Acute abducens nerve palsy with acute disseminated encephalomyelitis-like presentation following COVID-19 vaccination

We report two adult cases of abducens nerve palsy presenting immediately (within weeks) after they received the first dose of Covishield vaccination. Magnetic resonance imaging (MRI) of the brain obtained after the onset of diplopia demonstrated demyelinating changes. The patients had associated systemic symptoms. Post-vaccination demyelination typically known as acute disseminated encephalomyelitis (ADEM) associated with several vaccines is more common in children. Although the mechanism of the nerve palsy remains unclear, it is suspected to be related to the post-vaccine neuroinflammatory syndrome. Cranial nerve palsies and ADEM-like presentations may represent part of the neurologic spectrum following COVID-vaccination in adults, and ophthalmologists should be aware of these sequelae. Although cases of sixth nerve palsy following COVID vaccination are already reported, associated MRI changes have not been reported from India.

Clinical analysis and follow-up study of 32 children with first diagnosed acute disseminated encephalomyelitis / 国际儿科学杂志

Objective:To analyze the clinical features, risk factors of the prognosis and recurrence of first diagnosed acute disseminated encephalomyelitis(ADEM)in children.Methods:The clinical characteristics of 32 children with first diagnosed ADEM who were treated in the Department of Neurology of Shanghai Children′s Medical Center from January 1, 2012 to January 31, 2022 were analyzed retrospectively and followed up.Results:After follow-up, 27 of the 32 patients were diagnosed as ADEM and 4 were diagnosed as multiphasic disseminated encephalomyelitis(MDEM), 1 was diagnosed as ADEM followed by optic neuritis(ADEM-ON).Among the 27 patients diagnosed with ADEM, most of them were girls, 3~8 years old, had a history of infection before onset, occurred in summer and autumn.Common clinical symptoms included disturbance of consciousness or mental and behavior disorders(27/27, 100.0%), fever(13/27, 48.1%), paralysis(13/27, 48.1%), abnormal defecation(7/27, 25.9%), decreased vision(7/27, 25.9%)and so on.Brain MRI mainly involved subcortical white matter(20/27, 74.1%), cerebellum(10/27, 37.0%), deep nuclei such as thalamus and basal ganglia(7/27, 25.9%)and brainstem(3/27, 11.1%).In 10 cases, myelin oligodendrocyte glycoprotein(MOG)antibody tests showed positive results in 3(30.0%)and negative results in 7(70.0%).There were no significant differences in gender, age, history of infection before onset, season of onset, clinical symptoms, peripheral WBC, CRP, erythrocyte sedimentation rate, levels of WBC and protein in cerebrospinal fluid, electroencephalogram, brain and spinal cord MRI, treatment and prognosis between the MOG antibody positive and negative children(all P>0.05).16 cases (59.3%)were recovery completely, 11 cases (40.7%)were not, the rate of the infection before onset of the former was higher than the latter( P<0.05).27 cases (84.4%)had monophasic course and 5 cases (15.6%)had non-monophasic course, more of the nonmonophasic children′s brain MRI show multiple abnormal signals in the brain than the monophasic children( P<0.05). Conclusion:Children with first diagnosed ADEM require long-term clinical follow-up, and brain MRI show a higher risk of recurrence of multiple abnormal signals in the brain.The prognosis of ADEM children with a history of infection before onset is relatively good.

Acute disseminated encephalomyelitis (ADEM) associated with mosquito-borne diseases: Chikungunya virus X yellow fever immunization

Abstract Acute disseminated encephalomyelitis (ADEM) is a demyelinating autoimmune neuropathic condition characterized by extensive bilateral and confluent lesions in the cerebral white matter and cerebellum. The basal ganglia and gray matter may also be involved. In most cases, the symptoms are preceded by viral infection or vaccination. In this report, we present a case of ADEM associated with optic neuritis presenting alongside two potential triggering factors: chikungunya virus infection and yellow fever immunization.

The clinical and radiological features of myelin oligodendrocyte glycoprotein antibody associated disease / 中华神经科杂志

Objective To explore the clinical and radiological features of myelin oligodendrocyte glycoprotein (MOG) antibody associated disease.Methods The clinical data of 22 MOG antibody associated disease cases treated in the Department of Neurology,Qilu Hospital of Shandong University from January 2017 to June 2019 were retrospectively analyzed.The clinical data of MOG antibody associated disease were summarized,including clinical and imaging features.Results Of the 22 included patients with MOG antibody associated disease,the average age was 38.5 years,13 were male and nine were female.Among them,11 cases manifested as aquaporin-4 (AQP4)-negative neuromyelitis optica spectrum disorder (NMOSD),four cases optic neuritis,two cases transverse myelitis,one case acute disseminated encephalomyelitis (ADEM),two cases cortical encephalitis and two cases vestibular neuronitis.Magnetic resonance imaging (MRI) results showed that multiple anatomical areas were involved.Among the nine patients with optic nerve involvement,five patients had longitudinally extensive optic nerve lesions,which were longitudinally enhanced.In eight patients,MRI lesions in the spinal cord showed mostly long or short segments involvement,involving 2-5 spinal cord segments.Five cases involved the cervical spinal cord,six cases involved the thoracic spinal cord,and one case involved the lumbar spinal cord.Brain MRI abnormalities were found in 13 cases and the lesions were mostly patchy and point-shaped.MRI lesions demonstrated T2 hyperintensity and some of them could be strengthened,which may involve the basal ganglia,thalamus,radiographic crown,frontal temporal lobe,brain stem and other parts.Among them,16 patients were sensitive to high-dose intravenous/oral methylprednisolone in the acute phase.Seven patients had recurrence after two months to two years of follow-up.Conclusions MOG antibody associated disease include multiple manifestations.Among them,AQP4-negative NMOSD is the most common form.The clinical manifestations of patients showed diversity.Imaging is characterized by multiple parts involvement such as optic nerve,spinal cord,and brain.Most patients are sensitive to high-dose intravenous/oral methylprednisolone,and have a good prognosis in the acute phase,but some patients may relapse.

The clinical and radiological features of myelin oligodendrocyte glycoprotein antibody associated disease / 中华神经科杂志

Objective@#To explore the clinical and radiological features of myelin oligodendrocyte glycoprotein (MOG) antibody associated disease.@*Methods@#The clinical data of 22 MOG antibody associated disease cases treated in the Department of Neurology, Qilu Hospital of Shandong University from January 2017 to June 2019 were retrospectively analyzed. The clinical data of MOG antibody associated disease were summarized, including clinical and imaging features.@*Results@#Of the 22 included patients with MOG antibody associated disease, the average age was 38.5 years, 13 were male and nine were female. Among them, 11 cases manifested as aquaporin-4 (AQP4)-negative neuromyelitis optica spectrum disorder (NMOSD), four cases optic neuritis, two cases transverse myelitis, one case acute disseminated encephalomyelitis (ADEM), two cases cortical encephalitis and two cases vestibular neuronitis. Magnetic resonance imaging (MRI) results showed that multiple anatomical areas were involved. Among the nine patients with optic nerve involvement, five patients had longitudinally extensive optic nerve lesions, which were longitudinally enhanced. In eight patients, MRI lesions in the spinal cord showed mostly long or short segments involvement, involving 2-5 spinal cord segments. Five cases involved the cervical spinal cord, six cases involved the thoracic spinal cord, and one case involved the lumbar spinal cord. Brain MRI abnormalities were found in 13 cases and the lesions were mostly patchy and point-shaped. MRI lesions demonstrated T2 hyperintensity and some of them could be strengthened, which may involve the basal ganglia, thalamus, radiographic crown, frontal temporal lobe, brain stem and other parts. Among them, 16 patients were sensitive to high-dose intravenous/oral methylprednisolone in the acute phase. Seven patients had recurrence after two months to two years of follow-up.@*Conclusions@#MOG antibody associated disease include multiple manifestations. Among them, AQP4-negative NMOSD is the most common form. The clinical manifestations of patients showed diversity. Imaging is characterized by multiple parts involvement such as optic nerve, spinal cord, and brain. Most patients are sensitive to high-dose intravenous/oral methylprednisolone, and have a good prognosis in the acute phase, but some patients may relapse.

Neuritis óptica recurrente posterior a encefalomielitis diseminada aguda en pediatría. Reporte de un caso / Recurrent optic neuritis after acute disseminated encephalomyelitis in pediatrics. A case report

Abstract: Optic Neuritis (ON) is a condition caused by the inflammation of the optic nerve, causing diminished visual acuity and ocular pain. It is tightly related to Multiple Sclerosis (MS), often being the first demyelinating event. There is a 31% risk of recurrence during the first 10 years after the diagnosis, and 48% of the patients end up being diagnosed with Multiple Sclerosis1. Imaging studies like brain MRI (Magnetic Resonance Imaging) have a critical role in the diagnosis and prognosis of ON, as well as in the recognition of MS.2 The patient is a 11-year-old girl with recurrent ON and past medical history of Acute Disseminated Encephalomyelitis (ADEM) when she was 5 years old. ON diagnosis was based on clinical findings as well as on ophtalmologic, electrophysiologic and imaging studies. The recurrent episodes of ON improved after the use of high dose steroids. Recurrences were observed after titration of the dose, but remission was achieved after adjustment of treatment. During one of the recurrent episodes, blood work was performed to evaluate possible underlying infectious, demyelinating or autoinmmune process. Anti-MOG antibodies were found positive. The patient at the moment is not presenting with any other criteria suggesting MS or Optic Neuromyelitis, but long term follow up is adviced. Key words: Optic neuritis (ON), multiple sclerosis (MS), acute disseminated encephalomyelitis (ADEM), magnetic resonance imaging (MRI), cerebrospinal fluid (CSF), right eye (RE); visual acuity (VA), optic neuromyelitis (ONM)

Treinamento do assoalho pélvico na retenção urinária decorrente de encefalomielite aguda disseminada / Pelvic floor muscle training in neurogenic bladder due to acute disseminated encephalomyelitis syndrome

A dengue pode desencadear manifestações neurológicas como a Síndrome de Encefalomielite Aguda Disseminada (ADEM), de caráter inflamatório, desmielinizante, que pode ter dentre as consequências déficits motores e sensitivos, neurite ótica e disfunções vesicais, como a retenção urinária, tornando o indivíduo dependente da realização do cateterismo vesical intermitente (CVI). Desta forma, o objetivo deste estudo é descrever o tratamento fisioterapêutico de uma paciente com retenção urinária, decorrente de Síndrome de ADEM pós-dengue. Paciente do sexo feminino, 52 anos, internada em hospital de média complexidade com diagnóstico de ADEM. Na avaliação inicial apresentava grau de força muscular 3 de membros inferiores e 4 de membros superiores, 2 de musculatura do assoalho pélvico (MAP) e retenção urinária, necessitando realizar CVI. Foram realizados 32 atendimentos com duração de uma hora, incluindo o treinamento do assoalho pélvico em diferentes posturas e eletroestimulação de superfície da MAP com equipamento da marca Ibramed. Paciente recebeu alta hospitalar com evidente melhora da força muscular global e sem necessidade de CVI, apresentando micção voluntária e sem resíduo pós-miccional, favorecendo a prevenção de agravos do trato urinário inferior e superior. (AU)

Dengue can trigger neurological manifestations such as Acute Disseminated Encephalomyelitis (ADEM), with inflammatory, demyelinating condition, which may have, among the consequences, motor and sensory deficits, optic neuritis and bladder dysfunctions, such as urinary retention, making the individual dependent on intermittent bladder catheterization (IBC). Therefore, the objective of this study was to describe the physiotherapeutic treatment of a patient with urinary retention, due to ADEM syndrome after dengue. A 52-year-old female patient admitted to a hospital of medium complexity with a diagnosis of ADEM. In the initial evaluation, she presented a degree 3 of muscular strength in the lower limbs and 4 in the upper limbs, 2 in pelvic floor musculature (PFM) and urinary retention, requiring IBC. We performed 32 one hour sessions, including pelvic floor muscle training in different positions and surface electrostimulation of PFM with Ibramed brand equipment. Patient was discharged with evident improvement of global muscle strength and without IBC, presenting voluntary voiding and without post void residual, favoring the prevention of lower and upper urinary tract injuries. (AU)

Enfermedades desmielinizantes en niños: Encefalomielitis diseminada aguda y esclerosis múltiple / Demyelinizing diseases in children. Acute disseminated encephalomyelitis and multiple sclerosis

Se revisan dos de las principales enfermedades desmielinizantes en niños, la encefalomielitis aguda diseminada (EAD) y la esclerosis múltiple (EM). Por sus características fisiopatológicas, etiologías probables, manifestaciones clínicas, diagnóstico, tratamiento, pronóstico, evolución, así como alteraciones atípicas que complican su diagnóstico; cuanto más pequeño es el paciente se necesita estudiar más, antes de llegar al diagnóstico. El Grupo Internacional de Estudio de Esclerosis Múltiple Pediátrica publicó las definiciones operativas para enfermedades desmielinizantes adquiridas del sistema nervioso central: la EAD es monofásica, polisintomática y con encefalopatía. Su duración es de hasta 3 meses, con síntomas fluctuantes y hallazgos en resonancia magnética. La EM se define como síndrome aislado monofocal o polifocal, sin encefalopatía. Actualmente se consideran dos enfermedades diferentes y distinguibles desde el inicio de los síntomas.

The two main demyelinating diseases in children are reviewed. Acute disseminated encephalomyelitis (ADEM) and multiple sclerosis (MS). For its physiopathological characteristics, probable etiologies, clinical manifestations, diagnosis, treatment, prognosis, evolution, as well as atypical alterations that complicate its diagnosis, the smaller the child is, more study is needed before reaching the diagnosis. The International Study Group of Multiple Pediatric Sclerosis, published the operating definitions for demyelinating diseases acquired from the central nervous system in children: the ADEM is monophasic, polysymptomatic and with encephalopathy. Its duration is up to 3 months, with fluctuating symptoms and magnetic resonance findings. MS is an isolated monofocal or polyfocal syndrome, without encephalopathy. Currently, two different and distinguishable diseases are considered from the onset of symptoms.

Encefalomielitis aguda diseminada por Mycoplasma pneumoniae en un niño previamente sano / Acute disseminated encephalomyelitis due to Mycoplasma pneumoniae in a previously healthy boy

Se presenta el caso de un niño de 5 años sin antecedentes de enfermedad, que se internó en terapia intensiva por convulsiones tónico-clónicas focalizadas en la cara y en el hemicuerpo derecho, con documentación de temperatura axilar de 37,4°C. Se descartó la presencia de gérmenes comunes y la etiología viral a través de estudios de muestras de líquido cefalorraquídeo (LCR). Se sospechó la presencia de Mycoplasma pneumoniae por comprobarse inmunofluorescencia positiva en suero para anticuerpos de clase IgM. El diagnóstico se confirmó mediante la detección del ADN de dicho patógeno sobre la biopsia cerebral efectuada por el método de la reacción en cadena de la polimerasa (PCR) y una histología compatible con encefalomielitis aguda diseminada. El paciente recibió tratamiento con claritromicina y su evolución fue favorable. Al menos dentro de nuestros conocimientos, este es el primer caso en el que se detectó ADN de M. pneumoniae en una biopsia cerebral por el método de PCR.

We present here the case of a previously healthy 5 year-old boy hospitalized in an intensive care unit due to tonic-clonic seizures focused on the face and right side of the body, and axillary temperature of 37.4 °C. Common bacterial and viral etiology was ruled out through studies of cerebrospinal fluid (CSF) samples. Mycoplasma pneumoniae was suspected by a positive immunofluorescence serum test for IgM class antibodies. Finally, with a brain biopsy, M. pneumoniae was confirmed by polymerase chain reaction (PCR) and acute disseminated encephalomyelitis by pathological anatomy. The patient was treated with clarithromycin and had an uneventful evolution. At least to our knowledge, this is the first case in which M. pneumoniae DNA was detected by PCR in a brain biopsy.

Encefalomielitis diseminada aguda posinfecciosa en un paciente con sospecha de infección por virus dengue / Postinfectious acute disseminated encephalomyelitis in a patient with suspected dengue virus infection

Introducción: La infección por virus dengue es considerada una de las arbovirosis de mayor prevalencia en los países tropicales. La encefalomielitis diseminada aguda es un trastorno inflamatorio desmielinizante y multifocal que afecta al sistema nervioso central, de inicio agudo y curso clínico monofásico. El proceso inflamatorio se encuentra mediado por mecanismos inmunológicos y su relación con infecciones por el virus dengue aún no se establece con claridad. Objetivo: Describir un caso clínico con manifestaciones del sistema nervioso central después de una probable infección por el virus dengue. Presentación del caso: Paciente femenina de 50 años, con antecedentes de hipertensión arterial controlada. Quince días después de un cuadro de fiebre de 4 días de duración, que posiblemente fue por una infección por el virus dengue, comienza con síntomas y signos de afectación neurológica caracterizadas por ligera irritabilidad, dificultad para la concentración en una actividad específica de la vida cotidiana. Progresivamente se nota dificultad motora en el hemicuerpo izquierdo además de encontrarse agitada y distraída, motivo por el cual se decide su ingreso hospitalario. Se realiza el diagnóstico a través de los hallazgos en el examen físico, los estudios positivos de resonancia magnética nuclear y el resultado positivo de la IgM de dengue en sangre. Tanto la evolución clínica como la respuesta al tratamiento con esteroides fueron favorables. Conclusiones: El evento ocurrido en este caso sugiere que los facultativos deben tener presente el diagnóstico de encefalomielitis diseminada aguda en pacientes que han tenido infección previa o alta sospecha de esta por el virus dengue(AU)

Introduction: Dengue virus infection is one of the most prevalent arboviruses in tropical countries. Acute disseminated encephalomyelitis is an inflammatory demyelinating multifocal disorder affecting the central nervous system. Its onset is acute and its clinical course monophasic. The inflammatory process is mediated by immunological mechanisms, and its relationship to dengue virus infections is still not clear. Objective: Describe a clinical case of central nervous system manifestations after probable dengue virus infection. Case presentation: Female 50-year-old patient with a history of controlled hypertension. Fifteen days after a 4-day fever episode, possibly due to dengue virus infection, the patient starts presenting neurological signs and symptoms, such as slight irritability and difficulty to concentrate on a specific activity of daily living. The patient notices progressive motor difficulty in her left hemibody and she feels agitated and distracted. It is therefore decided for her to be hospitalized. A diagnosis is made based on physical examination findings, positive nuclear magnetic resonance studies, and the positive result of the dengue IgM blood test. Both the patient's clinical evolution and her response to treatment with steroids were favorable. Conclusions: The event herein described suggests that physicians should consider the diagnosis of acute disseminated encephalomyelitis in patients with previous infection or high suspicion of infection with dengue virus(AU)

Ensayo pictórico: diagnóstico diferencial radiológico en esclerosis múltiple / Pictory essay: radiological differential diagnosis of multiple sclerosis

La esclerosis múltiple (EM) es la enfermedad inflamatorio-desmielinizante del Sistema nervioso central más prevalente en adultos. La resonancia magnética (RM) juega un rol cada vez más importante en el estudio de esta patología, en especial en su diagnóstico precoz, por lo que la diferenciación imagenológica de variantes frecuentes e infrecuentes de EM con otras patologías de sustancia blanca que comprometen encéfalo y médula espinal es esencial. Mediante una revisión pictórica se ilustrarán características típicas en RM del compromiso por EM y de variantes menos habituales de lesión desmielinizante, y se ilustrarán hallazgos característicos de lesiones relacionadas a vasculopatías inflamatorias y no inflamatorias, encefalomielitis diseminada aguda (ADEM), neuromielitis óptica (NMO) y enfermedades vasculares de la médula espinal que pueden simular EM, con énfasis en el diagnóstico diferencial radiológico.

Multiple sclerosis (MS) is the most prevalent inflammatory-demyelinating disease of the central nervous system in adult population. Magnetic resonance imaging (MRI) has an increasingly important role, especially in early diagnosis, so the imaging differentiation of frequent and infrequent variants of MS with other white matter diseases of brain and spinal cord is essential. Through a pictorial essay we show typical MR features of MS and more infrequent variants of demyelinating lesions and illustrate characteristic imaging findings of inflammatory and non-inflammatory vasculopathies, acute disseminated encephalomyelitis (ADEM), neuromyelitis optica (NMO) and vascular diseases of spinal cord that may simulate MS, with emphasis on imaging differential diagnosis.

Enfermedades desmielinizantes / Demyelinating disorders

Las enfermedades desmielinizantes constituyen un grupo de afecciones de etiología autoinmune dirigida contra la mielina del sistema nervioso central. En muchos casos, el inicio del cuadro es precedido por una infección viral inespecífica. La esclerosis múltiple evoluciona con recaídas y remisiones con déficit neurológicos polifocales, siendo los más frecuentes la neuritis óptica, la mielitis transversa y el compromiso de tronco encefálico. Se caracteriza por lesiones hiperintensas que se observan en una resonancia magnética nuclear (RMN) en T2 y FLAIR peri-ventriculares y peri-callosas, cerebelo, tronco y médula espinal. La neuromielitis óptica se caracteriza por la presencia de neuritis óptica y mielitis transversa asociada a síndrome de área postrema y diencefálico. Las lesiones en RMN se distribuyen en los sectores ricos en acuaporina-4 (AQP-4): hipotálamo, peri tercer y cuarto ventrículo, nervios ópticos y médula espinal. Los anticuerpos anti AQP4 ayudan al diagnóstico aunque no son esenciales para el mismo. La encefalomielitis diseminada aguda es un cuadro clásicamente monofásico caracterizado por una encefalopatía aguda asociada a lesiones en RMN hiperintensas en T2 y FLAIR bilaterales, asimétricas, de gran tamaño y de bordes irregulares. En los tres casos, el líquido cefalorraquídeo (LCR) puede mostrar pleocitosis e hiperproteinorraquia. La presencia de bandas oligoclonales en LCR es característica de la esclerosis múltiple. En todos los casos, el tratamiento agudo incluye corticoides a altas dosis por vía endovenoso y en caso de no respuesta, plasmaféresis. Tanto la esclerosis múltiple como la neuromielitis óptica requieren tratamiento a largo plazo para evitar nuevas recaídas ya que se trata de enfermedades recurrentes.

Demyelinating diseases are a group of conditions of autoimmune etiology directed against the myelin of the central nervous system. In many cases, the onset of the illness is preceded by a nonspecific viral infection. Multiple sclerosis is a disease that evolves with relapses and remissions with polyfocal neurological deficits, being the most frequent optic neuritis, transverse myelitis and encephalic trunk involvement. Typically, magnetic resonance image (MRI) shows peri-ventricular, peri-callosal, cerebellum, brain stem and spinal cord hyperintensive lesions in T2 and FLAIR weighted images. Optic neuromyelitis is characterized by the presence of optic neuritis and transverse myelitis associated with the postrema and diencephalic area syndrome. MRI lesions are distributed in sectors rich with aquaporine-4 channels (AQP-4): hypothalamus, third and fourth ventricle, optic nerves and spinal cord. Finding anti AQP4 antibodies is useful for the diagnosis although they are not essential for it. Acute disseminated encephalomyelitis is typically a monophasic condition characterized by acute encephalopathy associated with hyperintense MRI large, bilateral and irregular asymmetric lesion in T2 and FLAIR weighted images. In all three cases, cerebral spine fluid (CSF) can show pleocytosis and hyperproteinorrachia. The presence of oligoclonal bands in CSF is characteristic of multiple sclerosis. In all cases, acute treatment includes high dose intravenous corticosteroids and plasmapheresis in non-responsive cases. Both multiple sclerosis and optic neuromyelitis require long-term treatment to prevent relapse and recurrent diseases.

Convulsion, disturbance of consciousness, fever / 中华实用儿科临床杂志

The child was a preschool girl who was admitted for the first time due to " convulsions,disturbance of consciousness and fever for 2 days".The girl was diagnosed as acute disseminated encephalomyelitis (ADEM).After immunotherapy,the girl recovered.During the course of hormone reduction,the cranial magnetic resonance imaging showed aggravated,and neurological symptoms were followed.There was a transient decrease of blood cells.After repeat hormone treatment,the girl was improved again,but the intracranial lesions still showed progressive aggravation tendency.The course of central nervous system demyelinating in this patient was unknown.When her younger sibling prestentd fever,hepatosplenomegaly,blood cell decreased and was diagnosed with familial haemophagocytosis syndrome(FHPS),through case discussion and gene testing,the girl was finally diagnosis "central nervous system involvement at the onset of FHPS".The case suggests unexplained central nervous system demyelinating lesions,regardless of whether there are abnormalities of blood system,should pay attention to haemophagocytosis syndrome.Positive immunotherapy should be given after diagnosis,and stem cell transplantation should be given as soon as possible to improve prognosis.

Interleukin-6, S-Nitrosothiols, and Neurodegeneration in Different Central Nervous System Demyelinating Disorders: Is There a Relationship?

BACKGROUND AND PURPOSE: A few groups have suggested that activated cytokines and nitrosative stress are closely involved in the pathogenesis of different demyelinating disorders induced by the neuroinflammatory destruction of neurons. The purpose of this study was to elucidate the associations of cytokines and S-nitrosothiols (RSNO) with the severity of neurodegeneration during relapse in demyelinating disorders of the central nervous system. METHODS: We measured levels of interleukin-6 (IL-6), erythropoietin, RSNO, and phosphorylated neurofilament heavy chain (pNfh) in cerebrospinal fluid (CSF) samples obtained from patients with different demyelinating disorders: multiple sclerosis (MS, n=52), acute disseminated encephalomyelitis (ADEM, n=9), and neuromyelitis optica spectrum disorders (NMOSD) with aquaporin-4 immunoglobulin G (AQP4-IgG, n=12). We compared these levels with those measured in a control group (n=24). RESULTS: We found that IL-6 in CSF was elevated in NMOSD with AQP4-IgG and ADEM patients as well as in MS patients after the destruction of soluble IL-6. Erythropoietin levels were lower in MS, while RSNO levels were higher in NMOSD with AQP4-IgG and MS patients than in the control group. CSF pNfh levels were elevated in MS and ADEM patients. CONCLUSIONS: These results confirm that IL-6 is activated in different demyelinating disorders, with this elevation being more prominent in the CSF of NMOSD with AQP4-IgG and ADEM patients. Moreover, S-nitrosylation is activated in demyelinating disorders with spinal-cord injury and neurodegeneration in these patients. However, we found no correlation between these biochemical markers, and so we could not confirm whether IL-6-mediated nitric oxide production is involved in spinal-cord lesions.

Anti-myelin oligodendrocyte glycoprotein antibody and central nervous system immune demyelinating diseases / 中华实用儿科临床杂志

Myelin oligodendrocyte glycoprotein (MOG) is a component of central nervous system myelin.Anti-MOG antibodies are important in both pediatric and adult demyelination and the clinical association of MOG antibody-induced demyelination has been refined to include neuromyelitis optica spectrum disease,optic neuritis,and acute disseminated encephalomyelitis.But there are still many questions about epidemiological,clinical,radiological,and prognostic profile in patients with MOG antibody-associated demyelination.No consensus has been reached on detecting this antibody.This review will summarize the advances in anti-Myelin oligodendrocyte glycoprotein antibody.

Desmielinización del sistema nervioso central asociado a encefalomielitis diseminada aguda: Reporte de un caso / Demyelination of central nervous system associated with acute disseminated encephalomyelitis: Case report

Resumen Las enfermedades desmielinizantes inflamatorias comprenden una serie de desórdenes de origen autoinmune que afectan la mielina a nivel del sistema nervioso central (SNC) y periférico. Pueden ser monofásicas, multifásicas, progresivas, monofocales o multifocales, y su diagnóstico suele ser de exclusión. Se presenta el caso de una paciente de 15 años que debuta con cefalea, ataxia, hemiparesia, oftalmoparesia y alteración de la conciencia. En la resonancia magnética nuclear se observaron lesiones compatibles con enfermedad desmielinizante. Se realizó diagnóstico de encefalomielitis diseminada aguda. La paciente respondió favorablemente al tratamiento con corticoides.

Abstract Inflammatory demyelinating diseases comprise a series of autoimmune disorders affecting myelin at the level of the central nervous system (CNS) and peripheral nervous system. They can be monophasic, multiphasic, progressive, monofocal or multifocal, and their diagnosis is usually of exclusion. We report the case of a 15-year-old female patient with headache, ataxia, hemiparesis, ophthalmoplegia and altered consciousness. Magnetic resonance imaging showed lesions compatible with demyelinating disease. Acute disseminated encephalomyelitis was diagnosed. The patient responded to treatment with corticosteroids.

Complicaciones neurológicas del virus chikunguya y dengue / Neurological complications of chikungunya and dengue virus

Resumen Los virus chikungunya (CHIKV) y dengue (DENV) son arbovirus transmitidos por el mosquito Aedes. La infección por ambos virus es casi privativa de regiones tropicales y subtropicales , pero en los últimos años han aparecido casos en la mayoría de los países, lo que ha aumentado el interés en estos virus. En esta revisión se describe las manifestaciones neurológicas de CHIKV y DENV. Los cuadros neurológicos observados en pacientes infectados por estos virus son en la mayoría de los casos encefalitis y encefalopatía. También hay cuadros de mielitis y encefalomielitis aguda diseminada(ADEM) post infecciosas. Más infrecuentemente se observa compromiso periférico: síndrome de Guillain Barré(SGB) y mononeuritis. La miositis y la parálisis periódica se observan aunque raramente en la infección por DENV. El diagnóstico se realiza por serología Ig M específica o por detección de ARN viral en suero y LCR. El tratamiento es de sostén pues no hay tratamiento específico. Los pacientes con cuadros post infecciosos/inmunomediados se tratan con inmunoglobulina(IG) EV o plasmaféresis(SGB)y con metilprednisolona/IG en pacientes con ADEM. Es fundamental realizar el diagnóstico precoz de la infección por DENV y CHIKV para tratar a tiempo las complicaciones sistémicas y neurológicas, en especial las encefalitis y cuadros postinfecciosos.

Abstract Chikungunya (CHIKV) and dengue (DENV) viruses are arboviruses transmitted by the Aedes mosquito. Infection by both viruses is almost deprived of tropical and subtropical regions, but in recent years cases have appeared in most countries, which has increased the interest in these viruses. This review describes the neurological manifestations of CHIKV and DENV. The neurological pictures observed in patients infected by these viruses are in most cases encephalitis and encephalopathy. There are also post-infectious myelitis and disseminated acute encephalomyelitis (ADEM). Peripheral involvement is more rarely observed: Guillain Barré syndrome (GBS) and mononeuritis. Myositis and periodic paralysis are seen but rarely in DENV infection. The diagnosis is made by specific IgM serology or by detection of viral RNA in serum and CSF. The treatment is supportive as there is no specific treatment. Patients with post-infectious / immunomediated squares are treated with immunoglobulin (IG) EV or plasmapheresis (GBS) and with methylprednisolone / GI in patients with ADEM. Early diagnosis of infection by DENV and CHIKV is essential to treat systemic and neurological complications, especially encephalitis and postinfectious disease.

Role of MRI in acute disseminated encephalomyelitis

Background: Acute disseminated encephalomyelitis is an acute demyelinating disorder of the central nervous system and is characterised by multifocal white matter involvement. Diffuse neurological signs with multifocal lesions in brain and spinal cord characterise the disease. It do not invade central nervous system. Aim: To evaluate role of MRI in acute disseminated encephalomyelitis. Materials and methods: A prospective study of 10 cases which was conducted in the pediatric department in Dhiraj hospital. MRI brain was done on 1.5 Tesla MRI machine. Results: ADEM can be distinguished from acute viral encephalitis because the disease is not the result of primary tissue invasion by an infectious organism. It was thought to be immune-mediated and is characterized neuropathologically by perivenular inflammation and demyelination. Conclusion: ADEM is more common in female and more associated with previous infection as compared to previous vaccination. Neurological deficit is mostly associated with it. CSF showed raised protein in almost cases. It showed hyperintense lesion on T2W and FLAIR sequences. Periventricular area of brain is commonly associated.

Clinical and imageology follow-up study of acute disseminated encephalomyelitis / 中华实用儿科临床杂志

Objective To explore the clinical manifestations and recovery from neuroimaging abnormalities of acute disseminated encephalomyelitis (ADEM),in order to improve the understanding of ADEM.Methods A total of 47 children with ADEM in Department of Neurology,Children′s Hospital of Soochow University were followed up for 1 year.The clinical manifestations,auxiliary examination (especially neuroimaging),treatment and prognosis were analyzed.The recovery conditions from clinical and neuroimaging abnormalities were summarized.Results Among 47 cases,37 cases (78.72%) had prodromic infection history,and 5 cases (10.64%) had the history of vaccination.The clinical manifestations were varied,including 36 cases (76.60%) with somatic discomfort,30 cases (63.83%) with fever,30 cases (63.83%) with disturbance of consciousness,28 cases (59.57%) with epileptic seizure,24 cases (51.06%) with headache and dizziness,22 cases (46.81%) with nausea and vomiting,15 cases (31.91%) with dyskinesia,15 cases (31.91%) with cranial nerve injury,9 cases (19.14%) with incoordination,and 8 cases (17.02%) with mental and behavior disorders.Abnormal lesions presented multiple,asymmetric patchy and large patchy signal image showed by brain magnetic resonance imaging (MRI),and 7 cases (14.89%) involved the spinal cord.All patients received the treatment of Methylprednisolone [15-20 mg/(kg·d)] combined with intravenous gamma globulin (total 2 g/kg,3 to 5 days).The dose of Methylprednisolone gradually decreased after it had been used for 3 days.In the end,it was replaced by an oral administration of Prednisone [1.0-2.0 mg/(kg·d)],and the total treatment period was for 2-3 months.The follow-ups of 47 children with ADEM showed that the MRI lesions of all the children were reduced and the MRI lesions in 4 (8.51%) of them disappeared completely after 0.5 month of follow-up;the lesions disappeared completely in 13 cases (27.66%) after 1 month of follow-up;the lesions disappeared completely in 23 cases (48.94%) after 3 months of follow-up;the lesions disappeared completely in 32 cases (68.09%) after 6 months of follow-up;the lesions disappeared completely in 43 cases (91.49%) after 1 year of follow-up;and finally there were 4 cases (8.51%) with residual lesions.Conclusion The clinical manifestations of children with ADEM are varied.Most children with ADEM have a favorable prognosis.Imaging lesions of 68.09% of the cases completely disappeared after the onset of 6 months.Imaging lesions in 91.49% of the cases completely disa-ppeared at the onset of 1 year.Only a small fraction of patients present permanent lesions.