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A case of haemophagocytic syndrome presenting with oculogyric crises
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Resumo(s)
Haemophagocytic lymphohistiocytosis
(HLH), also called haemophagocytic syndrome (HPS), is a rare disorder resulting
in abnormal proliferation of histiocytes in
tissues and organs, including the CNS. HLH
can present as a primary disease or occur as
a secondary reactive disease. Clinical
features are high fever, splenomegaly, cytopenia
of two or more cell lines, hypertriglyceridaemia
and haemophagocytosis.
CNS involvement varies between 10% and
73%, and clinical manifestations include
seizures, decreased sensorium, brainstem
symptoms, ataxia or demyelinating peripheral
neuropathy.
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Contexto Educativo
Citação
J Neurol Neurosurg Psychiatry 2010 81: 469-471