[Infection-triggered familial or recurrent acute necrotizing encephalopathy]. / Encefalopatía aguda necrosante familiar o recurrente desencadenada por infecciones.

Acute necrotizing encephalopathy (ANE) presents in children after common viral infections. Most cases of ANE are non-familial and non-recurrent and have been mainly reported in Asian patients, although ANE affects children worldwide. Recently, missense mutations in the gene encoding the nuclear pore protein Ran Binding Protein 2 (RANBP2) have been found in several families with familial or recurrent cases of ANE. We describe a Spanish family with familial and recurrent ANE without mutations in RANBP2. Mutations in RANBP2 are not the sole susceptibility alleles for familial or recurrent ANE.

Use of therapeutic plasma exchange in the management of acute hemorrhagic leukoencephalitis: a case report and review of the literature.

BACKGROUND: Acute hemorrhagic leukoencephalitis (AHLE) is a rare, fatal, central nervous demyelinating disease characterized by a rapid fulminant clinical course. Successful management requires early diagnosis, aggressive management of cerebral edema, and immunosuppression. Therapeutic plasma exchange (TPE) is infrequently used and commences after initial management fails. CASE REPORT: A 31-year-old man presented with right arm weakness, whose symptoms rapidly progressed to hemiplegia and aphasia. The patient was initially managed with glucocorticosteroids. Decompressive craniotomy and brain biopsies were performed when his intracranial pressure increased. Brain biopsy findings were consistent with AHLE. Mycoplasma pneumonia immunoglobulin G and immunoglobulin M serologies revealed recent infection. Despite surgical and medical management, he decompensated on Day 11, and TPE was initiated. The patient received a total of 10 TPE treatments. On the fourth day of TPE treatment, he was extubated. Twenty-one days after TPE began, he was ambulating with near normal muscle strength and was discharged. Four months after initial presentation, the patient has normal strength and is working full-time. CONCLUSIONS: AHLE has a fulminant course requiring accurate and rapid diagnosis. Successful therapy requires aggressive management of intracranial pressure and immunosuppression. Two other reports of AHLE document successful management with TPE. Each of these patients survived with minimal neurologic impairments. Given the likely immune-mediated nature of this disease, combined treatment of steroids, surgery, and TPE may lead to shorter hospital stays and improved neurologic outcomes. Clinical studies are needed to further study the effect of TPE on neurologic outcome in AHLE.

Association of herpetic encephalitis with acute retinal necrosis syndrome.

Bilateral acute retinal necrosis syndrome developed shortly after a severe acute encephalitis in two previously healthy adults. The clinical and laboratory data suggested that herpes simplex virus was the cause of the encephalitis and acute retinal necrosis in these patients. To the authors' knowledge, this is the first report of an association of herpetic encephalitis with acute retinal necrosis in the literature.

[Pathomorphological characteristics of the experimental infection in animals infected with viruses of the rabies antigenic group]. / Patomorfologicheskie osobennosti éksperimental'noi infektsii u zhivotnykh, zarazhennykh virusami antigennoi gruppy beshenstva.

Comparison of pathomorphological lesions in the brains of white mice inoculated with viruses isolated in Africa from shrews, in America from bats, in Czechoslovakia from rodents (6 strains) and classified into the rabies group, with those observed in the same animal species infected with street rabies virus and virus of human acute encephalomyelitis (HAE) established the peculiar features of the reactive processes distinguishing these infections from each other. In contrast to the reactions after inoculation with street rabies virus and HAE, 24 hours after inoculation of white mice with Lagos bat, Ib An 27 377, 297 BF, 548 BF, 808 BF, 482 SG, 598 SG, and 638 SG strains hemorrhagic foci were observed; within 48 hours multiple perivascular cuffs were formed in the brain, and degeneration of the Ammon horn down to its disappearance and formation of multiple voids in the brain tissue were observed. Cytoplasmic inclusions in neurons of the cerebrum cortex and Ammon horn in the form of small paired formations with a pinpoint basophilic granule inside could be detected 24 hours postinfection. Later in the disease, the number of inclusions found diminished sharply.

[Anatomoclinical study of 29 cases of acute necrotizing encephalitis. Demonstration of herpes virus in 24 cases]. / Etude anatomo-clinique de 29 cas d'encéphalite nécrosante aiguë. Mise en évidence du virus herpétique dans 24 cas

The authors studied the cerebral lesions in 29 cases of acute necrotizing encephalitis. Electron microscopy showed the presence of typical herpes virus particles in 24 cases. In 16 of them the virus had been isolated from specimens of the brain taken by biopsy and in 8 other cases from autopsy specimens. In 3 acute cases the search for the virus was negative and it was not carried out in the two cases with prolonged evolution. Only 6 patients have survived. Some peculiar varieties have beeen observed, i.e. two adolescents had temporal and almost entirely unilateral lesions, and two aged females had severely hemorrhagic necrotic areas which could have been confounded with vascular lesions. In these two later cases typical particles were present. Finally, one case showed within the same nuclei at once typical herpes virus particles and other viral structures of vermicular form, the origin of which could not be precised.