Clinical and Encephalographic Changes at Lennox–gastaut Syndrome

Мухин, К. Ю.

doi:10.17650/2073-8803-2015-10-2-19-31

Rus. ž. det. nevrol.

2015

DOI: 10.17650/2073-8803-2015-10-2-19-31

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Clinical and Encephalographic Changes at Lennox–gastaut Syndrome

К. Ю. Мухин¹

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Cited by 3 publications

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Diagnostics and age-related evolution of Lennox–Gastaut syndrome. Management in diverse patient age periods

Belousova

Бурд

Ермоленко

et al. 2022

Èpilepsiâ paroksizmalʹnye sostoâ.

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Lennox–Gastaut syndrome is an epileptic encephalopathy with onset in childhood. The classical triad of diagnostic criteria is well known: 1) presence of various types of refractory epileptic seizures (tonic, atypical absences, myoclonic, tonic/atonic drop attacks, generalized tonic-clonic, focal); 2) cognitive disorders with frequent behavioral disorders (not always evident by the beginning of epileptic seizures); 3) generalized, slow (≤2.5 Hz) spike-wave activity of wakefulness and generalized paroxysmal fast activity on sleep electroencephalogram. Despite the seizure onset usually occurring before the age of 8 (peak at 3–5) years old, the Lennox–Gastaut syndrome is often featured with a lifelong course. Many patients with this syndrome suffer from refractory epilepsy in adulthood, however, not always being provided a proper syndromological diagnosis. Expanding the criteria to diagnose the Lennox–Gastaut syndrome discussed here would allow to choose a proper treatment algorithm. Rufinamide is the drug of the second choice in the adjunctive therapy of epileptic seizures associated with Lennox–Gastaut syndrome. However, a pediatric-to-adult clinic transition of patients with Lennox–Gastaut syndrome may pose some obstacles. Herein, an effective patient management requires not only seizure control, but also improvement of patient's quality of life by influencing cognitive and behavioral issues, sleep disorders, disability (both physical and social), educational problems and employment.

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Diagnostics and age-related evolution of Lennox–Gastaut syndrome. Management in diverse patient age periods

Belousova

Бурд

Ермоленко

et al. 2022

Èpilepsiâ paroksizmalʹnye sostoâ.

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Differential diagnosis of Drave's syndrome and Gasto's Lenox syndrome on the example of a clinical case

2017

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Independent Occurrence of Two Cases of Lennox — Gastaut Syndrome and Neurofibromatosis in the Family: A Clinical Observation

Rakhmanina,

Levitina,

Khramova

et al. 2024

Урал. мед. журнал

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Developmental and epileptic encephalopathies (DEE) are the most difficult problem in epileptology. Lennox — Gastaut syndrome (LGS) is a developmental and epileptic encephalopathy with onset in childhood, manifested by: frequent polymorphic seizures, including tonic axial ones; severe cognitive impairment; slow activity (with a frequency of <2.5–3.0 Hz) sharp-slow wave in the interictal period on the electroencephalogram, as well as runs of fast activity with a frequency of 10–20 Hz, often associated with tonic seizures; and resistance to therapy. Presumably genetic or forms with an unknown cause account for 20–30 % of cases of LGS, but a family history of epilepsy among patients with LGS is observed in only 2.5 %.The purpose of this work was to describe a familial case of LGS in two siblings.Materials and methods. A retrospective description of the medical history of 3 siblings born in a consanguineous marriage was performed.Results and discussion. There was a similarity in the course of the disease in the two brothers, absolute resistance to drug antiepileptic therapy and temporary effectiveness of hormonal therapy for LGS with a decrease in the duration of the effect from course to course. In addition, the rare parallel existence in this family of another autosomal dominant disease — neurofibromatosis type 1 — is shown.Conclusion. A detailed description of LGS within one family will not only expand doctors’ knowledge of this disease and the difficulties of its treatment, but also improve understanding of the genetic mechanisms of the development of DEE.

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Clinical and Encephalographic Changes at Lennox–gastaut Syndrome

Cited by 3 publications

References 22 publications

Diagnostics and age-related evolution of Lennox–Gastaut syndrome. Management in diverse patient age periods

Diagnostics and age-related evolution of Lennox–Gastaut syndrome. Management in diverse patient age periods

Differential diagnosis of Drave's syndrome and Gasto's Lenox syndrome on the example of a clinical case

Independent Occurrence of Two Cases of Lennox — Gastaut Syndrome and Neurofibromatosis in the Family: A Clinical Observation

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