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. 2016 Feb 11:5:38-43.
doi: 10.1016/j.ebcr.2016.01.004. eCollection 2016.

Challenges in identifying Lennox-Gastaut syndrome in adults: A case series illustrating its changing nature

Jesus Eric Piña-Garza  1 , Steve Chung  2 , Georgia D Montouris  3 , Rodney A Radtke  4 , Trevor Resnick  5 , Robert T Wechsler  6
Affiliations

Challenges in identifying Lennox-Gastaut syndrome in adults: A case series illustrating its changing nature

Jesus Eric Piña-Garza et al. Epilepsy Behav Case Rep. .

Abstract

The variable presentation and progression of Lennox-Gastaut syndrome (LGS) can make it difficult to recognize, particularly in adults. To improve diagnosis, a retrospective chart review was conducted on patients who were diagnosed as adults and/or were followed for several years after diagnosis. We present 5 cases that illustrate changes in LGS features over time. Cases 1 and 2 were diagnosed by age 8 with intractable seizures, developmental delay, and abnormal EEGs with 1.5-2 Hz SSW discharges. However, seizure type and frequency changed over time for both patients, and the incidence of SSW discharges decreased. Cases 3, 4, and 5 were diagnosed with LGS as adults based on current and past features and symptoms, including treatment-resistant seizures, cognitive and motor impairment, and abnormal EEG findings. While incomplete, their records indicate that an earlier LGS diagnosis may have been missed or lost to history. These cases demonstrate the need to thoroughly and continuously evaluate all aspects of a patient's encephalopathy, bearing in mind the potential for LGS features to change over time.

Keywords: Adult; Diagnosis; EEG, electroencephalogram; Features; LGS; LGS, Lennox–Gastaut syndrome; Lennox–Gastaut syndrome; SSW, slow spike–wave discharge; SW, spike–wave discharge; Symptoms.

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Figures

Fig. 1
Fig. 1
Panel A. Case 1, age 8. EEG at LGS diagnosis, showing generalized 1.5–2 Hz SSW discharges with frontal predominance, intermittent spike–waves predominating in the left occipital region, and poorly formed posterior rhythm. Panel B. Case 1, age 12. EEG 4 years after diagnosis, showing low-amplitude background activity with infrequent low-amplitude generalized discharges and infrequent multifocal sharp waves.
Fig. 2
Fig. 2
Panel A. Case 3, age 19. EEG at presentation and diagnosis, showing high-amplitude slowing with irregular high-amplitude generalized SW discharges. Panel B. Case 3, age 12. EEG 7 years before diagnosis, showing generalized ≤ 1 Hz SSW discharges. Panel C. Case 3, age 25. EEG 6 years after diagnosis, showing bursts of high-amplitude background slowing.
Fig. 3
Fig. 3
Case 5, age 32. EEG at presentation and diagnosis, showing irregular posterior dominant 4–7 Hz rhythm and bursts of atypical 2–3 Hz SW complexes.
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