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Epilepsy in neurofibromatosis type 1.

Publication ,  Journal Article
Pecoraro, A; Arehart, E; Gallentine, W; Radtke, R; Smith, E; Pizoli, C; Kansagra, S; Abdelnour, E; McLendon, R; Mikati, MA
Published in: Epilepsy Behav
August 2017

OBJECTIVES: To describe the characteristics of epilepsy in patients with Neurofibromatosis type 1 (NF1). METHODS: Analysis of a cohort of consecutive NF1 patients seen in our NF1 clinic during a three-year period. RESULTS: Of the 184 NF1 patients seen during that period, 26 had epilepsy and three had febrile seizures. Of the 26, 17 (65%) had localization-related epilepsy, seven of whom (41%) were drug resistant. Six (23%) had apparently primary generalized epilepsy (0/6 drug resistant), two (8%) Lennox-Gastaut syndrome, and one (4%) West syndrome (all three were drug-resistant). As compared to the patients with no epilepsy, those with epilepsy were more likely to have MRI findings of mesial temporal sclerosis (MTS) (23% vs. 5%, p=0.0064), and cerebral hemisphere tumors (31% vs. 10%, p=0.0079), but not of the other MRI findings including neurofibromatosis bright objects, or optic gliomas. Three of the six patients with MTS underwent temporal lobectomy with subsequent control of their seizures with confirmation of MTS on pathology in 3/3 and presence of coexisting focal cortical dysplasia (FCD) in 2/3. We also have observed three additional patients outside the above study with the association of NF1, MTS, and intractable epilepsy. SIGNIFICANCE: Epilepsy is relatively common in NF1, often occurs in patients with brain tumors or with MTS which can coexist with FCD, can be associated with multiple types of epilepsy syndromes, and when localization-related is often drug-resistant. Patients with NF1 and MTS can respond to medial temporal lobectomy and may have coexisting medial temporal lobe cortical dysplasia.

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Published In

Epilepsy Behav

DOI

EISSN

1525-5069

Publication Date

August 2017

Volume

73

Start / End Page

137 / 141

Location

United States

Related Subject Headings

  • Young Adult
  • Psychosurgery
  • Neurology & Neurosurgery
  • Neurofibromatosis 1
  • Malformations of Cortical Development
  • Male
  • Magnetic Resonance Imaging
  • Humans
  • Hemispherectomy
  • Female
 

Citation

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ICMJE
MLA
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Pecoraro, A., Arehart, E., Gallentine, W., Radtke, R., Smith, E., Pizoli, C., … Mikati, M. A. (2017). Epilepsy in neurofibromatosis type 1. Epilepsy Behav, 73, 137–141. https://doi.org/10.1016/j.yebeh.2017.05.011
Pecoraro, Anthony, Eric Arehart, William Gallentine, Rodney Radtke, Edward Smith, Carolyn Pizoli, Sujay Kansagra, Elie Abdelnour, Roger McLendon, and Mohamad A. Mikati. “Epilepsy in neurofibromatosis type 1.Epilepsy Behav 73 (August 2017): 137–41. https://doi.org/10.1016/j.yebeh.2017.05.011.
Pecoraro A, Arehart E, Gallentine W, Radtke R, Smith E, Pizoli C, et al. Epilepsy in neurofibromatosis type 1. Epilepsy Behav. 2017 Aug;73:137–41.
Pecoraro, Anthony, et al. “Epilepsy in neurofibromatosis type 1.Epilepsy Behav, vol. 73, Aug. 2017, pp. 137–41. Pubmed, doi:10.1016/j.yebeh.2017.05.011.
Pecoraro A, Arehart E, Gallentine W, Radtke R, Smith E, Pizoli C, Kansagra S, Abdelnour E, McLendon R, Mikati MA. Epilepsy in neurofibromatosis type 1. Epilepsy Behav. 2017 Aug;73:137–141.
Journal cover image

Published In

Epilepsy Behav

DOI

EISSN

1525-5069

Publication Date

August 2017

Volume

73

Start / End Page

137 / 141

Location

United States

Related Subject Headings

  • Young Adult
  • Psychosurgery
  • Neurology & Neurosurgery
  • Neurofibromatosis 1
  • Malformations of Cortical Development
  • Male
  • Magnetic Resonance Imaging
  • Humans
  • Hemispherectomy
  • Female