RESUMEN
Subacute sclerosing panencephalitis is an infrequent central nervous system viral disease and is a late manifestation of persistent infection by a mutant form of measles virus. Since it affects mainly children and teenagers, the diagnosis in older ages is difficult. Its main clinical symptoms are cognitive impairment, behavioral disturbances and myoclonia. We report two males, aged 21 and 22 years old, presenting with the disease with atypical manifestations. One had a catatonic syndrome and the other, amaurosis. The recognition of the different presentation forms of the disease, endemic in developing countries, allows an earlier diagnosis and a more efficient treatment, when available
Asunto(s)
Humanos , Masculino , Adulto , Panencefalitis Esclerosante Subaguda/etiología , Virus SSPE/patogenicidad , Panencefalitis Esclerosante Subaguda/diagnóstico , Panencefalitis Esclerosante Subaguda/tratamiento farmacológico , Virus SSPE/efectos de los fármacos , Inosina Pranobex/uso terapéutico , Mioclonía/etiología , Mioclonía/tratamiento farmacológico , Ácido Valproico/uso terapéutico , Espectroscopía de Resonancia MagnéticaRESUMEN
Central nervous system (CNS) lesions were studied from weanling hamsters inoculated with the HBS strain of subacute sclerosing panencephalitis (SSPE) virus. The animals showed clincial signs of acute encephalitis between 8 and 18 days post-inoculation (PI), but all survivors were clinically recovered by day 21 PI. Nevertheless, 13 of 14 hamster brains examined by light and electron microscopy between days 21 and 59 PI had chronic lesions which contained morphologic evidence of persistent viral infection. The lesions developed preferentially in the subependymal areas of the lateral and fourth ventricles and involved degeneration of ependyma with subsequent damage to adjacent parenchyma. All CNS cell types were involved in degeneration. Viral inclusions occurred in both CNS parenchymal cells and in inflammatory cells. Giant cells were particularly common. No budding virus was seen in chronically infected animals, a finding in accord with previous studies. Demyelination was a common constituent of most lesions. It occurred in the presence of inflammatory cells and macrophages, and in later lesions, some remyelination was seen. It is suggested that the damage to myelin is a secondary phenomenon and is not a cellular immune reaction. The possible reasons underlying the latent nature of the virus and the similarities between this condition, canine distemper encephalomyelitis and human SSPE are discussed. It is concluded that the experimental chronic disease is a valid model for the study of human SSPE and may have usefulness in the understanding of other chronic CNS conditions of man, e.g. multiple sclerosis. Additional Key Words: Latent infection; Paramyxovirus; Slow Viruses; Demyelination; Inmmunologic defects; Multiple Sclerosis.