LAUSR

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 10mgwas given intravenously up to 60mg 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 continuous, but up to ten episodes could occur within 1 h, varying in duration from 5 to 45 s, regardless of external factors but certainly exacerbated by stimuli such as aspiration, nursing care or positional changes. On day 20, a tracheostomy was inserted, and then, an attempt was made to release the patient from sedation and mechanical ventilation that was unsuccessful. On day 22, due to the increased incidence and duration of seizures, his anticonvulsive treatment was further increased to IV levetiracetam 1500 mg BD, IV sodium valproate 800 mg BD and zonisamide 150 mg BD via PEG (Table 1). Sedation was increased, whilst fentanyl was discontinued to avoid any epileptogenic action. Clobazam 10 mg TDS was added via PEG whilst on extended spasms. The above combination of antiepileptic drugs failed to control seizures that continued at a frequency of up to 60 episodes per day, and thus, phenobarbital 30 mg TDS was added via PEG following loading with 600 mg IV. Phenobarbital was increased in the following days up to 90 mg TDS whilst monitoring daily blood levels. The patient continued the same epileptic activity, which deteriorated in the course of developing a respiratory infection and fever * Panayiotis Maghsoudlou [email protected]