Successful Treatment of Super-Refractory Status Epilepticus With Cannabis Oil in a Patient With Neuronal Ceroid Lipofuscinosis

Athena Michaelides, Spyridon Bekas, Antigoni Papaioannou, Athina Lazaridou, Paola Nicolaides

Abstract


Herein we describe a case of a 25-year-old man diagnosed with the “variant late infantile-onset” neuronal ceroid lipofuscinosis (NCL) who has a novel mutation in the CLN6 gene located on chromosome 15. The patient developed seizures at the age of 10 years along with progressive symptoms of ataxia, spasticity, cognitive decline and visual difficulties. At the age of 25 years, the seizures were drug-resistant, and the patient was put in an anesthesia-induced coma until the use of cannabis oil in combination with Food and Drug Administration (FDA)-approved anti-seizure drugs proved to be lifesaving. Treatment for NCL currently consists of symptomatic relief of seizures, and supportive therapies for associated symptoms of behavior, language and visual difficulties. Although limited, recent data on the efficacy of cannabinoids for treatment-resistant epilepsy have been published. Since cannabis oil is not currently FDA approved for most formulations, its use in drug-resistant epilepsy is anecdotal and remains highly controversial.




J Neurol Res. 2020;10(6):245-247
doi: https://doi.org/10.14740/jnr626

Keywords


Epilepsy; Drug-resistant seizure; Neuronal ceroid lipofuscinosis; CLN6

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