Encefalopatía necrotizante aguda asociada a influenza A / Influenza A-associated acute necrotizing encephalopathy

INTRODUCCIÓN: La encefalopatía necrotizante aguda (ENA) es una patología rara, caracterizada por compromiso de conciencia y presencia de múltiples lesiones encefálicas simétricas localizadas principalmente en tá lamo. Se asocia a alta letalidad e importantes secuelas. OBJETIVO: Describir el caso de un paciente escolar con ENA asociada a influenza-A con evolución favorable. CASO CLÍNICO: Paciente de 6 años de edad, con historia de 3 días de evolución de síntomas respiratorios altos asociados a fiebre (39 °C). Veinticuatro horas previo a la consulta destacaba compromiso de conciencia cualicuantitativo. Se realizó punción lumbar con proteinorraquia leve. En resonancia magnética (RM) se identificó focos de restricción a la difusión bilaterales de distribución simétrica, talámicos, en cuerpos mamila res, periacueductales, de tegmento pontino, hipocampales y en ambas cápsulas externas, asociado a componente hemorrágico y edema vasogénico, sugerente de ENA. Recibió tratamiento empírico con metilprednisolona y oseltamivir. Posteriormente, se recibió resultado positivo para virus influenza- AH1. Dado diagnóstico, se decidió administrar inmunoglobulina, evolucionando lento pero favora blemente. Al alta levemente bradipsíquico, con disminución de agudeza visual, lenguaje espontáneo y marcha con apoyo. A los 6 meses de seguimiento presentaba lenguaje y marcha normales, persis tiendo alteración visual a derecha. CONCLUSIÓN: Nuestro paciente presentó una ENA cuyo diagnóstico y manejo oportunos se asociaron a una favorable evolución neurológica en el largo plazo. Si bien la ENA es una patología infrecuente, la morbimortalidad asociada es altísima, por lo que resulta de gran importancia tener un alto grado de sospecha, a fin de solicitar estudio imagenológico dirigido, buscar causas infecciosas relacionadas e iniciar un manejo oportuno.

INTRODUCTION: Acute necrotizing encephalopathy of childhood (ANEC) is a rare disease characterized by alteration of consciousness and multiple symmetric brain lesions mainly involving the thalamus. It presents a high mortality rate and severe sequelae. OBJECTIVE: To describe a school-age patient with influenza A-related ANEC with favorable evolution. CLINICAL CASE: Six-year-old boy with 3 days history of upper respiratory symptoms and fever (39 °C). One day previous to admission, he presented altered state of consciousness. A lumbar puncture was performed, showing a mild increase of protein level in CSF. MRI showed bilateral foci of symmetric restricted signal in the thalamus, mammillary bodies, periaqueductal gray, ventral tegmentum, hippocampus, and in both external capsules, which was compatible with ANEC. The patient received empirical treatment with methylprednisolone and oseltamivir. Subsequently, a positive result was received for influenza. Considering diagnosis and severity of illness, it was decided to administer immunoglobulin. The patient got better slowly but favorably. At discharge, he still was mildly bradypsychic with decreased visual acuity, spontaneous speech and walking with assistance. At 6 months of follow-up, the patient presented normal speech and gait, with persistent visual impairment in the right eye. CONCLUSIONS: Our patient presented ANEC, whose timely diagnosis and management were associated with a favorable neurological evolution in the long term. Although ANEC is an infrequent pathology, it has very high morbidity and mortality rates, so it is very important to have a high degree of suspicion in order to request a targeted imaging study, search for related infectious causes, and start proper treatment.

A Case of Acute Hemorrhagic Encephalomyelitis associated with Mycoplasma Pneumoniae Infection / 대한소아신경학회지

Acute hemorrhagic encephalomyelitis (AHEM) is a severe form of encephalitis characterized by fulminant clinical course and presence of hemorrhagic necrosis of the white matter. Mycoplasma pneumoniae (M. pneumoniae) have severe central nervous syndrome complication with encephalitis as that most common pediatric manifestations, but have been extremely rare report with AHME. A 10-year-old boy was referred to emergency room because of drowsy mental status, weakness of left side extremities and truncal ataxia. His deep tendon reflexes were hyperactive, neck stiffness sign and Babinski sign were both positive. Motor power were decreased on the both left upper and lower extremities. The sequences of T2-weighted and gradient recalled echo (GRE) showed hyper-intense lesions on multifocal white natter areas with hemorrhagic signal. Cerebrospinal fluid (CSF) analysis showed the pleocytosis with neutrophil dominant. The results of deoxyribonucleic acid (DNA) test and culture of M. pneumoniae were reported negative in CSF, but immunoglobulin M (IgM) was positive in blood. He received intravenous high dose corticosteroid and macrolide. After discharge, his neurologic function gradually returned to normal including sitting and standing without support. We reported the previously healthy boy with M. pneumonia related AHEM. The early diagnosis with brain MRI and the aggressive immunosuppressive treatment may be beneficial for recovery.

Utility of Follow-up Diffusion Tensor Imaging in Acute Hemorrhagic Leukoencephalitis: a Case Report

Acute hemorrhagic leukoencephalitis (AHLE) is an acute, rapidly progressing, fulminant demyelinating disease. It is a rare disease of the central nervous system with high mortality; survivors commonly present with significant neurological deficit. We report the case of a 16-month-old girl who survived AHLE and presented with the associated neurologic deficit. The patient came into the emergency department with febrile seizure. She showed bilateral pinpoint-sized pupils and hyperactive deep tendon reflexes. Her mental status was initially drowsy and rapidly progressed to stupor. Extensive demyelination and microbleeds were found in the cerebral white matter, thalamus and left cerebellum on Magnetic resonance imaging (MRI) scans. Her mental status was improved by intravenous administration of immunoglobulin and methylprednisolone. Five months after being discharged, increased white matter connectivity was found on color-coded follow-up MR diffusion tensor imaging (DTI) as compared to previous MRI. We therefore suggest adding the DTI technique when a follow-up MRI is performed in patients with AHLE. It could be useful to visualize the status of axonal injury and to encourage patients and their parents to continue the rehabilitation program.

Utility of Follow-up Diffusion Tensor Imaging in Acute Hemorrhagic Leukoencephalitis: a Case Report

Acute hemorrhagic leukoencephalitis (AHLE) is an acute, rapidly progressing, fulminant demyelinating disease. It is a rare disease of the central nervous system with high mortality; survivors commonly present with significant neurological deficit. We report the case of a 16-month-old girl who survived AHLE and presented with the associated neurologic deficit. The patient came into the emergency department with febrile seizure. She showed bilateral pinpoint-sized pupils and hyperactive deep tendon reflexes. Her mental status was initially drowsy and rapidly progressed to stupor. Extensive demyelination and microbleeds were found in the cerebral white matter, thalamus and left cerebellum on Magnetic resonance imaging (MRI) scans. Her mental status was improved by intravenous administration of immunoglobulin and methylprednisolone. Five months after being discharged, increased white matter connectivity was found on color-coded follow-up MR diffusion tensor imaging (DTI) as compared to previous MRI. We therefore suggest adding the DTI technique when a follow-up MRI is performed in patients with AHLE. It could be useful to visualize the status of axonal injury and to encourage patients and their parents to continue the rehabilitation program.

Leucoencefalitis hemorrágica aguda: manifestaciones clínicas, hallazgos radiológicos y neuropatológicos / Acute hemorrhagic leukoencephalitis: clinical presentation, radiologic and neuropathologic features

La Leucoencefalitis Hemorrágica Aguda o enfermedad de Hurst es una enfermedad rara, caracterizada clínicamente por inicio súbito, curso clínico severo, usualmente fatal que se presenta posterior a una infección viral o vacunación. Patológicamente se caracteriza por desmielinización perivenular y necrosis hemorrágica difusa del sistema nervioso central. Se considera que representa una forma hiperaguda y severa de la Encefalomielitis Aguda Diseminada, la cual es una entidad inflamatoria con una base fisiopatológica autoinmune postinfecciosa. A continuación, se expone el caso de una paciente adulta, que ingresó al servicio de urgencias con cuadro clínico típico de migraña y antecedente de cefaleas previas de similares características. Quien doce horas posterior a su ingreso desarrolló de forma rápidamente progresiva depresión del estado de conciencia, signos neurológicos focales y signos de hipertensión de fosa posterior, que llevaron a desenlace fatal en tan solo 96 horas del inicio del cuadro clínico con hallazgos patológicos postmortem que confirman leucoencefalitis hemorrágica aguda. Se revisan las características clínicas, los hallazgos radiológicos y patológicos de esta entidad clínico-patológica poco común.

Acute hemorrhagic leukoencephalitis or Hurst disease is a rare disorder characterized by its severe neurological involvement, rapid progression and fatal outcome in a few days. The disease is usually a post infectious condition. Under microscope, it is identified by a perivenular demyelination and a diffuse hemorrhagic necrosis. This entity is thought to represent a hyperacute severe form of acute disseminated encephalomyelitis, which is an inflammatory autoimmune post infectious disorder. We describe the case of an adult woman, who visits the emergency room with migraine-like symptoms and a previous clinical history of similar headaches. Twelve hours later she developed focal neurologic findings, stupor and signs of endocraneal hypertension, her clinical status continued to worsen and in 96 hours she succumbed. The autopsy confirm acute hemorrhagic Leukoencephalitis. Reviewed clinical, radiological and pathological characteristics of this uncommon disease.

Acute necrotizing encephalopathy.

Acute necrotizing encephalopathy (ANEC) is a rare disease well recognized in Japan but has not yet been reported from Indian subcontinent. We describe here a case of ANEC with the neuroimaging findings. P. vivax infection was detected as an associated finding and the treatment given.

Clinical Spectrum and Prognostic Factors of Acute Necrotizing Encephalopathy in Children

This study was conducted to investigate the etiology, the clinical characteristics and prognosis of acute necrotizing encephalopathy (ANE) in Korean children. Six children (1 yr to 7 yr) patients with ANE were enrolled. They were diagnosed by clinical and radiological characteristics and their clinical data were retrospectively analyzed. In a search of clinically plausible causes, brain MRI in all patients, mitochondrial DNA studies for mitochondrial encephalomyopathy, lactic acidosis, and strokelike episodes (MELAS) and myoclonus epilepsy and ragged red fibers (MERRF) in four patients, and genomic typing on HLA DRB/HLA DQB genes in three patients were performed. All had precedent illnesses and the main initial symptoms included mental change (83%), seizures (50%), and focal deficits (50%). MRI revealed increased T2 signal density in the bilateral thalami and/or the brainstem in all patients. Mitochodrial DNA studies for MELAS and MERRF were negative in those children and HLA-DRB1*1401, HLA-DRB3*0202, and HLA-DQB1*0502 seemed to be significant. A high dose steroid was given to all patients, which seemed to be partly effective except for 2 patients. In conclusion, ANE is relatively rare, but can result in serious neurological complication in children. Early detection and appropriate treatment may lead to a better neurological outcome.

Acute hemorrhagic leukoencephalitis mimicking herpes simplex encephalitis: case report

Leucoencefalite hemorrágica aguda (AHLE) é forma grave da encefalomielite disseminada aguda, caracterizada por curso clínico fulminante e necrose hemorrágica da substância branca. Relatamos o caso de uma paciente de 57 anos de idade que desenvolveu estado confusional agudo uma semana após infecção respiratória. Ressonância magnética do encéfalo mostrou alterações de sinal bilateralmente em lobos frontal e temporal sugestivas de encefalite herpética. O estudo microbiológico do líquor foi negativo, assim como a reação de cadeia da polimerase (PCR) para o vírus herpes simplex. Diagnosticou-se AHLE e a paciente foi tratada com metilprednisolona 1g/dia durante 5 dias. Apesar do tratamento, a paciente apresentou sequelas neurológicas compatíveis com a gravidade de seu quadro clínico.

Acute Necrotizing Encephalopathy in Korean Infants and Children: Imaging Findings and Diverse Clinical Outcome

OBJECTIVE: The purpose of our study was to describe acute necrotizing encephalopathy in Korean infants and children, and we sought to evaluate the prognostic factors. MATERIALS AND METHODS: Acute necrotizing encephalopathy was diagnosed in 14 Korean infants and children. We retrospectively analyzed the neuroimaging findings including the follow-up changes. The clinical course of the disease was graded, and we evaluated prognostic factors including age, serum level of the aminotransferase, hemorrhage, and localized atrophy of the brain. RESULTS: This encephalopathy predominantly affected the bilateral thalami (n=14), pons (n=12), and midbrain (n=10) in a symmetrical pattern. Hemorrhage was observed in eight patients (57%). On the follow-up images (n=12), the brain lesions were reduced in extent for all patients, and generalized atrophy was seen in six patients. Localized tissue loss was observed in five patients and a complete resolution occurred for one patient. All the patients survived and two recovered completely; mild (n=6) to severe (n=6) neurological deficits persisted in the remaining 12 patients. The significant prognostic factors identified in this study were the presence of hemorrhage (p = 0.009) and localized atrophy (p = 0.015). CONCLUSION: Acute necrotizing encephalopathy in Korean patients showed the characteristic patterns of the post-infectious encephalopathy as described in the literature. The high survival rate and the relatively favorable clinical course observed for the present study suggest a more diverse spectrum of disease severity than was previously described. The presence of hemorrhage and localized tissue loss on MR images may suggest a poor prognosis.

Posterior leukoencephalopathy in a girl with acute haemorrhagic leukoencephalitis.

In children, posterior leukoencephalopathy is frequently associated with hypertensive encephalopathy, anticancer chemotherapy, treatment with immunosuppressive drugs in patients with organ transplantation, transfusion or human immunodeficiency virus infection. Posterior leukoencephalopathy in these children appears as a complicating illness and resolves once precipitating factor (e.g. cancer chemotherapy) is removed. Here we are reporting a fatal case of acute haemorrhatic leukoencephalitis in a 13 year old girl, imaging abnormalities are also suggestive of posterior leukoencephalopathy. Posterior leukoencephalopathy in our patient possibly, is a part of post-infectious haemorrhagic leukoencephalitis, rather than because of ischaemia or cerebral oedema secondary either to abrupt increase in blood pressure or following administration of immunosuppressive drugs.

Tc-99m-HMPAO SPECT Findings in Clinically Suspected HSE

Herpes simplex virus type I (HSV-I) causes an acute necrotizing encephalitis that selectively affects tempioral and frontal lobes. The sequelae and mortality of herpes simplex encephalitis(HSE) may be reduced by available antiviral therapy and therefore early diagnosis of HSE is essential. We have assessed the potential of brain perfusion scintigraphy using Tc-99m-HMPAO single photon emission computed tomoglaphy(SPECT) in HSE. Nine Tc-99m-HMPAO SPECTs were performed in eight patients with clinically suspected herpes simplex encephalitis. The examinations were made between 18 days and 10 months after onset of encephalitic symptoms The SPECT images in seven patients revealed decreased accumulation ol radioactivity in the affected temporal, frontal or parietal lobes. In a 30 yr-old female patient, two SPECT images were obtained on 18th day after the onset of symptoms and 3 months later. Her initial SPECT showed increased accumulation of radioactivity in the affected temporal and inferior frontal lobes. 3 month later follow-up SPECT image revealed the decreased radioactivity in the affected temporal, inferior frontal, and inferior parietal lobes. We found that the SPECT done at early stage of HSE may show increased cerebral perfusion due to inflammatory or other pathologic mechanism and the later stage SPECT may show decreased cerebral perfusion due to decreased cerebral metabolism caused by neuronal death We suggest that Tc-99m-HMPAO SPECT may support the clinical diagnosis of HSE.