Successful treatment of cannabinoid administration against refractory epilepsy in Batten disease: a case report
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LETTER TO THE EDITOR
Successful treatment of cannabinoid administration against refractory epilepsy in Batten disease: a case report Fotini Georgiou 1 & Panayiotis Maghsoudlou 2 & Panagiotis Loukaidis 3 & D. Markoullaki 1 & Christiana Giorgi 1 & Ioanna Christodoulou 1 & Chrystalla Matsentidou 1 Received: 8 May 2020 / Accepted: 24 September 2020 # Fondazione Società Italiana di Neurologia 2020
Dear Editor, In recent years, the use of cannabinoids for therapeutic purposes has gained ground, particularly in CNS disorders [1]. We present a case with refractory epilepsy on the background of neuronal ceroid lipofuscinosis 6-syndrome (CLN6; Batten Disease) that responded effectively when conventional antiepileptic therapy and cannabinoid treatment were combined. A 25-year-old male patient, with a history of Batten Disease, was admitted to Limassol General Hospital for the treatment of multiple seizures. Before admission, he experienced 8–12 seizures daily, and an electroencephalogram (EEG) showed an increased cerebral seizure disposition, unable to discriminate between primary focal or generalised cerebral seizure. The patient weighed 40 kg at the time of admission to the hospital, with kyphoscoliosis, upper and lower extremity muscle atrophy, dependent on the care of his relatives who mobilised him on a wheelchair. He was haemodynamically stable, afebrile and presented with tonic and tonicclonic convulsions. A computerised tomography brain and laboratory tests were within normal limits. His anti-epileptic treatment administered via percutaneous endoscopy gastrostomy (PEG) included levetiracetam 1000 mg twice-daily (BD), sodium valproate 400 mg BD, lamotrigine 125 mg BD, zonisamide 100 mg in the morning (OM)/150 mg at night (ON) and 10 mg diazepam suppositories given as required (Table 1). Due to the large number of attacks on admission (up to 50 episodes per day), diazepam
* Panayiotis Maghsoudlou [email protected] 1
Department of Intensive Care, Limassol General Hospital, Limassol, Cyprus
2
University College London, London, UK
3
Department of Neurology, Limassol General Hospital, Limassol, Cyprus
10 mg was given intravenously up to 60 mg daily. On day 7 of hospital admission, with approximately 80 seizures per day (Fig. 1), he was transferred to the intensive care unit (ICU) where he was immediately intubated under general anaesthesia and sedated with propofol 1% 10 ml/h, midazolam 10 mg/ h and fentanyl 10 ml/h (0.1 mg/h). At the time, his antiepileptic treatment was modified to intravenous (IV) levetiracetam 1000 mg BD, sodium valproate 400 mg BD with the addition of IV phenytoin 150 mg three times a day (TDS), following a loading dose of 750 mg. Lamotrigine 125 mg BD and zonisamide 100 mg OM/150 mg ON were continued via PEG. Further up-titration of anti-epileptic treatment is shown in Table 1. Despite sedation and anti-epileptic treatment, the patient continued to experience focal, tonic, clonic, tonic-clonic and generalised convulsions with up to 60 episodes per day (Fig. 1). Seizures were not continu
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