Childhood Epilepsies

Question 1

Childhood Epilepsies

Benign childhood epilepsy with centrotemporal spikes - Demographics

Answer 1

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.

Question 2

Childhood Epilepsies

Benign childhood epilepsy with centrotemporal spikes - Age range of onset

Answer 2

Onset is from age 1 to 14 years; peak 8 or 9 years.

Question 3

Childhood Epilepsies

Benign childhood epilepsy with centrotemporal spikes - Semiology

Answer 3

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).

Question 4

Childhood Epilepsies

Benign childhood epilepsy with centrotemporal spikes - Etiology

Answer 4

This is a genetically determined syndrome; possibly with multiple genes leading to the phenotype.

Question 5

Childhood Epilepsies

Benign childhood epilepsy with centrotemporal spikes - Genetic testing or metabolic screening

Answer 5

Normal

Question 6

Childhood Epilepsies

Benign childhood epilepsy with centrotemporal spikes - Imaging

Answer 6

Normal

Question 7

Childhood Epilepsies

Benign childhood epilepsy with centrotemporal spikes - Interictal EEG

Answer 7

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.

Question 8

Childhood Epilepsies

Benign childhood epilepsy with centrotemporal spikes - Ictal EEG

Answer 8

Ipsilateral rolandic regions and consists of slow waves mixed with fast rhythms.

Question 9

Childhood Epilepsies

Benign childhood epilepsy with centrotemporal spikes - Differential diagnosis

Answer 9

The diagnosis is usually pretty straightforward

Question 10

Childhood Epilepsies

Benign childhood epilepsy with centrotemporal spikes - Prognosis

Answer 10

Invariably excellent

Question 11

Childhood Epilepsies

Benign childhood epilepsy with centrotemporal spikes - Management

Answer 11

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.

Question 12

Childhood Epilepsies

Panayiotopoulos syndrome - Demographics

Answer 12

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.

Question 13

Childhood Epilepsies

Panayiotopoulos syndrome - Age range of onset

Answer 13

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.

Question 14

Childhood Epilepsies

Panayiotopoulos syndrome - Semiology

Answer 14

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.

Question 15

Childhood Epilepsies

Panayiotopoulos syndrome - Etiology

Answer 15

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.

Question 16

Childhood Epilepsies

Panayiotopoulos syndrome - Genetic testing or metabolic screening

Answer 16

Normal

Question 17

Childhood Epilepsies

Panayiotopoulos syndrome - Imaging

Answer 17

Normal

Question 18

Childhood Epilepsies

Panayiotopoulos syndrome - Interictal EEG

Answer 18

Etiology

Question 19

Childhood Epilepsies

Panayiotopoulos syndrome - Ictal EEG

Answer 19

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

Question 20

Childhood Epilepsies

Panayiotopoulos syndrome - Differential diagnosis

Answer 20

Migraine and other causes of syncope.

Question 21

Childhood Epilepsies

Panayiotopoulos syndrome - Prognosis

Answer 21

Typically excellent. The risk of epilepsy in adult life appears to be no higher than in the general population.

Question 22

Childhood Epilepsies

Panayiotopoulos syndrome - Management

Answer 22

Prophylactic treatment with anti-epileptic medication may not be needed for most patients.

Question 23

Childhood Epilepsies

Idiopathic childhood occipital epilepsy of Gastaut (ICOE-G) (previously known as childhood epilepsy with occipital paroxysms) - Demographics

Answer 23

Both sexes are equally affected. The disorder accounts for about 2–7% of benign childhood focal seizures. Less prevalent than PS.

Question 24

Childhood Epilepsies

Idiopathic childhood occipital epilepsy of Gastaut (ICOE-G) (previously known as childhood epilepsy with occipital paroxysms) - Age range of onset

Answer 24

Onset is between 3 and 15 years of age with a mean of around 8.

Question 25

Childhood Epilepsies

Idiopathic childhood occipital epilepsy of Gastaut (ICOE-G) (previously known as childhood epilepsy with occipital paroxysms) - Semiology

Answer 25

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.

Question 26

Childhood Epilepsies

Idiopathic childhood occipital epilepsy of Gastaut (ICOE-G) (previously known as childhood epilepsy with occipital paroxysms) - Etiology

Answer 26

ICOE-G is considered to be a late-onset phenotype of BCSSS. The seizures are of a purely occipital lobe origin.

Question 27

Childhood Epilepsies

Idiopathic childhood occipital epilepsy of Gastaut (ICOE-G) (previously known as childhood epilepsy with occipital paroxysms) - Genetic testing or metabolic screening

Answer 27

Normal

Question 28

Childhood Epilepsies

Idiopathic childhood occipital epilepsy of Gastaut (ICOE-G) (previously known as childhood epilepsy with occipital paroxysms) - Imaging

Answer 28

Normal

Question 29

Childhood Epilepsies

Idiopathic childhood occipital epilepsy of Gastaut (ICOE-G) (previously known as childhood epilepsy with occipital paroxysms) - Interictal EEG

Answer 29

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.

Question 30

Childhood Epilepsies

Idiopathic childhood occipital epilepsy of Gastaut (ICOE-G) (previously known as childhood epilepsy with occipital paroxysms) - Ictal EEG

Answer 30

Sudden appearance of an occipital discharge that consists of fast rhythms with lower amplitude then interictal discharges

Question 31

Childhood Epilepsies

Idiopathic childhood occipital epilepsy of Gastaut (ICOE-G) (previously known as childhood epilepsy with occipital paroxysms) - Differential diagnosis

Answer 31

Mainly from cryptogenic or symptomatic occipital epilepsy ; coeliac disease; migraine with aura; and basilar or acephalgic migraine.

Question 32

Childhood Epilepsies

Idiopathic childhood occipital epilepsy of Gastaut (ICOE-G) (previously known as childhood epilepsy with occipital paroxysms) - Prognosis

Answer 32

Remission occurs in 50–60% of patients within 2–4 years of onset.

Question 33

Childhood Epilepsies

Idiopathic childhood occipital epilepsy of Gastaut (ICOE-G) (previously known as childhood epilepsy with occipital paroxysms) - Management

Answer 33

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.

Question 34

Childhood Epilepsies

Benign (isolated) focal seizures of adolescence - Demographics

Answer 34

These are single seizures or occur in a cluster of up to five over 36 hours. They never occur again.

Question 35

Childhood Epilepsies

Benign (isolated) focal seizures of adolescence - Age range of onset

Answer 35

72% male preponderance. They may account to 1/5 of patients who have a simple focal seizure in the second decade of life.

Question 36

Childhood Epilepsies

Benign (isolated) focal seizures of adolescence - Semiology

Answer 36

Seizures start and end within the second decade of life with a peek around 13 to 15 years

Question 37

Childhood Epilepsies

Benign (isolated) focal seizures of adolescence - Etiology

Answer 37

Motor or somati sensory seizures are the most common types. The seizures usually evolve into a tonic clonic seizure.

Question 38

Childhood Epilepsies

Benign (isolated) focal seizures of adolescence - Genetic testing or metabolic screening

Answer 38

Normal

Question 39

Childhood Epilepsies

Benign (isolated) focal seizures of adolescence - Imaging

Answer 39

Normal

Question 40

Childhood Epilepsies

Benign (isolated) focal seizures of adolescence - Interictal EEG

Answer 40

Normal

Question 41

Childhood Epilepsies

Benign (isolated) focal seizures of adolescence - Ictal EEG

Answer 41

Usually not recorded

Question 42

Childhood Epilepsies

Benign (isolated) focal seizures of adolescence - Differential diagnosis

Answer 42

The definitive diagnosis cannot be made until the patient has been free of seizures for 1-5 years

Question 43

Childhood Epilepsies

Benign (isolated) focal seizures of adolescence - Prognosis

Answer 43

Invariably excellent

Question 44

Childhood Epilepsies

Benign (isolated) focal seizures of adolescence - Management

Answer 44

No drug management is required.