ABSTRACT
Abstract Background Progressive multifocal leukoencephalopathy (PML) - immune reconstitution inflammatory syndrome (IRIS) in people living with HIV/AIDS (PLWHA) has been rarely described in low- and middle-income countries. Objective To describe the prevalence of PML-IRIS among PLWHA with PML and its main features in a tertiary hospital in Brazil. Methods We performed a retrospective cohort study. We included PLWHA with PML-IRIS patients admitted at Instituto de Infectologia Emílio Ribas, São Paulo, Brazil, between 2011 and 2021. We retrieved information on neurological manifestations, neuroimaging findings, treatments, and outcomes. Results We identified 11 (11.8%) PML-IRIS cases among 93 patients with definite PML. Eight (73%) cases were men and had a median (IQR) age of 41 (27-50) years. Seven (63.6%) patients developed unmasking PML-IRIS and 4 (36.4%) had paradoxical PML-IRIS. The median (IQR) time from initiation of combined antiretroviral therapy (cART) to IRIS diagnosis was 49 (30-70) days. Ten (90.9%) patients received corticosteroids. There were 4 (36%) in-hospital deaths and 3 were associated with hospital-acquired pneumonia. Among the 7 (64%) patients who survived, 5 (71.5%) had sequelae at discharge. One year after the PML-IRIS diagnosis, 6 (54.5%) patients were alive. Conclusion The prevalence of PML-IRIS was 11.8%. Most patients had unmasking PML-IRIS. In-hospital mortality and morbidity were high. One-year survival was similar to that described in some high-income countries.
Resumo Antecedentes A síndrome inflamatória de reconstituição imune (SIRI) da leucoencefalopatia multifocal progressiva (LEMP) em pessoas vivendo com HIV/Aids (PVHA) foi raramente descrita em países de baixa e média renda. Objetivo Descrever a prevalência da SIRI-LEMP- em PVHA com LEMP e suas principais características em um hospital no Brasil. Métodos Foi realizado um estudo de coorte retrospectivo. Incluímos PVHA com SIRI-LEMP admitidos no Instituto de Infectologia Emílio Ribas, São Paulo, Brasil, entre 2011 e 2021. Recuperamos informações sobre manifestações neurológicas, neuroimagem, tratamento e desfecho. Resultados Identificamos 11 (11,8%) casos de SIRI-LEMP entre 93 pacientes com LEMP definitiva. Oito (73%) casos eram homens e a mediana de idade (amplitude interquartile - AIQ) foi de 41 (27-50) anos. Sete (63,6%) pacientes desenvolveram SIRI-LEMP "desmascarada" e 4 (36,4%) casos apresentaram SIRI-LEMP "paradoxal". A mediana de tempo (AIQ) desde o início da terapia antirretroviral combinada (cART) até o diagnóstico de SIRI foi de 49 (30-70) dias. Dez (90,9%) pacientes receberam corticoide. Houve 4 (36%) óbitos intra-hospitalares e 3 foram associados à pneumonia hospitalar. Dos 7 (64%) pacientes que sobreviveram, 5 (71,5%) ficaram com sequelas na alta. Um ano após o diagnóstico de SIRI-LEMP, 6 (54,5%) pacientes estavam vivos. Conclusão A prevalência de SIRI-LEMP foi de 11,8%. A maioria dos pacientes apresentava SIRI-LEMP "desmascarada". A mortalidade e morbidade hospitalar foram altas. A sobrevida em 1 ano foi semelhante à descrita em alguns países de alta renda.
ABSTRACT
ABSTRACT Toxic leukoencephalopathy (TLE) is a rare neurological debilitating and fatal condition. It has been previously associated with exposure to leukotoxic offenders such as chemotherapy, cranial radiation, certain drugs, and environmental factors. Currently, it is a commoner white matter syndrome resulting from increased substance abuse, classically by inhaled heroin and other opioids. Herein, we report a case of fatal TLE unveiled in an autopsy of a drug abuser. A 24-year-old male was found dead on the roadside. A day before, he was located in a state of delirium. In this case, the autopsy findings and histopathology characteristics of cerebral cortex involvement particularly directed to speculate the heroine as the principal offender.
ABSTRACT
Objective:To investigate the clinical manifestations, imaging features and prognosis of posterior reversible encephalopathy syndrome with spinal cord involvement (PRES-SCI).Methods:The clinical data of 1 patient with PRES-SCI admitted to the Department of Neurology of the First Affiliated Hospital of Zhengzhou University in November 2021 were analyzed, and the data of 38 patients with PRES-SCI reported in domestic and foreign databases and this patient were collected for pooled analysis.Results:The main clinical manifestations of 39 PRES-SCI patients (including this patient) included headache (79.5%, 31/39), visual disturbance (79.5%, 31/39), vomiting (46.2%, 18/39), disturbance of consciousness (38.5%, 15/39), limb weakness (28.2%, 11/39) and seizure (23.1%, 9/39). There were up to 97.4% (38/39) of patients who had significantly elevated blood pressure. The imaging feature was long-segment spinal cord lesion involving central gray matter. Approximately 89.7% (35/39) of the spinal cord lesions originated from the junction of the medulla oblongata and the cervical spinal cord. The median number of abnormal spinal cord segments corresponding to the vertebral body was 9 (the shortest was 4 and the longest was the entire spinal cord). Thirty-eight patients had brain lesions, and the most frequently involved sites were medulla oblongata (82.1%, 32/39), occipital lobe (46.2%, 18/39), pons (43.6%, 17/39), parietal lobe (41.0%, 16/39), and cerebellum (38.5%, 15/39). Among 38 patients completing the follow-up, 31 patients (81.6%) were clinically recovered.Conclusions:Posterior reversible encephalopathy syndrome could involve the spinal cord. For patients with long-segment spinal cord lesions and significantly increased blood pressure or other risk factors, PRES-SCI should be considered. Timely identification and treatment could make most patients recovery.
ABSTRACT
RESUMEN INTRODUCCIÓN: Los pacientes con compromiso del sistema inmune pueden desarrollar una enfermedad neurológica incapacitante e incluso mortal, como lo es la leucoencefalopatía multifocal progresiva (LMP) producida por el virus de John Cunningham (JC). PRESENTACIÓN DEL CASO: Se presenta el caso de un hombre de 26 años con diagnóstico reciente de infección por virus de la inmunodeficiencia humana (VIH) que presentó síntomas constitucionales, déficit neurológico progresivo por hemiparesia espástica izquierda, disminución de la agudeza visual y cambios comportamentales. En las imágenes de resonancia magnética (IRM) cerebral contrastada se encontró afectación subcortical difusa de la sustancia blanca con compromiso de las fibras en U que, correlacionado con una prueba de reacción en cadena de la polimerasa (PCR) para virus JC en LCR, confirmó el diagnóstico de LMP. DISCUSIÓN: La LMP puede manifestarse por medio de síntomas cognitivos usualmente imperceptibles para el clínico, pero también como déficit sensorio-motor y visual que se puede corroborar en las IRM al identificar las lesiones típicas en la sustancia blanca, o bien por medio de detección del virus por PCR en líquido cefalorraquídeo. El manejo específico de la causa que desencadenó la inmunosupresión sigue siendo el pilar de tratamiento. CONCLUSIÓN: La mínima sospecha diagnóstica en aquellos pacientes con factores de riesgo y manifestaciones clínicas concordantes con la enfermedad debe llevar a que se confirme el diagnóstico y que se inicie prontamente el manejo terapéutico en búsqueda de restablecer la respuesta inmune.
ABSTRACT INTRODUCTION: Patients with immunocompromised or weakened immune system can develop a disabling and even life-threatening neurological disorder such as progressive multifocal leukoencephalopathy (PML) caused by John Cunningham (JC) virus. CASE PRESENTATION: We present the case of a 26-year-old man with a recent diagnosis of human immunodeficiency virus (HIV) infection who presented constitutional symptoms, progressive neurological deficit due to left spastic hemiparesis with decreased visual acuity and behavioral changes. The brain Magnetic Resonance Imaging (MRI) showed diffuse subcortical involvement of the white matter including the U-fibers, which, correlated with a detection of JC virus DNA by polymerase chain reaction (PCR) cerebrospinal fluid, confirmed the diagnosis of PML. DISCUSSION: PML can range from subtle cognitive impairment imperceptible to the clinician to sensory-motor deficits and visual disturbances that can be corroborated in MRI by identifying the typical lesions in the white matter or by detecting the virus by PCR in cerebrospinal fluid. The specific management of the cause that triggered the immunosuppression continues to be the mainstay of treatment. CONCLUSION: At the minimum diagnostic suspicion in patients with risk factors and clinical manifestations consistent with the disease should proceed to confirm the diagnosis and promptly immune reconstitution.
Subject(s)
Leukoencephalopathy, Progressive Multifocal , Immunocompromised Host , HIV , JC VirusABSTRACT
Abstract Posterior reversible encephalopathy syndrome (PRES) is a rare disease attributed to an increase in blood pressure that exceeds the autoregulatory capabilities of the cerebral vasculature, resulting in brain edema. Although PRES primarily affects adults, the pediatric population is also at risk. Radiologists must be aware of that risk because the imaging features on brain MRI are often atypical, especially in pediatric patients. Over a 6-year period, nine pediatric patients were diagnosed with PRES at our institution. Here, those patients are evaluated retrospectively regarding demographic characteristics, clinical profiles, imaging aspects, and outcomes. In this pictorial essay, we review the typical and atypical imaging findings of PRES in pediatric patients, demonstrating that it should be considered in patients with a clinical profile suggestive of the diagnosis, given that prompt, effective treatment is important for full recovery, thus avoiding major morbidity and mortality in such patients.
Resumo A síndrome de encefalopatia posterior reversível (PRES) é uma doença rara relacionada com uma falha no mecanismo autorregulatório vascular, resultando em edema cerebral. Apesar de primariamente descrita em adultos, a população pediátrica encontra-se em igual risco de desenvolver esta condição e o radiologista deve estar atento porque frequentemente a ressonância magnética apresenta características de imagem atípicas. Em um período de seis anos, nove pacientes pediátricos com o diagnóstico de PRES foram avaliados, retrospectivamente, no que diz respeito a sua demografia, antecedentes médicos, características de imagem e prognóstico. Neste ensaio analisamos características de imagem típicas e atípicas de pacientes pediátricos com o diagnóstico de PRES, mostrando que este diagnóstico deve ser considerado quando está presente um quadro clínico adequado, uma vez que o tratamento rápido e eficaz é importante para a recuperação completa, evitando morbimortalidade maior neste grupo etário.
ABSTRACT
ABSTRACT Objective: Inhalant users may develop toluene leukoencephalopathy, a devastating neuropsychiatric disorder. We present a case of toluene-induced damage to the corticospinal and the corticonuclear tracts, which presented with involuntary emotional expression dis-order. Methods: Case study of a 20-year-old man with a 3-year history of frequent solvent abuse was admitted to the Neuropsychiatry Unit of the National Institute of Neurology and Neurosurgery because "he could not speak or walk" but would keep "laughing and crying without reason". Results: Neuropsychiatric examination revealed pathological laughter and crying, facial and speech apraxia, a bilateral pyramidal syndrome, and lack of control of urinary sphincter. Magnetic resonance imaging revealed a highly selective bilateral damage to the pyramidal system and the somatosensory pathway. SPECT imaging showed left fronto-parietal hypoperfusion. Conclusions: This document provides support for the understanding of involuntary emotional expression disorders as a differential diagnosis in the clinical practice of psychiatrists, as well as the functional anatomy of these conditions.
RESUMEN Objetivo: Los usuarios de inhalantes pueden contraer leucoencefalopatía por tolueno, un trastorno neuropsiquiátrico devastador. Se presenta un caso de daño inducido por tolueno en el tracto corticoespinal y corticonuclear, que se manifestó con un trastorno involuntario de la expresión emocional. Métodos: Un varón de 20 años con antecedente de 3 años de abuso de solventes ingresó en la Unidad de Neuropsiquiatría del Instituto Nacional de Neurología y Neurocirugía porque «no podía hablar ni caminar¼ y presentaba episodios súbitos de risa y llanto sin razón aparente. Resultados: La valoración neuropsiquiátrica reveló risa y llanto patológicos, apraxia facial y fonatoria, síndrome piramidal bilateral y ausencia de control del esfínter urinario. La resonancia magnética cerebral mostró un daño bilateral muy selectivo del sistema piramidal y la vía somatosensorial. La imagen de tomografía computarizada por emisión monofotónica mostró hipoperfusión frontoparietal izquierda. Conclusiones: Este documento proporciona apoyo para la comprensión de los trastornos de la expresión emocional involuntaria como diagnóstico diferencial en la práctica clínica de los psiquiatras, así como de la anatomía funcional de estas condiciones.
ABSTRACT
RESUMEN La leucoencefalopatía multifocal progresiva es una enfermedad desmielinizante del sistema nervioso central producido por un virus del género Polyomavirus. Las manifestaciones clínicas pueden ser motoras, sensitivas o cognitivas. Se presenta el caso de un paciente masculino de 32 años de edad con un cuadro de 24 horas de evolución de debilidad de miembro superior e inferior izquierdos que inició de manera insidiosa y progresiva, acompañada de disartria y confusión. Por sospecha de vasculitis cerebral versus enfermedad desmielinizante se inicia bolos de corticoides con lo cual mejora la debilidad. Se solicita estudios de laboratorio en la que se confirma sida. La resonancia magnética con Gadolinio en el que se observa lesiones compatibles con leucoencefalopatía multifocal progresiva. Se inicia tratamiento antirretroviral y es dado de alta sin otras complicaciones.
ABSTRACT Progressive multifocal leukoencephalopathy is a demyelinating disease of the central nervous system caused by a virus of the Polyomavirus genus. The clinical manifestations can be motor, sensory or cognitive. We present the case of a 32-year-old male patient with a 24-hour evolution of weakness in the left upper and lower limb that began insidiously and progressively, accompanied by dysarthria and confusion. Due to suspicion of cerebral vasculitis versus demyelinating disease, corticosteroid boluses are started, which improves weakness. Laboratory studies are requested in which AIDS is confirmed. Gadolinium magnetic resonance imaging shows lesions compatible with progressive multifocal leukoencephalopathy. Antiretroviral treatment is started and he is discharged without other complications.
ABSTRACT
OBJECTIVES@#Reversible posterior leukoencephalopathy syndrome (RPLS) is a rare clinical imaging syndrome. The causes of RPLS are complex and diverse, the pathogenesis is not yet clear. The onset is urgent and the onset age span is large, ranging from children to the elderly. The clinical symptoms of RPLS have no significant specificity, which can be manifested as headache, blurred vision, disturbance of consciousness or seizures. Clinicians have little knowledge on the disease, which may lead to misdiagnosis or missed diagnosis. This study aims to analyze and summarize the MRI changes and clinical characteristics regarding RPLS patients, so as to provide basis for rapid diagnosis and timely intervention for this disease.@*METHODS@#The clinical data and complete imaging data of 77 patients with RPLS diagnosed in Xiangya Hospital of Central South University from January 2012 to March 2021 were retrospectively collected. The main image data include T1 weighted imaging (T1WI), T2 weighted imaging (T2WI), T2 liquid attenuation inversion recovery (T2-FLAIR), diffusion weighted imaging (DWI) (b value=1 000×10-6 mm2/s), and apparent diffusion coefficient (ADC). The case group included 63 patients who underwent DWI examination, and 71 normal controls matched in age and sex. The characteristics of patients' magnetic resonance signals and the ADC value of 19 regions of interest (ROI) were analyzed. The differences in bilateral ADC value in the case group, the difference of ADC value between the case group and the normal control group, and the difference of ADC value in the case group before and after treatment were compared.@*RESULTS@#Compared with the normal control group, the ADC value of the right frontal lobe, bilateral parietal lobe, bilateral thalamus, bilateral head of caudate nucleus, left lenticular nucleus, right internal capsule, bilateral temporal lobe and pons in the case group were significantly higher (all P<0.01). There was no significant difference in ADC value of bilateral sides of the case group and before and after treatment in the case group (all P>0.01). The lesions of RPLS were widely distributed and multiple, usually high signal in the posterior parieto temporo occipital lobe or pons of the brain, and involved the cortex and subcortical white matter. Most of them were bilateral, but not completely symmetrical.@*CONCLUSIONS@#The imaging manifestations of RPLS and the occurrence and development of clinical symptoms are basically synchronous. The imaging manifestations are specific. Magnetic resonance imaging can show the range of involvement of RPLS. ADC value can provide information on the severity of the disease and predict the prognosis. There are few reversible diseases. It is very important to fully understand and timely diagnose the disease.
Subject(s)
Child , Humans , Aged , Posterior Leukoencephalopathy Syndrome/etiology , Retrospective Studies , Magnetic Resonance Imaging/methods , Brain/pathology , Diffusion Magnetic Resonance ImagingABSTRACT
Clinical data of a child with leukoencephalopathy with brainstem and spinal cord involvement and lactate elevation (LBSL) diagnosed in the First Affiliated Hospital of Zhengzhou University in June 2020 were retrospectively analyzed.A female patient with 1 year and 2 months old presented with 10 months of developmental delay and 1 month of recurrent seizures.Physical examinations showed grade Ⅲ muscle strength of limbs, slightly higher muscle tone, active reflex of bilateral knee tendons, normal reflex of bilateral achilles tendons, and positive Babinski sign.Brain magnetic resonance imaging (MRI) showed bilateral cerebral hemisphere atrophy and diffuse abnormal signals.The whole exome sequencing test showed two heterozygous mutations of the DARS2 gene in the present case.There are no reports of early-onset LBSL gene mutation in Chinese population.A total of 6 related foreign literatures have been reported.All affected cases present psychomotor development disorders and other encephalopathy manifestations.Brain MRI involvement and DARS2 gene mutations are found in previous reports.Therefore, for children with developmental retardation, epilepsy, and diffuse abnormal signals in both cerebral hemispheres examined by brain MRI, genetic testing is recommended to confirm the diagnosis, guide prenatal diagnosis and genetic counseling.
ABSTRACT
Leukoencephalopathy with vanishing white matter (VWM) is one of the most prevalent inherited childhood white-matter disorders, and the pathogenic gene has been confirmed as EIF2B gene. VWM is characterized by chronic progressive neurological deterioration with cerebellar ataxia, usually less prominent spasticity and relatively mild mental decline. There are episodes of rapid and major neurological deterioration provoked by stresses, such as fever, minor physical trauma and acute fright, which is a characteristic clinical feature of VWM. Brain magnetic resonance imaging findings are diagnostic in almost all patients,and the disappearance of the cerebral white matter occurs in a diffuse "melting away" pattern. The onset of VWM can be at any age from fetal stage to adult stage, and the clinical phenotypes vary immensely. Gene diagnosis is the golden standard for VWM. This article reported a patient with a course of 17 years, who was misdiagnosed as Wilson′s disease because of low serum ceruloplasmin, and was finally diagnosed as VWM by reinterpretation of whole exome sequencing, which is worthy of clinicians′ vigilance and consideration.
ABSTRACT
Adult-onset leukoencephalopathy with axonal spheroids and pigmented glia (ALSP) is a rare autosomal-dominant progressive leukodystrophy, caused by mutations of colony stimulating factor-1 receptor (CSF1R) gene. Age of onset is usually between 40 and 50 years old and the clinical presentations include dementia, apraxia, behavioral changes, pyramidal and extrapyramidal signs. Varying clinical manifestations have led to misdiagnoses. Magnetic resonance imaging (MRI) typically reveals white matter changes with T2-Flair/DWI hyperintensity and atrophy especially for thinning of the corpus callosum. Here, we report a young woman experiencing hypomnesia for 2 years with lower extremities weakness and rigidity for 1 month. Considering the evidence of clinical manifestations, imaging and genetic test, this patient was diagnosed with ALSP.
ABSTRACT
RESUMEN Introducción: La leucoencefalopatía multifocal progresiva es una enfermedad desmielinizante del sistema nervioso central, de etiología viral. Se presenta en pacientes con enfermedades inmunosupresoras y la localización en fosa posterior es rara. Debido a sus formas clínicas inespecíficas se hace infrecuente su diagnóstico lo que conlleva a daño irreversible y/o a la muerte del paciente. Objetivo: Orientar sobre la posibilidad de leucoencefalopatía multifocal progresiva cerebelosa en pacientes inmunodeprimidos con manifestaciones neurológicas de daño en fosa posterior. Caso clínico: Paciente masculino, de 25 años de edad, sin antecedentes de enfermedades aparentes, que comienza con lenguaje escandido, temblor mixto dismetría y ataxia. Se diagnostica leucoencefalopatía multifocal progresiva cerebelosa por cuadro clínico, neuroimagen y presencia de virus JC en líquido cefalorraquídeo, además de una inmunosupresión severa causada por virus de inmunodeficiencia humana diagnosticado por pruebas serológicas. Conclusiones: Considerar leucoencefalopatía multifocal progresiva cerebelosa en todo paciente con manifestaciones neurológicas de afectación en fosa posterior y estudiar causas de inmunosupresión subyacente.
ABSTRACT Introduction: Progressive multifocal leukoencephalopathy is a demyelinating disease of viral etiology that affects the central nervous system. It presents in patients with immunosuppressive conditions and location in the posterior fossa is rare. Due to its unspecific clinical forms, its diagnosis is infrequent, leading to irreversible damage and/or the patient's death. Objective: Instruct about the possibility of cerebellar progressive multifocal leukoencephalopathy in immunocompromised patients with neurological manifestations of posterior fossa damage. Clinical case: A case is presented of a male 25-year-old patient without apparent pathological antecedents who started out with slurred speech, mixed tremor, dysmetria and ataxia. Cerebellar progressive multifocal leukoencephalopathy was diagnosed by clinical picture, neuroimaging and the presence of JC virus in the cerebrospinal fluid, alongside severe immunosuppression caused by human immunodeficiency virus diagnosed by serological testing.
ABSTRACT
Resumen El síndrome de leucoencefalopatía posterior reversible (PRES) es un desorden neurológico agudo caracterizado por cefalea, alteración de la conciencia, convulsiones y alteraciones visuales, con imágenes de edema vasogénico reversible en regiones cerebrales posteriores. Nos propusimos describir una serie de casos de pacientes trasplantados que desarrollaron PRES, caracterizando su presentación, evolución clínica, imágenes y terapéutica. Se analizaron historias clínicas informatizadas desde enero 2009 hasta enero 2019. Se recabaron datos demográficos, antecedentes clínicos, motivos y días de internación, tiempos desde el trasplante a la presentación clínica y diagnóstico. Se evaluó la mejoría/resolución en estudios por imágenes y la supervivencia anual. Se identificaron 27 pacientes con PRES; 22 trasplantados de órgano sólido de 1647 totales (1.3%) y 5 de médula ósea de 617 totales (0.8%). La media de edad fue de 38.2 años (DS 19.5), 62.9% de sexo femenino, 59.2% (16) antes del año del trasplante. Las comorbilidades más frecuentes enfermedad renal (14; 51%) e hipertensión arterial (11; 40%). Se realizó tomografía axial computarizada (TAC) a 23 pacientes (85.1%), siendo patológica en 11 (47.8%), y resonancia magnética nuclear (RMN) a 25 (92.6%), mostrando patrón característico en 17 (62.9%), con mejoría/resolución antes del año en 20 (74%). El tratamiento fue sintomático, modificando la inmunosupresión. Se registraron 5 óbitos durante la internación y otros 3 antes del año, con una supervivencia anual del 70.3% (19). La población de trasplantados, en crecimiento en nuestro medio, es particularmente susceptible al PRES. Tanto su presentación en estudios por imágenes, como su comorbilidad, difieren de otras poblaciones.
Abstract Posterior reversible encephalopathy syndrome (PRES) is an acute neurological disorder characterized by headache, encephalopathy, seizures and visual disturbances, with reversible vasogenic edema in posterior brain areas. The aim of this research was to describe a case series of transplanted patients who developed PRES, characterize their presentation, treatment, clinical and imaging evolution. Electronic medi cal records were analyzed from January 2009 to January 2019. Demographic data, clinical backgrounds, causes of admission, hospital length of stay and time from transplantation to PRES were collected. Image improvement/ resolution and annual survival were assessed. We identified 27 patients with PRES; 22 of 1647 total solid-organ transplant (1.3%) and 5 of 617 total bone marrow transplant (0.8%). The mean age at presentation was 38.2 years (SD 19.5), 62.9% female, 59.2% (16) before the year of transplantation. The most common comorbidities were kidney disease (14; 51%) and high blood pressure (11; 40%). Computed axial tomography (CT) was per formed in 23 patients (85.1%), with pathological findings in 11 (47.8%). Magnetic resonance imaging (MRI) of 25 (92.6%), showed a characteristic pattern in 17 (62.9%) with improvement/resolution before the year in 20 (74%). Treatment was symptomatic, modifying immunosuppression. Five deaths were recorded during hospital stay and another 3 before the year of admission, with an annual survival of 70.3% (19 patients). Organ transplant trend is growing in our region. These patients are particularly susceptible to PRES, with a different imaging presentation and comorbidities from other populations.
Subject(s)
Humans , Male , Female , Posterior Leukoencephalopathy Syndrome/etiology , Posterior Leukoencephalopathy Syndrome/epidemiology , Posterior Leukoencephalopathy Syndrome/diagnostic imaging , Hypertension , Seizures , Magnetic Resonance Imaging , Follow-Up StudiesABSTRACT
Resumen Se presenta el caso de una paciente de 66 años con antecedente de enfermedad renal crónica desde hace 8 años y en terapia con diálisis peritoneal, quien acudió al servicio de urgencias por presentar deterioro del estado de conciencia asociado a convulsiones y emergencia hipertensiva. La paciente fue diagnosticada con síndrome de encefalopatía posterior reversible y luego de instaurado el tratamiento indicado la sintomatología cedió; después de un mes de evolución se observó resolución de las lesiones halladas en la resonancia magnética. Esta patología se asocia principalmente a hipertensión arterial severa de rápida instauración en correlación con diversas entidades clínicas como lo fue la hipertensión arterial en el presente caso.
Abstract We present the case of a 66-year-old patient with a history of chronic kidney disease for 8 years, on peritoneal dialysis therapy, who went to the emergency service for presenting deterioration in the state of consciousness associated with seizures, and hypertensive emergency, after treatment the symptoms subside and resolution of the lesions found on the MRI after one month of evolution, classified as posterior reversible encephalopathy syndrome. Considering this diagnosis in patients with chronic kidney injury associated with arterial hypertension.
ABSTRACT
RESUMEN La leucoencefalopatía multifocal progresiva es una enfermedad desmielinizante secundaria a la infección por el virus de John Cunnigham, con baja incidencia a pesar de su alta seroprevalencia en la población general. Su principal factor de riesgo es la inmunosupresión, incluyendo la infección por el virus de la inmunodeficiencia humana (VIH), cánceres hematológicos, enfermedades inflamatorias crónicas y medicamentos inmunosupresores sistémicos. Después de la infección primaria, el virus queda latente y por mutaciones en su genoma adquiere la capacidad neuroinvasiva e infecta a los oligodendrocitos, a los que lleva a su destrucción, con el consecuente proceso desmielinizante, mientras se enfrenta a la inmunidad celular del huésped. El diagnóstico se basa en manifestaciones clínicas secundarias al compromiso encefálico, clásicamente supratentorial, así como la demostración de la presencia de genoma viral o anticuerpos en suero o líquido cefalorraquídeo y hallazgos imagenológicos e histopatológicos de lesiones en la sustancia blanca cerebral. El tratamiento, en general, consiste en la recuperación de la función inmune alterada, con reparos cuando esta se presenta en el contexto de un estado de reconstitución inmune. En este escrito se revisan los aspectos básico-clínicos más relevantes de esta enfermedad.
SUMMARY The progressive multifocal leukoencephalopathy is a demyelinating disease secondary to infection to John Cunningham Virus, it has a low incidence despite a high seroprevalence in the general population. The principal risk factor for its development is an immunosuppression, including Human Immunodeficiency Virus infection, hematologic neoplasms, chronic inflammatory diseases and systemic immunosuppressive drugs. After the primary infection, the virus stays in a latent state, acquiring a neuroinvasive capacity following a set of mutations in its genome. After infecting oligodendrocytes it takes them to destruction with the consequent demyelinating process whilst its fight to the host's cellular immune system. The diagnosis is based on a set of clinical findings secondary to encephalic compromise, classically supratentorial, in addition to a demonstration of viral genome or antibodies in serum or cerebrospinal fluid and the presence of diagnostic images and histopathologic findings on the cerebral white matter. Its treatment is based on the enhancement of the disturbed immune function, with the exception of immune reconstitution state where other strategies are applied. In this paper we will review the more relevant basic and clinical aspects of this disease.
Subject(s)
Transit-Oriented DevelopmentABSTRACT
Resumen La encefalopatía posterior reversible es un síndrome clínico-radiológico caracterizado por hipertensión arterial, cefalea, alteraciones visuales, convulsiones y delirium. Radiológicamente, se observa edema vasogénico en regiones subcorticales con predilección por los lóbulos posteriores del cerebro. Ha sido asociada con trasplante de órgano sólido, hipertensión arterial y uso de medicamentos inmunosupresores, como los anticalcineurínicos. Se reporta el caso de un niño de seis años quien inició con cefalea, cifras tensionales elevadas, irritabilidad, agresividad y por momentos agitado cinco días después de recibir un trasplante ortotópico de corazón y un día después de inicio de tacrolimus. El tratamiento incluyó manejo sintomático con antihipertensivos y anticonvulsivantes, además del aumento progresivo del inmunosupresor hasta niveles terapéuticos en sangre para disminuir el riesgo de rechazo del injerto. El síndrome de encefalopatía posterior reversible debe ser considerado en pacientes trasplantados de corazón que reciben inhibidores de la calcineurina y presentan síntomas neurológicos.
Abstract Posterior reversible encephalopathy is a clinic-radiological syndrome characterized by arterial hypertension, headache, visual disturbances, seizures and delirium. Radiologically, vasogenic edema is observed in subcortical regions with predilection for the posterior lobes of the brain. It has been associated with solid organ transplantation, arterial hypertension and the use of immunosuppressive drugs such as anticalcineurin. We report the case of a six-year-old boy who started with headache, elevated blood pressure, irritability, aggressiveness and at times agitated five days after receiving an orthotopic heart transplant and one day after starting tacrolimus. His treatment included symptomatic management with antihypertensives and anticonvulsants, in addition to a progressive increase in immunosuppressant to therapeutic blood levels to reduce the risk of graft rejection. Reversible posterior encephalopathy syndrome should be considered in heart transplant recipients receiving calcineurin inhibitors who present neurological symptoms.
ABSTRACT
Resumen La enfermedad de sustancia blanca representa una enfermedad poco común en usuarios que se apersonan al Organismo de Investigación Judicial, por este motivo al valorar un caso es necesario investigar los antecedentes de la medicina legal para establecer mecanismos causales con hechos representados en la sociedad. Además, es necesario abordar la enfermedad de sustancia blanca desde su aparición, incidencia, aspectos clínicos, hallazgos ante una posible autopsia, explorar el desarrollo de fisiopatología como indagar acerca de las formas de diagnosticar la enfermedad y valorar las opciones terapéuticas. Estos conocimientos son base para establecer una relación médico legal y analizar si el caso de una niña de 9 años tiene correlación con una historia de trauma en cabeza que no evidencia cambios inflamatorios, aumento de volumen de tejidos blandos o duros, excoriaciones, equimosis, hematomas, cicatrices, gradas o callos óseos y con valoraciones médicas que indican tetraparesia flácida en ausencia de hallazgos patológicos al reporte de tomografía axial computarizada y resonancia magnética de columna cervicodorsal.
Abstract White matter disease represents a rare disease in users who go to the Organismo de Investigación Judicial (Judicial Investigation Agency) for this reason, when evaluating a case, it is necessary to investigate the antecedents of legal medicine to establish causal mechanisms with facts represented in society. In addition, it is necessary to address white matter disease from its appearance, incidence, clinical aspects, findings in a possible autopsy, explore the development of pathophysiology such as inquire about ways to diagnose the disease, and assess therapeutic options. This knowledge is the basis for establishing a legal medical relationship and analyzing whether the case of a 9-year-old girl has a correlation with a history of head trauma that does not show inflammatory changes, increased soft or hard tissue volume, excoriations, bruising, bruising. , scars, bleachers or calluses and with medical evaluations that indicate flaccid tetraparesis in the absence of pathological findings on the report of computed tomography and magnetic resonance imaging of the cervicodorsal spine.
Subject(s)
Humans , Female , Child, Preschool , Leukoencephalopathies , White Matter , Costa RicaABSTRACT
Anti-contactin associated protein-like 2 (CASPR2) antibody encephalitis is a rare autoimmune encephalitis with variable clinical symptoms and atypical imaging manifestations. The prognosis of the patients with severe disease is poor. Reversible posterior leukoencephalopathy syndrome is rarely reported in autoimmune encephalitis. The clinical data, diagnosis and treatment of a patient with anti-CASPR2 antibody encephalitis complicated with reversible posterior encephalopathy syndrome were reported, in order to improve the understanding of clinicians on the rare disease complicated with atypical imaging manifestations.
ABSTRACT
JC virus (JCV) is a member of polyomaviridae family that infects approximately 70% of the population worldwide. JCV constantly stays in a latent state after the primary infection. In immunosuppressed individuals, especially under the circumstances of low cellular immune function, JCV may be reactivated and lead to severe clinical manifestations. In recent years, the correlation between JCV and complications after renal transplantation has captivated widespread attention. JCV-associated nephropathy (JCVAN) has been reported. Here, latest research progresses on the epidemiology, molecular biology, in vivo infection process, JCV and complications after renal transplantation, and the relationship between JCV and BKV were reviewed, aiming to provide reference for the adjustment of immunosuppressive regimen following renal transplantation.