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 80,221 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Acute necrotizing encephalopathy is a relatively new disease. The characteristic clinical findings are of febrile illness followed by rapid deterioration in mental status and seizures. The hallmark of the disease is multifocal bilateral symmetric lesions affecting the thalamus, hypothalamus, brainstem tegmentum, cerebral white matter, and cerebellum. The etiology is unknown, but immune-mediated mechanism was suggested. We present a 12-year-old previously healthy girl who developed increased sleepiness progressing to stupor and coma. Magnetic resonance imaging (MRI) of the brain showed the characteristic findings previously described in acute necrotizing encephalopathy. Her mental status improved dramatically with steroid treatment, and the MRI findings resolved completely within 6 months. Following the acute illness, she developed a complex neuropsychiatric disorder consistent with basal ganglia syndrome.
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PMID:Acute necrotizing encephalopathy presenting as a basal ganglia syndrome. 1141 19

Acute necrotizing encephalopathy (ANE) is a devastating and rapidly progressive neurologic disorder that occurs in healthy children after common viral infections. Typically, ANE is sporadic and does not recur. However, familial (ANE1) and recurrent cases have been reported and were recently linked to mutations in RANBP2 (RAN-binding protein 2). We report here a multiply affected kindred with recurrent familial ANE. These affected male siblings (a set of twins and their older brother) all presented with prodromal fever and upper respiratory tract infection that progressed within 72 hours to seizures, coma, and ultimately death, a course that is typical of ANE. It should be noted that 1 brother was treated with early aggressive management, including corticosteroids, and he survived for an additional 5 years. This represents the second reported case of familial ANE in the United States and the only case of male siblings with consanguineous parents. We hope that early recognition and growing awareness can lead to more effective treatment and better outcomes in the future.
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PMID:Acute necrotizing encephalopathy in 3 brothers. 2014 83

Acute necrotizing encephalopathy is characterized by fever, seizures, acute encephalopathy, and rapid progression to coma. It is usually associated with viral illness and shows characteristic brain magnetic resonance imaging features, including symmetrical involvement of bilateral thalami, brain stem, white matter, and cerebellum. After the first report of recurrent or familial cases in 2003, similar cases were found exclusively in American and European regions. The association with Ran-binding protein 2 gene was identified in 75% of familial or recurrent cases. This report describes a previous healthy 22-month-old boy who recurrently manifested typical clinical and radiological characteristics of acute necrotizing encephalopathy. His neurological outcome worsened with repeated episodes. There was no family history of acute necrotizing encephalopathy and no mutation in the coding region of Ran-binding protein 2 (RANBP2) gene. This is the first reported case of recurrent acute necrotizing encephalopathy in a non-Caucasian family.
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PMID:Recurrent acute necrotizing encephalopathy in a Korean child: the first non-Caucasian case. 2256 10

Acute necrotizing encephalopathy (ANE) may be suspected when a young child presents with abrupt onset of altered mental status, seizures, or both. Definitive clinical diagnosis is based on magnetic resonance imaging (MRI) results. ANE is associated with influenza virus infections. Preliminary data suggests that up to 25% of ANE patients die, and up to 25% of ANE survivors develop substantial neurologic sequelae. Here, we describe a case of a comatose 22-month-old girl who was admitted to our hospital because of febrile illness and seizures. On day 13 of her illness, she died from ANE associated with infection from parainfluenza virus. Brain MRI results indicated diffuse bilateral symmetric signal changes in both basal ganglia, thalami, periventricular white matter, pons, and cerebral white matter, as well as generalized swelling of the brain.
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PMID:A case of acute necrotizing encephalopathy associated with parainfluenza virus infection. 2257 76

Acute necrotizing encephalopathy predominately affects young children in Japan, Taiwan, and Korea. It manifests with fever, altered mental status, and seizures 2-5 days after the onset of upper respiratory infection. It is commonly associated with influenzas A, B, and H1N1. The hallmark of the encephalopathy involves multifocal, symmetric brain lesions affecting the bilateral thalami, brainstem tegmentum, cerebral periventricular white matter, cerebellum, and medulla, as visualized by computed tomography and magnetic resonance imaging. Prognoses were uniformly dismal before 1980, with high mortality rates and severe neurologic sequelae in survivors. We describe a previously healthy 4-year-old Caucasian girl who presented with fever, alterations of consciousness, and convulsions. Nasal swab revealed her to be influenza A-positive, and her magnetic resonance imaging was diagnostic of the disease. Prompt recognition of the disease and treatment with hypothermia and anti-inflammatory agents led to a favorable outcome.
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PMID:Favorable outcomes in acute necrotizing encephalopathy in a child treated with hypothermia. 2263 35

Acute necrotizing encephalopathy (ANE) has a characteristic imagimg finding of bilateral symmetrical thalamic lesions. Acute encephalopathy with biphasic seizures and late reduced diffusion (AESD) is characterized by biphasic clinical course and high intensity of subcortical white matter in MRI diffusion images appearing around the late seizure. We herein report a case of an 8-month-old girl who presented with fever and status epilepticus associated with human herpes 6 infection. Although MRI first demonstrated images of ANE, typical AESD images were observed several days after the onset. We therefore concluded that this case had a combination of ANE and AESD. A proper therapeutic strategy should be established, and acute encephalopathy needs to be better clarified by identifying diagnostic markers and improving the genetical analysis.
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PMID:[A case of acute necrotizing encephalopathy and acute encephalopathy with biphasic seizures and late reduced diffusion]. 2324 May 32

Acute necrotizing encephalopathy of childhood (ANEC) is a disease characterized by respiratory or gastrointestinal infection and high fever accompanying with rapid alteration of consciousness and seizures. This disease is nearly exclusively seen in East Asian infants and children who had previously been completely healthy. Serial magnetic resonance imaging examinations have demonstrated symmetric lesions involving the thalami, brainstem, cerebellum, and white matter in this disease. The condition accompanies a poor prognosis with high morbidity and mortality rates. A 22-month-old toddler with ANEC hospitalized in Amirkola Children Hospital is being reviewed.
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PMID:Acute necrotizing encephalopathy of childhood; a case report. 2466 98

Acute necrotizing encephalopathy of childhood (ANEC) is a disease, characterized by a respiratory or gastrointestinal infection, accompanied with fever, rapid alteration of consciousness, and seizures. The clinical characteristics of ANEC include acute encephalopathy following a viral infection, seizure, altered consciousness, and absence of cerebrospinal fluid (CSF) pleocytosis, with an occasional increase in the level of proteins. This disease is almost exclusively seen in previously healthy infants and children from East Asia. Serial magnetic resonance imaging (MRI) examinations have demonstrated symmetric lesions involving the thalami, brainstem, cerebellum, and white matter. ANEC has a poor prognosis with high morbidity and mortality rates. Herein, we present three cases of ANEC, who were referred to Bu-Ali Hospital of Ardabil, Iran during two weeks. Report of these three cases promoted the idea of an epidemic. The purpose of this case series was to raise the issue that ANEC may occur as an epidemic.
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PMID:Three Cases of Acute Necrotizing Encephalopathy: Is It an Epidemic or Only Incidental? 3225 27

Acute necrotizing encephalopathy (ANE) is a rare disease in childhood. We reviewed the 10-year data from a local pediatric department, reported the clinical characteristics, laboratory tests, neuroimaging findings, and outcome of the acute necrotizing encephalopathy cases and identified the potential factors affecting the outcome. Eight episodes of acute necrotizing encephalopathy among 7 patients were recorded, in which all of them had an initial presentation of fever and seizure. We identified that acute necrotizing encephalopathy patients with a severe score of Glasgow Coma Scale (GCS) on presentation, brainstem involvement in magnetic resonance imaging (MRI) of the brain, and higher MR imaging scores were associated with worse outcome. Association of outcome with acute necrotizing encephalopathy severity score, platelet count, and serum alanine aminotransferase level did not reach a statistically significant level. These results highlight the importance of combined clinical, laboratory, and neuroimaging findings in determining the prognostic outcome of acute necrotizing encephalopathy patients.
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PMID:Presentation and Outcome of Acute Necrotizing Encephalopathy of Childhood: A 10-Year Single-Center Retrospective Study From Hong Kong. 3249 3

Acute necrotizing encephalopathy (ANE) is a recently identified, uncommon encephalopathy affecting children. ANE is characterized by a preceding viral illness followed by seizures and rapid progressive neurologic deterioration. The diagnosis of ANE is made based on clinical presentation and characteristic multifocal brain lesions seen on computed tomography (CT). We report a previously healthy two-year-old boy who presented to our emergency department (ED) after a seizure in the setting of fever and diarrhea. He was ultimately diagnosed with ANE and treated with steroids and IVIG. Early identification of this high morbidity condition by its typical clinical picture and characteristic radiologic findings is key to allow for optimal treatment.
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PMID:Acute necrotizing encephalopathy: A case report. 3286 30


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