Although it is relatively rare for children with CDKL5 disorder to experience status epilepticus, it can happen by the very nature of the severe seizure disorder which is generally the most prominent symptom of the disorder. However, non-convulsive status is more common amongst this population of children. The following article has been written as a guideline.
Status epilepticus is defined as a seizure lasting for thirty minutes or longer, or multiple seizures spaced so close together within thirty minutes that the patient does not recover between them. Status epilepticus – regardless of its cause – is associated with increased morbidity and mortality in all age and remains the most common neurological medical emergency in the UK.
At the onset of a seizure, it is first important to ensure that the patient is not in a potentially harmful position, and assess their airway, breathing and circulation. Help should be called for immediately – whether it is an ambulance or medical staff, depending on where the seizures begin. As soon as the proper equipment is available, the patient’s blood glucose concentration should be checked and high flow oxygen administered.
As is outlined in Figure 1, if intravenous (IV) access has not yet been established, then 0.5 mg/kg of buccal midazolam should be given. IV access should be gained rapidly such that 0.1 mg/kg of lorazepam can be administered if the convulsions persist for ten minutes following the first line oral treatment. On the occasion that IV access cannot be obtained, a second dose of buccal midazolam can be given or, alternatively, a rectal dose of paraldehyde could be administered. The patient should be carefully monitored after the first dose of lorazepam and, if their seizures continue for ten minutes, a second dose of lorazepam should be given. If the patient still does not recover, they are said to have entered the second phase of status epilepticus – established status epilepticus. This phase of the condition is first treated by setting up an IV infusion of 20 mg/kg of phenytoin to be administered over 20 minutes. As an adjuvant to phenytoin, a 0.8ml/kg mixture of paraldehyde can also be infused at the discretion of senior medical staff. Alternatively, 20 mg/kg of phenobarbital can be infused over five minutes. It is at this point that the staff of the intensive care unit should be notified of the impending transfer of the patient for intensive treatment; the patient is now entering refractory status epilepticus.
Figure 1: A flow chart outlining the treatment of status epilepticus in children.
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Authors: James Higham, Dr Sam Amin August 2015 Review August 2016