Focal cortical dysplasia. Clinical-radiological-pathological associations.

نویسندگان

  • I Pascual-Castroviejo
  • J L Hernández-Moneo
  • M L Gutiérrez-Molina
  • J Viaño
  • S I Pascual-Pascual
  • R Velazquez-Fragua
  • C Morales
  • D Quiñones
چکیده

INTRODUCTION The term focal cortical dysplasia (FCD) describes a particular migration disorder with a symptomatology mainly characterised by drug-resistant epileptic seizures, typical neuroradiological images, and histological characteristics, as well as a very positive response to surgical treatment in the majority of cases. MATERIAL AND METHODS A total of 7 patients were studied, comprising 6 children with a mean age of 34.3 months and one 25-year-old male with very persistent focal seizures and MRI images that showed FCD. RESULTS Three of the patients (all girls) were operated on while very young, with extirpation of the FCD and the surrounding area; with the histopathology study showed agreement between the MRI images and the macroscopic study of the slices. The histology study showed findings typical of a Taylor-type FCD (poor differentiation between the cortical grey matter and the subcortical white matter, and balloon cells). Three years after the FCD extirpation, the same 3 patients remained seizure-free with no anti-epilepsy medication. Two others have seizure control with medication, another (the adult) is on the surgical waiting list, and the remaining patient refused the operation. CONCLUSION Taylor-type FCD is associated with a high percentage of all drug-resistant focal seizures, and it needs to be identified and extirpated as soon as possible. Well planned and well-performed surgery that leaves no remains of dysplasia can cure the disease it in many cases.

برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

ثبت نام

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

منابع مشابه

Towards in vivo focal cortical dysplasia phenotyping using quantitative MRI

Focal cortical dysplasias (FCDs) are a range of malformations of cortical development each with specific histopathological features. Conventional radiological assessment of standard structural MRI is useful for the localization of lesions but is unable to accurately predict the histopathological features. Quantitative MRI offers the possibility to probe tissue biophysical properties in vivo and...

متن کامل

Gangliocytoma associated with focal cortical dysplasia in a young-adult: a case report.

Neoplasms and (non-neoplastic) focal dysplasias may coexist as a cause of seizures in both the developing and mature brain. Low grade neoplastic lesions (ganglioglioma/gangliocytoma) may present with seizures, and distinction of these lesions from focal cortial dysplasia is difficult on standard radiological imaging. We report a 24-year-old man who had complaints of tonic-clonic seizures for on...

متن کامل

PI3K/AKT pathway mutations cause a spectrum of brain malformations from megalencephaly to focal cortical dysplasia.

Malformations of cortical development containing dysplastic neuronal and glial elements, including hemimegalencephaly and focal cortical dysplasia, are common causes of intractable paediatric epilepsy. In this study we performed multiplex targeted sequencing of 10 genes in the PI3K/AKT pathway on brain tissue from 33 children who underwent surgical resection of dysplastic cortex for the treatme...

متن کامل

Surgical Treatment of Intractable Epilepsy Associated with Focal Cortical Dysplasia

Focal cortical dysplasias (FCDs), initially thought to be rare, are a common cause of drugrefractory epilepsy in both children and adults. Successful resection and subsequent characterization of FCDs was first described by Taylor et al. in 1971 from pathological specimens obtained in patients treated for intractable temporal lobe epilepsy (Tassi, et al.,2001; Becker, et al.,2002; Tassi, et al.,...

متن کامل

Automatic Delineation of Focal Cortical Dysplasia Lesions on MRI

Rationale: MRI has allowed the recognition of focal cortical dysplasia (FCD) in an increasing number of patients. The precise delineation of FCD is important since freedom from seizures after surgery is closely related to the extent of resection of the pathological tissue. FCD lesions are difficult to delineate on MRI due to their ill-defined boundaries and the complexity of the cortex. We prev...

متن کامل

ذخیره در منابع من


  با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید

برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

ثبت نام

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

عنوان ژورنال:
  • Neurologia

دوره 27 8  شماره 

صفحات  -

تاریخ انتشار 2012