Clinical and imaging features of acute encephalopathy with biphasic seizures and late reduced diffusion in children / 中华儿科杂志

Objective: To explore the clinical and imaging features of acute encephalopathy with biphasic seizures and late reduced diffusion(AESD) in children. Methods: For the case series study, 21 children with AESD from Peking University First Hospital, Provincial Children's Hospital Affiliated to Anhui Medical University, Children's Hospital of Fudan University, and Shanxi Children's Hospital who were diagnosed and treated from October 2021 to July 2023 were selected. Clinical data were collected to summarize their clinical information, imaging, and laboratory tests, as well as treatment and prognostic characteristics. Descriptive statistical analysis was applicated. Results: Of the 21 cases with AESD, 11 were males and 10 were females, with the age of onset of 2 years and 6 months (1 year and 7 months, 3 years and 6 months). Of the 21 cases, 18 were typical cases with biphasic seizures. All typical cases had early seizures within 24 hours before or after fever onset. Among them, 16 cases had generalized seizures, 2 cases had focal seizures, and 7 cases reached the status epilepticus. Of the 21 cases, 3 atypical cases had late seizures in biphasic only. The late seizures in the 21 cases occurred on days 3 to 9. The types of late seizures included focal seizures in 12 cases, generalized seizures in 6 cases, and both focal and generalized seizures in 3 cases. Diffusion-weighted imaging (DWI) test on days 3 to 11 showed reduced diffusion of subcortical white matter which was named "bright tree sign" in all cases. The diffuse cerebral atrophy predominantly presented in the front-parietal-temporal lobes was found in 19 cases between day 12 and 3 months after the onset of the disease. Among 21 cases, 20 had been misdiagnosed as autoimmune encephalitis, central nervous system infection, febrile convulsions, posterior reversible encephalopathy syndrome, acute disseminated encephalomyelitis, and hemiconvulsion-hemiplegia-epilepsy syndrome. All the cases received high-dose gammaglobulin and methylprednisolone pulse therapy with poor therapeutic effect. By July 2023, 18 cases were under follow-up. Among them, 17 cases were left with varying degrees of neurologic sequelae, including 11 cases with post-encephalopathic epilepsy; 1 recovered completely. Conclusions: AESD is characterized by biphasic seizures clinically and "bright tree sign" on DWI images. Symptomatic and supportive treatments are recommended. The immunotherapy is ineffective. The prognosis of AESD is poor, with a high incidence of neurological sequelae and a low mortality.

Clinical and imaging analysis of neurological complications in critically ill children infected with SARS-CoV-2 Omicron / 中华危重病急救医学

OBJECTIVE@#To summarize clinical predictors and imaging characteristics of critically ill children infected with SARS-CoV-2 Omicron with neurological complications in Shenzhen during the peak of the first round of infections.@*METHODS@#The clinical data of 11 critically ill children with neurological complications infected with SARS-CoV-2 Omicron in Shenzhen Children's Hospital from December 12 to 31, 2022, were retrospectively collected and analyzed. Laboratory test results related to liver parenchymal injury, histiocytic injury, inflammation, and coagulation function were collected, and imaging characteristics including CT and/or magnetic resonance imaging (MRI) were analyzed. The differences in CT/MRI score, acute necrotizing encephalopathy severity scale (ANE-SS) score and total score (CT/MRI score + ANE-SS score) were compared between the two groups with different prognosis during hospitation.@*RESULTS@#Among 11 children, 7 were male and 4 were female. The age ranged from 10 months to 16 years. There were 5 cases of acute necrotizing encephalopathy (ANE) and 6 cases of acute fulminant cerebral edema (AFCE). During hospitalization, 3 patients survived and 8 patients died of multiple organ dysfunction syndrome (MODS), including 2 cases of ANE and 6 cases of AFCE. All cases had fever (> 38.5 centigrade), and 3 cases had ultra-high fever (> 41 centigrade). Within 48 hours of onset, all cases had disorders of consciousness and 9 cases had seizures. The 8 dead children had complications with multisystem involvement, including shock, respiratory failure, disseminated intravascular coagulation (DIC), liver failure, renal failure or myocardial damage, and the laboratory predictors related to hepatocellular injury [alanine aminotransferase (ALT), aspartate aminotransferase (AST)], histocyte injury [creatine kinase (CK), lactate dehydrogenase (LDH)], inflammation [procalcitonin (PCT), interleukin-6 (IL-6), serum ferritin (SF)], coagulation function (D-dimer) and blood glucose (Glu) increased in different quantities, of which PCT was specifically increased in 6 cases with AFCE, PLT was specifically decreased in 3 cases with AFCE, and ALT and LDH were significantly increased in 2 cases with ANE. Imaging analysis showed subarachnoid hemorrhage, basal ganglia and thalamus lesions in all 6 cases with AFCE, while thalamus lesions in all 5 cases with ANE. The ANE-SS score of 8 deceased children ranged from 2 to 7 (of which 6 cases were ≥ 5), and the ANE-SS score of 3 surviving children ranged from 0 to 2. Eight dead children had a CT/MRI score of 1-4 (of which 6 cases were 4), and 3 surviving children had a CT/MRI score of 1-2 (of which 2 cases were 1). The total score of 8 deceased children was 6-10 (of which 6 cases ≥ 8), and 3 surviving children was 1-4.@*CONCLUSIONS@#The neurological complications of critically ill children infected with SARS-CoV-2 Omicron in Shenzhen progressed rapidly to ANE and AFCE, with high mortality. High fever (> 40 centigrade), convulsion/disturbance of consciousness, and multiple organ failure were the most common symptoms in ANE and AFCE cases. PCT increased and PLT decreased specifically in AFCE cases. Poor prognosis (death) was more common in age < 4 years old, predictors of ALT, AST, CK, LDH, PCT, D-dimer, Glu, IL-6 increased significantly, PLT decreased significantly. The common imaging feature of ANE and AFCE is the involvement of dorsal thalamus, a new imaging sign of AFCE (subarachnoid hemorrhage) was found. The higher the ANE-SS score, CT/MRI score and total score, the greater the risk of death.

Manifestaciones neurológicas graves de la gripe durante la temporada gripal 2018-2019: serie de casos de 13 pacientes pediátricos / Severe neurological manifestations of influenza during 2018-2019 influenza season: Case series of 13 pediatric patients

La gripe se asocia al aparato respiratorio, especialmente en invierno, y puede causar complicaciones neurológicas. Se evaluó a pacientes pediátricos con manifestaciones neurológicas graves por gripe desde septiembre de 2018 hasta febrero de 2019 para determinar características clínicas, neuroimagenología, tratamiento y resultados. El objetivo fue evaluar la encefalitis asociada a la gripe y destacar diferentes manifestaciones neurológicas y cambios de neuroimagenología. El estudio incluyó a 13 pacientes. Los síntomas neurológicos ocurrieron tras los síntomas típicos de la gripe. Los cambios de neuroimagenología incluyen alteraciones de señal de la sustancia blanca cortical y subcortical, edema localizado o generalizado y lesiones multifocales simétricas bilaterales en el tálamo y la médula del cerebelo. Las opciones terapéuticas incluyen metilprednisolona en inyección intravenosa, inmunoglobulina intravenosa, plasmaféresis y oseltamivir. Es fundamental considerar la encefalitis asociada a la gripe en pacientes con convulsiones, la encefalopatía con hallazgos radiológicos compatibles, e iniciar el tratamiento lo antes posible

Influenza is mostly associated with the respiratory tract system, especially in the winter season. Various neurological complications could occur due to influenza infection. Pediatric patients who had severe neurological manifestations due to influenza infection from September 2018 to February 2019 were evaluated for clinical characteristics, neuroimaging studies, treatment, and outcome. We aimed to assess Influenza-associated encephalitis in children, emphasize different neurological manifestations and neuroimaging changes. Thirteen patients were included in the study. Neurological symptoms occurred after flu-like symptoms. Neuroimaging changes of influenza-associated encephalitis/encephalopathy include cortical and subcortical white matter signal alterations, localized or generalized edema, and bilateral symmetrical multifocal lesions on the thalamus and cerebellar medulla. Pulse methylprednisolone, intravenous immunoglobulin, plasma exchange, and oseltamivir are the therapy choices. It is essential to consider influenza-associated encephalitis in patients with seizures, encephalopathy with supporting radiological findings, especially during the influenza season and starting treatment as fast as possible for better outcomes.

Síndrome de Susac: Una causa poco frecuente de encefalopatía / Susac syndrome, a rare cause of encephalopathy

El síndrome de Susac es una enfermedad rara causada por oclusión autoinmune de la microvasculatura del cerebro, de la retina y del oído interno, lo que provoca la triada clínica característica de disfunción del sistema nervioso central, trastornos visuales y déficit vestíbulo-coclear. El diagnóstico se basa en las manifestaciones clínicas y en estudios complementarios que demuestren el compromiso de los tres sistemas. Existen diferentes tratamientos que incluyen combinaciones de varios fármacos inmunosupresores como corticoides, inmunoglobulina intravenosa, micofenolato mofetilo, entre otros. Presentamos el caso de una mujer de 26 años que manifestó hipoacusia izquierda, zumbidos y episodios de vértigo recurrente. Cuatro semanas después agregó visión borrosa bilateral, ataxia cerebelosa y encefalopatía. La resonancia magnética de cerebro mostró múltiples lesiones redondeadas hiperintensas en t2 y FLAIR (fluid-attenuated inversion recovery), hipointensas en t1 a nivel medial del cuerpo calloso, cápsula interna, cerebelo y pedúnculo cerebeloso medio derecho. La audiometría evidenció hipoacusia perceptual bilateral a predominio del oído izquierdo y en la angiografía por tomografía de coherencia óptica se observó obstrucción de arterias de la capa profunda de la retina. Se diagnosticó síndrome de Susac y se inició tratamiento con pulsos de metilprednisolona por 5 días y mantenimiento con micofenolato, revirtiendo totalmente la encefalopatía, con persistencia de leve ataxia e hipoacusia. Es importante conocer la triada clínica característica y los estudios complementarios necesarios para arribar al diagnóstico, ya que muchas veces se puede demorar el tratamiento inmunosupresor. Nuestro caso tuvo una excelente respuesta a los corticoides.

Susac syndrome is a rare disorder caused by autoimmune-mediated occlusions of microvessels in the brain, retina and inner ear. These occlusions lead to a characteristic clinical triad of central nervous system dysfunction, visual disturbances and vestibule-cochlear deficits. The diagnosis is based on clinical manifestations and complementary studies, which demonstrate the involvement of three systems. There are different treatments that include various immunosuppressive drugs combinations such as corticosteroids, intravenous immunoglobulin, mycophenolate mofetil, among others. We present the case of a 26-year-old woman with left hearing loss, tinnitus and episodes of recurrent vertigo, four weeks after bilateral blurred vision, cerebellar ataxia and encephalopathy. Magnetic resonance imaging of the brain showed multiple rounded hyperintense lesions in t2 and fluid-attenuated inversion recovery (FLAIR), hypointense in t1, at the middle level of the corpus callosum, internal capsule, cerebellum and right middle cerebellar peduncle. The audiometry evidenced bilateral perceptual hearing loss, predominantly in the left ear. Angiography by optical coherence tomography showed obstruction in the deep layer retina arteries. The Susac syndrome was diagnosed and treatment started with methylprednisolone pulses therapy, intravenously 1000 mg/ day for 5 days, followed by maintenance with mycophenolate, which completely reversed the encephalopathy, with persistence of mild ataxia and hearing loss. It is important to know the clinical triad characteristic and the complementary studies necessary to arrive at the diagnosis, since immunosuppressive treatment can often be delayed. Our case had an excellent response to corticosteroids.

Langerhans' cell histiocytosis with neurological injuries diagnosed from a single cutaneous lesion

Abstract: Histiocytoses are rare diseases caused by the proliferation of histiocytes. The pathogenesis remains unknown and the highest incidence occurs in pediatric patients. The clinical presentations can be varied, in multiple organs and systems, and the skin lesions are not always present. Evolution is unpredictable and treatment depends on the extent and severity of the disease. It is described the case of a patient with various neurological symptoms, extensively investigated, who had its was diagnosed with histiocytosis from a single skin lesion. This report highlights the importance of Dermatology in assisting the investigation of difficult cases in medical practice.

Endocarditis infecciosa: ¿afectan su pronóstico las complicaciones neurológicas? / Neurologic complications of infective endocarditis: do they have an impact on prognosis?

De acuerdo a la literatura, en un 20 a 40% de las endocarditis infecciosas se producen complicaciones neurológicas. Nuestro objetivo fue determinar la frecuencia de compromiso neurológico en pacientes con endocarditis infecciosa de válvulas izquierdas, considerando la presentación clínica, tipo de lesión en las imágenes y su influencia en la evolución. Se incluyeron en forma prospectiva 98 pacientes con endocarditis infecciosa de válvulas izquierdas. El 47% (46 casos) presentó compromiso neurológico. En 28 casos (61%) el síntoma fue el déficit focal, en 9 (20%) la alteración de estado de conciencia; el 17% (8) fue asintomático y 2% (1 caso) presentó convulsiones. En las imágenes, la isquemia cerebral (76%) fue la lesión más frecuente. El tamaño de la vegetación mayor a 1 cm se asoció a compromiso neurológico (57% vs. 31%, p = 0.01). Aquellos pacientes con compromiso neurológico tuvieron mayor frecuencia de reemplazo valvular (70% vs. 44%, p = 0.01) y la mortalidad hospitalaria también fue mayor (20% vs. 15%, p = 0.5). El tiempo total de internación fue significativamente más prolongado en sujetos con compromiso neurológico (32 ± 27 vs. 21 ± 15 días, p = 0.01) y la discapacidad fue mayor en los que tenían manifestaciones neurológicas (74% vs. 36% p = 0.0001). En nuestra serie, el síntoma más frecuente fue el déficit focal, y en las imágenes la isquemia cerebral fue el patrón más común. Los casos con lesión neurológica presentaron mayor tiempo de internación y grado de discapacidad al alta, pero no mayor mortalidad hospitalaria.

Neurologic complications of infective endocarditis have been observed in 20-40% of cases. Our aim was to determine the frequency of neurologic involvement, clinical manifestations, lesional patterns and evolution in patients with infective endocarditis. This was a prospective cohort study. We included 98 patients with left-sided infective endocarditis. Forty seven percent presented neurologic involvement at some time of the disease. The frequency of symptoms was: focal deficit 61%, sensory disturbance 17% and seizures 2%, while 20% remain asymptomatic. The most prevailing lesion was cerebral ischemia (76%). Vegetations larger than 1 cm were associated to neurologic involvement (57% vs. 31%, p = 0.01). Valvular replacement was more common among patients with neurologic involvement (70% vs. 44%, p = 0.01). Hospital mortality was 20% in patients with neurologic manifestation versus 15% of those without it (p = 0.5). The length of stay was significantly prolonged in patients with neurologic affection (32 ± 27 vs. 21 ± 15 days, p = 0.01) and a favorable Rankin assessment at the discharge was less likely in patients with neurologic involvement (36% vs. 74%, p = 0.0001). In this cohort, the most frequent clinical manifestation was focal deficit and the most prevalent pattern of lesion was cerebral ischemia. Those with neurologic involvement presented an increased length of stay and more disability at the discharge but not statistical significant difference in hospital mortality.

Tuberculoma cerebral en paciente joven inmunocompetente / Cerebral tuberculoma in young immunocompetent patient

RESUMEN La tuberculosis con afección del sistema nervioso central es una afectación infrecuente pero muy grave de esta enfermedad, representa el 1% de todos los casos de tuberculosis. Reportamos el caso de una joven HIV negativa, con daño del sistema nervioso central de tipo miliar a nivel cerebral y sin enfermedad pulmonar. La tuberculosis puede afectar extensamente a sujetos inmunocompetentes y este fenómeno ha sido descripto en muchas series a los largo del tiempo. Ante la sospecha clínica, el seguimiento de un protocolo específico para confirmar el diagnóstico es de vital importancia para el temprano diagnóstico y correcto manejo de una situación que puede comprometer la vida y generar a largo plazo secuelas graves. Se presenta el caso por el reto diagnóstico que ha supuesto y rara presentación en paciente inmunocompetente.

ABSTRACT Tuberculosis with central nervous system involvement is an uncommon but very serious disease, with a frecuence of 1% of all cases of tuberculosis. We report the case of a young HIV negative woman, with central nervous system damage of the miliary type in the brain without lung disease. Tuberculosis can extensively affect immunecompetent subjects and this phenomenon has been described in many series over the time. In the presence of clinical suspicion, the follow-up of a specific protocol to confirm the diagnosis is of vital importance for the early diagnosis and correct management of a situation that can compromise life and generate long-term serious sequelae. The case is presented because of diagnostic challenge and a rare presentation in an immunocompetent patient.

Outcome of isolated fetal choroid plexus cyst detected in prenatal sonography among infertile patients referred to Royan Institute: a 3-year study

Several studies have assessed the correlation of fetal choroid plexus cyst [CPC] and the risk of congenital anomalies, but few ones have discussed isolated CPC [with no other abnormal sonographic finding]. The aim of this study was to determine the outcome of isolated fetal choroid plexus cyst and to specify its clinical significance. This cross sectional study was carried out at Royan Institute in Tehran, Iran, between April 2009 and December 2012. All prenatal sonographies in this period of time were assessed using a computerized database and fetuses who had isolated CPC were recruited in the study. Sonography reports, mother serum screening test results, fetal echocardiography and amniocentesis were evaluated until birth. A follow-up phone call was made to all individuals to learn about the neonatal outcomes. Overall, 6240 prenatal sonographies were performed in this setting during this period. Isolated CPC was detected in 64 fetuses. The results of double test [N=30], triple test [N=5] and fetal echocardiography [N =24] were normal. Quadruple test result showed 3 abnormal out of 29 cases that all had normal karyotypes. Four samples were dropped out due to premature rupture of membranes [N=3] and intrauterine fetal death [N=1]. It was found that the outcomes of all remaining fetuses [N=60] were normal and no anomaly ones were seen until birth. Isolated CPC is a benign regressive condition with no clinical significance

Von Hippel-Lindau Syndrome: Demonstration of Entire Disease Spectrum with 68Ga-DOTANOC PET-CT

Von Hippel-Lindau (VHL) syndrome is a rare neoplastic disorder characterized by central nervous system (CNS) and visceral tumors. We here present 68Ga-labelled [1, 4, 7, 10-tetraazacyclododecane-1, 4, 7, 10-tetraacetic acid]-1-Nal3-Octreotide positron emission tomography computed tomography findings in a 52 year old female with VHL syndrome, demonstrating both CNS and visceral tumors.

Chronic renal disease and reversible posterior leukoencephalopathy syndrome after liver transplantation / Enfermedad renal crónica y síndrome de leucoencefalopatía posterior reversible después del trasplante del hígado

This is a case report of a patient who developed chronic renal dysfunction and neurologic emergency with multiple cranial lesions after liver transplantation. Immune-complex glomerulonephritis was confirmed on the basis of histopathologic evaluation of the renal biopsy. According to clinical features and brain magnetic resonance imaging follow-up, neuroradiographic atypical reversible posterior leukoencephalopathy syndrome (RPLS) was finally diagnosed.

Este es un reporte de caso de un paciente que desarrolló una disfunción renal crónica y requirió emergencia neurológica con múltiples lesiones craneales luego de un trasplante del hígado. La evaluación histopatológica de la biopsia renal permitió confirmar una glomerulonefritis por complejos inmunes. De acuerdo con las características clínicas y el seguimiento mediante tomografía por resonancia magnética del cerebro (de la resonancia magnética cerebral, finalmente se diagnóstico un síndrome de leucoencefalopatía posterior reversible atípico neuroradiográfico (SLPR).

Avaliação radiológica do cérebro pediátrico: qual exame devo pedir? / Radiological evaluation of the pediatric brain: what should i ask for?

Neste trabalho objetivamos esclarecer quais as principais indicações, contra-indicações, vantagens e desvantagens, de acordo com a faixa etária, de cada exame nas diversas condições patológicas do cérebro em pediatria.

In this work we aimed to clarify the main indications, contraindications, advantages anddisadvantages, according to the age, of each examination in various pathological conditions of the brain in pediatrics.

Intracranial menifestations of tuberculosis: an imaging study

In this paper we report various intracranial manifestations of tuberculosis in 48 patients who presented at our hospitals. The object of this study was to see different patterns of intracranial tuberculosis seen on cross sectional imaging in the city of Lahore and its suburbs. In a total of 48 patients, 21 had meningitis, 16 patients were found to have tuberculomas which also included 6 who had accompanying meningitis. Vasculitis resulting in lacunar infarcts was seen in 3 patients. Hydrocephalus was seen in 5 patients and choroid plexus tuberculosis [choroid plexitis] was seen in 3. Intracranial tuberculosis, therefore can have many presentations such as meningitis, tuberculomas or rare varieties such as abscess, cerebritis or choroid plexus involvement. It can also lead to devastating complications such as hydrocephalus and infarcts

Experience of cranial ultrasonography in admitted symptomatic neonates.

In infants who have experienced perinatal injury or who have neurological symptoms, it is often difficult on the basis of history and clinical examination to come to a proper diagnosis. Cranial ultrasonography of the symptomatic neonates can often diagnose the underlying pathology. Cranial ultrasonography of 28 admitted neonates with neurological symptoms was done at Community Based Medical College Hospital (CBMCH), Mymensingh. In 11 cases (39%) there were positive findings in ultrasonography. Of them, 7 (25%) were Hypoxic ischaemic encephalopathy (HIE) and 4 (14%) had dilated ventricles. No abnormal ultrasonography findings were noted in the remaining neonates. Before closure of the fontanelles, Ultrasonography can help as a cheaper, easier and acceptable method to diagnose some neurologic problems in children.

Non-epileptic manifestations in patients with single enhancing computed tomography lesions.

OBJECTIVES AND METHODS: Single enhancing lesions are common computed tomographic (CT) abnormality in patients with epilepsy. In this series we are reporting 13 unusual cases with varied non-epileptic neurological manifestations in patients with ring or disk enhancing CT lesions. RESULTS: Acute, stroke like non-vascular focal neurological deficits (hemiparesis in four patients, and crural monoparesis, Broca's aphasia, homonymous hemianopia, hemichorea in one patient each) were frequent non-epileptic manifestations. Episodic vascular type of headache was seen in three patients, one patient had headache because of raised intracranial pressure. One patient presented with acute confusional state. All these patients were treated symptomatically, and with oral corticosteroids. CT lesions disappeared in 8-12 weeks time in all patients except in one patient with chorea where the lesion calcified. Significant clinical improvements were noted in all the patients. CONCLUSIONS: Several non-epileptic manifestations can also be associated with single enhancing CT lesions, and like epileptic disorders these disorders also have a benign course. Corticosteroids, probably, hasten the clinical improvement and produce early resolution of the CT lesions.