An Italian consensus on the management of Lennox-Gastaut syndrome.
Seizure
; 101: 134-140, 2022 Oct.
Article
in En
| MEDLINE
| ID: mdl-35981474
ABSTRACT
PURPOSE:
Although international guidelines exist, the clinical heterogeneity of Lennox-Gastaut syndrome (LGS) and the increasing availability of new and repurposed drugs (e.g., fenfluramine and cannabidiol) requires a practical guide to patient management in the clinical context. We report the results of a consensus survey among 42 Italian experts in the diagnosis and treatment of LGS.METHODS:
The consensus procedure followed a modified Delphi approach. Statements were formulated, based on the most recent published evidence and the clinicians' personal experience, then discussed, and agreed upon by the experts through a two-round voting procedure. Approval of a statement was reached with an average score ≥7.RESULTS:
Thirteen statements dealing with three main topics (i.e., clinical diagnosis and prognosis, impact on the Quality of Life (QoL), and treatment strategies) were generated. Six statements achieved a level of agreement sufficient for approval on the first voting round. Following the discussion and a few consequent amendments, most of the statements increased their level of agreement and all 13 were approved.CONCLUSIONS:
Overall, the statements draw a slightly more benign picture of this rare and severe disease, highlighting the possibility of remission - albeit modest -, an apparent trend towards lower mortality, and the availability of several effective drugs, to which greater accessibility would be hoped for. Valproate remains a major therapeutic option in LGS patients although lamotrigine, rufinamide, topiramate, cannabidiol, and clobazam are popular therapeutic options in Italy, allowing for a tailor-made antiseizure therapy.Key words
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Main subject:
Cannabidiol
/
Lennox Gastaut Syndrome
Type of study:
Diagnostic_studies
/
Guideline
/
Qualitative_research
Aspects:
Patient_preference
Limits:
Humans
Language:
En
Journal:
Seizure
Journal subject:
NEUROLOGIA
Year:
2022
Document type:
Article
Affiliation country:
Italy