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1/21. Necrotizing encephalopathy and macrocephaly with mitochondrial complex I deficiency.

    A neonate presented in the first weeks after birth with vomiting. He was unresponsive, with hypotonia, macrocephaly, and lactic acidosis. The cranial computed tomographic scan revealed a hypodense brain, with increased brain volume and extensive cerebral edema. He died at 6 weeks of age; postmortem examination revealed necrotizing encephalopathy with marked brain edema, spongiosis, thalamic necrosis, and basal ganglia calcifications. Enzyme studies of the mitochondrial respiratory chain revealed complex I deficiency in both muscle and liver.
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ranking = 1
keywords = necrotizing
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2/21. Clinical diversity in acute necrotizing encephalopathy.

    Acute necrotizing encephalopathy is a novel disease entity, proposed by Mizuguchi et al in 1995, that shows a characteristic selective and symmetric involvement of the thalamus, brain stem, and cerebellum. It usually leaves sequelae. The etiology of acute necrotizing encephalopathy is unknown. We describe here six patients aged 6 months to 5 years (four boys and two girls). Four cases were typical, and the patients' cranial computed tomographic scans and magnetic resonance imaging showed irreversible symmetric involvement of the thalamus, brain stem, and cerebellum. Three of the patients died, and one was left with severe sequelae. In the other two patients, who had selective reversible thalamic involvement, the disease was mild; one also showed transient unilateral thalamic involvement. These patients recovered completely. We consider the illness in these two patients to fit the criteria of the mild form of acute necrotizing encephalopathy. We believe that acute necrotizing encephalopathy has some clinical diversity, as is seen in other neurologic disorders, and that a mild form could exist.
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ranking = 2564.7999184841
keywords = acute necrotizing, necrotizing
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3/21. Varicella-associated acute necrotizing encephalopathy with a good prognosis.

    A patient with acute necrotizing encephalophathy (ANE) following varicella infection with a good prognosis is reported. A somatosensory evoked magnetic field (SEF) study using a 37-channel-magnetoencephalography system demonstrated normal latency and strength of the first component (N20m) elicited by median nerve stimulation, despite bilateral symmetrical thalamic lesions on MRI. The normal SEF findings and the good prognosis suggested a reversible breakdown of the blood-brain barrier, and an edematous process as the brain pathology. Furthermore, our results support the idea of distinct generators for the three earliest cortical SEF components (N20m, P30m, N45m).
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ranking = 1831.28565606
keywords = acute necrotizing, necrotizing
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4/21. Acute necrotizing encephalopathy presenting as a basal ganglia syndrome.

    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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ranking = 371.25713121201
keywords = acute necrotizing, necrotizing
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5/21. Brain MRI findings in influenza A-associated acute necrotizing encephalopathy of childhood.

    Acute necrotizing encephalopathy following influenza A is frequently reported from japan and taiwan but is very rarely seen in Western countries. We describe a 10-year-old boy with acute necrotizing encephalopathy, who developed symmetrical thalamic and brain stem lesions seen on magnetic resonance imaging (MRI). Serological confirmation of influenza A was made 2 weeks after the onset of symptoms. The child made a full recovery. This case is interesting because of its rarity in European countries, the striking brain MRI findings and the good neurological outcome.
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ranking = 1832.28565606
keywords = acute necrotizing, necrotizing
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6/21. Acute necrotizing encephalopathy: diffusion MR imaging and localized proton MR spectroscopic findings in two infants.

    In this report, we describe the findings of diffusion MR imaging and proton MR spectroscopy in two infants with acute necrotizing encephalopathy in which there was characteristic symmetrical involvement of the thalami. diffusion MR images of the lesions showed that the observed apparent diffusion coefficient (ADC) decrease was more prominent in the first patient, who had more severe brain damage and a poorer clinical outcome, than in the second. Proton MR spectroscopy detected an increase in the glutamate/glutamine complex and mobile lipids in the first case but only a small increase of lactate in the second. diffusion MR imaging and proton MR spectroscopy may provide useful information not only for diagnosis but also for estimating the severity and clinical outcome of acute necrotizing encephalopathy.
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ranking = 736.51426242402
keywords = acute necrotizing, necrotizing
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7/21. Autosomal dominant acute necrotizing encephalopathy.

    OBJECTIVE: To define the clinical and biochemical abnormalities of an autosomal dominant form of acute encephalopathy. methods: The clinical details of 11 affected family members in comparison with 63 unaffected relatives were analyzed. RESULTS: Affected children become comatose after onset of a febrile illness. Outcomes include full recovery, permanent neurologic impairment, and death. Recurrences produce more severe impairments. Lesions of necrotizing encephalopathy of the thalamus and brainstem are present on autopsy and MRI. oxidative phosphorylation of intact mitochondria from a muscle biopsy shows loose coupling. Unaffected family members, including obligate carriers, share no clinical characteristics, demonstrating incomplete penetrance. CONCLUSIONS: Characteristic pathology and MRI findings define this disorder of autosomal dominant acute encephalopathy. Leigh syndrome and sporadic acute necrotizing encephalopathy share similarities but are distinct.
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ranking = 1832.28565606
keywords = acute necrotizing, necrotizing
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8/21. Acute necrotizing encephalopathy of childhood associated with influenza type B virus infection in a 3-year-old girl.

    Acute necrotizing encephalopathy of childhood represents a novel entity of acute encephalophathy, predominantly affecting infants and young children living in taiwan and japan. It manifests with symptoms of coma, convulsions, and hyperpyrexia after 2 to 4 days of respiratory tract infections in previously healthy children. The hallmark of acute necrotizing encephalopathy of childhood consists of multifocal and symmetric brain lesions affecting the bilateral thalami, brainstem tegmentum, cerebral periventricular white matter, or cerebellar medulla. The etiology and pathogenesis of this kind of acute encephalopathy remain unknown, and there is no specific therapy or prevention. The prognosis is usually poor, and less than 10% of patients recover completely. We report a 3-year-old previously healthy girl presenting with acute necrotizing encephalopathy of childhood associated with influenza type B virus infection, which resulted in severe neurologic sequelae. We also review the current knowledge of the clinical, neuroimaging, and pathologic aspects of acute necrotizing encephalopathy of childhood.
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ranking = 1103.771393636
keywords = acute necrotizing, necrotizing
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9/21. diffusion-weighted MR imaging findings of acute necrotizing encephalopathy.

    Multiple, symmetrical brain lesions affecting the bilateral thalami and cerebral white matter, which often show a concentric structure on CT and MR images, characterize acute necrotizing encephalopathy (ANE) of childhood. We describe the imaging findings of a 2-year-old child with ANE obtained with diffusion-weighted MR imaging. We discuss the significance of these findings, as well as the pathophysiology of ANE lesions, with reference to the appearance of the disease as revealed by diffusion-weighted MR imaging.
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ranking = 1831.28565606
keywords = acute necrotizing, necrotizing
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10/21. Acute fatal necrotizing hemorrhagic encephalitis caused by Epstein-Barr virus in a young adult immunocompetent man.

    Epstein-Barr Virus (EBV) encephalitis is a rare (<1%) and generally self-limited disease with few sequelae. This neurological complication has been reported almost exclusively in the course of acute primary infection and in paediatric patients. We describe a case of a young adult immunocompetent man who developed an acute fatal necrotizing haemorrhagic encephalitis as the only manifestation of an acute EBV infection. EBV-dna was tested positive in several CSF samples by qualitative and quantitative PCR. Serological profile showed: absence of IgM against Viral capsid Antigen (VCA) in three different consecutive samples, presence of IgG against VCA and IgG seroconversion for Epstein Barr Nuclear Antigen (EBNA). EBV-dna was detected by qualitative PCR in autoptic brain material. Clinical course was not influenced by antiviral therapy with acyclovir. In conclusion to our knowledge, this is the only case of acute necrotizing haemorrhagic EBV encephalitis with a fatal outcome, in an adult immunocompetent man.
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ranking = 371.25713121201
keywords = acute necrotizing, necrotizing
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