Document Detail


Progressive facial hemiatrophy and epilepsy: a common underlying dysgenetic mechanism.
MedLine Citation:
PMID:  9109892     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Progressive facial hemiatrophy (PFH), a rare disorder characterized by progressive and self-limited atrophy of the skin and the subcutaneous tissues, is often associated with epilepsy but the link between these two conditions is poorly understood. The cause of PFH remains unclear. We report four patients with PFH associated with partial epilepsy in whom brain MRI showed cerebral dysgenesis. The four patients (two men, two women; age range: 24 to 73 years) developed parasagittal PFH in their second decade. Seizures started before the age of 20 years in three patients and were refractory simple, or complex partial seizures. All the patients had focal MRI showing cortical dysgenesis, ipsilateral to PFH, consisting of cortex thickening, gyral effacement, and blurring of the white-gray interface. The underlying white matter was hyperintense on T2-weighted sequences, with nodular areas in two patients. These areas were stable over time, without contrast enhancement, and were consistent with the MRI characteristics of cystic encephalomalacia. These neuroradiologic features suggest a localized cerebral hemispheric defect of congenital origin. Because cells participating in the formation of the fronto-nasal bud derive from common progenitors with the cells that give rise to the cerebral hemisphere, we suggest that an early malformative process affecting one side of the rostral neural tube could underlie both cerebral dysgenesis and facial hemiatrophy.
Authors:
S Dupont; M Catala; D Hasboun; F Semah; M Baulac
Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Neurology     Volume:  48     ISSN:  0028-3878     ISO Abbreviation:  Neurology     Publication Date:  1997 Apr 
Date Detail:
Created Date:  1997-05-15     Completed Date:  1997-05-15     Revised Date:  2005-11-17    
Medline Journal Info:
Nlm Unique ID:  0401060     Medline TA:  Neurology     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  1013-8     Citation Subset:  AIM; IM    
Affiliation:
Unité d'Epileptologie Clinique Neurologique Paul Castaigne, Hôpital de la Pitié-Salpêtrière, Paris, France.
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MeSH Terms
Descriptor/Qualifier:
Adult
Aged
Animals
Brain / abnormalities,  pathology
Cricetinae
Disease Progression
Epilepsy / complications*,  diagnosis
Facial Hemiatrophy / complications*,  pathology
Female
Humans
Magnetic Resonance Imaging
Male
Middle Aged

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