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October 2018; 8 (5) Research

Late-onset Lennox-Gastaut syndrome

Diagnostic evaluation and outcome

Kelsey M. Smith, Jeffrey W. Britton, Gregory D. Cascino
First published September 28, 2018, DOI: https://doi.org/10.1212/CPJ.0000000000000527
Kelsey M. Smith
Department of Neurology, Mayo Clinic, Rochester, MN.
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Jeffrey W. Britton
Department of Neurology, Mayo Clinic, Rochester, MN.
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Gregory D. Cascino
Department of Neurology, Mayo Clinic, Rochester, MN.
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Citation
Late-onset Lennox-Gastaut syndrome
Kelsey M. Smith, Jeffrey W. Britton, Gregory D. Cascino
Neurol Clin Pract Oct 2018, 8 (5) 397-402; DOI: 10.1212/CPJ.0000000000000527

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Abstract

Background We describe the clinical features and outcome in patients with late-onset Lennox-Gastaut syndrome (LGS).

Methods Adult patients evaluated between January 1, 2000, and March 1, 2017, who presented with onset of LGS ≥10 years were identified. Data abstracted included age at seizure onset, seizure types, etiology, treatments, EEG and neuroimaging results, cerebrospinal fluid (CSF) findings, and autoimmune evaluation.

Results Ten patients (8 females) were identified. The mean age at onset of seizures consistent with LGS was 16.5 years (range, 10–32 years). Seizure types included tonic, atonic, and tonic-clonic seizures (all), myoclonic seizures (n = 3), and atypical absence seizures (n = 7). Five patients had normal intellectual function at onset. Prolonged video-EEG monitoring recorded seizures and generalized interictal epileptiform discharges in all. All patients had drug-resistant epilepsy (range of antiseizure drugs tried, 7–16). Two patients had a history of intrathecal methotrexate to treat acute lymphoblastic leukemia. Two patients had malformations of cortical development. CSF analysis (n = 5) showed a mild elevation in the protein level without other abnormalities. Autoantibody determinations in the serum (n = 4) or the CSF (n = 5) and genetic testing (n = 5) were negative. At final follow-up, all but 1 patient was disabled and required a caregiver, and none were driving. One patient died of probable sudden unexpected death in epilepsy (SUDEP).

Conclusions Late-onset LGS represents a rare, treatment-resistant generalized epilepsy that is disabling and may be associated with progressive cognitive impairment. The elevated CSF protein level in our cohort could have been due to high seizure burden but increases the possibility of an inflammatory component to the pathophysiology of this disorder.

Footnotes

  • Funding information and disclosures are provided at the end of the article. Full disclosure form information provided by the authors is available with the full text of this article at Neurology.org/cp.

  • Editorial, page 375

  • Received May 23, 2018.
  • Accepted July 16, 2018.
  • © 2018 American Academy of Neurology
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