Childhood Epilepsies Flashcards
Childhood Epilepsies
Benign childhood epilepsy with centrotemporal spikes - Demographics
There is a 1.5 male predominance. Prevalence is around 15% in children aged 1–15 years with seizures. Incidence is 10–20 per 100;000 children aged 0–15 years.
Childhood Epilepsies
Benign childhood epilepsy with centrotemporal spikes - Age range of onset
Onset is from age 1 to 14 years; peak 8 or 9 years.
Childhood Epilepsies
Benign childhood epilepsy with centrotemporal spikes - Semiology
The cardinal features of rolandic seizures are infrequent; often single; focal seizures consisting of: unilateral facial sensorimotor symptoms (30% of patients) oropharyngolaryngeal manifestations (53%) speech arrest (40%) t hypersalivation (30%). Secondarily generalized tonic–clonic seizures (GTCSs) are reported in around half of children with rolandic seizures. focal motor status or hemiconvulsive status epilepticus is more likely to occur than secondarily generalized convulsive status epilepticus; which is exceptional. Status it may be induced by carbamazepine or lamotrigine. Febrile seizures are common (10–20%) before rolandic seizures (thus composing part of the spectrum of benign childhood seizure susceptibility syndrome).
Childhood Epilepsies
Benign childhood epilepsy with centrotemporal spikes - Etiology
This is a genetically determined syndrome; possibly with multiple genes leading to the phenotype.
Childhood Epilepsies
Benign childhood epilepsy with centrotemporal spikes - Genetic testing or metabolic screening
Normal
Childhood Epilepsies
Benign childhood epilepsy with centrotemporal spikes - Imaging
Normal
Childhood Epilepsies
Benign childhood epilepsy with centrotemporal spikes - Interictal EEG
Central temporal spikes (CTS) - usually maximal at C3–C4 or C5–C6. CTSs may be unilateral; but are more often bilateral. They are abundant; usually occur in clusters; amplify during stages I–IV; and can also be triggered by sensory stimulation of fingers and toes. These are extreme (giant) somatosensory evoked spikes (ESESs); which correspond to mid- or long-latency somatosensory evoked potentials with peaks at 35– 80 ms. Can be elicited by asking the child to tap fingers from both hands. The frequency of spikes is not associated with prognosis.
Childhood Epilepsies
Benign childhood epilepsy with centrotemporal spikes - Ictal EEG
Ipsilateral rolandic regions and consists of slow waves mixed with fast rhythms.
Childhood Epilepsies
Benign childhood epilepsy with centrotemporal spikes - Differential diagnosis
The diagnosis is usually pretty straightforward
Childhood Epilepsies
Benign childhood epilepsy with centrotemporal spikes - Prognosis
Invariably excellent
Childhood Epilepsies
Benign childhood epilepsy with centrotemporal spikes - Management
Children with rolandic seizures may not need AEDs; particularly if the seizures are infrequent; mild or nocturnal; or the onset is close to the natural age remission in this age limited disorder. Treatment is warranted in children with frequent seizures or generalized seizures. Carbamazepine is the preferred AED.
Childhood Epilepsies
Panayiotopoulos syndrome - Demographics
Even though the syndrome is also referred to as early onset benign childhood occipital epilepsy; it not is strictly a occipital epilepsy; since autonomic symptoms are the first manifestation; and EEG abnormalities involve many other brain regions Both genders are equally affected. 1/3 out of 1000 children are affected.
Childhood Epilepsies
Panayiotopoulos syndrome - Age range of onset
Onset is from age 1 to 14 years; 76% of cases start at 3–6 years of age (peak 4 or 5 years). Approximately 20% of children have a preceding history of the febrile seizures.
Childhood Epilepsies
Panayiotopoulos syndrome - Semiology
Seizures comprise autonomic symptoms (mainly emetic; but also include pallor or; less often; flushing or cyanosis; mydriasis or; less often; miosis; incontinence); behavioral changes; unilateral deviation of the eyes and other manifestations. Consciousness and speech; as a rule; are preserved at seizure onset. Later; ‘syncopal-like symptoms’ are common; and other ictal manifestations may also occur such as convulsions or hemiconvulsions. Typically; the seizures last a long time; with the mean duration time of nine minutes. Two thirds of the seizures happen at night during sleep.
Childhood Epilepsies
Panayiotopoulos syndrome - Etiology
Probably genetically determined. The pathophysiology Is related to diffuse and multifocal cortical hyper-excitability; and a epileptic electrical discharge (irrespective of localisation) activates; at its onset; susceptible autonomic centers to produce autonomic seizures.
Childhood Epilepsies
Panayiotopoulos syndrome - Genetic testing or metabolic screening
Normal
Childhood Epilepsies
Panayiotopoulos syndrome - Imaging
Normal
Childhood Epilepsies
Panayiotopoulos syndrome - Interictal EEG
Etiology
Childhood Epilepsies
Panayiotopoulos syndrome - Ictal EEG
The seizure discharge mainly consists of rhythmic theta or delta activity ; usually mixed with small spikes. Onset is unilateral; often posterior; but may also be anterior and not strictly localized to one electrode
Childhood Epilepsies
Panayiotopoulos syndrome - Differential diagnosis
Migraine and other causes of syncope.
Childhood Epilepsies
Panayiotopoulos syndrome - Prognosis
Typically excellent. The risk of epilepsy in adult life appears to be no higher than in the general population.
Childhood Epilepsies
Panayiotopoulos syndrome - Management
Prophylactic treatment with anti-epileptic medication may not be needed for most patients.
Childhood Epilepsies
Idiopathic childhood occipital epilepsy of Gastaut (ICOE-G) (previously known as childhood epilepsy with occipital paroxysms) - Demographics
Both sexes are equally affected. The disorder accounts for about 2–7% of benign childhood focal seizures. Less prevalent than PS.
Childhood Epilepsies
Idiopathic childhood occipital epilepsy of Gastaut (ICOE-G) (previously known as childhood epilepsy with occipital paroxysms) - Age range of onset
Onset is between 3 and 15 years of age with a mean of around 8.
Childhood Epilepsies
Idiopathic childhood occipital epilepsy of Gastaut (ICOE-G) (previously known as childhood epilepsy with occipital paroxysms) - Semiology
Seizures are purely occipital and primarily manifest with elementary visual hallucinations; blindness or both. They are usually frequent and diurnal; develop rapidly within seconds and are brief; lasting from a few seconds to 1–3 min; and; rarely; longer. Deviation of the eyes; often associated with ipsilateral turning of the head; is the most common (in about 70% of cases) non-visual ictal symptom. Post-ictal headache; mainly diffuse; but also severe; unilateral and pulsating; or indistinguishable from migraine headache; occurs in half the patients.
Childhood Epilepsies
Idiopathic childhood occipital epilepsy of Gastaut (ICOE-G) (previously known as childhood epilepsy with occipital paroxysms) - Etiology
ICOE-G is considered to be a late-onset phenotype of BCSSS. The seizures are of a purely occipital lobe origin.
Childhood Epilepsies
Idiopathic childhood occipital epilepsy of Gastaut (ICOE-G) (previously known as childhood epilepsy with occipital paroxysms) - Genetic testing or metabolic screening
Normal
Childhood Epilepsies
Idiopathic childhood occipital epilepsy of Gastaut (ICOE-G) (previously known as childhood epilepsy with occipital paroxysms) - Imaging
Normal
Childhood Epilepsies
Idiopathic childhood occipital epilepsy of Gastaut (ICOE-G) (previously known as childhood epilepsy with occipital paroxysms) - Interictal EEG
Occipital paroxysms; often demonstrating fixation-off sensitivity. Giants somatosensory potentials may occur. Spikes are more common during sleep. It is the datable if occipital photosensitivity is part of this syndrome.
Childhood Epilepsies
Idiopathic childhood occipital epilepsy of Gastaut (ICOE-G) (previously known as childhood epilepsy with occipital paroxysms) - Ictal EEG
Sudden appearance of an occipital discharge that consists of fast rhythms with lower amplitude then interictal discharges
Childhood Epilepsies
Idiopathic childhood occipital epilepsy of Gastaut (ICOE-G) (previously known as childhood epilepsy with occipital paroxysms) - Differential diagnosis
Mainly from cryptogenic or symptomatic occipital epilepsy ; coeliac disease; migraine with aura; and basilar or acephalgic migraine.
Childhood Epilepsies
Idiopathic childhood occipital epilepsy of Gastaut (ICOE-G) (previously known as childhood epilepsy with occipital paroxysms) - Prognosis
Remission occurs in 50–60% of patients within 2–4 years of onset.
Childhood Epilepsies
Idiopathic childhood occipital epilepsy of Gastaut (ICOE-G) (previously known as childhood epilepsy with occipital paroxysms) - Management
In contrast to other phenotypes of the BCSSS; patients with ICOE-G often suffer from frequent seizures and therefore medical treatment; mainly with carbamazepine; is likely to be mandatory .Secondarily GTCSs are probably unavoidable without medication.
Childhood Epilepsies
Benign (isolated) focal seizures of adolescence - Demographics
These are single seizures or occur in a cluster of up to five over 36 hours. They never occur again.
Childhood Epilepsies
Benign (isolated) focal seizures of adolescence - Age range of onset
72% male preponderance. They may account to 1/5 of patients who have a simple focal seizure in the second decade of life.
Childhood Epilepsies
Benign (isolated) focal seizures of adolescence - Semiology
Seizures start and end within the second decade of life with a peek around 13 to 15 years
Childhood Epilepsies
Benign (isolated) focal seizures of adolescence - Etiology
Motor or somati sensory seizures are the most common types. The seizures usually evolve into a tonic clonic seizure.
Childhood Epilepsies
Benign (isolated) focal seizures of adolescence - Genetic testing or metabolic screening
Normal
Childhood Epilepsies
Benign (isolated) focal seizures of adolescence - Imaging
Normal
Childhood Epilepsies
Benign (isolated) focal seizures of adolescence - Interictal EEG
Normal
Childhood Epilepsies
Benign (isolated) focal seizures of adolescence - Ictal EEG
Usually not recorded
Childhood Epilepsies
Benign (isolated) focal seizures of adolescence - Differential diagnosis
The definitive diagnosis cannot be made until the patient has been free of seizures for 1-5 years
Childhood Epilepsies
Benign (isolated) focal seizures of adolescence - Prognosis
Invariably excellent
Childhood Epilepsies
Benign (isolated) focal seizures of adolescence - Management
No drug management is required.
