrum disorder (AQP4 + NMOSD) and multiple sclerosis (MS). MATERIAL/METHODS: Two blinded raters evaluated spinal cord magnetic resonance imaging (MRIs) of myelitis patients with MOGAD (n = 63), AQP4 + NMOSD (n = 37), and MS (n = 26), assessing for marked central canal T2-hyperintensity and its evolution. If there were conflicting results, a third neurologist assessed the MRI. RESULTS: Marked central canal T2-hyperintensity was more frequent in patients with MOGAD (18/63[29%]) than MS (1/26[4%]; p = 0.01) myelitis but did not differ from AQP4 + NMOSD (13/37[35%]; p = 0.49). Marked central canal T2-hyperintensity had completely resolved on follow-up axial MRI for most MOGAD (12/14[86%]) and AQP4 + NMOSD (10/10[100%]; p = 0.49) patients. CONCLUSIONS: Marked central canal T2-hyperintensity is a common transient radiologic accompaniment of MOGAD and AQP4 + NMOSD myelitis, but not MS myelitis.

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.

Clinical and Radiologic Features Among Children With Myelin Oligodendrocyte Glycoprotein Antibody-Associated Myelitis.

BACKGROUND: Myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease (MOGAD) often manifests as optic neuritis, transverse myelitis(TM), and acute disseminated encephalomyelitis. Patients with a TM phenotype are at high risk for neurological sequelae, so recognizing the characteristics of MOG-IgG myelitis is essential for early, accurate diagnosis and treatment. METHODS: This was a single-center retrospective study. Pediatric MOG antibody-associated disease patients who had clinical myelitis were recruited for this study. Data on clinical and radiologic features and outcomes were retrospectively collected. RESULTS: Thirty-four patients (age range: 6 months to 13 years; median age, 7 years; female, 16) were enrolled in this study. As one patient had two clinical episodes of myelitis, 35 episodes were included. Isolated transverse myelitis was the initial manifestation in 28 (82%) patients. The most frequent clinical features of MOG-IgG myelitis were weakness and neurogenic bladder, and 80% were better than wheelchair-dependent at the nadir. There was a high presentation of weakness (91%), bowel/bladder dysfunction (63%), and sensory dysfunction (46%), and 80% were better than wheelchair-dependent at the nadir. In addition, seven patients (20%) had radicular pain, and six had flaccid areflexia. Magnetic resonance imaging features were often longitudinally extensive (63%) and prominently involved gray matter (H-sign) (63%), accompanied by leptomeningeal enhancement (4/14.29%) and spinal root enhancement (6/14.43%). At the final follow-up (median, 28 months; range, 8-109 months), 10 patients (29%) had developed one or more relapses, spinal cord lesions resolved entirely in 11 of 22 children (50%), and none had appreciable spinal cord atrophy. At the final follow-up, most patients had favorable outcomes, with median (interquartile range) Expanded Disability Status Scale scores of 0 (range, 0-2), four patients (12%) had sphincter dysfunction, and one patient had gait problems. CONCLUSIONS: Pediatric MOG-IgG myelitis clinically presents with weakness and bowel and bladder dysfunctions. Prominent involvement of the gray matter, leptomeningeal enhancement, and spinal root enhancement are common in pediatric MOG-IgG myelitis.

Longitudinal extensive transverse myelitis due to tuberculosis: A case report.

Neurotuberculosis is a potentially fatal disease that can present with upper or lower motor neuron symptoms. Longitudinally extensive transverse myelitis (LETM) is characterized by contiguous inflammatory lesions of the spinal cord extending to three or more vertebral segments. The causes of LETM include infections, neoplasm, and autoimmune diseases. Mycobacterium tuberculosis is a rare cause of transverse myelitis. Here, we report a 21-year-old Afghan female who was referred with chronic progressive quadriparesis and showed LETM on cervical MRI. This report indicates that tuberculosis should be considered as a differential diagnosis of LETM, especially in endemic areas.

Congestive myelopathy due to craniocervical junction arteriovenous fistulas mimicking transverse myelitis: a multicenter study on 27 cases.

BACKGROUND: The purpose was to clarify diagnostic clues and pitfalls in cranio-cervical junction arteriovenous fistulas (CCJ AVFs) with congestive myelopathy. METHODS: In a multicenter observational study by the Neurospinal Society of Japan, we described the demographics, clinical courses, imaging findings, and outcomes of consecutive patients with CCJ AVFs presenting with congestive myelopathy between 2009 and 2019. RESULTS: Twenty-seven patients were included (mean age, 70 years; male, 96%). Progressive symptoms within one day to one month were more common (63%) than chronic symptoms. Myelopathic symptoms were characterized by ascending paralysis beginning from the legs, involving the trunk and arms, and sometimes ending in the brainstem. Fifteen patients (56%) received a misdiagnosis, including acute transverse myelitis. The most common MRI findings were venous congestive edema of the cervical cord (96%) and the brainstem (63%) and surrounding vascular flow voids (100%). The mean extension of congestive edema was 5.5 ± 2.9 vertebral segments. The most common angiographic findings were a dural AVF (78%) at the C1 level (81%) with descending venous drainage (85%). Seven patients (26%) were administered steroids, which resulted in neurological decline in 3. Neurosurgical obliteration of the AVF led to improvements in MRI findings in 75% and a functional status in 67%; however, 44% remained dependent. CONCLUSIONS: The myelopathy of CCJ AVFs was characterized by acute ascending paralysis in elderly men. A misdiagnosis was common because of the acute presentation due to a longitudinally extensive spinal cord lesion. Dilated vessels on MRI were a key finding for the correct diagnosis. What is already known on this topic? Slowly progressive myelopathy is a well-known symptom that results from impaired spinal venous drainage due to thoracolumbar AVFs. Although cranio-cervical junction arteriovenous fistulas (CCJ AVFs) constitute a treatable cause of congestive myelopathy, detailed information is not currently available due to their rarity. What does this study add? CCJ AVFs often presented with acute ascending myelopathy in elderly men due to a longitudinally extending cervical cord lesion with surrounding flow voids. Steroid pulse therapy was not effective or even harmful to congestive myelopathy, while neurosurgical treatment effectively obliterated AVFs. How might this study affect research, practice or policy? The results obtained revealed diagnostic clues and pitfalls from the largest dataset of patients with CCJ AVFs in a multicenter cohort.