The 20th Congress of RSCANP,

Băile Felix, 18-21.09.2019

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The 42st National Conference of Child and Adolescent Neurology and Psychiatry and Allied Professions with international participation


Autor: Adelheid Wiemer-Kruel
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Idiopathic focal epilepsies are very common epilepsies in childhood, rarer in infancy and adolescence. There are different forms at different ages. The prognosis is good for the typical ones, only in atypical forms or associated syndromes suspect, especially with respect to cognitive functions. In the article the main forms are described more intensively and examples are given, concerning the Watanabe syndrome, the Benign childhood epilepsy in children with centro-temporal spikes (BECTS) or Rolandic Epilepsy and the Benign childhood epilepsy with occipital paroxysms, with the 2 subtypes, the Panayiotopoulos syndrome and the Gastaut type.



Idiopathic focal epilepsies are characterized by focal seizures and focal EEG-findings. They are based on genetic dispositions, so the patients often have a positive family history concerning benign epilepsies, which are related to special ages, and remit spontaneously. The patients do neither show anatomical lesions nor neurological or serious cognitive deficits and do not have a history of relevant previous diseases.

The seizures are mostly short and rare, but in the beginning they can occur more often. The type of seizures is different from patient to patient, however mostly the same in one patient. The EEG is characterized by a normal background activity and high-amplitude sharp waves, sometimes with different independent localisations. Short generalized sharp wave complexes are possible. The focal findings change for the worse during sleep, but do not alter their shape.

The terms idiopathic and benign are not identical, because idiopathic means an underlying genetic disposition. This is not clear in some benign focal epilepsies, and on the other hand there is a controversial discussion wether some of the benign epilepsies are really independent entities.

Table 1 shows the different syndromes which belong to the idiopathic focal epilepsies.

Table 1: Benign focal epilepsies of childhood and adolescence

In the ILAE classification:

1.  Benign childhood epilepsy in children with centra-temporal spikes

  1. Benign childhood epilepsy with occipital paroxysms

Not in the ILAE classification:

1.  Benign partial epilepsy in infancy (Watanabe syndrome)

  1. Atypical benign childhood focal epilepsy
  2. Benign partial epilepsy with affective symptoms (benign psychomotor epilepsy)
  3. Benign focal epilepsy of childhood with extreme somatosensory potentials
  4. Benign frontal lobe epilepsy of childhood
  5. Benign focal seizures during adolescence
  6. Idiopathic photosensitive occipital lobe epilepsy (Tassinari)

In controversial relation:

1.  Landau-Kleffner syndrome

  1. CSWS syndrome

A new syndrome:

Autosomal dominant Rolandic epilepsy with dyspraxia

Some authors submit the Landau-Kleffner and the CSWS syndrome to the benign focal epilepsies. This is quite controversial. Both syndromes are remitting during adolescence, but the prognosis with regard to cognitive functions is very unfavourable. Doose, a German pediatric epileptologist, is discussing that there are different manifestations of idiopathic focal epilepsies, at one end the Rolandic epilepsy with centra-temporal sharp waves without symptoms, and at the other end the CSWS syndrome. The cause is supposed in a hereditary disturbance of brain maturation. In the ILAE-classification, the Landau-Kleffner and the CSWS syndrome are submitted to the focal epilepsies with generalized seizures.

There is a new syndrome, the autosomal dominant Rolandic epilepsy with dyspraxia, which is similar to the Rolandic epilepsy, but different from it with regard to dyspraxia and cognitive impairment as the dominant features. So this epilepsy is not really “benign”, same to Landau-Kleffner and CSWS syndrome.

Table 2: Benign focal epilepsies in children

  1. Benign partial epilepsy in infancy (Watanabe syndrome)
  2. Benign childhood epilepsy in children with centro-temporal spikes (BECTS, Rolandic epilepsy)
  3. Benign childhood epilepsy with occipital paroxysms early onset (Panayiotopoulos syndrome) – late onset (Gastaut type)

Benign partial epilepsy in infancy (Watanabe syndrome)

The first description of the benign partial epilepsy in infancy was given by Watanabe, 1987. There is an autosomal dominant inheritance (Vigevano, Guipponi). The gene is located on the long branch of chromosome 19. It is not the same as the one responsible for the benign neonatal seizures, which has been proven by different genetic studies. The first manifestation of the epilepsy is about an age of 4-8 months. The seizures manifest in clusters during 2-4 days, 8-10 seizures perday, brief, for one to two minutes. The semiology is motor arrest, reduced reagibility, staring, mild oral automatisms, mild convulsions.

The mean duration of the active epilepsy is about 3 months and there is a remission in all cases. The EEG shows a normal background activity and often a normal interictal pattern without spikes.The children have no brain damage, normal neurologic findings, and a normal development before and after epilepsy. There is a high incidence of seizures at the same age in the family. The prognosis is excellent, also because it is easy to treat. A low dose of carbamazepin will be sufficient, but phenobarbital or valproat are also possible.

Case report:

Lea, born on March 10th, 2004, is the first child of a 28 years old father and a 25 years old mother. Her father and his sister had seizures when they were about 6 months old. They received no AED, but calcium and   magnesium.   The   seizures   stopped afterwards, and they had a normal cognitive development. The pregnancy with Lea, her birth, weight, length, Apgar index (10-10) were normal. At the end of the 5th month she was very calm after awakening, showed no reaction, with a duration of only a few seconds. After that she seemed quite normal again. One week later, after drinking, she suddenly rolled her eyes, stared, the skin colour turned to blue, and she had tonic extremities for a few seconds. In September 2004 she had several events: rolling of the eyes upwards, tonic extremities (no myoclonias), a slightly cyanotic appearance, mostly for 60 seconds. In the following 26 hours she had 9 seizures. Between the seizures she was tired, but unchanged in behaviour. She was admitted to hospital. Her neurology, lumbar puncture, screening for metabolic diseases, CCT, MRT and several EEGs were normal. The seizures were stopped by intravenous injection of DZP and PB. In the following time she had a normal development and no change of behaviour. Four weeks later, in October 2004, she had again 3 seizures within 9 hours, and further 4 attacks within the following 3 days.

In October she was admitted to our clinic. She seemed to be a normal child, had a normal EEG, in wakeful state and during sleep. The next cluster of seizures occured on 14th – 15th of the month and derived from the occipital median region. Clinically she showed a deviation of the eyes to the right side, a turn of the head from the left to the right side, moving of the body to the right side, no reaction, staring, and a cyanotic skin. The features of the following seizures were tonic members and clonic movements of the mouth. The diagnosis of a Watanabe syndrome was made by the family history of seizures in infancy, the normal history and neurology of the child, the normal MRT and interictal EEG, the time of onset of the focal seizures at 5 months, the seizure semiology, the seizures manifesting in clusters, and the seizure onset in the EEG occipital medial. The treatment consisted of a low dose of CBZ, 300 mg/day ( body weight 8,2 kg, drug blood level 4,0 ug/ml).

After that she was seizure-free and had a normal development. Even after a sudden reduction of CBZ from 300 to 120 mg in December, 2004, at the age of 9 months, she had no seizures, but after a further reduction to 2 x 30 mg in January, 2005, she had a small seizure for a few seconds. The dose was elevated once more up to 120 mg/day, and she was free from attacks again. In October, 2005, a new start of reduction of CBZ was done more slowly, and since that time there was no relaps.

Benign childhood epilepsy in children with centro-temporal spikes

The Benign childhood Epilepsy in children with Centro-Temporal Spikes (BECTS) or Rolandic epilepsy was first described in 1597 by doctor Martinius Rulandus, but the name will be derived from the Italian anatomist and physiologist Luigi Rolando (1773-1831). The first publication of the clinical course has been done by Nayrac and Beaussart (1958).

It is one of the most common epilepsies in childhood ( 5-20% of. all epilepsies in children). There is a predominance of the male gender (3:2).

From the aetiologic point of view, 6-10% have a conspicuous neonatal period, 4-5% a mild head injury, and 7-10% febrile convulsions. A multi-factorial genesis is suspected and genetic factors are important. 40% of the close relatives have seizures or EEG patterns with spike waves. As to brothers and sisters, 15% have a Rolandic focus and an epilepsy, 19% only a Rolandic focus in the EEG, and 11% of the parents have had seizures. Genetic studies demonstrate that the typical centro-temporal SWs have an autosomal recessive inheritance with variable penetrance (chromosome 15q14, Neubauer). Only 10% of the children with a Rolandic focus get seizures.

The manifestational age of the Benign childhood epilepsy in children with centro-temporal spikes is 3-13 years, in 75% between 5-10 years. The focal seizures are usually short and rare, but secondary generalisation  is possible. The seizures occur mainly during night-time, 15% during day- and night-time and 20-30% only during day-time. During day-time thereare mainly simple focal seizures, during the night more often with secondary generalisation. The seizures present, being full conscious, with somato-sensible paresthesias of the tongue, the lips, the oral mucosa and cheek, or unilateral tonic, clonic, and tonic-clonic fits of the muscles of the face, the lips, tongue and the pharynx, or with disturbance of the language (dysarthria), or with hypersalivation because of dysphagia. The seizures can spread to the ipsilateral arm and foot, can become complex partial or secondarily generalized. The Grand mal seizure is in 60% the most common one, only 30% have simple focal seizures. This depends on age. In younger children there are more often generalized seizures, in older ones more often hemi-facial focal ones. The seizures last in general 30 seconds to 2 minutes, but 5-10% of the children have a status epilepticus. The frequency is rare in 2/3 of the cases, with a 2-12 months distance between the seizures. 13-21% of the children have only a single seizure, 50% less than 5, and only 21% have frequent seizures, mostly in clusters. 7-16% of the patients have a postictual paresis.

The background activity of the EEG is normal. Besides of that there are high-amplitude sharp- and sharp-slow-waves of a characteristic shape in the centra-temporal region and more rare in the neighbourhood. Secondary generalisation is possible. The spike waves tend to secondary bilateralisation or spreading to the contralateral side (60% unilateral, 40% bilateral). The most dominant feature in EEG pattern is the SW activation during sleep. In 30% of the patients SW are only seen during sleep. Mostly in sleep EEGs, short generalized SSW-and SW-complexes are possible. There is a complete remission of the SWs during puberty. SWs in the centra-temporal region were found in 1,5% – 2,5% of all normal school children. MRI is normal, without cortical lesions in the centra-temporal region.

With regard to neuropsychological aspects, no neurologic and mental deficits,and no mental regression during the course of the epilepsy should be found. However, slight problems are often seen, like learning difficulties or reduced concentration. Newer studies have proven specific memory difficulties, visual as well as verbal, and impaired phonologic processing skills. These difficulties are not explained by the interictual EEG-activity.

If seizures are rare, no therapy is recommended. Remission will be during puberty, with or without therapy. In general, Benign focal epilepsy in children with centra-temporal spikes is easy to treat: the drug of first choice in Germany is sulthiame (Ospolot) (dosage: 3-6 mg/kg/day). 86% of the patients will be free from attacks by this medication. Other possible drugs are clobazam, carbamazepin,oxcarbazepin, and valproat,but one should be careful with carbamazepin and oxcarbazepin, because seizure aggravation is noticed, particularly with higher doses. Levetiracetam (Keppra), 30-40(-60) mg/kg/ day, seems to get in Germany the therapy of second choice. Till now it is not the official way to treat, because levetiracetam is still not licensed for a monotherapy, but only allowed in combinations. There is a study in progress (HEAD-Study), comparing sulthiame with levetiracetam in the treatment of Rolandic epilepsies. – If the patient is free from attacks during (1-)2 years, therapy can be stopped.

The prognosis of Benign focal epilepsy in children with centro-temporal spikes is excellent: no SWs and no more seizures at least at an age of 15-16 years, independent of the former seizure frequency. Already at an age of 6 years, 50% of the epilepsies are in remission. The psychosocial prognosis is also very good, even if there had been some difficulties during the acute phase of the epilepsy.

Case report:

Luca, born November 26th, 1999, has no family history of epilepsy. His pregnancy, birth and development were normal. At 3 1/2 years, his mother realised during sleep clonic movements of the left side, and he was slightly blue. His speech was indistinct for a certain time after recovery. After admission at hospital, his neurology, lumbar puncture, MRT, ECG, EEG were normal. The second and third seizure happened one year later (3/2005). He was unable to speak, but could understand everything. He was vomiting and had a hypersalivation. The EEG showed a normal background activity and a sharp wave focus right centra-temporal (C4/T4). In sleep EEG, a mild seizure was registered. Because of rare and mild seizures no therapy was necessary. In 4/2007, his EEG was normal. The boy was in the second class of a regular school and had no school problems.

Benign childhood epilepsy with occipital paroxysms

The first description of the Benign childhood epilepsy with occipital paroxysms was made by Gastaut, 1981. The seizures start with visual symptoms, often followed by motor or psycho-motor manifestations, sometimes with postictual migrainous headache. Remission of the epilepsy happens during adolescence or at least when adult. The EEG shows distinct repetitive occipital paroxysms after closing of the eyes. The children have no neurologic problems. The syndrome is rarer than Rolandic epilepsy. Panayiotopoulos (1989, 1999) mentioned it more often, 20% of the benign childhood focal epilepsies, but there are quite variable data. Two subunits of the syndrome are existing, the early onset or Panayiotopoulos syndrome or the late onset or Gastaut type. The last one is much rarer, 2-7% of the benign focal epilepsies.

Benign childhood epilepsy with occipital paroxysms – Panayiotopoulos syndrome

The early onset of Panayiotopoulos syndrome is often associated with febrile convulsions in the history (16-30%) and with epilepsies in the family in about 7%. The age of onset of the epilepsy is 2-8 years, on an average 4-5 years. Seizures mainly occur during night; in 2/3 of the cases only during night, in 1/3 during day and night or during day-time. Normally only a few seizures, i.e. 2-3, will be seen.

The semiology of the seizures is characterized by deviation and opening of the eyes and mainly by vomiting (72-74%). The deviation of the eyes may take minutes or hours, sometimes with turning of the head to the same side. In the beginning, consciousness and speech are regulary preserved, but in the course impairment of consciousness, also hemiclonic convulsions of the face and members are possible (25%), as well as secondary generalisation (20%). Autonomous signs as palour, mydriasis or change of mood and behaviour may appear before or during seizures. Visual symptoms and postictual headache are rare. The duration of the seizures is 5-10 minutes in 2/3 of the cases, in 1/3 of the cases, however, the fits last for some hours as focal or autonomous status epilepticus (on the average about 2 hours). The children are normal in respect to their neurology and psychology, as well as they have normal MRT. The EEG shows unilateral or bilateral high voltage sharp slow waves in the occipital region, blocked by eye opening (“fixation off” sensitivity, like late onset variant). A change of the side of the focus is often found.

The diagnosis results from the following symptoms: normal child, onset of the epilepsy between 2 and 8 years, occipital EEG focus; and ictual vomiting with tonic eye deviation. The therapy depends on the frequency of attacks, which is normally low and so no therapy is required. A small dose of carbamazepin seems to be sufficient. Alternatives are valproat and phenobarbital, but these are not well documented. The prognosis is excellent. The duration of the epilepsy is about 1-2 years and remission will take place before 12 years of age.

Case report:

Gizem, born on January 25th, 1996, of Turkish origin, had her first seizure in June, 2000, at the age of 4 1/2 during holidays in Turkey. It started with, “Mama, I must vomit”, then deviation of the eyes to the right side. She lost her orientation, could not speak, was atonic, and had myoclonic movements of the right side. The duration of the status was 2 hours. After that she did not speak during 3 days. The EEG showed an SW focus over the left occipital region (01), sometimes on the right side (02), too. The therapy in Turkey was started with phenobarbital in a high dosage, perhaps too much, so that she could not speak for 3 days. Two weeks later, the EEG in Germany showed a dominant slowing over the left temporo-occipital region. The MRI demonstrated a periventricular leucomalacia, but no cortical lesion. At first the diagnosis of a hemiplegic migraine was made and phenobarbital was stopped. However, after stopping phenobarbital, the EEG revealed a great activity of SWs, much more over the left than over the right occipital region (01»02), nearly a continuous activity. A therapy was started with valproat, but it was not well tolerated, so phenobarbital was given once more. In the EEG multifocal SWs (01, T5, 02) from the aspect of benign epileptic potentials of childhood were found, so the therapy was changed to sulthiame (5 mg/kg/ day), and because of the still presenting high SW activity clobazam was added (2×2,5 mg). Gizem had no more seizure, so clobazam was slowly reduced with time and stopped without any relapse. Since 2007, Gizem is without any therapy and still free from attacks and SWs. EEGs of Gizem under PB, 2001

Benign childhood epilepsy with occipital paroxysms 

– late onset or Gastaut type –

In this syndrome the genetic factors are very important, too. 30-40% have a positive family history of febrile convulsions or epilepsy (Gobbi u. Guerrini, 1997). The manifestational age of the epilepsy is about 3-16 years, on the average 8 years. There is no difference in gender. The epilepsy is characterized by simple partial seizures during day-time, visual symptoms like hallucinations and illusions (macropsia, micropsia), scotomas or amaurosis, small circular multicoloured pattern. The duration is short, i.e. 5-15 seconds, seldom extended to 1 -2 minutes, and 15-20 minutes are very rare. Complex partial just as hemiclonic or generalized seizures are possible, as well as, most commonly, deviation of the eyes with ipsilateral turning of the head. 2/3 of the patients have postictual migrainous headache. Background activity in EEG is normal, and in the occipital and temporo-posterior region there are high amplitudic SWs, rhythmic repeated only by closed eyes. The ictual EEG shows SWs, spreading from the occipital to the centro-temporal region. The differential diagnosis is migraine, but here, no EEG changes are seen and no SWs during the appearance of symptoms. It is important to exclude symptomatic occipital epilepsies by MRI. The therapy of choice is carbamazepin and sulthiame. The prognosis is relatively good, remission is possible after 2-4 years.

The following idiopathic focal epilepsies should be mentioned to complete the whole spectrum of possibilities, but altogether they are rare.

Atypical benign childhood focal epilepsy

The Atypical benign childhood focal epilepsy was first described in 1982 by Aicardi and Chevrie and later confirmed by Deonna and Doose (1992). The beginning of the epilepsy is usually at an age of 2-6 years, with typical focal and generalized seizures, atypical absences, myoclonias and atonic seizures. The epilepsy is therapy resistent, but in contrast to other syndromes as the Landau-Kleffner or CSWS syndrome, no psychomotor development regression is seen.Theremission of the epilepsy will be at the age of 9-12 years. The EEG pattern during awakening shows focal spikes and sharp waves, changing from one hemisphere to the other, but in sleep EEG there is an almost continuous slow spike wave activity. Deonna (1986) described a possible combination of Rolandic epilepsy with myoclonic astatic epilepsy. If seizures are very frequent, a regression of the mental status, worsening of behaviour and concentration is going to happen. Doose (1992) spoke of a similarity of the syndrome with the Lennox-Gastaut syndrome or the Pseudo-Lennox-Gastaut syndrome. Besides central or centro-temporal, also frontal sharp waves are seen in the EEGs, and permanent mental and oral regression is noticed with often bioelectrical status.

The differentiation to Lennox-Gastaut syndrome is that there are no tonic seizures, a better prognosis and an enormous activation of SW activity during sleep. The therapy is worthful to be done with sulthiame and carbamazepin.

Benign partial epilepsy with affective symptoms (benign psychomotor epilepsy)

It is not really clear wether the Benign partial epilepsy with affective symptoms is an independent syndrome. In some studies the benign psychomotor epilepsy is said to be 10% of all benign focal epilepsies, in others it really does not exist (Gobbi and Guerrini, 1997). Some mention it as a variant of Rolandic epilepsy (Dalla Bernardina, 1992) – a very controversial discussion. It has a positive family history in 38% of the cases, and the patients have – like in all benign epilepsies – no neurological deficits. The beginning of the epilepsy is between 2-9 years. The characteristics are seizures at day or night with sudden fear and panic, loud crying, holding tight to mothers, chewing, swallowing, gurgling sounds, hypersalivation, impaired consciousness, with a normal duration of 1-2 minutes, but it can happen several times per day. In the EEG there are fronto-temporal or parieto-temporal, uni- or bilateral SWs and in 60% also short generalized SW-complexes. The prognosis is good, and remission will happen before 18 years. For therapy carbamazepin and phenobarbital are recommended.

Benign focal epilepsy of childhood with extreme somato-sensoric potentials

The first description of the Benign focal epilepsy of childhood with extreme somato-sensoric potentials was given by De Marco and Negrini, 1973. Tapping on the sole of the foot produces extremely high somato-sensoric evocated potentials in the contralateral parietal region. The EEG shows high potentials of 200 – 400 uV. 44% of the patients have febrile convulsions before. The gender is predominantly male (3:1). The epilepsy begins at an age of 4-8 years. There are rare seizures during day time.

Initially, somato-sensoric potentials are found, then typical focal SWs, at first only during the night then also during day-time, and at least seizures may occur, mainly with version, as well as somato-sensoric seizures, generalized tonic-clonic seizures without involving the facial muscles. In the EEG the SWs are mainly located in the parietal region. Because of rare seizures, therapy is often not necessary.

Benign frontal lobe epilepsy of childhood

The Benign frontal lobe epilepsy of childhood is not a definite syndrome. It is said to be 10% of all idiopathic focal epilepsies (Loiseau, 1991). The prognosis is good. The manifestational age is 6 months to 9 years. Short tonic seizures occur with dystone posturing during night.

The consciousness is not impaired. The seizure frequency is high, almost every night. The EEG pattern consists of interictal unilateral frontal spike wave complexes. The therapy is carbamazepin.

Benign focal seizures during adolescence

The first description of Benign focal seizures during adolescence was done by Loiseau and Orgogozo, 1978. The syndrome is characterized by single attacks or clusters of seizures (i.e. 2-5), simple or complex partial in a short time in healthy teenagers. In 80% of the patients there will be only one seizure, otherwise 2-5 seizures within 36 hours. The manifestational age is 10-20 years (12-13 years), and it will be in 60% secondarily generalized tonic-clonicseizures. The interictal EEG is normal as well as the MRI.

Idiopathic photosensitive occipital lobe epilepsy (Tassinari)

The Idiopathic photosensitive occipital lobe epilepsy (Tassinari) is an age related benign epilepsy induced by specific stimuli like television, computers, optic stimulation. There is an incidence of family history in 25-30%. The manifestational age is about 5-18 years, beginning with visual symptoms like optic hallucinations, blindness, turning of the head to one side, feeling sick, vomiting, headache, often secondary generalisation of the seizures. The EEG shows a photoparoxysmal reaction with occipital spikes. Valproat is an effective therapy.

Wether the Landau-Kleffner and CSWS syndrome are really belonging to the idiopathic focal epilepsies is discussed controversial, as already mentioned above.

Landau-Kleffner syndrome

The Landau-Kleffner syndrome is an age related epileptic syndrome with a manifestational age of 3-5 years. The children were normal before and had a normal MRI. The syndrome is characterized by acquired abrupt or creeping motor and often also sensory aphasia. Because of the aphasia, or independent from it, behavioural disturbances occur, even autistic symptoms. Seizures are possible, but not imperative. The EEG pattern shows focal and multifocal SWs, particularly in the temporal region, perisylvic, and during sleep EEG there is a CSWS. The aetiology of the syndrome is unknown. Seizures are easy to control by antiepileptic therapy, but in contrast to that the CSWS only very difficult and often not at all.

First line antiepileptic drugs are sulthiame, clobazam, levetiracetam, valproat, ethosuximide and for CSWS dexamethason. In puberty, normalisation of the EEG is typical. With respect to that the prognosis is good, but not for the verbal and mental outcome! 63,6% of the patients will be mentally retarded, and only 18,2% will have complete speech normalisation (Rossi, 1999).

CSWS syndrome

The CSWS syndrome affects normal or primary slightly retarded children at an age of 2-10 years. The EEG pattern is multifocal with bilateral synchronisation und generalisation to a CSWS during sleep. The occurrence of the CSWS is accompanied of complex mental and speech development disturbances, global regression, behaviour problems, and difficulties in motorcoordination. The prognosis is poor with a great tendency for relapses. Remission will be before or during puberty, seldom without defects and deficits in mental and speech development. The therapy is very difficult. Sulthiame, levetiracetam, valproat, clobazam and dexamethason are used, only to mention the most important drugs in this connection.

Idiopathic focal epilepsies in adults

There are also idiopathic focal epilepsies in adults, i.e. the reading epilepsy and the autosomal dominant noctal frontal lobe epilepsy. Of the reading epilepsy, the manifestational age is 15-18 years, with male predominance. It is characterized by simple focal motor seizures of the masticatory muscles. The main therapeutic rational is valproat. The autosomal dominant noctal fronta\ lobe epilepsy has its manifestational age at 11-12 years, with complex partial seizures, in 70% with auras. The therapy will be carbamazepin.


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