Abstract
We describe a 19-year-old woman with hemophagocytic syndrome and adult Still's disease who showed rare features of central neurological involvement, including cerebellar symptoms and the sudden onset of unconsciousness with pleocytosis in the cerebrospinal fluid during the early course of the illness. As this patient's serum showed a high level of interferon-gamma and soluble interleukin 2 receptor, this might play a pathologic role in the development of central nervous system symptoms. Intensive treatment consisting of methylprednisolone pulse therapy followed by the oral administration of methylprednisolone and cyclosporine, as well as plasma exchange, was found to achieve good results.
MeSH terms
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Adult
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Anti-Inflammatory Agents / administration & dosage
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Cyclosporine / administration & dosage
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Female
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Histiocytosis, Non-Langerhans-Cell / etiology*
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Histiocytosis, Non-Langerhans-Cell / therapy
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Humans
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Immunosuppressive Agents / administration & dosage
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Influenza A virus / immunology*
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Influenza, Human / complications*
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Influenza, Human / therapy
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Meningoencephalitis / complications*
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Meningoencephalitis / drug therapy
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Meningoencephalitis / virology
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Methylprednisolone / administration & dosage
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Plasma Exchange
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Still's Disease, Adult-Onset / complications*
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Still's Disease, Adult-Onset / therapy
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Treatment Outcome
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Unconsciousness / etiology*
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Unconsciousness / therapy
Substances
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Anti-Inflammatory Agents
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Immunosuppressive Agents
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Cyclosporine
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Methylprednisolone