Encephalomyelitis in a patient with monkeypox: an unusual complication.

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

Optic neuromyelitis after vaccination against SARS-CoV-2.

Neuromyelitis optica is an autoimmune demyelinating astrocytopathy of the central nervous system that primarily affects the optic nerve and spinal cord. It is considered a multifactorial disease associated with antibodies against aquaporin 4, with complement cascade activation and lymphocytic infiltration leading to axonal loss and causing significant morbidity and disability. In addition, cases of inflammatory diseases of the central nervous system have been described after vaccination against SARS-CoV-2, mainly acute disseminated encephalomyelitis. Also, a few cases of neuromyelitis optica spectrum disorder, mostly aquaporin 4+, have been reported. We describe a patient who developed symptoms suggestive of acute disseminated encephalomyelitis the next day after vaccination against SARS-CoV-2. Three months later, a longitudinally extensive transverse myelitis compatible with aquaporin 4+ neuromyelitis optica was successfully treated with an interleukin 6 inhibitor. There is no proven association and research is needed to establish whether optic neuromyelitis is related to vaccination; this is a single case report from which no conclusion can be drawn.

Etiology and prognosis of encephalitis in French Guianese children: a retrospective record-based study.

Acute encephalitis is an important cause of mortality and morbidity in children. We retrospectively identified children (≤15 years of age) admitted with suspected encephalitis at the Intensive Care Unit of the Pediatric Department of Cayenne Hospital between January 2007 and December 2018. A total of 30 children with acute encephalitis were identified. The incidence rate varied from 0 to 10.40 cases/100000 children under 15 years. Proven encephalitis was diagnosed in 73% of patients. Nine cases of acute disseminated encephalomyelitis were diagnosed. The causes of infection (44%) were Haemophilus influenzae, followed by Cryptococcus spp and Varicella Zoster Virus. Four children (13%) died: one case of Streptococcus pneumoniae, one of Haemophilus influenzae, one of Mycobacterium tuberculosis and one with no identified cause. Seventeen percent of children had moderate to severe neurological sequelae. The only factor associated with poor outcome was young age at the time of hospitalization (p = 0.03). Conclusion: This study highlights both vaccine-preventable pathogens and acute disseminated encephalomyelitis as the leading causes of childhood encephalitis in French Guiana.

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

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.

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.

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)

Encefalomielitis aguda diseminada y colitis ulcerosa: descripción de un caso clínico / Acute disseminated encephalomyelitis and ulcerative colitis: description of a clinical case in magnetic resonance

Acute disseminated encephalomyelitis (ADEM) is an acute demyelinating disorder of the central nervous system, characterized by an acute or subacute onset of neurological symptoms and focal signs in association with multifocal demyelinating findings on neuroimaging. Spontaneous resolution has been reported within days of onset, corticosteroids therapy accelerates the resolution time. ADEM has been associated with previous infections, and its relationship with other autoinmune disease is unclear. A case of ADEM is reported, in a patient previously diagnosed with ulcerative pancolitis, whose neurological symptoms resolved after 3 days of hospital admission without evident sequelae after receiving a short empirical treatment with ampicilin, ceftriaxone, acyclovir and intravenous steroids.

La encefalomielitis aguda diseminada (EAD) es un trastorno desmielinizante agudo del Sistema Nervioso Central, caracterizado por la presentación aguda de síntomas y signos neurológicos en asociación con hallazgos multifocales de desmielinización en las neuroimágenes. Se describe en la literatura resolución espontánea a los pocos días de presentación, con secuelas variables. La terapia corticoesteroidal aceleraría este tiempo de resolución. Se ha relacionado con eventos infecciosos previos, y su relación con otras enfermedades autoinmunes no es clara. Se presenta un caso de EAD en paciente con diagnóstico previo de pancolitis ulcerosa cuya sintomatología neurológica se resuelve tras 3 días del ingreso hospitalario, sin secuelas evidentes, tras recibir tratamiento empírico corto con Ampicilina, Ceftriaxona, Aciclovir y corticoides endovenosos en dosis de estrés.

[Association between acute disseminated encephalomyelitis and Guillain-Barré syndrome in an adult]. / Asociación de encefalomielitis diseminada aguda y síndrome de Guillain-Barré en un adulto.

Acute disseminated encephalomyelitis (ADEM) and Guillain-Barré Syndrome (GBS) are commonly recognized as separated entities involving different parts of the nervous system. However, they share some features such as: autoimmune pathogenesis, myelin injury and previous history of viral infections or vaccination. We report the case of a 41 year-old man who developed fever, lower limbs weakness and obtundation fifteen days after an acute gastroenteritis. Neurological examination showed patellar hypereflexia, bilateral Babinski and neurogenic bladder. Twenty-four hours later he developed flaccid paraparesis, generalized areflexia and respiratory failure that was supported by mechanical ventilation. Cerebrospinal fluid showed mononuclear pleocytosis and elevated proteins. Electrodiagnosis showed important reduction of conduction velocity on both peroneal nerves. Magnetic Resonance Imaging revealed white matter lesions in brain, pons and thoracic levels of the spinal cord. Diagnosis of the association between ADEM and GBS (ASADEM-GBS) was made and treatment with corticosteroids and intravenous immunoglobulin was started. The patient recovered motor, sensory and bladder functions and he was able to walk six months later. ASADEM-GBS is an uncommon entity generally considered of poor outcome; however a rapid diagnosis and treatment can substantially improve the prognosis.

Asociación de encefalomielitis diseminada aguda y síndrome de Guillain-Barré en un adulto / Association between acute disseminated encephalomyelitis and Guillain Barré syndrome in an adult

La encefalomielitis diseminada aguda (EMDA) y el síndrome de Guillain-Barré (SGB) son reconocidas como entidades distintas, que afectan diferentes sectores del sistema nervioso, pero que comparten varias características tales como la patogenia autoinmune, el impacto sobre la mielina y el antecedente de infección viral o vacunación una a cuatro semanas previas al cuadro clínico. Se presenta un paciente varón de 41 años de edad que consultó por presentar fiebre, debilidad en miembros inferiores y somnolencia dos semanas posteriores a episodio agudo de gastroenteritis. Al ingreso se constató deterioro del sensorio (obnubilación) hiperreflexia patelar, Babinski bilateral y vejiga neurogénica. Veinticuatro horas después desarrolló paraplejía flácida y arreflexia generalizada, requiriendo asistencia respiratoria mecánica por insuficiencia respiratoria. El líquido cefalorraquídeo mostró pleocitosis mononuclear e hiperproteinorraquia. El estudio electrofisiológico evidenció importante disminución de las velocidades de conducción en ambos nervios ciáticos poplíteos externos, compatible con polineuropatía desmielinizante. La resonancia magnética nuclear mostró imágenes compatibles con desmielinización en cerebro, protuberancia y segmentos medulares dorsales. Se realizó diagnóstico de ASEMDA-SGB e inició tratamiento con metilprednisolona e inmunoglobulina intravenosa. Evolucionó favorablemente, recuperando las funciones motoras, vesical y la sensibilidad, siendo capaz de deambular luego de seis meses. La asociación de EMDA y SGB (ASEMDA-SGB) es una condición infrecuente, generalmente señalada como de mal pronóstico, en la cual un diagnóstico precoz y un rápido y enérgico tratamiento pueden mejorar substancialmente la evolución.

Acute disseminated encephalomyelitis (ADEM) and Guillain-Barré Syndrome (GBS) are commonly recognized as separated entities involving different parts of the nervous system. However, they share some features such as: autoimmune pathogenesis, myelin injury and previous history of viral infections or vaccination. We report the case of a 41 year-old man who developed fever, lower limbs weakness and obtundation fifteen days after an acute gastroenteritis. Neurological examination showed patellar hypereflexia, bilateral Babinski and neurogenic bladder. Twenty-four hours later he developed flaccid paraparesis, generalized areflexia and respiratory failure that was supported by mechanical ventilation. Cerebrospinal fluid showed mononuclear pleocytosis and elevated proteins. Electrodiagnosis showed important reduction of conduction velocity on both peroneal nerves. Magnetic Resonance Imaging revealed white matter lesions in brain, pons and thoracic levels of the spinal cord. Diagnosis of the association between ADEM and GBS (ASADEM-GBS) was made and treatment with corticosteroids and intravenous immunoglobulin was started. The patient recovered motor, sensory and bladder functions and he was able to walk six months later. ASADEM-GBS is an uncommon entity generally considered of poor outcome; however a rapid diagnosis and treatment can substantially improve the prognosis.

Encefalomielitis aguda diseminada: reporte de un caso y revisión de la literatura / Acute disseminated encephalomyelitis: a case report and review of the literature

Introducción: la encefalomielitis aguda diseminada (EAD) es un trastorno neurológico caracterizado por inflamación del cerebro y médula espinal causado por un daño a la mielina, afectando al sistema nervioso central de manera difusa. Esta afección puede manifestarse de manera espontánea o secundaria a infecciones o a vacunación. La mayoría de las veces evoluciona de manera monofásica con manifestaciones clínicas inespecíficas, por lo que la sospecha diagnóstica es fundamental. Objetivo: Presentar un caso clínico de una patología infrecuente, con una revisión actualizada del tema. Caso clínico: Presentamos el caso de una paciente de 7 años de edad, de sexo femenino, portadora de EAD y se exponen la forma clínica de presentación, los hallazgos neurorradiológicos y el tratamiento realizado. Conclusión: La EAD es una patología poco frecuente, que puede tener un curso mortal. El diagnóstico definitivo se realiza a través de RM y el tratamiento más efectivo pareciera ser los corticoides, aunque un porcentaje de pacientes evoluciona espontáneamente a la mejoría.

[Disseminated acute encephalomyelitis in children]. / Encefalomielitis aguda diseminada en niños.

INTRODUCTION: Disseminated acute encephalomyelitis (DAE) is an autoimmune inflammatory condition, usually monophasic with gradual resolution, polysymptomatic, usually in relation to a virus, bacteria or immunizations but may also occur in the absence of any obvious infection. OBJECTIVE: To describe DAE, its aetiology, clinical features, diagnosis and treatment. Also to analyze these aspects with regard to a series of 13 children with DAE. RESULTS: The clinical condition started with fever in 61% of the patients. The commonest neurological findings were sensory deterioration, convulsions and motor disorders (paresias). The cerebrospinal fluid was abnormal in four patients. MR showed hyperintense lesions of multifocal distribution, predominantly in the subcortical white matter. All patients were treated with intravenous methylprednisolone at a dose of 20 30 mg/kg. Clinical follow up showed complete recovery in 11/13 patients and mild sequelae in two cases. CONCLUSIONS: Diagnosis of DAE is based on suggestive clinical data and MR findings. MR is very sensitive in detecting demyelinating lesions and is the method of choice for confirmation of the diagnosis. DAE is characterized by a satisfactory clinical course with simultaneous resolution of the demyelinating lesions.

Acute disseminated encephalomyelitis: an unusual cause of encephalitic syndrome in childhood.

Acute disseminated encephalomyelitis is a rare central nervous system demyelinating disease that occurs most frequently in children. It usually runs a monophasic course, beginning with fever, headache, and meningeal signs and rapidly progressing to coma when appropriate diagnosis and treatment are not provided. We report a case of a 14-year-old patient to alert emergency physicians to consider acute disseminated encephalomyelitis when presented with any child with encephalitic signs with nonspecific cerebrospinal fluid findings, failure to detect any causative agent, and only mild alterations on computerized tomography scan. The role of magnetic resonance imaging for the diagnosis is emphasized.