Document Detail

Cerebromeningeal haemophagocytic lymphohistiocytosis.
MedLine Citation:
PMID:  1345827     Owner:  NLM     Status:  MEDLINE    
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, predominantly 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 haemophagocytic lymphohistiocytosis. An awareness of this condition is important because treatments are available.
J I Henter; G Elinder
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Publication Detail:
Type:  Case Reports; Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Lancet     Volume:  339     ISSN:  0140-6736     ISO Abbreviation:  Lancet     Publication Date:  1992 Jan 
Date Detail:
Created Date:  1992-02-03     Completed Date:  1992-02-03     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  2985213R     Medline TA:  Lancet     Country:  ENGLAND    
Other Details:
Languages:  eng     Pagination:  104-7     Citation Subset:  AIM; IM    
Department of Paediatrics, Karolinska Institute, St Göran's Children's Hospital, Stockholm, Sweden.
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MeSH Terms
Brain Diseases / etiology,  pathology*,  therapy
Child, Preschool
Combined Modality Therapy
Histiocytosis, Non-Langerhans-Cell / complications,  pathology*,  therapy
Meningoencephalitis / etiology,  pathology*,  therapy
Comment In:
Lancet. 1992 Feb 1;339(8788):312   [PMID:  1346323 ]

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