Hemimegalencephaly and neurofibromatosis

Hemimegalencephaly, which previously has been associated with a poor clinical course characterized by intractable seizures and severe encephalopathy, was found without these conditions in two children with neurofibromatosis. These children showed relatively similar and favourable prognostic features...

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Bibliographic Details
Published inNeuropediatrics Vol. 21; no. 4; p. 179
Main Authors Cusmai, R, Curatolo, P, Mangano, S, Cheminal, R, Echenne, B
Format Journal Article
LanguageEnglish
Published Germany 01.11.1990
Subjects
Brain - abnormalities
Brain - pathology
Child, Preschool
Diseases in Twins - diagnosis
Diseases in Twins - genetics
Dominance, Cerebral - physiology
Electroencephalography
Follow-Up Studies
Humans
Infant
Magnetic Resonance Imaging
Male
Neurofibromatosis 1 - diagnosis
Neurofibromatosis 1 - genetics
Neurofibromatosis 1 - pathology
Neuroma, Acoustic - diagnosis
Neuroma, Acoustic - genetics
Neuroma, Acoustic - pathology
Tomography, X-Ray Computed
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Summary:Hemimegalencephaly, which previously has been associated with a poor clinical course characterized by intractable seizures and severe encephalopathy, was found without these conditions in two children with neurofibromatosis. These children showed relatively similar and favourable prognostic features: no presence of seizures before one month, seizures controlled or absent, no focal neurological signs and peculiar EEG findings. In our opinion the absence of heterotopias and hamartomas can be related with less severe outcome.
ISSN:0174-304X
DOI:10.1055/s-2008-1071490