HOW TO TREAT 33 contain details about the child ’ s emergency contacts , diagnosis , medications , seizure types and how to manage a seizure ( that may include administration of emergency medication , such as midazolam ).
ausdoc . com . au 12 JULY 2024
HOW TO TREAT 33 contain details about the child ’ s emergency contacts , diagnosis , medications , seizure types and how to manage a seizure ( that may include administration of emergency medication , such as midazolam ).
Individuals with epilepsy have twice the risk of being involved in an MVA compared with the general population ; however , taking ASM regularly and avoiding risk factors for provoking seizures can reduce this risk . 35 People with epilepsy may be eligible to hold a driver ’ s licence . The guidelines for assessing fitness to drive in individuals with epilepsy can be found on the Austroads website .
Medical management
ASM is the mainstay of epilepsy treatment . Starting a medication is an individualised decision made by a specialist in discussion with the patient and their family . Not all people with epilepsy require ASM . 36 ASM reduces the risk of future seizures but does not change the underlying disorder or longterm prognosis .
The choice of ASM is based on seizure type , epilepsy syndrome and comorbidities ( see tables 3 and 4 ). The general principles for the use of ASM appear in box 5 .
WHEN TO STOP MEDICATIONS In general , a trial of discontinuing ASM may be considered if a child has been seizure free for at least two years . 37 There are some circumstances where medications can be stopped sooner , for example , after epilepsy surgery , or where it can be predicted that lifelong medications are required , such as juvenile myoclonic epilepsy .
Unless there is a life-threatening side effect , never suddenly stop ASM as this may exacerbate the seizures and cause withdrawal symptoms . Reduce a single medication gradually , usually over 2-3 months .
CANNABIDIOL For the past two decades , the use of cannabidiol in paediatric epilepsy has captured the attention of the public through anecdotal reports in the mainstream media .
As a result , families often enquire about using cannabidiol ( CBD ) for their child ’ s epilepsy . Evidence demonstrates the efficacy of CBD in certain epilepsy syndromes ; however , there are high rates of side effects , including diarrhoea , weight loss , increased seizures , drowsiness and elevated 41 , 42 transaminases . CBD significantly increases blood levels of the active metabolite of clobazam and its risk of side effects . 40
Epidyolex ( a pure CBD preparation ) was listed on the PBS in 2021 for Dravet syndrome and in 2023 for LGS , if initiated by a neurologist . 43
KETOGENIC DIET The classic ketogenic diet is a high-fat , low-protein and low-carbohydrate diet that shifts metabolism to using fats as the primary fuel source . 44
The mechanism of action of the ketogenic diet ( KD ) is poorly understood , but it has demonstrated benefit in several epilepsy syndromes , including infantile spasms , and is the gold-standard treatment for the metabolic disease glucose transport protein 1 ( GLUT-1 ) deficiency syndrome . 37
The KD is an important alternative treatment in patients with medically refractory epilepsy who are not candidates for surgery .
Table 3 . Common anti-seizure medications used in children
Anti-seizure medication
In an orally fed child , compliance can be an issue because of the diet ’ s restrictive nature . There are variations to the diet , such as the modified Atkins diet , which allow more protein and may be more acceptable .
Contraindications include predominantly metabolic disorders , where the patient is unable to process fats , including fatty acid oxidation disorders . 42
Side effects include dehydration , hypoglycaemia , metabolic acidosis , renal stones and gastrointestinal symptoms . 44 It is essential the initiation and maintenance of the KD is supervised by a dietitian and paediatric neurologist .
Surgical management
Epilepsy surgery may offer a cure for children with epilepsy or be used to reduce seizure frequency or severity to improve quality of life .
Epilepsy surgery can be divided into resection ( removal of tissue ), laser interstitial thermal therapy ( LiTT , destruction of tissue ), disconnection ( interruption of spread of seizure ) or brain stimulation ( vagal nerve or deep brain stimulation ) — see figure 11 . 45
Sudden unexpected death in epilepsy
Sudden unexpected death in epilepsy describes the sudden death of a person with epilepsy without a known cause . It is the most common epilepsy-related cause of death , affecting about one in 5000 children . 46
Factors associated with an increased risk of sudden unexpected death in epilepsy ( SUDEP ) include
Side effects |
Precautions |
Maintenance dose |
|
|
mg / kg / day |
Carbamazepine Common : dizziness , ataxia , blurred vision , hyponatraemia
Significant : Stevens – Johnson syndrome ( SJS ), toxic epidermal necrolysis , multiorgan hypersensitivity syndrome
Clobazam Common : dizziness , hypersalivation , ataxia , slurred speech , respiratory depression , behavioural changes
Ethosuximide
Common : gastrointestinal symptoms and weight loss
Lamotrigine Common : visual disturbance , dizziness , ataxia , headache , insomnia
Significant : SJS frequent generalised tonic – clonic seizures , nocturnal generalised tonic – clonic seizures , medication non-adherence and associated intellectual disability . 47
Certain epilepsy syndromes , including Dravet syndrome , and epilepsies secondary to mutations in certain genes ( including SCN8A , SCN2A , DEPDC5 , KCNQ1 , KCNH2 and SCN5A ) are also associated with an increased risk of SUDEP . 48
It is integral to management to provide education to patients and their families about the importance of seizure control , medication adherence and avoidance of seizure triggers to reduce the risk of SUDEP .
Seizure monitors and alert devices
There is currently inadequate
Increased risk of severe skin reactions in people of Asian ancestry with the HLA-B * 1502 allele
May worsen absence and myoclonic seizures
Respiratory depression , sleep apnoea , ventilatory insufficiency and severe hepatic insufficiency are contraindications
Not suitable as monotherapy for seizure types other than absence seizures
Slow dose increases will reduce the risk of SJS
Treatment with valproate increases lamotrigine concentrations
Dosing interval
10-40 8-12 hourly 1g
0.5-1 8-12 hourly 60mg
10-40 12 hourly 1.5g
10-15 ( without valproate )
5 ( with valproate ),
Maximum daily dose
12 hourly 700mg ( without valproate )
Levetiracetam Common : behavioural and mood changes 20-40 12 hourly 3g
Oxcarbazepine Common : dizziness , tremor , ataxia , nystagmus , visual changes , hyponatraemia
Significant : SJS , toxic epidermal necrolysis , multi-organ hypersensitivity syndrome
Sodium valproate Common : increased appetite , weight gain , tremor , hair loss , hyperammonaemia , raised aminotransferases , thrombocytopenia
Significant : liver failure , pancreatitis , bone marrow suppression , reduced BMD
Topiramate Common : cognitive impairment , weight loss , reduced sweating
Source : Australian Medicines Handbook 38-40
Significant : kidney stones , metabolic acidosis , hyperthermia
Box 5 . General principles for the use of anti-seizure meds
• Start at a low dose , and increase slowly to help avoid / reduce side effects .
• Aim to control seizures at the lowest effective dose , ideally with one ASM .
• Monitor for side effects ; drowsiness and gastrointestinal symptoms are common for all medications if dose is increased rapidly .
• Give each ASM an adequate trial before changing / stopping medication .
• Slowly reduce ASM when stopping treatment .
May worsen absence and myoclonic seizures
Do not use if there is a history of hypersensitivity or skin reactions with carbamazepine
Monitor FBC , LFT , EUC and vitamin D every 6-12 months
Avoid use in confirmed or suspected mitochondrial disorders and in children aged under two because of the risk of hepatotoxicity
If used in females of child-bearing age , counsel regarding the need for contraception because of the risk of teratogenicity
Ensure adequate hydration to reduce the risk of kidney stones
Monitor creatinine and serum bicarbonate every 6-12 months
30-45 8-12 hourly 1.8g
10-30 12 hourly 2.5g
200mg ( with valproate )
3-6 12 hourly 500mg
Table 4 . Common anti-seizure medication choices for different seizure types in children
Seizure type
Focal
Generalised tonic – clonic
Absence
Myoclonic
Tonic or atonic
Source : NICE 2022 15
First-line ASM
Carbamazepine Lamotrigine Levetiracetam Oxcarbazepine Zonisamide
Lamotrigine Levetiracetam Sodium valproate
Ethosuximide Lamotrigine Levetiracetam Sodium valproate
Sodium valproate Levetiracetam
Sodium valproate Lamotrigine
Other ASMs that may be useful
Lacosamide Topiramate Brivaracetam Perampanel Pregabalin Sodium valproate Phenobarbital Phenytoin Vigabatrin
Clobazam Perampanel Topiramate Brivaracetam Lacosamide Phenobarbital Primidone Zonisamide
Brivaracetam Clobazam Clonazepam Phenobarbital Topiramate Zonisamide
Clobazam Rufinamide Topiramate
ASMs that may worsen seizures
Carbamazepine Gabapentin Oxcarbazepine Phenobarbital Phenytoin Pregabalin Vigabatrin
Carbamazepine Gabapentin Lamotrigine Oxcarbazepine Phenytoin Pregabalin Vigabatrin
Carbamazepine Gabapentin Oxcarbazepine Pregabalin Vigabatrin