ABSTRACT
ABSTRACT Background: Neurological manifestations of COVID-19 are still incompletely understood. Neurological manifestations may be due to direct viral effect on neurons and glial cells, to an immune-mediated response to the virus, or to a hypercoagulable state and associated endothelial damage, as well as to severe systemic disease with prolonged intensive care unit stay. Objective: To describe two patients with severe SARS-CoV-2 infection and delayed recovery of consciousness after sedation withdrawal, in whom MRI disclosed multifocal white matter brain lesions, compatible with the diagnosis of acute disseminated encephalomyelitis. Methods: Observational report of two cases of severe COVID-19 infection in patients from two tertiary hospitals in São Paulo, Brazil. Results: These patients underwent neurologic and systemic evaluation for delayed awakening after sedation withdrawal. MRI displayed multifocal centrum semiovale lesions, suggestive of demyelinating inflammation. Cerebrospinal fluid (CSF) polymerase chain reaction (PCR) for SARS-CoV-2 was negative in both cases. Conclusion: A recurrent pattern of multifocal white matter lesions can occur in COVID-19 patients, possibly associated with delayed awakening. Additional studies are necessary to elucidate the role of the viral infection and of inflammatory and immune-mediated associated changes in neurological manifestations of COVID-19.
RESUMO Introdução: As manifestações neurológicas causadas pela COVID-19 ainda não estão completamente elucidadas. O comprometimento neurológico pode decorrer de um efeito viral direto em neurônios ou em células gliais, a efeito imunomediado em resposta à infecção viral, ou de um efeito secundário a estados de hipercoagulabilidade e danos endoteliais, assim como decorrente de complicações sistêmicas graves relacionadas a cuidados intensivos prolongados na unidade de terapia intensiva. Objetivo: Descrever dois pacientes com recuperação tardia do nível de consciência após a retirada da sedação associados à infecção grave pelo SARS-CoV-2, que apresentaram lesões multifocais de substância branca, compatíveis com o diagnóstico de encefalomielite disseminada aguda. Métodos: Estudo observacional, com relato de dois casos de infecção grave pela COVID-19, em dois hospitais terciários na cidade de São Paulo, Brasil. Resultados: Os pacientes foram submetidos à investigação sistêmica e neurológica para avaliação de estado alterado de consciência após retirada de sedação. A ressonância magnética de crânio evidenciou lesões multifocais no centro semioval, sugestivos de processo inflamatório desmielinizante. Análise liquórica evidenciou PCR negativo para SARS-CoV-2 em ambos os casos. Conclusão: Lesões multifocais de substância branca podem ocorrer em pacientes com COVID-19, possivelmente associadas a estados alterados de consciência. Estudos adicionais são necessários para determinar o processo fisiopatológico da infecção viral e dos estados inflamatórios e imunomediados na gênese das manifestações neurológicas causadas pela COVID-19.
Subject(s)
Humans , Coronavirus Infections , Brain , Brazil , Encephalomyelitis, Acute Disseminated/diagnostic imaging , BetacoronavirusABSTRACT
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.
Subject(s)
Humans , Male , Adult , Chikungunya virus/immunology , Optic Neuritis/diagnostic imaging , Encephalomyelitis, Acute Disseminated/virology , Encephalomyelitis, Acute Disseminated/diagnostic imaging , Chikungunya Fever/complications , Magnetic Resonance Imaging , Encephalomyelitis, Acute Disseminated/complications , Chikungunya Fever/diagnosisABSTRACT
La encefalomielitis diseminada aguda es una enfermedad desmielinizante del sistema nervioso central que generalmente se presenta como un trastorno monofásico asociado con síntomas neurológicos multifocales y encefalopatía. Suele ser precedido de una infección viral generalmente de vía aérea superior y, con menor frecuencia, de antecedente vaccinal. Se presenta un paciente de 4 años de edad con ataxia, encefalopatía, descenso progresivo del Score de Glasgow, imágenes de Sistema Nervioso Central compatibles con encefalomielitis diseminada aguda y adecuada respuesta al pasaje de pulsos de metilprednisolona. El objetivo de la publicación es enfatizar la presentación proteiforme de esta enfermedad y mejorar el reconocimiento de la misma en forma oportuna
The Acute Disseminated Encephalomyelitis is a demyelinating disease of the central nervous system that usually presents itself as a single-phase disorder associated with encephalopathy and multifocal neurologic symptoms. It tends to be preceded by a viral infection usually upper airway and, less frequently, of vaccinal antecedent. We present the case of a 4 years old child with clinic of ataxia, encephalopathy with progressive decline in Glasgow, history of fever in previous days, images of Central Nervous System compatible with ADEM and appropriate response to pulses of methylprednisolone. We decided to present this case to emphasize the varied presentation of this disease and improve recognition of it in a timely manner
Subject(s)
Male , Child, Preschool , Encephalomyelitis, Acute DisseminatedABSTRACT
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)
Subject(s)
Humans , Optic Neuritis , Encephalomyelitis, Acute Disseminated , Magnetic Resonance Imaging , Cerebrospinal Fluid , Antibodies , Multiple SclerosisABSTRACT
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)
Subject(s)
Humans , Urinary Retention , Pelvic Floor , Encephalomyelitis, Acute Disseminated , Urination , Urinary Bladder, Neurogenic , Dengue , Muscle StrengthABSTRACT
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.
Subject(s)
Humans , Child , Encephalomyelitis, Acute Disseminated/diagnostic imaging , Multiple Sclerosis/diagnostic imaging , Steroids/therapeutic use , Syndrome , Brain/physiopathology , Brain/diagnostic imaging , Magnetic Resonance Imaging , Drug Therapy, Combination , Encephalomyelitis, Acute Disseminated/drug therapy , Immunotherapy , Multiple Sclerosis/drug therapyABSTRACT
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.
Subject(s)
Humans , Male , Child, Preschool , Encephalomyelitis, Acute Disseminated/diagnosis , Encephalomyelitis, Acute Disseminated/therapy , Mycoplasma pneumoniae/pathogenicity , Biopsy/methods , Immunoglobulin M , Cerebrospinal Fluid/microbiology , Polymerase Chain Reaction/methods , Fluorescent Antibody Technique/methodsABSTRACT
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)
Subject(s)
Humans , Female , Middle Aged , Dengue/complications , Encephalomyelitis, Acute Disseminated/complications , Encephalomyelitis, Acute Disseminated/diagnosis , Clinical Evolution , Encephalomyelitis, Acute Disseminated/diagnostic imagingABSTRACT
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.
Subject(s)
Humans , Multiple Sclerosis/diagnostic imaging , Magnetic Resonance Imaging , Tomography, X-Ray Computed , Neuromyelitis Optica/diagnostic imaging , Diagnosis, Differential , Encephalomyelitis, Acute Disseminated/diagnostic imaging , Susac Syndrome/diagnostic imagingABSTRACT
OBJECTIVE@#To study the clinical features of children with acute disseminated encephalomyelitis (ADEM) and related recurrence factors.@*METHODS@#A retrospective analysis was performed for the clinical data and prognosis of 73 children with ADEM who were hospitalized from November 2011 to January 2017.@*RESULTS@#Among the 73 children, 41 (56%) had a history of infection before onset and 7 (10%) had a history of vaccination. All children had the symptoms of encephalopathy, including disturbance of consciousness in 47 children (64%) and mental and behavioral disorders in 54 children (74%). Pyrexia was observed in 53 children (73%), dyskinesia in 47 children (64%), headache in 47 children (64%) and vomiting in 40 children (55%). Brain MRI was performed for 65 children and the results showed involvement of the subcortical white matter (83%, 54/65), the deep nuclei (60%, 39/65), the brain stem (58%, 38/65) and the cerebellum (42%, 27/65). Spinal cord involvement was observed in 20 children (20/43, 47%). A total of 15 children experienced recurrence during follow-up. Compared with the non-recurrence group, the recurrence group had significantly higher percentages of children with deep nucleus involvement (P2 weeks (P2 weeks) may be associated with the recurrence of ADEM.
Subject(s)
Child , Humans , Encephalomyelitis, Acute Disseminated , Magnetic Resonance Imaging , Prognosis , Recurrence , Retrospective StudiesABSTRACT
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.
Subject(s)
Humans , Male , Female , Child, Preschool , Child , Adolescent , Neuromyelitis Optica/diagnosis , Encephalomyelitis, Acute Disseminated/diagnosis , Multiple Sclerosis/diagnosis , Magnetic Resonance Imaging , Neuromyelitis Optica/cerebrospinal fluid , Neuromyelitis Optica/drug therapy , Contrast Media , Encephalomyelitis, Acute Disseminated/cerebrospinal fluid , Encephalomyelitis, Acute Disseminated/drug therapy , Aquaporin 4 , Multiple Sclerosis/cerebrospinal fluid , Multiple Sclerosis/drug therapyABSTRACT
Guillain-Barré syndrome (GBS) and acute disseminated encephalomyelitis (ADEM) are demyelinating neurologic disorders with different target organs. Although they share similar pathogenetic mechanism, reports of simultaneous occurrence of the 2 disorders are rare. A 2 year 6 month old girl visited our hospital for fever, cough, and general weakness. Although the muscle power of extremities showed mild weakness and voiding difficulty, initial deep tendon reflex of both knees and ankles was normal. A nerve conduction study to evaluate the weakness revealed the absence of F waves. Cerebrospinal fluid analysis demonstrated pleocytosis with lymphocyte predominance and elevated protein levels. Magnetic resonance imaging showed abnormal T2 hyperintensity in pons, medulla and spinal cord. Serum anti-GD1b antibody was positive. Based on clinical findings, laboratory findings, nerve conduction study, and neuroimaging, the diagnosis of GBS and ADEM was made. This is the first case of GBS accompanied by ADEM in Korea.
Subject(s)
Female , Humans , Ankle , Cerebrospinal Fluid , Cough , Demyelinating Diseases , Diagnosis , Encephalomyelitis , Encephalomyelitis, Acute Disseminated , Extremities , Fever , Guillain-Barre Syndrome , Knee , Korea , Leukocytosis , Lymphocytes , Magnetic Resonance Imaging , Nervous System Diseases , Neural Conduction , Neuroimaging , Peripheral Nervous System , Pons , Reflex, Stretch , Spinal CordABSTRACT
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.
Subject(s)
Humans , Biomarkers , Central Nervous System , Cerebrospinal Fluid , Cytokines , Demyelinating Diseases , Encephalomyelitis, Acute Disseminated , Erythropoietin , Immunoglobulin G , Interleukin-6 , Intermediate Filaments , Multiple Sclerosis , Neuromyelitis Optica , Neurons , Nitric Oxide , Recurrence , S-NitrosothiolsABSTRACT
This study showed that laboratory markers of recent infection by dengue, Zika or chikungunya arboviruses were detected in the biological samples of approximately one-third of patients with encephalitis, myelitis, encephalomyelitis or Guillain-Barré syndrome, in a surveillance programme in Piauí state, Brazil, between 2015-2016. Fever and myalgia had been associated with these cases. Since in non-tropical countries most infections or parainfectious diseases associated with the nervous system are attributed to herpesviruses, enteroviruses, and Campylobacter jejuni, the present findings indicate that in tropical countries, arboviruses may now play a more important role and reinforce the need for their surveillance and systematic investigation in the tropics.
Subject(s)
Humans , Chikungunya virus/genetics , Chikungunya virus/immunology , Dengue Virus/genetics , Dengue Virus/immunology , Zika Virus/genetics , Zika Virus/immunology , Acute Disease , Reverse Transcriptase Polymerase Chain Reaction , Guillain-Barre Syndrome/diagnosis , Guillain-Barre Syndrome/virology , Encephalitis/diagnosis , Encephalitis/virology , Encephalomyelitis, Acute Disseminated/diagnosis , Encephalomyelitis, Acute Disseminated/virology , Enzyme-Linked Immunospot Assay , Myelitis, Transverse/diagnosis , Myelitis, Transverse/virology , Nervous System Diseases/diagnosis , Nervous System Diseases/virologyABSTRACT
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.
Subject(s)
Humans , Chikungunya virus , Dengue Virus , Encephalitis, Viral , Encephalomyelitis, Acute DisseminatedABSTRACT
Acute disseminated encephalomyelitis (ADEM) and Guillain-Barré syndrome (GBS) are both rare post-infectious neurological disorders. The co-existence of these conditions has often been reported despite of low incidence. We describe a 20-year-old male, who presented with acute flaccid paralysis and encephalopathy. The patient showed reversible MRI lesions suggesting ADEM. This case showed anti-GT1a IgG and anti-GM1 IgM antibodies positivity. We suggest that certain immunogenicity within central and peripheral nervous system may share a common autoimmune process during the disease course.
Subject(s)
Humans , Male , Young Adult , Antibodies , Brain Diseases , Encephalomyelitis, Acute Disseminated , Gangliosides , Guillain-Barre Syndrome , Immunoglobulin G , Immunoglobulin M , Incidence , Magnetic Resonance Imaging , Nervous System Diseases , Paralysis , Peripheral Nervous SystemABSTRACT
PURPOSE: This study aimed to describe the clinical characteristics and outcomes of children with acute combined central and peripheral nervous system demyelination (CCPD); and compare with the children of isolated acute central or peripheral nervous system demyelination. METHODS: A retrospective chart review of 145 children with acute demyelinating disease between 2010 and 2015 was undertaken in children with younger than 18 years old. Among these, 96 fulfilled criteria (clinical features and positive neuroimaging or electromyography/nerve conduction studies) for either acute central (group A, n=60, 62.5%) or peripheral (group B, n=30, 31.3%) nervous system demyelination, or a CCPD (group C, n=6, 6.3%). RESULTS: Significant differences among the groups (A vs B vs C) were evident for occurrence of disease between 2013-2015 (45.0% vs 43.3% vs 83.3%; P=0.024), admission to intensive care unit (8.3% vs 26.7% vs 50.0%; P=0.027), length of hospitalization (median, 9.7 vs 12.3 vs 48.3 days; P<0.001), treatment with steroids (88.3% vs 10.0 vs 100.0%; P=0.003), immunoglobulins (13.3% vs 100.0% vs 100.0%; P=0.002) and plasmapheresis (0.0% vs 3.3% vs 50.0%; P=0.037) and severe disability at discharge (3.3% vs 16.7% vs 33.3%; P=0.012). Children of group C showed good response to simultaneous use of immunoglobulin and high-dose corticosteroids and earlier try of plasmapheresis, however, two patients had moderate degree of neurological disability. CONCLUSION: Systemic studies using neuroimaing and electromyography/nerve conduction studies in all patients with demyelinating disease will be necessary to verify the combined or isolated disease, because CCPD might have the poorer outcome than isolated disease.
Subject(s)
Child , Humans , Adrenal Cortex Hormones , Demyelinating Diseases , Encephalomyelitis, Acute Disseminated , Guillain-Barre Syndrome , Hospitalization , Immunoglobulins , Intensive Care Units , Miller Fisher Syndrome , Myelitis, Transverse , Nervous System , Neuroimaging , Optic Neuritis , Peripheral Nervous System , Plasmapheresis , Retrospective Studies , SteroidsABSTRACT
Influenza vaccine is rarely associated with neurological adverse effects such as Guillain Barré syndrome, encephalitis or aseptic meningitis. We report the case of a male patient that presented two episodes of acute encephalitis in consecutive years, 16 and 20 days after his annual influenza vaccine shot, respectively. In both instances, patient required ICU admission and evolved with fast recovery and no sequels. The first episode was managed empirically as herpetic encephalitis but an extensive study was performed in the second episode without identifying any microorganism. Neuroimaging studies also discarded acute disseminated encephalomyelitis. Mild pleocytosis of mononuclear predominance was detected in both cases in CSF. Naranjo score punctuated 8 points indicating a probable causal relationship. Acute encephalitis is a rare adverse effect of influenza vaccine and occurs several days after immunization. It has a broad differential diagnosis, and appears to be of self-limited duration and associated with good prognosis.
La vacuna influenza en raras ocasiones se asocia a efectos adversos neurológicos tales como síndrome de Guillain Barré, encefalitis o meningitis aséptica, entre otros. Presentamos el caso clínico de un paciente de sexo masculino de 66 años que presentó dos episodios de encefalitis en años consecutivos, 16 y 20 días después de recibir la vacuna anual de influenza, respectivamente. En ambos casos requirió ingreso a UCI y evolucionó con una rápida recuperación y sin secuelas. El primer episodio fue manejado empíricamente como una encefalitis herpética y en el segundo se hizo un extenso estudio microbiológico que no identificó microorganismos. Los estudios de neuroimágenes descartaron una encefalomielitis aguda diseminada. En ambos casos se pesquisó pleocitosis de predominio mononuclear en el LCR. El score de Naranjo dio 8 puntos indicando una probable relación causal. La encefalitis aguda es un efecto adverso muy infrecuente tras la vacuna influenza y ocurre varios días después de la inmunización. Tiene un amplio diagnóstico diferencial, parece ser autolimitado y de buen pronóstico.
Subject(s)
Humans , Male , Aged , Influenza Vaccines/adverse effects , Encephalomyelitis, Acute Disseminated/etiology , Meningoencephalitis/etiology , Recurrence , Diagnosis, Differential , Encephalomyelitis, Acute Disseminated/diagnosis , Meningoencephalitis/diagnosisABSTRACT
The International Pediatric Multiple Sclerosis Study Group (IPMSSG) put forward the 2007 version of the diagnostic criteria for multiple sclerosis and other immune-mediated demyelinating diseases of the central nervous system in children in 2007 ("2007 version" for short). In 2012, IPMSSG proposed the new diagnostic criteria with reference to the latest research achievements of 150 members ("2012 version" for short). The 2012 version of the consensus statements covers the diagnostic criteria for acute disseminated encephalomyelitis, clinically isolated syndrome, neuromyelitis optica, and multiple sclerosis in children. As the two IPMSSG members in China, the authors give an interpretation of the 2012 version of the consensus statements with reference to related literature and clinical and scientific experience. The authors focus on how the 2012 version comprehensively and thoroughly elaborates on the clinical features, diagnostic criteria, influencing factors, and new ideas of acute demyelinating diseases of the central nervous system in children. These become more operable in clinical diagnosis and treatment of multiple sclerosis and other immune-mediated demyelinating diseases of the central nervous system in children.