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J Neurol Neurosurg Psychiatry 2008;79:547-552 doi:10.1136/jnnp.2007.118216
  • Research paper

Electroclinical and imaging findings in ulegyria and epilepsy: a study on 25 patients

  1. G Kuchukhidze1,
  2. I Unterberger1,
  3. J Dobesberger1,
  4. N Embacher1,
  5. G Walser1,
  6. E Haberlandt2,
  7. T Gotwald3,
  8. H Maier4,
  9. M Ortler5,
  10. S Felber3,
  11. G Bauer1,
  12. E Trinka1
  1. 1
    Department of Neurology, Innsbruck Medical University, Innsbruck, Austria
  2. 2
    Department of Paediatrics IV, Division of Neuropaediatrics, Innsbruck Medical University, Innsbruck, Austria
  3. 3
    Department of Radiology II, Innsbruck Medical University, Innsbruck, Austria
  4. 4
    Department of Neuropathology, Innsbruck Medical University, Innsbruck, Austria
  5. 5
    Department of Neurosurgery, Innsbruck Medical University, Innsbruck, Austria
  1. E Trinka, Department of Neurology, Innsbruck Medical University, Anichstrasse 35, A-6020 Innsbruck, Austria; Eugen.Trinka{at}uki.at
  • Received 12 February 2007
  • Revised 20 July 2007
  • Accepted 22 July 2007
  • Published Online First 6 August 2007

Abstract

Purpose: Ulegyria refers to cerebral cortex scarring, which results from a perinatal ischaemic brain injury. It presents with a characteristic gyral pattern: small circumvolutions with atrophy at sulci bottom and spared apex. Ulegyria is frequently associated with epilepsy, cerebral palsy and mental disability. We analysed electroclinical and MRI features in patients with ulegyria and epilepsy.

Patients and methods: We reviewed 25 patients (14 males/11 females) with ulegyria and epilepsy from the database (about 5000 patients with epilepsy) of our unit. Patients were examined clinically, underwent high resolution MRI, EEG recordings, positron emission tomography, single photon emission computed tomography and neuropsychological testing. Two patients with refractory seizures underwent epilepsy surgery.

Results: Mean age of patients was 34 years (5–66) at the reassessment time. The majority (16/25, 64%) had a history of perinatal asphyxia. 15 patients had delayed developmental milestones; 20 had learning disabilities and five patients were severely disabled. Mean age at seizure onset was 4.2 years (1–18). 17 patients (68%) had medically intractable epilepsy. 11 patients (44%) had occipital lobe seizures. The majority (n = 24, 96%) had parieto-occipital lesions on MRI. In 13 patients (52%), ulegyria was bilateral. 12 patients (48%) had hippocampal sclerosis. Two patients underwent epilepsy surgery with an excellent postoperative outcome (Engel class IA and IC).

Conclusion: Patients with ulegyria often have a history of perinatal asphyxia and present with pharmacoresistant seizures. Their presurgical assessment is complicated because of frequent dual pathology (hippocampal sclerosis) and bilateral lesions.

Footnotes

  • Competing interests: None.

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