Diacomit

Diacomit should only be administered under the supervision of a paediatrician / paediatric neurologist experienced in the diagnosis and management of epilepsy in infants and children. Posology Paediatric population The dose of stiripentol is calculated on a mg/kg body weight basis.

The daily dosage may be administered in 2 or 3 divided doses. The initiation of adjunctive therapy with stiripentol should be undertaken gradually using upwards dose escalation to reach the recommended dose of 50 mg/kg/day administered in conjunction with clobazam and valproate.

Stiripentol dosage escalation should be gradual, starting with 20mg/kg/day for 1 week, then 30mg/kg/day for 1 week. Further dosage escalation is age dependent: - children less than 6 years should receive an additional 20 mg/kg/day in the third week, thus achieving the recommended dose of 50 mg/kg/day in three weeks; - children from 6 to less than 12 years should receive an additional 10 mg/kg/day each week, thus achieving the recommended dose of 50 mg/kg/day in four weeks; - children and adolescents 12 years and older should receive an additional 5 mg/kg/day each week until the optimum dose is reached based on clinical judgment.

1). g. exposure to gastric acid in an empty stomach). ), carbonated drinks, fruit juice or food and drinks that contain caffeine or theophylline. Children aged less than 3 years The pivotal clinical evaluation of stiripentol was in children of 3 years of age and over with SMEI.

The clinical decision for use of stiripentol in children with SMEI less than 3 years of age needs to be made on an individual patient basis taking into consideration the potential clinical benefits and risks. 1). Data are limited about the use of stiripentol under 12 months of age.

For these children the use of stiripentol will be done under the close supervision of the doctor. Patients aged ≥ 18 years of age Long-term data has not been collected in a sufficient number of adults to confirm maintenance of effect in this population.

Treatment should be continued for as long as efficacy is observed. Dose adjustments of other antiepileptics used in combination with stiripentol Despite the absence of comprehensive pharmacology data on potential drug interactions, the following advice regarding modification of the dose and dosage schedules of other anti-epileptic medicinal products administered in conjunction with stiripentol is provided based on clinical experience.

Summary of the safety profile The most common side effects with stiripentol are anorexia, weight loss, insomnia, drowsiness, ataxia, hypotonia and dystonia. Tabulated list of adverse reactions Adverse reactions encountered most often are as follows: very common (≥ 1/10), common (≥ 1/100 to < 1/10), uncommon (≥ 1/1,000 to < 1/100), rare (≥ 1/10,000 to < 1/1,000), very rare (< 1/10,000), not known (cannot be estimated from the available data).

Within each frequency grouping, undesirable effects are presented in order of decreasing severity. System Organ Class (MedDRA terminology) Very common Common Uncommon Rare Not known Blood and lymphatic system disorders Neutropenia Thrombocytopenia * Metabolism and nutrition disorders Anorexia, loss of appetite, weight loss 8 Psychiatric disorders Insomnia Aggressiveness, irritability, behaviour disorders, opposing behaviour, hyperexcitability, sleep disorders Nervous system disorders Drowsiness, ataxia, hypotonia, dystonia Hyperkinesias Eye disorders Diplopia Gastrointestinal disorders Nausea, vomiting Skin and subcutaneous tissue disorders Photosensitivity, rash, cutaneous allergy, urticaria General disorders and administration site conditions Fatigue Investigations Raised γ-GT Liver function test abnormal Infections and infestations Pneumonia, aspiration pneumonia * Thrombocytopenia data are derived from both clinical trials and post-marketing experience.

5) and may regress when the dose of these medicinal products is reduced. Reporting of suspected adverse reactions Reporting suspected adverse reactions after authorisation of the medicinal product is important. It allows continued monitoring of the benefit/risk balance of the medicinal product.

Healthcare professionals are asked to report any suspected adverse reactions via the national reporting system listed in Appendix V.

Carbamazepine, phenytoin and phenobarbital These substances should not be used in conjunction with stiripentol in the management of Dravet’s syndrome. 2). Growth rate of children Given the frequency of gastrointestinal adverse reactions to treatment with stiripentol and valproate (anorexia, loss of appetite, nausea, vomiting), the growth rate of children under this combination of treatment should be carefully monitored.

Blood count Neutropenia may be associated with the administration of stiripentol, clobazam and valproate. Blood counts should be assessed prior to starting treatment with stiripentol. Unless otherwise clinically indicated, blood counts should be checked every 6 months.

Liver function It should be assessed prior to starting treatment with stiripentol. Unless otherwise clinically indicated, liver function should be checked every 6 months. 2). 5). In vitro studies suggested that stiripentol phase 1 metabolism is catalyzed by CYP1A2, CYP2C19 and CYP3A4 and possibly other enzymes.

Caution is advised when combining stiripentol with other substances that inhibit or induce one or more of these enzymes. Paediatric population The pivotal clinical studies did not include children below 3 years old. As a consequence, it is recommended that children between 6 months and 3 years of age are carefully monitored whilst on stiripentol therapy.

This medicine contains less than 1 mmol sodium (23 mg) per capsule, that is to say essentially ‘sodium-free’. 5

1. A past history of psychoses in the form of episodes of delirium.