Acute fulminant subacute sclerosing panencephalitis with absent measles and PCR studies in cerebrospinal fluid
ABSTRACT This report describes an atypical case of rapidly progressive subacute sclerosing panencephalitis presenting as transient visual agnosia and myoclonus in a 14-year-old male. There were no typical periodic complexes in serial electroencephalographic monitoring; cerebrospinal fluid measles antibody titer was negative. The diagnosis was made by molecular and histologic examination of open brain biopsy tissue.
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ABSTRACT: Subacute sclerosing panencephalitis is a progressive neurological disorder of children and young adults caused by a measles virus that became defective by persisting in the host. According to the results of clinical trials, antiviral and/or immunomodulatory therapy can slow the progression of the disease and improve life expectancy in patients. However, its long-term effects and eventual outcome remain debatable due to conflicting results and its lack of effect on the rapidly progressive form of the disease. Possible future therapies for subacute sclerosing panencephalitis are RNAi and antiapoptotic agents, which are currently in the hypothetical and experimental stages of research.Expert Review of Neurotherapeutics 04/2012; 12(4):485-92. DOI:10.1586/ern.12.21 · 2.83 Impact Factor
05/2013; 24(2). DOI:10.1007/s00062-013-0218-x
Article: Subacute sclerosing panencephalitis[Show abstract] [Hide abstract]
ABSTRACT: A 19-year-old female patient presented in an acute state of akinetic mutism. Serological analysis of serum and cerebrospinal fluid demonstrated the presence of antibodies to measles virus. CT scan carried out during this acute phase of relapse demonstrated white matter enhancement affecting the cortical white matter of the frontal lobes and corpus callosum. These features indicate that active demyelination occurs during acute relapse in subacute sclerosing panencephalitis (SSPE) and suggest that immunotherapy should be considered during this acute phase.Neuroradiology 10/1989; 31(5):433-434. DOI:10.1007/BF00343870 · 2.37 Impact Factor