Evolución del síndrome de Lennox-Gastaut en la edad adulta

Rodríguez-Rodríguez, Samuel; Salas-Puig, J; Jc, Alvarez-Carriles; Temprano-Fernandez, T; C, Antón-González; García-Martínez, A.N.

doi:10.33588/rn.5205.2010580

Cited by 4 publications

(25 citation statements)

References 19 publications

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“…In relation to seizure type, Rodríguez et al observed that 100% of cases suffered tonic seizures and atypical absences; drop-attacks were also very common, what was not found in our study. Nine (75%) presented generalized tonic-clonic seizures; 5 (41.7%), while myoclonic and focal seizures were seen just in one patient [18]. Ferlazzo et al (2010) found a similar frequency of seizures as our research [16].…”

Section: Seizure Types At Recruiting Pointsupporting

confidence: 77%

“…In Ferlazzo´s patients, the evolution of EEG findings was characterized by the normalization of background activity in 44% as well as a clear reduction of epileptiform discharges during wakefulness in 74% over the years [16]. Those results are in disagreement with Rodríguez´s findings, who observed a slow and disturbed activity with frequent intermixed generalized slow spike-wave discharges, as it is seen in childhood [18].…”

Section: Electrographic Patternmentioning

confidence: 66%

“…Data collected in literature report that approximately 20% may have a preceding history of West syndrome. In Rodríguez´s series it occurred in 3/12 cases (25%) [18]. Nevertheless, it is remarkable that although in trisomy 21 infants, West syndrome is the epilepsy most frequently found, in any patient reported by Ferlazzo with late-onset LGS in Down syndrome, had this antecedent [17].…”

Section: West Syndrome Past Historymentioning

confidence: 72%

“…Yagi found that 12 out of 102 patients with LGS worked normally and 36 had part-time or sheltered jobs [22]. In Ferlazzo´s series, 26/27 patients at the end of follow-up had moderate to severe cognitive impairment and in Rodríguez´s study, 11/12 had a severe mental retardation [16,18].…”

Section: Neurodevelopment Impairmentmentioning

confidence: 90%

“…Ferlazzo et al has also developed a multicentric research about electroclinical characteristics of 27 adult patients with this syndrome, who were studied from 1960 to 1976 [16]. This same author reported LGS in some older cases with Down syndrome [17] and more recently, has been published a Spanish study of 12 cases about the evolution of LGS in adult ages [18].…”

Section: Introductionmentioning

confidence: 99%

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Electroclinical Features of Lennox-Gastaut Syndrome in Adulthood and Adolescence

2011

Neurol Neurophysiol

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Introduction: Lennox-Gastaut Syndrome (LGS) is characterized by seizures which may have inconspicuous semiological features so they may be unrecognized while patients are continuously deteriorating. To confirm its clinical and electrographic characteristics is mandatory, which has therapeutic and prognostic implications. Those features have not been completely elucidated in LGS in adulthood. Purpose: We performed a descriptive study to investigate seizure types, interictal and ictal EEG characteristics and cognitive outcome in adult LGS subjects. Methods: We evaluated 28 cases with development impairment and several refractory seizure types, which included tonic seizures, in order to make a screening of LGS. We confirm LGS diagnosis in 24 patients older than 12 years who were assessed by video-EEG, particularly to record seizure types and EEG findings as well as cognitive outcome. Results: During this stage of the disease, all patients presented tonic seizures (TS) during wakefulness and sleep, 12/24 had atypical absences, more rarely other seizure types. Some cases (5/24) have evolved from West syndrome and in 62.5% aetiology was cryptogenic. Interictal EEG showed normal background activity in 13/24 patients, slow spike-wave discharges during wakefulness was diffuse just in 4/24; bursts of diffuse fast rhythms (DFR) in sleep were seen in all patients. A moderate to severe cognitive impairment was observed in 18/24 patients, but all of them experience deterioration after the epilepsy had begun. Conclusions: In adult LGS patients a standard waking EEG may be normal. TS during sleep and the presence of DFR are paramount to confirm the diagnosis.

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Section: Seizure Types At Recruiting Pointsupporting

confidence: 77%

Section: Electrographic Patternmentioning

confidence: 66%

Section: West Syndrome Past Historymentioning

confidence: 72%

Section: Neurodevelopment Impairmentmentioning

confidence: 90%

Section: Introductionmentioning

confidence: 99%

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Electroclinical Features of Lennox-Gastaut Syndrome in Adulthood and Adolescence

2011

Neurol Neurophysiol

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Late-Onset Lennox-Gastaut Syndrome with Chromosome 15q Duplication in Sisters

Honorato

Santos

Silva

et al. 2022

JNRP

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Our study reports two nontwin sisters with late-onset Lennox-Gastaut syndrome and chromosome 15q duplication, showing the evolution, symptoms, diagnosis, and treatment of these patients, with the aim of increasing knowledge about this extremely rare association. They had a variety of generalized seizures types, intellectual disability, electroencephalogram with generalized epileptiform discharges less than 3 Hz, dysmorphisms, and genetic studies with the presence of duplicated chromosome 15. Cases reported here may be related to chromosomal changes inherited from their asymptomatic mother.

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A comprehensive systematic literature review of the burden of illness of Lennox–Gastaut syndrome on patients, caregivers, and society

Cross,

Benítez,

Roth

et al. 2024

Epilepsia

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Fully elucidating the burden that Lennox–Gastaut syndrome (LGS) places on individuals with the disease and their caregivers is critical to improving outcomes and quality of life (QoL). This systematic literature review evaluated the global burden of illness of LGS, including clinical symptom burden, care requirements, QoL, comorbidities, caregiver burden, economic burden, and treatment burden (PROSPERO ID: CRD42022317413). MEDLINE, Embase, and the Cochrane Library were searched for articles that met predetermined criteria. After screening 1442 deduplicated articles and supplementary manual searches, 113 articles were included for review. A high clinical symptom burden of LGS was identified, with high seizure frequency and nonseizure symptoms (including developmental delay and intellectual disability) leading to low QoL and substantial care requirements for individuals with LGS, with the latter including daily function assistance for mobility, eating, and toileting. Multiple comorbidities were identified, with intellectual disorders having the highest prevalence. Although based on few studies, a high caregiver burden was also identified, which was associated with physical problems (including fatigue and sleep disturbances), social isolation, poor mental health, and financial difficulties. Most economic analyses focused on the high direct costs of LGS, which arose predominantly from medically treated seizure events, inpatient costs, and medication requirements. Pharmacoresistance was common, and many individuals required polytherapy and treatment changes over time. Few studies focused on the humanistic burden. Quality concerns were noted for sample representativeness, disease and outcome measures, and reporting clarity. In summary, a high burden of LGS on individuals, caregivers, and health care systems was identified, which may be alleviated by reducing the clinical symptom burden. These findings highlight the need for a greater understanding of and better definitions for the broad spectrum of LGS symptoms and development of treatments to alleviate nonseizure symptoms.

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Evolución del síndrome de Lennox-Gastaut en la edad adulta

Cited by 4 publications

References 19 publications

Electroclinical Features of Lennox-Gastaut Syndrome in Adulthood and Adolescence

Electroclinical Features of Lennox-Gastaut Syndrome in Adulthood and Adolescence

Late-Onset Lennox-Gastaut Syndrome with Chromosome 15q Duplication in Sisters

A comprehensive systematic literature review of the burden of illness of Lennox–Gastaut syndrome on patients, caregivers, and society

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