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.

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.

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.

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.

Outcome and risk of recurrence in a large cohort of idiopathic longitudinally extensive transverse myelitis without AQP4/MOG antibodies.

BACKGROUND: Longitudinally extensive transverse myelitis (LETM) is classically related to aquaporin (AQP4)-antibodies (Ab) neuromyelitis optica spectrum disorders (NMOSD) or more recently to myelin oligodendrocyte glycoprotein (MOG)-Ab associated disease. However, some patients remain negative for any diagnosis, despite a large work-up including AQP4-Ab and MOG-Ab. Data about natural history, disability outcome, and treatment are limited in this group of patients. We aimed to (1) describe clinical, biological, and radiological features of double seronegative LETM patients; (2) assess the clinical course and identify prognostic factors; and (3) assess the risk of recurrence, according to maintenance immunosuppressive therapy. METHODS: Retrospective evaluation of patients with a first episode of LETM, tested negative for AQP-Ab and MOG-Ab, from the French nationwide observatory study NOMADMUS. RESULTS: Fifty-three patients (median age 38 years (range 16-80)) with double seronegative LETM were included. Median nadir EDSS at onset was 6.0 (1-8.5), associated to a median EDSS at last follow-up of 4.0 (0-8). Recurrence was observed in 24.5% of patients in the 18 following months, with a median time to first relapse of 5.7 months. The risk of recurrence was lower in the group of patients treated early with an immunosuppressive drug (2/22, 9%), in comparison with untreated patients (10/31, 32%). CONCLUSIONS: A first episode of a double seronegative LETM is associated to a severe outcome and a high rate of relapse in the following 18 months, suggesting that an early immunosuppressive treatment may be beneficial in that condition.

An acute transverse myelitis attack after autologous stem cell transplantation: A rare case.

Transverse myelitis is a quite rare complication of hematopoietic stem cell transplantation. The case is here reported of a 49 year old male with diffuse large B cell lymphoma in complete remission who developed transverse myelitis after autologous stem cell transplantation. The patient presented with numbness and sensory loss of the bilateral lower extremities and difficulty in urinating on the 20th day after cell transplantation. Millimetric hyperintensity was detected in the C5-C6 and T2-T5 segments of the spinal cord on cervical and thoracic vertebral magnetic resonance imaging. Treatment was initiated of pulse steroid and intravenous immunoglubulin followed by plasmapheresis and cyclophosphamide due to inadequate response. The patient then started a rehabilitation program and was discharged in the 9th month after stem cell transplantation when most of the symptoms were relieved. To the best of our knowledge, this is the first case reported in literature of TM development after autologous stem cell transplantation.

Clinical, laboratory features, and prognostic factors in adult acute transverse myelitis: an Italian multicenter study.

OBJECTIVES: We compared the clinical, laboratory, and radiological features of different subgroups of acute transverse myelitis (ATM) diagnosed according to the criteria established by the Transverse Myelitis Consortium Working Group (TMCWG) as well as of non-inflammatory acute transverse myelopathies (NIATM) to identify possible short- and long-term prognostic factors. METHODS: A multicenter and retrospective study comprising 110 patients with ATM and 15 NIATM admitted to five Italian neurological units between January 2010 and December 2014 was carried out. RESULTS: A significantly higher frequency of isolated sensory disturbances at onset in ATM than in NIATM patients (chi-square = 14. 7; P = 0.005) and a significantly higher frequency of motor symptoms in NIATM than ATM (chi-square = 12.4; P = 0.014) was found. ATM patients with high disability at discharge had more motor-sensory symptoms without (OR = 3.87; P = 0.04) and with sphincter dysfunction at onset (OR = 7.4; P = 0.0009) compared to those with low disability. Higher age (OR = 1.08; P = 0.001) and motor-sensory-sphincter involvement at onset (OR = 9.52; P = 0.002) were significantly associated with a high disability score at discharge and after a median 1-year follow-up. CONCLUSIONS: The diagnosis of ATM may prevail respect to that of NIATM when a sensory symptomatology at onset occurs. In ATM, patients older and with motor-sensory involvement with or without sphincter impairment at admission could experience a major risk of poor prognosis both at discharge and at longer time requiring a timely and more appropriate treatment.

Impact of the hematocrit value of the blood prime unit on patient hematocrit values after therapeutic plasma exchange in children weighing 10 kg or less.

We report the impact of measuring the hematocrit (HCT) of blood prime units (BPUs) on postprocedure patient HCT values in a small child with transverse myelitis undergoing therapeutic plasma exchange (TPE). Initially, the BPU HCT values were not measured, according to our apheresis policy of using our blood center's estimated HCT value. This approach resulted in unexpected increasing elevations of our patient's post-TPE HCT after the first two TPE procedures. Subsequent measurement of the BPU HCT prior to use stabilized the patient's post-TPE HCT. To our knowledge, this is the first case report describing the impact of using the measured BPU HCT vs the estimated HCT for very small children undergoing therapeutic apheresis. Our standard operating procedure for very small children has been updated after this patient's case to include measurements of the HCT values of BPUs for children who weigh 10 kg or less.

Feasibility and Safety of Transnasal High Flow Air to Reduce Core Body Temperature in Febrile Neurocritical Care Patients: A Pilot Study.

BACKGROUND: Fever is an important determinant of prognosis following acute brain injury. Current non-pharmacologic techniques to reduce fever are limited and induce a shivering response. We investigated the safety and efficacy of a novel transnasal unidirectional high flow air device in reducing core body temperature in the neurocritical care unit (NCCU) setting. METHODS: This pilot study included seven consecutive patients in the NCCU who were febrile (> 37.5 °C) for > 24 h despite standard non-pharmacologic and first-line antipyretic agents. Medical grade high flow air was delivered transnasally using a standard continuous positive airway pressure machine with a positive pressure of 20 cmH2O for 2 h. Core esophageal and tympanic temperature were continuously monitored. RESULTS: Mean age was 40 ± 14 yo, and 72% (5/7 patients) were men. Five patients had intracerebral or intraventricular hemorrhage, one subject had transverse myelitis, and the remaining patient had anoxic brain injury due to a cardiac arrest. After 2 h of cooling, core temperature was significantly lower than the baseline pre-cooling temperature (37.3 ± 0.5 °C vs. 38.4 ± 0.6 °C; p < 0.002). Mean transnasal airflow rate was 57.5 ± 6.5 liters per minute. Five of the seven subjects were normothermic at the end of the 2-h period. One subject with severe hyperthermia (39.7 °C) and the other with multiple interruptions to therapy due to technical reasons did not cool. The core temperature within 30 min of cessation of airflow increased and was similar to the pre-cooling baseline temperature (38.3 ± 0.4 °C vs. 38.4 ± 0.6 °C, p = NS). Rate of core cooling was 0.6 ± 0.15 °C per hour at this flow rate. No shivering response was observed. No protocol-related adverse events occurred. CONCLUSIONS: High flow transnasal air in a unidirectional fashion lowers core body temperature in febrile patients in the NCCU setting. No adverse events were seen, and the process showed no signs of shivering or any other serious side effects during short-term exposure. This pilot study should inform further investigation.

Myelitis: A Rare Presentation of Epstein Barr Virus.

EBV associated nervous system complications includes encephalitis, meningitis, cerebellitis, polyradiculomyelitis, transverse myelitis, cranial and peripheral neuropathies, and psychiatric abnormalities are usually more commonly seen in immunocompromised patients and rarely in immunocompetent patients. Here we are reporting a 13 years old boy developed headache, malaise, sore throat and low back pain with radiation to both lower limbs. Next day he felt numbness below umbilicus followed by acute onset weakness in both lower limbs and urinary retention. Motor exam revealed proximal muscle power MRC grade 4/5 and distal power 1/5 in right lower limb and proximal power 4-/5 and distal power 0/5 in left lower limb with normal power in both upper limbs. Deep tendon reflexes were bilaterally normal except absent ankle reflexes. Both plantars were mute. All the modalities of sensation including pain, touch, temperature, joint position and vibration were impaired below umbilicus. Routine investigations were normal. The magnetic resonance imaging (MRI) of thoracic spine showed intramedullary lesion in conus, which was iso-hyperintense on T1-weighted and hyperintense on T2- weighted images extending from D12thoracic vertebral level to L1 with cord expansion (Figures 1, 2). The MRI features were suggestive of conus myelitis. Cerebrospinal fluid (CSF) analysis revealed increased protein, normal cells, glucose and Chloride. CSF Polymerase chain reaction (PCR) was positive for Epstein Barr virus . The clinical and imaging findings were consistent with the diagnosis of myelitis and responded well to steroid plus acyclovir treatment. The clinicians should be aware of such uncommon etiology of a common disease.

Clinical course, therapeutic responses and outcomes in relapsing MOG antibody-associated demyelination.

OBJECTIVE: We characterised the clinical course, treatment and outcomes in 59 patients with relapsing myelin oligodendrocyte glycoprotein (MOG) antibody-associated demyelination. METHODS: We evaluated clinical phenotypes, annualised relapse rates (ARR) prior and on immunotherapy and Expanded Disability Status Scale (EDSS), in 218 demyelinating episodes from 33 paediatric and 26 adult patients. RESULTS: The most common initial presentation in the cohort was optic neuritis (ON) in 54% (bilateral (BON) 32%, unilateral (UON) 22%), followed by acute disseminated encephalomyelitis (ADEM) (20%), which occurred exclusively in children. ON was the dominant phenotype (UON 35%, BON 19%) of all clinical episodes. 109/226 (48%) MRIs had no brain lesions. Patients were steroid responsive, but 70% of episodes treated with oral prednisone relapsed, particularly at doses <10 mg daily or within 2 months of cessation. Immunotherapy, including maintenance prednisone (P=0.0004), intravenous immunoglobulin, rituximab and mycophenolate, all reduced median ARRs on-treatment. Treatment failure rates were lower in patients on maintenance steroids (5%) compared with non-steroidal maintenance immunotherapy (38%) (P=0.016). 58% of patients experienced residual disability (average follow-up 61 months, visual loss in 24%). Patients with ON were less likely to have sustained disability defined by a final EDSS of ≥2 (OR 0.15, P=0.032), while those who had any myelitis were more likely to have sustained residual deficits (OR 3.56, P=0.077). CONCLUSION: Relapsing MOG antibody-associated demyelination is strongly associated with ON across all age groups and ADEM in children. Patients are highly responsive to steroids, but vulnerable to relapse on steroid reduction and cessation.

Predictors of outcome in a large retrospective cohort of patients with transverse myelitis.

BACKGROUND: Transverse myelitis (TM) is an inflammatory disorder that can be idiopathic or associated with central nervous system autoimmune/dysimmune inflammatory diseases, connective tissue autoimmune diseases, or post-infectious neurological syndromes. Prognosis of initial TM presentations is uncertain. OBJECTIVE: To identify outcome predictors in TM. METHODS: Retrospective study on isolated TM at onset. Scores ⩾3 on the modified Rankin scale (mRS) marked high disability. RESULTS: A total of 159 patients were identified. TM was classified as follows: idiopathic (I-TM, n = 53), post-infectious (PI-TM, n = 48), associated with multiple sclerosis (MS-TM, n = 51), or neuromyelitis optica spectrum disorders/connective tissue autoimmune diseases/neurosarcoidosis ( n = 7). At follow-up (median, 55 months; interquartile range, 32-80), 42 patients were severely disabled, and patients with I-TM or PI-TM showed the worst outcomes. Predictors of disability were infectious antecedents, sphincter and pyramidal symptoms, high mRS scores, blood-cerebrospinal fluid barrier damage, lumbar magnetic resonance imaging (MRI) lesions on univariate analysis, and older age (odds ratio (OR), 1.1; 95% confidence interval (CI), 1.0-1.1), overt/subclinical involvement of the peripheral nervous system (PNS) (OR, 9.4; 95% CI, 2.2-41.0), complete TM (OR, 10.8; 95% CI, 3.4-34.5) on multivariate analysis. CONCLUSION: Our findings help define prognosis and therapies in TM at onset. Infectious antecedents and PNS involvement associate with severe prognosis. Nerve conduction studies and lumbar MRI could improve the prognostic assessment of this condition.

Acute demyelination following radiotherapy for glioma: a cautionary tale.

Radiotherapy is the mainstay of treatment after surgery for high-grade gliomas and is usually well tolerated. Radiation toxicity in the brain is usually classified according to the timing of side effects in relation to treatment, as either acute (during radiotherapy), early delayed (within 12 weeks of radiotherapy) or late delayed (months to years after radiotherapy). We report two cases of young women who developed severe acute demyelination within 4 months of radiotherapy for glioma, one of whom had a previous history of transverse myelitis. Both improved with corticosteroids and remain in tumour remission. These cases emphasise the importance of careful discussion with patients before starting radiotherapy if there is a previous history of central nervous system demyelination or multiple white matter lesions on MRI.

Acute transverse myelitis of the cervical spine secondary to psoas abscess.

BACKGROUND: Acute transverse myelitis is uncommon and presumably results from an autoimmune process or a preceding infection. Most cases of bacterial myelitis are due to hematogenous dissemination from urinary or respiratory tract infections or contiguous spreading from a neighboring infected structure. A psoas abscess rarely spreads to higher levels of the spinal cord. No cases of acute cervical myelitis due to a psoas abscess have been previously reported. CASE PRESENTATION: A 34-year-old man was transferred to our hospital due to progressive muscle weakness, sensory deficits and severe hypotension. Two weeks prior to admission, he had received low back injection to relieve back pain in a healthcare clinic. One day prior to admission, his condition had worsened. On admission, he was tetraplegic with absence of sensation below the level of the suprasternal fossa. A lumbar CT scan demonstrated an abscess in the left psoas, and the magnetic resonance imaging (MRI) scan of the entire spinal suggested a cervical spine infection. A cerebrospinal fluid (CSF) analysis performed before surgery indicated the possibility of bacterial infection. An operation was performed to drain the abscess. Microbiological cultivation revealed a Methicillin-resistant Staphylococcus aureus (MRSA) infection. The patient was administered with vancomycin for 10 days and followed by oral formulations of linezolid for 6 weeks. The patient's general condition improved, and he was successfully discharged. Six months later, a follow-up MRI revealed that the lesion of the cervical spine had been ameliorated, and the sensation and myodynamia of his upper limbs had partially recovered. CONCLUSION: This was a rare case of a high-level cervical spine pyogenic infection complicating psoas abscess. An invasive paravertebral injection procedure was thought to be the initial damaging event that created a port of entry for MRSA into the psoas muscle and caused a subsequent psoas abscess. This case indicated that evaluation of higher levels of the spine is warranted when a psoas abscess coexists with severe weakness.

Evaluation and management of longitudinally extensive transverse myelitis: a guide for radiologists.

Longitudinal extensive transverse myelitis (LETM) is defined as an intramedullary spinal cord T2 signal abnormality extending craniocaudally over at least three vertebral bodies on an MRI study. Timely and appropriate diagnosis greatly facilitates patient management. The radiologist should review the relevant clinical information and determine the patient demographics and acuity of symptoms. Herein, we review the spectrum of diseases causing LETM and propose interpretation to guide the radiologist when presented with the MRI finding of LETM.

Neuromyelitis optica spectrum disorder (NMOSD): A new concept.

The relationship between neuromyelitis optica (NMO) and multiple sclerosis (MS) has long been controversial. NMO was previously considered a form of MS involving predominantly the spinal cord and optic nerve. However, since the discovery of NMO-IgG/aquaporin-4 (AQP4) antibody, an NMO-specific autoantibody to AQP4, some unique clinical features, and magnetic resonance imaging (MRI) and other laboratory findings in NMO, have been further clarified. AQP4 antibody is now the most important laboratory finding for the diagnosis of NMO. Besides typical NMO, some patients with recurrent optic neuritis or recurrent longitudinally extensive transverse myelitis alone are also often positive for AQP4 antibody. Moreover, studies of AQP4 antibody-positive patients have revealed that brain and brainstem lesions are not uncommon in NMO, and some patterns appear to be unique to NMO. All these findings have expanded the NMO concept into 'NMO spectrum disorder' (NMOSD), and new criteria have recently been published. A new antigenic target, myelin oligodendrocyte glycoprotein (MOG), has also been discovered recently. This new antibody seems to correspond to around 20% of seronegative patients, but its specificity needs to be evaluated more precisely, especially in pediatric populations. These recent findings may also have therapeutic impact, as it has been demonstrated that many MS drugs can exacerbate NMO. This report provides an overview of the clinical and neuroimaging features of NMOSD, followed by its treatment.