DI-096 Refractory lance–adams syndrome: pharmacotherapy management and iatrogenic complications

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Abstract

Background

Lance–Adams syndrome (LAS) is a chronic post-hypoxic myoclonus that may appear after a period of cerebral hypoxia. Many different antiepileptic drugs (AED) have been used for the symptomatic control of LAS. In the absence of response to classic AED, it is necessary to consider new off-label therapeutic options which may cause unpredictable adverse events.

Purpose

To present the pharmacotherapy approach to a case of refractory LAS, describing treatment related adverse events and its management.

Material and methods

We describe the case of a 35-year-old active male smoker with antecedents of three consecutive cardiac arrests that led to the development of LAS. The clinical information was collected from the electronic medical records (Cerner Millennium). A literature review was conducted looking for evidence of the use of perampanel, 5-hydroxytryptophan (5-HT) and sodium oxybate in LAS. Adverse events information was obtained from the drugs’ EPAR.

Results

At admission, the patient was treated with levetiracetam, sodium valproate and then sedated with propofol and sodium thiopental. As myoclonus was not controlled, piracetam, zonisamide (to reduce the use of sedative drugs) and clonidine were added to the previous treatment, without obtaining improvement. Sodium oxybate was added, but it was discontinued early due to the risk of respiratory arrest. 5-HT also was added with no significant outcome and severe diarrhoea as an adverse event. Finally, perampanel (24 mg/day—maximum daily dose doubled) was added to the treatment, achieving myoclonus improvement. Simultaneously, the patient had behavioural disorders that were linked with perampanel treatment, needing addition of risperidone. Finally, LAS control was achieved and the patient was discharged with levetiracetam, gabapentin, perampanel and risperidone treatment.

Conclusion

The refractory nature of LAS forced the medical team to use off-label drugs and supratherapeutic doses, with increased frequency of adverse events. The drug related events were identified and properly managed, allowing treatment continuation and ensuring patient improvement.

References and/or acknowledgements

Suchitra Malhotra and Kumar Mohinder. Lance–Adams syndrome: Difficulties surrounding diagnosis, prognostication, and treatment after cardiac arrest. Anesth Essays Res2012;6:218–22.

References and/or acknowledgements

No conflict of interest

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