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1.
J Assoc Physicians India ; 72(6): 94-96, 2024 Jun.
Article in English | MEDLINE | ID: mdl-38881142

ABSTRACT

A 38-year-old gentleman, following an uncomplicated dengue fever 2 weeks back, developed acute onset bilateral lower limb weakness and numbness for 5 days, associated with bladder and bowel incontinence and a band-like sensation in T4 dermatome. On examination, he had paraparesis with normal cranial nerves except for left upper motor neuron-type 7th cranial nerve palsy and normal higher mental function. Magnetic resonance imaging (MRI) of the brain and spine detected multiple demyelinating lesions. A diagnosis of postdengue acute disseminated encephalomyelitis (ADEM) was made as part of postinfective inflammatory process after the fever had subsided. Cerebrospinal fluid study ruled out active infection. He was treated with intravenous steroids and is currently recovering. An interesting point in our case was that the patient had significant imaging findings in MRI of the brain with no symptoms or signs suggestive of intracranial involvement-ADEM without evidence of encephalitis.


Subject(s)
Dengue , Encephalomyelitis, Acute Disseminated , Magnetic Resonance Imaging , Humans , Male , Encephalomyelitis, Acute Disseminated/diagnosis , Encephalomyelitis, Acute Disseminated/drug therapy , Encephalomyelitis, Acute Disseminated/etiology , Adult , Dengue/complications , Dengue/diagnosis , Brain/diagnostic imaging , Brain/pathology
2.
Eur J Paediatr Neurol ; 50: 86-95, 2024 May.
Article in English | MEDLINE | ID: mdl-38705015

ABSTRACT

BACKGROUND AND OBJECTIVES: Patients with myelin oligodendrocyte glycoprotein antibody-associated disorders (MOGAD) clinically present e.g. with acute disseminated encephalomyelitis (ADEM), optic neuritis (ON), transverse myelitis (TM) or aquaporin-4-IgG (AQP4-IgG) negative neuromyelitis optica spectrum disorders (NMOSD)-like phenotypes. We aimed to analyze and compare blood parameters in children with MOGAD, AQP4-IgG-positive NMOSD (hence NMOSD), multiple sclerosis (MS) and healthy controls (HC). METHODS: We evaluated differences in complete blood counts (CBC), neutrophil-to-lymphocyte ratio (NLR), platelet-to-lymphocyte ratio (PLR), monocyte-to-lymphocyte ratio (MLR) and C-reactive protein (CRP) between these four groups and within the groups between clinical attack, acute treatment and remission. RESULTS: Our cohort consisted of 174 children and adolescents with a total of 550 timepoints: 66 patients had MOGAD (202 timepoints), 11 NMOSD (76 timepoints), 58 MS (219 timepoints) and 39 were HC (53 timepoints). At clinical attack, leukocyte counts were elevated in MOGAD compared to remission (p < 0.001) and compared to all other groups (p < 0.001). NLR was high in MOGAD and NMOSD, and PLR was high in NMOSD, however, after correction for multiple testing these findings did not remain significant. While glucocorticoids caused an increase of leukocyte counts and NLR in NMOSD and MS, these values remained stable during acute treatment in MOGAD. In remission, NLR normalized in MOGAD, while it stayed high in NMOSD. PLR increased in NMOSD and was significantly higher compared to all other groups. DISCUSSION: Some blood parameters, mainly leukocyte and differential counts, might help clinicians to evaluate disease activity, differentiate relapses from pseudo-relapses and even distinguish between different disease entities.


Subject(s)
Myelin-Oligodendrocyte Glycoprotein , Neuromyelitis Optica , Humans , Child , Myelin-Oligodendrocyte Glycoprotein/immunology , Female , Male , Adolescent , Neuromyelitis Optica/blood , Neuromyelitis Optica/immunology , Autoantibodies/blood , Multiple Sclerosis/blood , Multiple Sclerosis/immunology , Child, Preschool , Aquaporin 4/immunology , Aquaporin 4/blood , C-Reactive Protein/analysis , Encephalomyelitis, Acute Disseminated/blood , Encephalomyelitis, Acute Disseminated/immunology , Encephalomyelitis, Acute Disseminated/diagnosis
3.
Neurol India ; 72(2): 379-383, 2024 Mar 01.
Article in English | MEDLINE | ID: mdl-38817174

ABSTRACT

Guillain-Barré syndrome is the most common cause of acute flaccid paralysis in children, but several diseases mimic GBS. We aimed to identify and report the clinical pointers and battery of tests required to differentiate Guillain-Barré syndrome from its observed mimics in the pediatric population admitted to our neuro-critical care unit. We conducted a retrospective record analysis of all pediatric patients admitted over ten years from 2008-2018, whose initial presentation was compatible with a clinical diagnosis of GBS. Eighty-three patients were at first treated as GBS, of which seven (8.4%) were found to have an alternate diagnosis-three cases of paralytic rabies, one case each of acute disseminated encephalomyelitis, cervical myeloradiculopathy, neuromyelitis optica, and a case of community-acquired Staphylococcus aureus pneumonia associated sepsis. Neurophysiological and neuro-virological testing, central nervous system imaging, and sepsis screening helped to confirm the alternate diagnosis. Our case series provides knowledge of subtle clinical differences along with the mindful use of diagnostic testing to facilitate the accurate diagnosis of GBS mimics.


Subject(s)
Guillain-Barre Syndrome , Tertiary Care Centers , Humans , Guillain-Barre Syndrome/diagnosis , Guillain-Barre Syndrome/physiopathology , Child , Retrospective Studies , Female , Male , Diagnosis, Differential , Child, Preschool , Adolescent , Intensive Care Units , Infant , Encephalomyelitis, Acute Disseminated/diagnosis
5.
BMJ Case Rep ; 17(4)2024 Apr 08.
Article in English | MEDLINE | ID: mdl-38589237

ABSTRACT

We reported a case of a school-going child, diagnosed with acute disseminated encephalomyelitis (ADEM) who presented with symptoms such as high fever, acute hemiplegia and ataxia and was referred for physiotherapeutic intervention. This case report aims to document the assessment and management of ADEM from the intensive care unit to the home setting by physical therapy. Also, the child developed ventilator-associated pneumonia and a right lower motor neuron facial injury for which the child was referred to paediatric physical therapy. Since then, continuing for 8 months has helped the child to be independent in all aspects of mobility with no complaints. The child showed improvement in WeeFIM scores and Sunnybrook facial grading after 99 sessions of intensive physical therapy for approximately 83 hours along with the home programme. It has been proven an efficient treatment method along with other medical lines of treatment for neurological impairment associated with ADEM.


Subject(s)
Encephalomyelitis, Acute Disseminated , Physical Therapy Modalities , Child , Humans , Encephalomyelitis, Acute Disseminated/diagnosis , Encephalomyelitis, Acute Disseminated/therapy
6.
Mult Scler Relat Disord ; 85: 105526, 2024 May.
Article in English | MEDLINE | ID: mdl-38489945

ABSTRACT

BACKGROUND: Eosinophils in cerebrospinal fluid (CSF) are an uncommon finding most often associated with parasitic infections, but have also been described in some neuroinflammatory disorders. Eosinophilic infiltration is not thought to be a typical feature of myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD). We aim to describe the rate of CSF eosinophil positivity in a cohort of pediatric MOGAD patients. METHODS: Single-center retrospective chart review of pediatric MOGAD patients. Clinical and laboratory data was collected from the electronic medical record and analyzed. RESULTS: Of 46 pediatric patients with positive serum myelin oligodendrocyte glycoprotein antibody (MOG-IgG) identified, 38 patients fulfilling internationally proposed MOGAD diagnostic criteria were included for analysis. 6 patients with MOGAD were excluded as no CSF data was available, and 2 patients with positive MOG-IgG but diagnosis more consistent with MS were excluded. Median age was 7.3 years, and 19/38 (50 %) were female. Acute disseminated encephalomyelitis (ADEM) was the most common presenting phenotype (23/38, 61 %), and other phenotypes included optic neuritis (10/38, 26 %), transverse myelitis (3/38, 8 %), and neuromyelitis optica spectrum disorder (NMOSD) (2/38, 5 %). 12 of 36 (33 %) patients with all lumbar puncture (LP) data available had CSF eosinophils present, with eosinophil mean of 3 % and range from 1 % to 18 % of CSF while blood cells. CONCLUSION: CSF eosinophils were present in one third of pediatric MOGAD patients, which is a higher rate than previously reported in either MOGAD or aquaporin-4 antibody positive NMOSD cohorts. Understanding the CSF composition of pediatric MOGAD patients helps to facilitate more prompt diagnosis and treatment and may shed light onto underlying pathologic mechanisms of disease with the goal to inform future therapeutic targets.


Subject(s)
Autoantibodies , Eosinophils , Myelin-Oligodendrocyte Glycoprotein , Humans , Myelin-Oligodendrocyte Glycoprotein/immunology , Female , Male , Child , Retrospective Studies , Eosinophils/immunology , Child, Preschool , Adolescent , Autoantibodies/cerebrospinal fluid , Autoantibodies/blood , Encephalomyelitis, Acute Disseminated/immunology , Encephalomyelitis, Acute Disseminated/cerebrospinal fluid , Encephalomyelitis, Acute Disseminated/blood , Encephalomyelitis, Acute Disseminated/diagnosis , Neuromyelitis Optica/cerebrospinal fluid , Neuromyelitis Optica/immunology , Neuromyelitis Optica/blood , Infant , Myelitis, Transverse/immunology , Myelitis, Transverse/cerebrospinal fluid , Myelitis, Transverse/blood , Optic Neuritis/immunology , Optic Neuritis/cerebrospinal fluid , Optic Neuritis/blood , Demyelinating Autoimmune Diseases, CNS/cerebrospinal fluid , Demyelinating Autoimmune Diseases, CNS/immunology , Demyelinating Autoimmune Diseases, CNS/blood
7.
BMC Pediatr ; 24(1): 92, 2024 Feb 02.
Article in English | MEDLINE | ID: mdl-38308211

ABSTRACT

BACKGROUND: The application of evoked potentials (EPs) to the diagnosis of acute disseminated encephalomyelitis (ADEM ) has not been investigated in detail. The aim of this study, therefore, was to analyze the value of multimodal EPs in the early diagnosis of pediatric ADEM. METHODS: This was a retrospective study in which we enrolled pediatric ADEM patients and controls (Cs) from neurology units between 2017 and 2021. We measured indices in patients using brainstem auditory evoked potentials (BAEPs), visual evoked potentials (VEPs) and somatosensory evoked potentials (SEPs), and then we analyzed their early diagnostic value in ADEM patients. RESULTS: The mean age of the ADEM group was 6.15 ± 3.28 years (range,1-12 years) and the male/female ratio was 2.1:1 The mean age of the Cs was 5.97 ± 3.40 years (range,1-12 years) and the male/female ratio was 1.3:1. As we used magnetic resonance imaging (MRI) as the diagnostic criterion, the sensitivity, specificity, and accuracy (κ was 0.88) of multimodal EPs were highly consistent with those of MRI; and the validity could be ranked in the following order with respect to the diagnosis of ADEM: multimodal Eps > single SEP > single VEP > single BAEP. Of 34 patients with ADEM, abnormalities in multimodal EPs were 94.12%, while abnormalities in single VEPs, BAEPs and SEPs were 70.59%,64.71%and 85.3%, respectively. We noted significant differences between single VEP/BAEPs and multimodal EPs (χ2 = 6.476/8.995,P = 0.011/0.003). CONCLUSIONS: The combined application of multimodal EPs was superior to BAEPs, VEPs, or SEPs alone in detecting the existence of central nerve demyelination, and we hypothesize that these modalities will be applicable in the early diagnosis of ADEM.


Subject(s)
Encephalomyelitis, Acute Disseminated , Evoked Potentials, Visual , Humans , Child , Female , Male , Infant , Child, Preschool , Encephalomyelitis, Acute Disseminated/diagnosis , Retrospective Studies , Evoked Potentials, Auditory, Brain Stem/physiology , Evoked Potentials, Somatosensory/physiology
8.
J Neurol ; 271(5): 2662-2671, 2024 May.
Article in English | MEDLINE | ID: mdl-38366070

ABSTRACT

BACKGROUND: Myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease (MOGAD) is an idiopathic central nervous system (CNS) demyelinating disease gaining recognition with wider availability of cell-based assay (CBA) testing and recently published diagnostic criteria. However, uncertainty remains regarding the interpretation of antibody titers, particularly cerebrospinal fluid (CSF) MOG antibody titers. METHODS: All MOG IgG CBA results performed by the provincial MitogenDx laboratory in Alberta from July 2017 to July 2023 were retrieved. Chart review was performed in patients with both serum and CSF testing and ≥ 1 positive MOG antibody result. Demographics, antibody titers, clinical and imaging features, treatment, and diagnosis were analyzed based on serum/CSF status. RESULTS: Among 4494 MOG CBA assays, there were 413 CSF samples in 402 patients, and 268 patients had at least one associated serum sample. Mean time between CSF and serum testing was 20.9 days (range 0-870 days), most with testing within 30 days. Five of the 268 patients had serum positive/CSF positive MOG antibodies, 4 with acute disseminated encephalomyelitis and 1 with longitudinally extensive transverse myelitis. Twenty-three patients had serum positive/CSF negative MOG and 13/23 with optic neuritis. CSF MOG antibody positive patients were younger, and more likely to remain MOG seropositive versus CSF negative patients. No seronegative patient had MOG antibodies in CSF. CONCLUSIONS: In province-wide testing, CSF MOG antibodies were rare, only in MOG seropositive patients and none with optic neuritis. Our study does not support a clear role for CSF MOG antibody testing in the majority of patients, although further study is required.


Subject(s)
Autoantibodies , Myelin-Oligodendrocyte Glycoprotein , Humans , Myelin-Oligodendrocyte Glycoprotein/immunology , Male , Female , Adult , Middle Aged , Autoantibodies/cerebrospinal fluid , Autoantibodies/blood , Aged , Adolescent , Young Adult , Child , Aged, 80 and over , Child, Preschool , Demyelinating Autoimmune Diseases, CNS/cerebrospinal fluid , Demyelinating Autoimmune Diseases, CNS/immunology , Demyelinating Autoimmune Diseases, CNS/diagnosis , Demyelinating Autoimmune Diseases, CNS/blood , Encephalomyelitis, Acute Disseminated/diagnosis , Encephalomyelitis, Acute Disseminated/cerebrospinal fluid , Encephalomyelitis, Acute Disseminated/immunology , Encephalomyelitis, Acute Disseminated/blood , Retrospective Studies , Optic Neuritis/cerebrospinal fluid , Optic Neuritis/immunology , Optic Neuritis/diagnosis , Optic Neuritis/blood
9.
Mult Scler Relat Disord ; 84: 105497, 2024 Apr.
Article in English | MEDLINE | ID: mdl-38364768

ABSTRACT

BACKGROUND: Prognostic markers for relapse and neurological disability following the first clinical event in children with myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) remain lacking. We investigated the clinical profiles and early prognostic factors associated with relapsing disease or impaired functional outcome in a large single-center cohort of pediatric MOGAD. METHODS: We retrospectively analyzed the clinical and paraclinical data and treatment outcomes of children with MOGAD seen at Children's Health in Dallas, Texas from 2009 to 2022. Univariate analyses were used to evaluate factors from initial event associated with relapsing disease course and impaired functional outcome (modified Rankin scale [mRS] >1) at final follow-up. RESULTS: Our cohort comprised of 87 children of diverse race/ethnicity. Presentation with acute disseminated encephalomyelitis (ADEM) was more frequent in children aged ≤8 years and Caucasian background, whereas presentation with optic neuritis was more common in children aged >8 years and other race/ethnicity. 44.3 % (27/61) had relapsing disease course, of whom 48.0 % had multiple relapses. 30.3 % (23/76) had final mRS >1. Children with abnormal electroencephalogram had reduced relapse risk. Children with ADEM presentation, severe disease, low MOG-IgG titer, and central and systemic inflammation (represented by cerebrospinal fluid pleocytosis and serum leukocytosis, respectively) at onset had higher likelihood of final mRS >1. CONCLUSION: Abnormal electroencephalogram at the first event was associated with reduced relapse risk while disease severity and peripheral inflammation significantly contributed to final neurological disability. Further studies are needed to validate these findings as early risk factors for disability and relapse and to identify optimal treatment strategies.


Subject(s)
Autoantibodies , Encephalomyelitis, Acute Disseminated , Child , Humans , Myelin-Oligodendrocyte Glycoprotein , Retrospective Studies , Encephalomyelitis, Acute Disseminated/diagnosis , Inflammation , Chronic Disease , Disease Progression , Recurrence
10.
Medicina (Kaunas) ; 60(1)2024 Jan 22.
Article in English | MEDLINE | ID: mdl-38276067

ABSTRACT

Acute disseminated encephalomyelitis (ADEM) is an inflammatory demyelinating disorder that typically follows an infection or recent vaccination. Symptoms such as encephalopathy and focal neurological deficits appear weeks after the initial illness, leading to swift and progressive neurological decline. While ADEM in the brain has been well documented, reports of ADEM, specifically in the spinal cord, are relatively limited. A 58-year-old male presented with rapidly progressive bilateral lower extremity tingling, numbness, and mild gait disturbance approximately two days prior to visiting the emergency room. Spinal magnetic resonance imaging revealed a diffuse, longitudinal, high-signal lesion with mild enlargement of the conus and proximal cauda equina. The lesions were predominantly localized in the distal conus and cauda equina, and serial electrodiagnostic studies showed that the lesions progressed toward the proximal conus in tandem with symptom evolution and lacked clear lateralization. The patient was subsequently treated with high-dose steroids for seven days (intravenous methylprednisolone, 1 mg/kg). The patient's lower extremity weakness gradually improved and he was able to walk independently under supervision three weeks after symptom onset. In this case of spinal ADEM in a middle-aged adult, high-dose steroid treatment led to outstanding neurological recovery from both the initial occurrence and subsequent attacks.


Subject(s)
Encephalomyelitis, Acute Disseminated , Spinal Cord Compression , Male , Middle Aged , Adult , Humans , Encephalomyelitis, Acute Disseminated/diagnosis , Encephalomyelitis, Acute Disseminated/drug therapy , Spinal Cord Compression/complications , Spinal Cord Compression/pathology , Brain/pathology , Magnetic Resonance Imaging/adverse effects , Brain Damage, Chronic
11.
Ther Apher Dial ; 28(1): 119-124, 2024 Feb.
Article in English | MEDLINE | ID: mdl-37646345

ABSTRACT

INTRODUCTION: Acute disseminated encephalomyelitis is a rare acute demyelinating disease of the central nervous system (CNS). The pathogenesis remains unclear but is suspected to be autoimmune. High doses of methylprednisolone (HDMP) are currently considered standard of treatment. Plasmapheresis (PE) is typically given in steroid refractory cases. There is currently limited evidence supporting its use in ADEM. MATERIALS AND METHODS: We report a 16-year-old girl with ADEM who improved rapidly after initiating PE. RESULTS: The patient presented with acute onset of multifocal CNS symptoms, including encephalopathy, requiring intensive care unit management. Despite HDMP administration, her clinical condition continued to deteriorate. PE was therefore initiated on the same day as HDMP. Her clinical condition improved significantly following the first session. She was extubated and discharged from the intensive care unit the following day. CONCLUSION: HDMP combined with PE may be an effective first-line treatment in patients with fulminant ADEM.


Subject(s)
Encephalomyelitis, Acute Disseminated , Myelitis, Transverse , Humans , Female , Adolescent , Encephalomyelitis, Acute Disseminated/therapy , Encephalomyelitis, Acute Disseminated/diagnosis , Encephalomyelitis, Acute Disseminated/etiology , Myelitis, Transverse/therapy , Myelitis, Transverse/complications , Plasmapheresis , Methylprednisolone/therapeutic use , Intensive Care Units , Magnetic Resonance Imaging
12.
Rev. bras. oftalmol ; 83: e0008, 2024. graf
Article in Portuguese | LILACS | ID: biblio-1535604

ABSTRACT

RESUMO A encefalomielite aguda disseminada é uma doença rara, aguda, inflamatória e desmielinizante do sistema nervoso central, presumivelmente associada, em mais de três quartos dos casos, a infecções (virais, bacterianas ou inespecíficas) e imunizações ou sem qualquer antecedente indentificável. Habitualmente, apresenta um curso monofásico com início de sintomas inespecíficos na fase prodrómica, podendo evoluir com alterações neurológicas multifocais e até à perda total da acuidade visual. Descrevemos o caso de um menino de 9 anos com quadro inicial de edema de papila causado por encefalomielite aguda disseminada devido a Bartonella henselae. Apesar da gravidade da doença, o diagnóstico e o tratamento precoce proporcionaram bons desfechos.


ABSTRACT Acute disseminated encephalomyelitis is a rare, acute, inflammatory, and demyelinating disease of the central nervous system. Presumably associated in more than three quarters of cases by infections (viral, bacterial, or nonspecific) and immunizations or without any identifiable antecedent. It usually presents a monophasic course with onset of nonspecific symptoms in the prodromal phase and may evolve with multifocal neurological changes and even visual acuity loss. We describe a case of a 9-year-old boy with an initial picture of papillary edema caused by acute disseminated encephalomyelitis due to Bartonella henselae. Despite the severity of the disease, early diagnosis and treatment provided good outcomes.


Subject(s)
Humans , Male , Child , Cat-Scratch Disease/complications , Encephalomyelitis, Acute Disseminated/etiology , Methylprednisolone/administration & dosage , Magnetic Resonance Imaging , Cat-Scratch Disease/diagnosis , Cat-Scratch Disease/drug therapy , Visual Acuity , Doxycycline/administration & dosage , Bartonella henselae , Encephalomyelitis, Acute Disseminated/diagnosis , Encephalomyelitis, Acute Disseminated/drug therapy , Slit Lamp Microscopy , Fundus Oculi , Headache
13.
Rev Med Inst Mex Seguro Soc ; 61(5): 695-701, 2023 Sep 04.
Article in Spanish | MEDLINE | ID: mdl-37773190

ABSTRACT

Background: Acute disseminated encephalomyelitis is an autoimmune and demyelinating disease. It is rare in adults. It has 3 main variants. One of them is Weston-Hurst syndrome, also called acute hemorrhagic leukoencephalitis. The objective was to share the experience in the diagnostic and therapeutic approach of this rare disease, as well as make a review of the current bibliography, in order to collaborate in the knowledge of this disease. Clinical case: 27-year-old woman, with a viral respiratory infection 2 weeks prior to the development of a neurological syndrome characterized by paresthesia, motor deficit, status epilepticus and acute encephalopathy, progressing rapidly to coma, with evidence in MRI of diffuse hemorrhagic lesions in cerebral white matter with demyelination and peripheral edema. It was administered steroid treatment for 5 days, with improvement of symptoms, but with motor and sensory deficits persisting. Conclusion: Acute disseminated encephalomyelitis and its variants are rare entities, with an important range of differential diagnosis, which must be identified and quickly treated to avoid their lethal or disabling outcome.


Introducción: la encefalomielitis aguda diseminada es una enfermedad autoinmune y desmielinizante. Es rara en el adulto. Cuenta con tres variantes principales. Una de ellas es el síndrome de Weston Hurst, también conocido como leucoencefalitis hemorrágica aguda. El objetivo fue compartir la experiencia en el abordaje diagnóstico y terapéutico de esta rara enfermedad, así como hacer una revisión de la bibliografía actual, a fin de colaborar con el conocimiento de esta. Caso clínico: mujer de 27 años con cuadro de infección respiratoria viral 2 semanas previas al desarrollo de síndrome neurológico caracterizado por parestesias, déficit motor, estatus epiléptico y encefalopatía aguda, el cual progresó a estado de coma y evidenció en resonancia magnética lesiones difusas hemorrágicas en sustancia blanca cerebral con desmielinización y edema periférico. Se inició tratamiento con esteroides por 5 días con mejora de síntomas, aunque persistió el déficit motor y sensitivo. Conclusión: la encefalomielitis aguda diseminada y la variante hemorrágica de esta son entidades raras, con una importante gama de diagnóstico diferencial, que deben ser identificadas y tratadas de forma rápida para evitar su letal o incapacitante desenlace.


Subject(s)
Encephalomyelitis, Acute Disseminated , Leukoencephalitis, Acute Hemorrhagic , Status Epilepticus , Adult , Female , Humans , Leukoencephalitis, Acute Hemorrhagic/diagnosis , Leukoencephalitis, Acute Hemorrhagic/etiology , Leukoencephalitis, Acute Hemorrhagic/pathology , Encephalomyelitis, Acute Disseminated/diagnosis , Encephalomyelitis, Acute Disseminated/etiology , Encephalomyelitis, Acute Disseminated/pathology , Magnetic Resonance Imaging/adverse effects
15.
Tijdschr Psychiatr ; 65(6): 388-391, 2023.
Article in Dutch | MEDLINE | ID: mdl-37434580

ABSTRACT

We describe a case of a 36-year-old woman with no psychiatric or somatic history who was presented to the emergency department with a profound change in mental status, more precisely a catatonic status and auditory hallucinations. Due to the unclear aetiology and suspicion of underlying psychiatric problems, the patient was admitted to the psychiatric ward. After discharge against medical advice, readmission was necessary due to deterioration and sudden onset of myoclonus. On further examination, acute disseminated encephalomyelitis (ADEM) was diagnosed. This case illustrates that ADEM can present itself as an initial psychiatric problem and emphasizes the importance of extensive medical clearance at presentation and continued attention for possible somatic origin, even when the initial clearance turns out to be negative.


Subject(s)
Catatonia , Encephalomyelitis, Acute Disseminated , Female , Humans , Adult , Encephalomyelitis, Acute Disseminated/diagnosis , Emergency Service, Hospital , Hospitalization
16.
Pediatr Neurol ; 146: 44-49, 2023 09.
Article in English | MEDLINE | ID: mdl-37429226

ABSTRACT

BACKGROUND: Acute disseminated encephalomyelitis (ADEM) is an immune-mediated demyelinating disorder of the central nervous system that is usually triggered by infections. We aimed to determine the temporal trends in the incidence of ADEM before and during the pandemic and their correlation with viral epidemiology. METHODS: We conducted a nationwide, population-based, retrospective, ADEM cohort study by using the Health Insurance Review and Assessment Service database. New-onset ADEM was defined as a patient (age <19 years) who was hospitalized with a diagnostic code of G04.0, G36.8, and G36.9 and a prescription of intravenous methylprednisolone. The National Infectious Disease Surveillance System was used to collect the nationwide viral epidemics. RESULTS: A total of 185 new-onset pediatric ADEM cases were included. The mean patient age was 7.0 ± 4.9 years. The incidence of ADEM was 0.34 to 0.48 of 100,000 persons per year before the pandemic, which dropped to 0.22 of 100,000 persons per year during the first pandemic year. The risk of ADEM occurrence was approximately 1.74% higher during the prepandemic years compared with the first pandemic year (odds ratio = 1.017, P = 0.009). There was a weak positive correlation between acute respiratory viral infection and ADEM incidence (r = 0.28, P = 0.03). CONCLUSION: This study demonstrates how infection control during the early coronavirus disease 2019 (COVID-19) pandemic influenced the incidence of ADEM. The low incidence of ADEM in the early COVID-19 pandemic may be related to the decline in acute respiratory viral infections, which are potential triggers of ADEM.


Subject(s)
COVID-19 , Encephalomyelitis, Acute Disseminated , Child , Humans , Young Adult , Adult , Child, Preschool , Encephalomyelitis, Acute Disseminated/diagnosis , Pandemics , COVID-19/epidemiology , COVID-19/complications , Cohort Studies , Retrospective Studies , Incidence
17.
Rinsho Shinkeigaku ; 63(8): 518-522, 2023 Aug 29.
Article in Japanese | MEDLINE | ID: mdl-37518016

ABSTRACT

The patient is an 18-year-old female. She had a history of acute disseminated encephalomyelitis at the age of 6 and 7. She visited our hospital due to acute disturbance of consciousness, quadriplegia, and numbness of left upper and lower extremities. Brain MRI showed multiple DWI/FLAIR high-signal lesions in the bilateral cerebral hemispheres, cerebellum, and brainstem. Qualitative test indicated that serum anti-MOG antibodies was positive, and she was diagnosed with anti-MOG antibody-positive polyphasic disseminated encephalomyelitis. Intravenous mPSL pulse therapy was performed twice, but the symptoms worsened. As a second line treatment, plasma exchange was started. However, she developed transfusion related acute lung injury. Alternatively, she was treated with immunoadsorption plasmapheresis. Her symptoms were significantly improved. This case seems to be valuable because there are few reports showing effectiveness of immunoadsorption therapy on anti-MOG antibody-related diseases, especially for polyphasic disseminated encephalomyelitis.


Subject(s)
Encephalomyelitis, Acute Disseminated , Female , Humans , Autoantibodies , Encephalomyelitis, Acute Disseminated/etiology , Encephalomyelitis, Acute Disseminated/therapy , Encephalomyelitis, Acute Disseminated/diagnosis , Myelin-Oligodendrocyte Glycoprotein , Oligodendroglia , Plasmapheresis/adverse effects
18.
Mult Scler Relat Disord ; 76: 104781, 2023 Aug.
Article in English | MEDLINE | ID: mdl-37295322

ABSTRACT

Postinfectious neurological syndromes (PINS), among which acute disseminated encephalomyelitis (ADEM), are inflammatory and mostly monophasic disorders. We previously reported that PINS patients can show relapses, or even disease progression. Here we describe a cohort of patients with progressive-PINS and >5 years of follow-up, that developed a progressive worsening without radiological/cerebrospinal fluid analysis evidence of inflammation. At onset 5 patients fulfilled diagnostic criteria for ADEM and none for MS. Progression occurred after a median of 22 months from onset (in 4/7 after 1/more relapses), manifesting as ascending tetraparesis with bulbar functions involvement in 5/7. Five/7 patients received high dose steroids and/or IvIG and 6/7 Rituximab(n = 4) and/or cyclophosphamide(n = 2), with no impact on disease progression in 6/7. NfL levels were higher in patients with progressive-PINS compared to monophasic-ADEM (p = 0.023) and healthy controls (p = 0.004). Progression is rare, but possible, in PINS. Immunotherapy seems to be ineffective in these patients, and elevated serum NfL in serum suggest persistent axonal damage.


Subject(s)
Encephalomyelitis, Acute Disseminated , Intermediate Filaments , Humans , Follow-Up Studies , Encephalomyelitis, Acute Disseminated/diagnosis , Disease Progression , Recurrence
19.
J Clin Neurosci ; 111: 57-70, 2023 May.
Article in English | MEDLINE | ID: mdl-36963124

ABSTRACT

BACKGROUND: Although global vaccination against COVID-19 infection has its excellence, potential side effects are yet of concern. Several lines of evidence have proposed ADEM occurrence after SARS-CoV-2 infection. Moreover, a large number of case reports and case series have also suggested the casual association between ADEM and COVID-19 vaccination. To better understand the development of ADEM following COVID-19 vaccination and its potential association, we aimed to systematically review ADEM cases reported after COVID-19 vaccination. METHODS: We conducted a comprehensive systematic search using three databases including PubMed, Scopus, and Web of Science. Studies that reported ADEM after COVID-19 vaccination were eligible to include in our study. Observational studies, case reports, and case series which reported cases of ADEM with sufficient detail to confirm clinical diagnosis following COVID-19 vaccination were eligible to enter our study. RESULTS: Twenty studies were included in our systematic review after the abstract and full-text screening with a total of 54 cases. Among included patients, 45 (85.1 %) developed ADEM after the first dose of the COVID-19 vaccine, and seven (12.9 %) cases experienced ADEM after the second dose. The median time interval between vaccination and neurological symptoms was 14 days which ranged from 12 h to 63 days. Twelve (22.2 %) patients experienced symptoms of muscle weakness, ten (18.5 %) presented unconsciousness, nine (16.6 %) patients had urinary complaints, nine (16.6 %) had visual impairments, and five (9.2 %) experienced a seizure. After treatments, four (13.8 %) patients died. Forty-six patients had clinical improvement (85.1 %), also improvement in brain MRI was observed among 44 (81.4 %) patients. CONCLUSION: In conclusion, it is not clear that ADEM could be a potential complication of COVID-19 vaccination based on the current evidence and further studies are needed. However, this rare condition should not trigger stopping the mass vaccination programs since the only way to eradicate the current pandemic of COVID-19 is to extend the number of immunized people.


Subject(s)
COVID-19 Vaccines , COVID-19 , Encephalomyelitis, Acute Disseminated , Humans , COVID-19/prevention & control , COVID-19/complications , COVID-19 Vaccines/adverse effects , Encephalomyelitis, Acute Disseminated/etiology , Encephalomyelitis, Acute Disseminated/diagnosis , Observational Studies as Topic , SARS-CoV-2 , Vaccination/adverse effects
20.
Arch. Soc. Esp. Oftalmol ; 98(2): 116-120, feb. 2023. ilus
Article in Spanish | IBECS | ID: ibc-215180

ABSTRACT

La encefalomielitis diseminada aguda es una enfermedad inflamatoria-desmielinizante inmunomediada que suele manifestarse tras una infección o vacunación en niños en edad escolar. Típicamente presenta una fase prodrómica con un cuadro pseudogripal seguida de una fase con clínica muy variada, pudiendo aparecer alteraciones neurooftalmológicas como oftalmoplejía o neuritis óptica.La etiología es variada, incluyendo enfermedades tumorales, vasculares, infecciosas, inflamatorias y desmielinizantes. El diagnóstico se basa en la historia clínica y en las características de la resonancia magnética cerebral, prueba de imagen de elección. El estudio del líquido cefalorraquídeo puede servir de ayuda en la orientación del cuadro clínico.El pronóstico es favorable, con excelente respuesta a los corticoides e inmunoglobulinas y con mínimas secuelas a largo plazo en la mayoría de los casos.Presentamos el caso de un varón de 8años con enfermedad desmielinizante aguda por adenovirus cuya manifestación fue un síndrome del ocho y medio. (AU)


Acute disseminated encephalomyelitis is an immune mediated inflammatory-demyelinizing disease that usually manifests after infection or vaccination in school-age children. It typically presents a prodromal phase with flu-like symptoms, followed by a phase with varied clinical symptoms, neuro-ophthalmological alterations such as ophthalmoplegia or optic neuritis may occur.The differential diagnosis includes tumor, vascular, infectious, inflammatory and demyelinating diseases. Diagnosis is based on the clinical history and the characteristics of brain magnetic resonance imaging, the gold standard test. The study of the cerebrospinal fluid can help to guide the clinical picture.The prognosis is favorable, with an excellent response to corticosteroids and immunoglobulins, with minimal long-term sequelae in most cases.We report the case of an 8-year-old male with acute demyelinating disease due to adenovirus whose manifestation was an eight-and-a-half syndrome. (AU)


Subject(s)
Humans , Male , Child , Encephalomyelitis, Acute Disseminated/diagnosis , Encephalomyelitis, Acute Disseminated/virology , Ophthalmoplegia/virology , Adenoviridae Infections/complications , Tomography, X-Ray Computed , Magnetic Resonance Imaging , Syndrome
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