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Cerebromeningeal Haemophagocytic Lymphohistiocytosis
Lancet 239:104-107, Henter,J.&Elinder,G., 1992
See this aricle in Pubmed

Article Abstract
We describe 3 children with a progressive encephalopathy that was characterised by irritability,convulsions,cranial nerve palsies,ataxia, nystagmus,walking difficulties,delayed psychomotor development,hemiplegia/ tetraplegia,visual disturbance,vomiting,neck stiffness,and non-specific signs of raised intracranial pressure.A final diagnosis was made in all 3 patients from necropsy material.The clinical features were ascribed to multiple inflammatory,predominately lymphocytic,reactions and raised intracranial pressure.This condition is an atypical form of haemophagocytic lymphohistiocytosis,which normally presents with fever, hepatosplenomegaly,and cytopenias.By contrast,the disease pattern in our 3 children was dominated by cerebromeningeal involvement,which can precede the typical systemic symptoms of haemophogocytic lymphohistiocytosis.An awareness of this condition is important because treatments are available.
 
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anemia
ataxia
cerebral edema
cranial nerve palsies
encephalopathy
familial
fever
gait disorder
hemiplegia
hemophagocytic lymphohistiocytosis,cerebromeningeal
hemophagocytosis
hepatomegaly
hepatosplenomegaly
hypertriglyceridemia
hypofibrinogenemia
immunologic disease
intracranial pressure,increased
irritability
lymphadenopathy
meningismus
nausea and vomiting
neuropathology
neuropathy
neutropenia
nystagmus
opisthotonus
pancytopenia
papilledema
pleocytosis of cerebrospinal fluid
psychomotor retardation
quadriplegia
seizure
splenomegaly
steroid therapy,CNS treatment and complications with
thrombocytopenia
treatment of neurologic disorder
walking,difficulty with

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