[Extrinsic bilateral ophthalmoplegia during Strümpell-Liechtenstein hemorrhagic encephalitis].
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Two cases that fulfil the clinical and neuropathological criteria of acute hemorrhagic encephalitis are described. Histological examination revealed additionally focal changes in the white matter characteristic for neuroaxonal dystrophy. The differences in the clinical course and morphological picture observed in both cases are discussed.
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A selective non-occlusive technique was developed for administration of BCNU (1,3-bis(2-chloroethyl)-1-nitrosourea) into the internal carotid artery of the dog, and the neuropathological effects in the brain were studied. One out of three dogs showed ipsilateral hemorrhagic necrotizing encephalitis at doses of 102 mg/sq m, and all of three dogs showed similar but more severe pathology at doses of 215 to 232 mg/sq m. This study and previous studies in the dog and monkey suggest definite thresholds above which cerebral toxicity occurs when BCNU is administered via the intracarotid route. Greater dilution of drug in the larger territory of supply of the human internal carotid artery allows somewhat higher doses in man. The pathology of the lesion induced by BCNU suggests a primary vascular injury as a pathogenic mechanism, consonant with similar findings following high-dose systemic BCNU administration in man. Investigators conducting ongoing and future trials of intracarotid BCNU in the human for the treatment of intracranial neoplasms should be especially vigilant for a similar toxic effect.
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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.
Clinical and electroencephalographic evolution, as well as the neuropathology, are described in a 25 year old man with a Hurst's acute haemorrhagic leucoencephalitis. Lesions, unusually symmetrical, coexists with those, without vascular necrosis nor haemorrhages, of the perivenous encephalomyelitis. The aspecific onset, course and results of complementary investigations appear on reviewing 44 clinical observations in the literature. Histologic examination of affected white matter is the only was to assess the diagnosis. Two features that may have been previously neglected are outlined: the predilection for 20 to 40 years old males, while the adolescent male, the most susceptible to perivenous encephalomyelitis after specific fevers, is not affected by Hurst's disease. Multiple sclerosis or a prodromal meningo-encephalitis, clinically undetected, could be not rare. Therapeutic results of surgical decompression, hyperosmolar substances and massive doses of corticosteroids (3 survivals out of 10 cases diagnosed on biopsy) cannto be evaluated at the present time. By analogy with the simple and the hyperacute forms of EAE, the myelinotoxicity may result from sensitized lymphocytes alone in the perivenous encephalomyelitis, from an association of circulating antibodies and lymphocytes in Hurst's disease. Whether the immunoglobulins enchance the cellular myelinotoxicity or induce an immune complex disease upon a perivenous encephalomyelitis is still to be determined.
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