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Occipital lobe epilepsy secondary to ulegyria

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Abstract

Objective

To analyse clinical and therapeutic aspects of epilepsy secondary to ulegyria in adults.

Patients

Out of 1,020 consecutive patients studied at a tertiary care epilepsy centre, eight cases of ulegyria were identified. All patients had comprehensive clinical evaluation, neuropsychological testing, interictal EEG, and brain magnetic resonance imaging (MRI). In addition, five patients had video–EEG monitoring. Ulegyria was confirmed by histological analysis in two patients who had successful epilepsy surgery.

Results

All patients had a history of perinatal asphyxia. In four of them there was psychomotor developmental delay. Mean age at onset of seizures was 5.8 years (range first week to 21 years). Brain MRI demonstrated predominant involvement of occipito–parietal cortical and subcortical areas. This posterior distribution of lesions was also supported by the presence of auras with occipital and parietal semiology in six patients, and signs of visuospatial dysfunction in five. Four patients had medically refractory epilepsy and two of them had significant improvement with surgical treatment.

Conclusions

In this group of adult epileptic patients with ulegyria brain MRI, ictal semiology, and neurological examination are consistent with occipital lobe epilepsy. Most patients have severe epilepsy, but in some of them epilepsy can be controlled with antiepileptic drugs, while in others surgical treatment can be effective. Brain MRI criteria of ulegyria are well established, and in two cases it was possible to confirm their diagnosis with histological analysis.

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References

  1. Azzarelli B, Meade P, Muller J (1980) Hypoxic lesions in areas of primary myelination. A distinct pattern in cerebral palsy. Childs Brain 7:132–145

    PubMed  Google Scholar 

  2. Barkovich AJ (1995) Pediatric neuroimaging. 2nd edition. Raven, New York

  3. Barkovich AJ, Kuzniecky RI, Jackson GD, Guerrini R, Dobyns WB (2001) Classification system for malformations of cortical development: update 2001. Neurology 57:2168–2178

    PubMed  Google Scholar 

  4. Commission on classification and terminology of the International League Against Epilepsy (1981) Proposal for revised clinical and electroencephalographic classification of epileptic seizures. Epilepsia 22:489–501

    PubMed  Google Scholar 

  5. Coulson WF, Bray PF (1969) An association of phenylketonuria with ulegyria. Dis Nerv Syst 30:129–132

    PubMed  Google Scholar 

  6. Engel JR (2001) A proposed diagnostic scheme for people with epileptic seizures and with epilepsy: Report of the ILAE Task Force on Classification and terminology. Epilepsia 42:796–803

    Article  PubMed  Google Scholar 

  7. Guerrini R, Dubeau F, Dulac O, Barkovich AJ, Kuzniecky R, Fett C, Jones-Gotman, M, Canapicchi R, Cross H, Fish D, Bonanni P, Jambaque I, Andermann F (1997) Bilateral parasagittal parietooccipital polymicrogyria and epilepsy. Ann Neurol 4:65–73

    Article  Google Scholar 

  8. Kornfeld M, Woodfin BM, Papile L, Davis LE, Bernard LR (1985) Neuropathology of ornithine carbamyl transferase deficiency. Acta Neuropathol (Berl) 65:261–264

    Article  PubMed  Google Scholar 

  9. Kuchna I, Kozlowski PB (1991) Sequelae of perinatal central nervous system damage after long-term survival. Neuropatol Pol 29:103–108

    PubMed  Google Scholar 

  10. Lahl R, Villagran R Teixeira W (eds) (2003) Neuropathology of focal epilepsies: An Atlas. John Libbey, London, pp 150–151

    Google Scholar 

  11. Lee BC, Hatfield G, Park TS, Kaufman BA (1997) MR imaging surface display of the cerebral cortex in children. Pediatr Radiol 27:199–206

    Article  PubMed  Google Scholar 

  12. Lopez-Gonzalez FJ, Macias M, Castro- Borrajo A, Vazquez-Lema C, Pereiro I (1996) Ulegyria and epilepsy. Rev Neurol (Paris) 24:572–573

    PubMed  Google Scholar 

  13. Muller J (1983) Congenital malformations of the brain. In: Rosenberg RN, Schocher SS (eds) The Clinical Neurosciences; Neuropathology. Vol III. Churchill Livingstone, New York Edinburgh London Melbourne, pp 1–33

  14. Norman MG (1981) On the morphogenesis of ulegyria. Acta Neuropathol (Berlin):331–332

    Google Scholar 

  15. Norman MG, McGillivray BC, Kalousek DK, Hill A, and Poskitt KJ (1995) Perinatal hemorrhagic and hypoxic-ischemic lesions. In: Norman MG, McGillivray BC, Kalousek DK, Hill A, Poskitt KJ (eds) Congenital malformations of the brain. Oxford University Press New York, pp 419–423

  16. Olive M, Ferrer I, Arbizu T, Calopa M, Ferrer X, Peres J (1992) Polymicrogyria and ulegyria. Diagnosis by magnetic resonance. Neurología 7:117–119

    PubMed  Google Scholar 

  17. Palmini A, Andermann E, Andermann F (1994) Prenatal events and genetic factors in epileptic patients with neuronal migration disorders. Epilepsia 35:965–973

    PubMed  Google Scholar 

  18. Rubinstein M, Denays R, Ham HR, Piepsz A, VanPachterbeke T, Haumont D, Noel P (1989) Functional imaging of brain maturation in humans using iodine-123 iodoamphetamine and SPECT. J Nucl Med 30:1982–1985

    PubMed  Google Scholar 

  19. Salanova V, Andermann F, Olivier A, Rasmussen T, Quesney LF (1992) Occipital lobe epilepsy: electroclinical manifestation, electrocorticography, cortical stimulation and outcome in 42 patients treated between 1930 and 1991. Brain 115:1655–1680

    PubMed  Google Scholar 

  20. Sisodiya SM (2000) Surgery for malformations of cortical development causing epilepsy. Brain 123:1075–1091

    Article  PubMed  Google Scholar 

  21. Tokumaru AM, Barkovich AJ, O’uchi T, Matsuo T, Kusano S (1999) The evolution of cerebral blood flow in the developing brain: evaluation with iodine- 123 iodoamphetamine SPECT and correlation with MR imaging. AJNR Am J Neuroradiol 20:845–852

    PubMed  Google Scholar 

  22. Villani F, D’Incerti L, Granata T, Battaglia G, Vitali P, Chiapparini L, Avanzini G (2003) Epileptic and imaging findings in perinatal hypoxicischemic encephalopathy with ulegyria. Epilepsy Res 55:235–243

    Article  PubMed  Google Scholar 

  23. Volpe JJ (2001) Hypoxic-ischemic encephalopathy: Neuropathology and pathogenesis. In:Vope JJ (ed) Neurology of the Newborn, 4th ed. WB Saunders, Philadelphia Pennsylvania, pp 296–330

  24. Wieser HG, Bloome WT, Fish D, Goldensohn E, Hufnagel A, King D, Sperling MR, Lüders H (2001) ILAE Commission Report: Proposal for a new classification of outcome with respect to epileptic seizures following epilepsy surgery. Epilepsia 42:282–286

    Article  PubMed  Google Scholar 

  25. Wolf HK, Zentner J, Hufnagel A, Campos MG, Schramm J, Elger CE, Wiestler OD (1993) Surgical pathology of chronic epileptic seizure disorders: experience with 63 specimens from extratemporal corticectomies, lobectomies and functional hemispherectomies. Acta Neuropathol (Berl) 86:466–472

    Article  PubMed  Google Scholar 

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Authors and Affiliations

  1. Dept. of Neurology, Hospital Ruber Internacional, La Masó 38, Mirasierra, 28034, Madrid, Spain

    A. Gil–Nagel MD, I. García Morales MD, PhD, A. Jiménez Huete MD, PhD, A. del Barrio PhD & D. G. Muñoz MD, PhD

  2. Dept. of Diagnostic Imaging, Hospital Ruber Internacional, Madrid, Spain

    J. Alvarez Linera MD

  3. Dept. of Neurosurgery, Hospital Ruber Internacional, Madrid, Spain

    C. Ruiz Ocaña MD, PhD

Authors
  1. A. Gil–Nagel MD
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  2. I. García Morales MD, PhD
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  3. A. Jiménez Huete MD, PhD
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  4. J. Alvarez Linera MD
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  5. A. del Barrio PhD
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  6. C. Ruiz Ocaña MD, PhD
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  7. D. G. Muñoz MD, PhD
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Correspondence to A. Gil–Nagel MD.

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Gil–Nagel, A., García Morales, I., Jiménez Huete, A. et al. Occipital lobe epilepsy secondary to ulegyria. J Neurol 252, 1178–1185 (2005). https://doi.org/10.1007/s00415-005-0829-5

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  • DOI: https://doi.org/10.1007/s00415-005-0829-5

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