Abstract | OBJECTIVE: DESIGN: Retrospective study on presentation and outcome of children and adolescents with the clinico-radiological syndrome of limbic encephalitis tested for specific neuronal autoantibodies (Abs) over 3.5 years. SETTING: Assessment, diagnosis, treatment and follow-up at 12 neuropaediatric and neurological departments in Europe, with Abs determined in Bonn, Germany and Oxford, UK. PATIENTS: Ten patients <18 years of age who presented with a disorder mainly affecting the limbic areas of <5 years' duration with MRI evidence of mediotemporal encephalitis (hyperintense T2/FLAIR signal, resolving over time). RESULTS: CONCLUSIONS:
Limbic encephalitis is a disease that can occur in childhood or adolescence with many of the hallmarks of the adult disorder, suggesting that both result from similar pathogenic processes. Since most of the cases were non-paraneoplastic, as now also recognised in adults, more systematic and aggressive immunotherapies should be evaluated in order to improve outcomes.
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Authors | E Haberlandt, T Bast, A Ebner, H Holthausen, G Kluger, R Kravljanac, J Kröll-Seger, G Kurlemann, C Makowski, K Rostasy, E Tuschen-Hofstätter, G Weber, A Vincent, C G Bien |
Journal | Archives of disease in childhood
(Arch Dis Child)
Vol. 96
Issue 2
Pg. 186-91
(Feb 2011)
ISSN: 1468-2044 [Electronic] England |
PMID | 20959359
(Publication Type: Journal Article)
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Chemical References |
- Autoantibodies
- Glucocorticoids
- Immunoglobulins, Intravenous
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Topics |
- Adolescent
- Autoantibodies
(blood)
- Brain
(pathology)
- Child
- Child, Preschool
- Female
- Follow-Up Studies
- Glucocorticoids
(therapeutic use)
- Humans
- Immunoglobulins, Intravenous
(therapeutic use)
- Limbic Encephalitis
(diagnosis, drug therapy, immunology)
- Magnetic Resonance Imaging
- Male
- Neuroblastoma
(diagnosis, drug therapy, immunology)
- Neurons
(immunology)
- Paraneoplastic Syndromes
(diagnosis, drug therapy, immunology)
- Prognosis
- Retrospective Studies
- Treatment Outcome
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