engleski

Two Cases of Subacute Sclerosing Panencephalitis in Croatia

Introduction: Subacute sclerosing panencephalitis (SSPE) is rare chronic progressive encephalitis caused by reactivation of the measles virus or an inappropriate immune response to the measles virus. Following the introduction of vaccination against measles in Croatia in 1968 the incidence of measles decreased rapidly. During the last epidemia in 1998 two children had measles, boy in age of 4 years and girl in age of 6 month followed by SSPE. Clinical details: 5-year-old girl presented with progressive change of personality and focal grand - mal seizures. 8-year-old boy presented with progressive memory loss, irritability, seizures, and involuntary muscle movement and myoclonic seizures. The diagnosis of SSPE was established in both cases by raised measles antibody titers in serum and CSF. Both children died in less then 6 months after the first neurological changes. The autopsies were performed. Neuropathological findings: The tissue samples yielded two pieces of brain tissue ranged 10 mm in diameter in both of the cases. Histologically there were perivascular infiltrations of lymphocytes, some plasma cells and macrophages. Neuronophagia was common and neurofibrillary degeneration was present occasionally. Red intranuclear inclusions bodies were present in both oligodendrocytes and neurons. Ultrastructurally, in both cases viral nucleocapsides were found. Genes of wild genotype D6 of measles virus were sequenced following RT-PCR amplification from brain tissue of both children. IFA were also positively stained in tissue samples of both brains. Conclusion: Two cases of SSPE in Croatia developed recently were slow-virus infection of the CNS associated with wild genotype D6 measles infection in 1998.

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