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A 51-year-old woman with COVID-19 infection developed coma and an impaired oculocephalic response to one side. MRI of the brain demonstrated acute multifocal demyelinating lesions, and CSF testing did not identify a direct cerebral infection. High-dose steroids followed by a course of IVIG was administered, and the patient regained consciousness over the course of several weeks. As more patients reach the weeks after initial infection with COVID-19, acute disseminated encephalomyelitis should be considered a potentially treatable cause of profound encephalopathy or multifocal neurological deficits.

A new outbreak of monkeypox has been reported worldwide with CNS complications like encephalitis or myelitis being extremely rare. We present a case of a 30-year-old man with PCR-confirmed diagnosis of monkeypox who developed rapid neurological deterioration with extensive inflammatory involvement of the brain and spinal cord on MRI. Because of the clinical and radiological resemblance to acute disseminated encephalomyelitis (ADEM), it was decided to indicate treatment with high-dose corticosteroids for 5 days (without concomitant antiviral management due to lack of availability in our country). Given the poor clinical and radiological response, 5 days of immunoglobulin G were administered. During follow-up the patient’s clinical condition improved, physiotherapy was started and all associated medical complications were controlled. To our knowledge, this is the first reported monkeypox case with severe CNS complications treated with steroids and immunoglobulin in the absence of specific antiviral treatment.

Background Acute disseminated encephalomyelitis (ADEM) is a rare immune-mediated inflammatory demyelinating disease of the central nervous system. We report a case of ADEM presenting with bilateral optic neuritis temporally associated with the ChAdOx1 vaccine against SARS-COVID19 virus. Case presentation A 36-year-old female presented with bilateral optic neuritis following her first dose of the ChAdOx1 vaccine. Initial MRI Brain showed evidence of demyelination within the subcortical white matter, with no radiological involvement of the optic nerves. Visual evoked potentials were consistent with bilateral optic neuritis which was confirmed radiologically on follow up MRI. She was treated with intravenous steroids with improvement both in symptoms and radiological appearance. A pseudo-relapse occurred which was treated with a further course of intravenous steroids followed by an oral taper. The clinical, radiological and serological results were most consistent with diagnosis of ADEM. Conclusions ADEM is an exceedingly rare complication of ChAdOx1 vaccine despite millions of doses. While it is imperative clinicians remain aware of neurological complications of vaccines, the importance of vaccination to control a pandemic should not be undermined.

Objective: The purpose of this study was to report three patients COVID-19 infection with severe respiratory syndrome requiring intubation, who developed acute demyelinating encephalomyelitis (ADEM). Methods: Patient data were obtained from medical records from the North Memorial Hospital, Robbinsdale, MN, USA. Results: Three patients (two men and one woman, aged 38-63) presented with fatigue, cough, and fever leading to acute respiratory distress syndrome secondary to COVID-19 infection requiring ventilatory support. Two patients were unresponsive and the third patient had severe diffuse weakness. MRI in all patients showed findings consistent with ADEM. CSF showed elevated protein in all patients with normal cell count and no evidence of infection, including negative COVID-19 PCR. All three patients were treated with intravenous corticosteroids and one improved markedly. The other two had minimal response to steroids and no further improvement after IVIG. Conclusion: Neurological complications from COVID-19 are being rapidly recognized. Our three cases highlight the occurrence of ADEM as a postinfectious/immune-mediated complication of COVID-19 infection, which may be responsive to corticosteroid treatment.

Myelin oligodendrocyte glycoprotein (MOG) antibody-associated diseases (MOGAD), which has been recognized as a distinct entity in patients with neuromyelitis optica spectrum disorders, often presents with acute disseminated encephalomyelitis (ADEM) symptoms in pediatric patients. Appropriate treatment based on accurate diagnosis is challenging in relapsing pediatric patients with MOGAD. An 11-year-old girl experienced relapses four times, exhibiting brainstem symptoms, an ADEM episode, seizures, and optic neuritis (ON). She was initially diagnosed with multiple sclerosis and received interferon beta-1a therapy with a mild effect on relapse suppression. She was then transferred from the pediatric department to the department of neurology of our hospital. Two months before her referral visit, she experienced left optic neuritis, and her annualized relapse rate reached 0.6. She desired to switch from the injectable treatment to oral dimethyl fumarate (DMF) administration. At that time, she was found to be seropositive for MOG antibody, but after that had no relapses for more than five years. Moreover, her seropositivity for serum MOG-antibody turned out to be seronegative. DMF showed long-term effects on suppressing relapses in a pediatric patient with MOGAD, revealing its potential as a treatment option for such patients.

Distinguishing aquaporin-4 IgG(AQP4-IgG)-negative neuromyelitis optica spectrum disorders (NMOSD) from opticospinal predominant multiple sclerosis (MS) is a clinical challenge with important treatment implications. The objective of the study was to examine whether expert clinicians diagnose and treat NMO/MS overlapping patients in a similar way. 12 AQP4-IgG-negative patients were selected to cover the range of clinical scenarios encountered in an NMO clinic. 27 NMO and MS experts reviewed their clinical vignettes, including relevant imaging and laboratory tests. Diagnoses were categorized into four groups (NMO, MS, indeterminate, other) and management into three groups (MS drugs, immunosuppression, no treatment). The mean proportion of agreement for the diagnosis was low ( p o  = 0.51) and ranged from 0.25 to 0.73 for individual patients. The majority opinion was divided between NMOSD versus: MS (nine cases), monophasic longitudinally extensive transverse myelitis (LETM) (1), acute disseminated encephalomyelitis (ADEM) (1) and recurrent isolated optic neuritis (RION) (1). Typical NMO features (e.g., LETM) influenced the diagnosis more than features more consistent with MS (e.g., short TM). Agreement on the treatment of patients was higher ( p o  = 0.64) than that on the diagnosis with immunosuppression being the most common choice not only in patients with the diagnosis of NMO (98 %) but also in those indeterminate between NMO and MS (74 %). The diagnosis in AQP4-IgG-negative NMO/MS overlap syndromes is challenging and diverse. The classification of such patients currently requires new diagnostic categories, which incorporate lesser degrees of diagnostic confidence. Long-term follow-up may identify early features or biomarkers, which can more accurately distinguish the underlying disorder.

We describe immune-related acute disseminated encephalomyelitis (ADEM) in a woman in her 60s. This was her third immune-related adverse event (IRAE) secondary to immunotherapy treatment for metastatic melanoma. Initially thought to be intracranial metastatic disease, the diagnosis was reconsidered due to atypical radiological findings. With the persistence and open-minded approach of the wider multidisciplinary team (MDT), a definitive diagnosis of ADEM was reached. Refractory to the conventional management of IRAEs with corticosteroids, the patient was successfully treated with intravenous immunoglobulins (IVIG), followed by tocilizumab and mycophenolate mofetil (MMF), ultimately resulting in complete radiological resolution of ADEM. This case highlights the importance of early suspicion, identification and management of IRAEs when oncology patients present acutely.

A 53-year-old man admitted to the critical care secondary to respiratory failure due to COVID-19 developed agitation and global hypotonia. Brain MRI revealed bilateral hyperintense lesions throughout the brain and cerebrospinal fluid identified oligoclonal bands. Intravenous high-dose glucocorticoids were administered followed by an oral tapering dose and the patient clinically improved. Acute disseminated encephalomyelitis should be considered in patients with COVID-19 who present with altered mentation and polyfocal neurological deficits.

Background Acute disseminated encephalomyelitis (ADEM) is a rare, inflammatory, demyelinating central nervous system disorder typically responsive to corticosteroid therapy. However, some cases are resistant to steroids, requiring alternative treatment options. Case presentation We present an 8-year-old male child with steroid-resistant ADEM who underwent treatment with the modified zipper technique, combining intravenous immunoglobulin (IVIG) and therapeutic plasma exchange (TPE). Despite initial high-dose intravenous methylprednisolone, which failed to improve his condition, the patient showed significant clinical and radiological improvement after five cycles of the modified zipper technique. Methods The patient received alternating cycles of TPE and IVIG (0.4 g/kg/day) over five cycles, with a 48-hour interval between each TPE and IVIG session. TPE sessions used fresh frozen plasma, followed by IVIG to enhance antibody clearance and immunomodulation. Results After two cycles of TPE, clinical improvement was observed. Follow-up MRI on day 15 showed marked regression of white matter lesions. The patient was discharged on day 20 with near-complete recovery. Follow-up at one, three, and six months showed no neurological deficits, and MRI lesions continued to regress. Conclusions This case suggests the modified zipper technique as a promising alternative for steroid-resistant ADEM. The combination of TPE and IVIG may provide beneficial effects, but further controlled studies are needed to evaluate its efficacy and safety in broader populations.

IntroductionSars-CoV-2 is a single-strained RNA virus belonging to Coronaviridae’s family. In pediatric age, the majority of patients is asymptomatic; however, several neurological manifestations associated with Sars-CoV-2 infection have been detected in a percentage of cases ranging from 17.3 to 36.4%. Acute disseminated encephalomyelitis (ADEM) has been recently included among the potential complications of Sars-Cov2 infection. The available data regarding pediatric patient show only one case.Case reportWe present a case regarding a 6-year-old patient suffering from Fisher-Evans syndrome who was given sirolimus and thalidomide therapy. After 10 days since the first positive nasopharyngeal swab for Sars-CoV-2, in which he had no symptoms, he presented an episode of generalized tonic-clonic seizure with spontaneous resolution. The patient underwent MRI which showed the typical picture of acute disseminated encephalomyelitis. His clinical course was favorable, with a good response to cortisone therapy and a progressive improvement of the neuroradiological and electroencephalographic picture.ConclusionsAccording to our knowledge, this is the second case of an acute disseminated encephalomyelitis following SARS-CoV-2 infection in a pediatric patient, characterized by monosymptomatic onset, in which the immunosuppressive therapy practiced for the Fisher-Evans syndrome has probably contributed to a favorable evolution of ADEM, in contrast to other case described in the literature.