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Neuropediatrics 1990; 21(4): 179-182
DOI: 10.1055/s-2008-1071490
Original article

© Georg Thieme Verlag KG Stuttgart · New York

Hemimegalencephaly and Neurofibromatosis

R.  Cusmai1 , P.  Curatolo1 , S.  Mangano2 , R.  Cheminal3 , B.  Echenne3
  • 1Institute of Child Neuropsychiatry, Rome University "La Sapienza", Via dei Sabelli 108, I-00185 Rome, Italy
  • 2Institute of Child Neuropsychiatry, Palermo University, Via Lancia di Brolo 10 bis, I-90145 Palermo, Italy
  • 3CHU Gui-de-Chauliac, F-34059 Montpellier Cedex, France
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Publication History

Publication Date:
19 March 2008 (online)

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Abstract

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.

Key words

Hemimegalencephaly - Neurofibromatosis - MRI - Prognosis