A Case of Multifocal Neurological Symptoms

2018 ◽  
pp. 9-14
Author(s):  
Aaron E. Miller ◽  
Tracy M. DeAngelis ◽  
Michelle Fabian ◽  
Ilana Katz Sand
Keyword(s):  
Acute Disseminated Encephalomyelitis ◽  
Myelin Oligodendrocyte Glycoprotein ◽  
High Dose ◽  
Neuromyelitis Optica Spectrum Disorder ◽  
Good Recovery ◽  
Related Disease ◽  
Demyelinating Disorder ◽  
Immune Mediated ◽  
Initial Onset ◽  
Adolescents And Adults

Acute disseminated encephalomyelitis (ADEM) is an immune-mediated demyelinating disorder characterized by multifocal involvement of the CNS and encephalopathy. Diagnostic criteria also require MRI lesions characteristic for demyelination and that new symptoms/findings not occur more than three months after initial onset. ADEM preferentially affects young children but also occurs in adolescents and adults. An antecedent infection or vaccine may be reported, but a clear causal relationship has not been established, and more often no clear provoking factor is identified. In addition to encephalopathy, patients present with symptoms reflecting multifocal CNS involvement such as optic neuritis, ataxia, hemiparesis, spinal cord syndrome, and seizures. Respiratory failure may occur. ADEM is typically treated with high-dose corticosteroids. Plasma exchange or intravenous immunoglobulin may also be used. The course is most often monophasic with good recovery. However, a subset of patients will go on to have recurrent disease, reflecting a diagnosis of multiple sclerosis, neuromyelitis optica spectrum disorder, or myelin oligodendrocyte glycoprotein related disease.

scholarly journals Use of Cyclophosphamide in a Child With Fulminant Acute Disseminated Encephalomyelitis

2018 ◽  
Vol 5 ◽  
pp. 2329048X1875463
Author(s):  
Hana Ayed ◽  
Mohammed W. Chaudhary ◽  
Raidah AlBaradie ◽  
Ali Mir
Keyword(s):  
Intravenous Immunoglobulin ◽  
Immunosuppressive Agents ◽  
Acute Disseminated Encephalomyelitis ◽  
High Dose ◽  
Intravenous Methylprednisolone ◽  
First Line ◽  
Demyelinating Disorder ◽  
Immune Mediated ◽  
Minimal Improvement ◽  
The Central Nervous System

Acute disseminated encephalomyelitis is an immune-mediated inflammatory demyelinating disorder of the central nervous system. The first-line treatment is usually high-dose intravenous methylprednisolone. Intravenous immunoglobulin and plasmapheresis have also shown to be beneficial. Immunosuppressive agents like cyclophosphamide have been used in adults with fulminant acute disseminated encephalomyelitis. We report a case of a 3-year-old boy with fulminant acute disseminated encephalomyelitis. Minimal improvement was seen with high-dose intravenous methylprednisolone, intravenous immunoglobulin, and plasmapheresis. Based on the reports of cyclophosphamide being used successfully to treat adult patients with fulminant acute disseminated encephalomyelitis, we used it in our patient who then showed dramatic and quick improvement. We suggest that if conventional treatment fails, cyclophosphamide could be tried in pediatric patients with fulminant acute disseminated encephalomyelitis.

scholarly journals Acute Disseminated Encephalomyelitis (ADEM): A Demyelinating Disease with Specific Morphological Features

2021 ◽  
pp. 106689692199356
Author(s):  
Fleur Cordier ◽  
Lars Velthof ◽  
David Creytens ◽  
Jo Van Dorpe
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Differential Diagnosis ◽  
Clinical Case ◽  
Demyelinating Disease ◽  
Acute Disseminated Encephalomyelitis ◽  
Demyelinating Diseases ◽  
Demyelinating Disorder ◽  
Immune Mediated ◽  
The Central Nervous System

Acute disseminated encephalomyelitis (ADEM) is a rare immune-mediated inflammatory and demyelinating disorder of the central nervous system. Its characteristic perivenular demyelination and inflammation aid in the differential diagnosis with other inflammatory demyelinating diseases. Here, we present a clinical case of ADEM, summarize its histological hallmarks, and discuss pitfalls concerning the most important neuropathological differential diagnoses.

Acute Disseminated Encephalomyelitis

2012 ◽  
Vol 27 (11) ◽  
pp. 1408-1425 ◽  
Author(s):  
Gulay Alper
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Acute Disseminated Encephalomyelitis ◽  
Protein Levels ◽  
Demyelinating Disorder ◽  
Immune Mediated ◽  
Elevated Protein ◽  
Demyelinating Lesions ◽  
Diagnostic Difficulties ◽  
The Central Nervous System

Acute disseminated encephalomyelitis is an immune-mediated inflammatory and demyelinating disorder of the central nervous system, commonly preceded by an infection. It principally involves the white matter tracts of the cerebral hemispheres, brainstem, optic nerves, and spinal cord. Acute disseminated encephalomyelitis mainly affects children. Clinically, patients present with multifocal neurologic abnormalities reflecting the widespread involvement in central nervous system. Cerebrospinal fluid may be normal or may show a mild pleocytosis with or without elevated protein levels. Magnetic resonance image (MRI) shows multiple demyelinating lesions. The diagnosis of acute disseminated encephalomyelitis requires both multifocal involvement and encephalopathy by consensus criteria. Acute disseminated encephalomyelitis typically has a monophasic course with a favorable prognosis. Multiphasic forms have been reported, resulting in diagnostic difficulties in distinguishing these cases from multiple sclerosis. In addition, many inflammatory disorders may have a similar presentation with frequent occurrence of encephalopathy and should be considered in the differential diagnosis of acute disseminated encephalomyelitis.

scholarly journals Acute Disseminated Encephalomyelitis: Current Perspectives

Children ◽  
2020 ◽  
Vol 7 (11) ◽  
pp. 210
Author(s):  
Renata Barbosa Paolilo ◽  
Kumaran Deiva ◽  
Rinze Neuteboom ◽  
Kevin Rostásy ◽  
Ming Lim
Keyword(s):  
Magnetic Resonance Imaging ◽  
Central Nervous System ◽  
Nervous System ◽  
Magnetic Resonance ◽  
Acute Disseminated Encephalomyelitis ◽  
Myelin Oligodendrocyte Glycoprotein ◽  
Resonance Imaging ◽  
Mri Findings ◽  
Immune Mediated ◽  
Magnetic Resonance Imaging Mri

Acute disseminated encephalomyelitis (ADEM) is an immune-mediated central nervous system (CNS) disorder, characterized by polyfocal symptoms, encephalopathy and typical magnetic resonance imaging (MRI) findings, that especially affects young children. Advances in understanding CNS neuroimmune disorders as well as the association of myelin oligodendrocyte glycoprotein antibody (MOG-Ab) with both monophasic and recurrent forms of ADEM have led to new insights into its definition, management and outcome. In this review, we aim to provide an update based on current epidemiologic, clinical, radiological and immunopathological aspects and clinical outcome of ADEM.

Clinical characteristics and prognosis of myelin oligodendrocyte glycoprotein antibody-seropositive paediatric optic neuritis in China

2018 ◽  
Vol 103 (6) ◽  
pp. 831-836 ◽  
Author(s):  
Honglu Song ◽  
Huanfen Zhou ◽  
Mo Yang ◽  
Shaoying Tan ◽  
Junqing Wang ◽  
...  
Keyword(s):  
Optic Neuritis ◽  
Ganglion Cell ◽  
Nerve Fibre ◽  
Clinical Characteristics ◽  
Age Of Onset ◽  
Acute Disseminated Encephalomyelitis ◽  
Myelin Oligodendrocyte Glycoprotein ◽  
Potential Biomarker ◽  
Significant Difference ◽  
Initial Onset

Background/AimsTo investigate clinical characteristics and prognosis of paediatric optic neuritis (PON) in patients seropositive for myelin oligodendrocyte glycoprotein antibody (MOG-Ab) in China.MethodsChildren displaying initial onset of optic neuritis (ON) were recruited from the Neuro-ophthalmology Department in the Chinese People’s Liberation Army General Hospital from January 2016 to August 2017. They were assigned into three groups based on antibody status: MOG-Ab-seropositive ON (MOG-ON), aquaporin-4 antibody-seropositive ON (AQP4-ON) and double seronegative ON (seronegative-ON).ResultsTotally 48 patients were assessed, including 25 MOG-ON (52.1%), 7 AQP4-ON (14.6%) and 16 seronegative-ON (33.3%). The MOG-ON and seronegative-ON cohorts had equal ratios of female/male, but the AQP4-ON cohort was predominantly females (100%). The patients with MOG-ON were significantly younger at onset compared with the AQP4-ON group. Of the MOG-ON eyes, 97.6% had good recovery of visual acuity (VA) (≥0.5) compared with33.3% of AQP4-ON eyes (p<0.001). However, there was no significant difference compared with the seronegative-ON eyes (82.6%, p=0.052). Two children in the MOG-ON group ended up being diagnosed with acute disseminated encephalomyelitis, while only one patient in the AQP4-ON group developed neuromyelitis optica during follow-up. Patients with MOG-ON had thicker peripapillary retinal nerve fibre layers overall and in the superior and inferior quadrants than in patients with AQP4-ON (p=0.005, p=0.002 and p=0.024, respectively). In addition, the macular ganglion cell-inner plexiform in MOG-ON eyes became significantly thicker than in AQP4-ON eyes (p=0.029). Orbital MRI revealed a larger proportion of patients with MOG-ON had intracranial optic nerve involvement than patients with seronegative-ON (51.2% vs 17.4%, p=0.009).ConclusionMOG-ON was the most common PON subtype in China. MOG-ON had different clinical features including earlier age of onset, equal female/male ratio, better recovery of VA and thicker peripapillary retinal nerve fibre and macular ganglion cell-inner plexiform layers. MOG-Abs may be a potential biomarker for determining visual prognosis with PON.

scholarly journals Case Report: Postvaccination Anti–Myelin Oligodendrocyte Glycoprotein Neuromyelitis Optica Spectrum Disorder

2020 ◽  
Vol 22 (2) ◽  
pp. 85-90 ◽  
Author(s):  
Neha Kumar ◽  
Kelsey Graven ◽  
Nancy I. Joseph ◽  
John Johnson ◽  
Scott Fulton ◽  
...  
Keyword(s):  
Neuromyelitis Optica ◽  
Transverse Myelitis ◽  
First Trimester ◽  
Acute Disseminated Encephalomyelitis ◽  
Myelin Oligodendrocyte Glycoprotein ◽  
Spectrum Disorder ◽  
Aquaporin 4 ◽  
Future Research ◽  
Neuromyelitis Optica Spectrum Disorder ◽  
The Central Nervous System

Abstract Stimulation of the immune response after vaccination can occasionally result in adverse effects, including demyelination of the central nervous system. The most common presentation of postvaccination demyelination is acute disseminated encephalomyelitis, but cases of optic neuritis, transverse myelitis, and multiple sclerosis relapses have been reported. More recently, an increasing number of postvaccination neuromyelitis optica spectrum disorder (NMOSD) cases have surfaced in the literature, especially in patients with aquaporin-4 antibodies. In this article, we report an unusual case of myelin oligodendrocyte glycoprotein antibody–related NMOSD after the receipt of multiple vaccines in a first-trimester pregnant woman from Africa. We review the reported cases of postvaccination demyelination in the past decade, with a focus on the relationship between NMOSD and vaccination in patients with aquaporin-4 or myelin oligodendrocyte glycoprotein antibodies. Finally, we discuss the clinical relevance of the present case and similar reported cases as it relates to patient care in the neuroimmunology clinic and identify potential areas for future research.

scholarly journals Pseudotumoral Demyelinating Lesions: A Presentation of Acute Disseminated Encephalomyelitis

2021 ◽  
pp. 289-296
Author(s):  
Rachid Belfkih ◽  
Omar Ghomari Khayat ◽  
Hind H’daidane ◽  
Fatima Zahra El Amrani
Keyword(s):  
Multiple Sclerosis ◽  
Brain Mri ◽  
Brain Biopsy ◽  
Acute Disseminated Encephalomyelitis ◽  
Neurological Deficits ◽  
High Dose ◽  
Good Recovery ◽  
Disease Diagnostics ◽  
Demyelinating Lesions ◽  
Demyelinating Disorders

Pseudotumoral forms of demyelination are related to central nervous system demyelinating disorders, usually considered to be an atypical presentation of multiple sclerosis including its different varieties such as Balo’s, Schilder’s, and Marburg diseases. These lesions could also be seen in myelin oligodendrocyte glycoprotein antibody-associated demyelination, acute disseminated encephalomyelitis (ADEM), and neuromyelitis optica spectrum disorder. The pseudotumoral aspect may be mistakenly considered as an abscess or a cancerous tumor, in which case, patients could endure unnecessary possibly harmful brain biopsy and have a delay in their disease diagnostics and management. Once latter differential diagnosis is discarded, pseudotumoral demyelination prompts uncertainties concerning the nature of the underlying demyelinating condition as prognosis and management differ from multiple sclerosis to other syndromes, especially whether a chronic treatment is needed or not. In this case report, we present a 35-year-old male patient hospitalized in the department of neurology for a rapidly progressive onset of encephalopathy and polyfocal neurological deficits, with pseudotumoral lesions shown on brain MRI. On further investigations, ADEM was the more likely diagnosis that could fit the patient’s clinical and radiological presentation. Thence, he was put on high dose of intravenous corticosteroids, with a followed good recovery within the first week of the treatment.

Diagnosis of Multiple Sclerosis

2021 ◽  
pp. 540-547
Author(s):  
W. Oliver Tobin
Keyword(s):  
Multiple Sclerosis ◽  
Central Nervous System ◽  
Nervous System ◽  
Demyelinating Disease ◽  
Acute Disseminated Encephalomyelitis ◽  
Myelin Oligodendrocyte Glycoprotein ◽  
Spectrum Disorder ◽  
Demyelinating Diseases ◽  
Neuromyelitis Optica Spectrum Disorder ◽  
The Central Nervous System

Multiple sclerosis is the most common idiopathic inflammatory demyelinating disease of the central nervous system (CNS), with a prevalence of 1 in 500 to 1 in 2,000 people, depending on geography and various other factors. Idiopathic inflammatory demyelinating diseases are a group of related disorders that include acute disseminated encephalomyelitis, neuromyelitis optica spectrum disorder, and myelin oligodendrocyte glycoprotein–immunoglobulin G–associated CNS demyelinating disease.

scholarly journals Acute disseminated encephalomyelitis with delayed onset and feasibility of the Miethke shunt and sensor reservoir system: a case report

2021 ◽  
Author(s):  
Gunnar Liminga ◽  
Anna Grabowska ◽  
Dýrleif Pétursdóttir ◽  
Kristina G. Cesarini ◽  
Elham Rostami ◽  
...  
Keyword(s):  
Case Report ◽  
Acute Disseminated Encephalomyelitis ◽  
High Dose ◽  
Vp Shunt ◽  
Delayed Onset ◽  
Reservoir System ◽  
Acute Visual Loss ◽  
First Case ◽  
Immune Mediated ◽  
System Disorder

AbstractAcute disseminated encephalomyelitis (ADEM) is an immune-mediated demyelinating central nervous system disorder with predilection for early childhood. Delayed onset of ADEM is rare, and herein we present a previously healthy 5-year-old boy, with an unusual clinical course of ADEM with high intracranial pressure (ICP) and acute visual loss that was at first diagnosed as idiopathic intracranial hypertension without papilledema (IIHWOP). The boy underwent acute neurosurgical intervention with ventriculoperitoneal (VP) shunt using Miethke valve and sensor reservoir system and received high-dose steroid treatment with symptom relieve within days. This is the first case report using this system in such a young child, and we find it feasible and valuable also in younger children when VP shunt with ICP measurement is indicated.

scholarly journals Recurrent Bilateral Optic Neuritis Associated with Myelin Oligodendrocyte Glycoprotein Antibody: A Case Report from Nepal

2021 ◽  
Vol 2021 ◽  
pp. 1-3
Author(s):  
Sangam Shah ◽  
Rajeev Ojha ◽  
Sanjeeta Sitaula ◽  
Dosti Regmi ◽  
Ragesh Karn ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Case Report ◽  
Cell Surface ◽  
Optic Neuritis ◽  
Myelin Sheath ◽  
Myelin Oligodendrocyte Glycoprotein ◽  
Neuromyelitis Optica Spectrum Disorder ◽  
Inflammatory Condition ◽  
Immune Mediated ◽  
Aquaporin 4 Antibody

Neuromyelitis optica spectrum disorder (NMOSD) is an immune-mediated inflammatory condition involving spinal cord and optic nerves. Diagnosis of NMOSD is done by aquaporin-4 antibody (AQP4) in patients with optic neuritis. Myelin oligodendrocyte glycoprotein (MOG) expressed on the oligodendrocyte cell surface and on the outermost cell surface of the myelin sheath may also be present in patients with NMOSD bilateral optic neuritis. Here, we describe a case of a thirty-nine-year-old-female with recurrent bilateral optic neuritis with positive anti-MOG antibody, and anti-MOG syndrome has not previously been reported from Nepal.

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