Taipa, R.Moreira, B.França, M.Maia, L.2011-07-042011-07-042010J Neurol Neurosurg Psychiatry 2010 81: 469-4710022-3050http://hdl.handle.net/10400.16/717Haemophagocytic 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.engjournal article