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Scientific meeting of the Netherlands Society of Child Neurology held in The Hague, 14th April 1988 Frontal lobe seizures in children; case presentation W. van Emde Boas, A.C.B. Peters (Heem- stede, Leiden) Three children, aged 14-16 years were referred for intensive EEG- and videomonitoring for the evaluation of paroxysmal behavioural disturb- ances. A diagnosis of epilepsy had been estab- lished in two of these children but there was doubt about the epileptic origin of the present attacks. Previous seizure recording in these 3 patients had yielded negative results. On the basis of intensive monitoring a posi- tive clinical- and EEG diagnosis of frontal lobe seizures could be established in all cases. Complex partial seizures originating from or- bital-frontal or mesiofrontal foci are character- ized by a rather peculiar clinical semiology, quite distinct from more well-known seizure types such as absence seizures, generalized fits or typical mesiotemporal- or frontocentral par- tial complex seizures. The seizures start abruptly, often with a scream or other explosive vocalisations and typ- ically include complex, often rather bizarre or apparently even theatrical bilateral motor beha- viour. Some patients, especially children, show autonomic symptoms during the attacks. Juvenile alternating hemiplegic migraine L.A.E.M. Laan*, H.G.J. Krouwer* *, P.M. Stuurman* * *, W.F.M. Arts*,A.W. de Weerd* (Den Haag*, Vlaardingen* *, Gorinchem* * *) A girl with normal development and intelli- gence is described, who suffered from an al- ternating hemiplegia. The symptoms followed by headaches started at the age of 13. The family history revealed no migraine. During an attack the clinical signs varied from hemiplegia and aphasia to vasomotor disturbances and dysfunc- tion of alternating parts of the brain stem. Laboratory tests of blood and cerebrospinal fluid, computerized tomography and four- Seizure frequency often is high, up to 20 or more seizures a day, but may vary widely within one patient. The duration is short, usually less than a minute and conciousness and behaviour are completely normal again immediately fol- lowing the cessation of the seizure. Seizures may occur at any time of the day but often are associated with sleep. The ictal EEG often shows unilateral or bilateral abnormalities over the frontal or central areas but may be negative or, unless adequate recording tech- niques are applied, even unreadable due to ma- jor movement artifact. Due to these factors these seizures often are misinterpreted as hysterical seizures or, when occurring during sleep, as nightmares or other parasomnias. In pediatric patients, as in adults, the inci- dence of this specific seizure syndrome is more common than is generally appreciated, especial- ly during puberty or adolescence but in some cases even during infancy or early childhood. Child-neurologists, pediatricians and youth- psychiatrists should be aware of this type of epilepsy that in most cases responds well to treatment with Carbamazepine or Phenytoin, alone or combined with Valproate. vessel-angiography were normal. EEG- examination showed generalized disturbances and F.I.R.D.A.; an interictal EEG was normal. Ergotamine, clonidine or propanolol had no ef- fects. The disease responded favourably to flu- narizine with a complete abolition of the at- tacks. The course of the disease, the EEG-disturbances, the normal findings at all other laboratory tests and the favourable reac- tion to flunarizine, all point to a juvenile form of alternating hemiplegic migraine. This entity has been described only a few times and can be considered as a variant of the also rare infantile alternating hemiplegia. 287

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Page 1: Frontal lobe seizures in children; case presentation

Scientific meeting of the Netherlands Society of Child Neurology held in The Hague, 14th April 1988

Frontal lobe seizures in children; case presentation

W. van Emde Boas, A.C.B. Peters (Heem- stede, Leiden)

Three children, aged 14-16 years were referred for intensive EEG- and videomonitoring for the evaluation of paroxysmal behavioural disturb- ances. A diagnosis of epilepsy had been estab- lished in two of these children but there was doubt about the epileptic origin of the present attacks. Previous seizure recording in these 3 patients had yielded negative results.

On the basis of intensive monitoring a posi- tive clinical- and EEG diagnosis of frontal lobe seizures could be established in all cases.

Complex partial seizures originating from or- bital-frontal or mesiofrontal foci are character- ized by a rather peculiar clinical semiology, quite distinct from more well-known seizure types such as absence seizures, generalized fits or typical mesiotemporal- or frontocentral par- tial complex seizures.

The seizures start abruptly, often with a scream or other explosive vocalisations and typ- ically include complex, often rather bizarre or apparently even theatrical bilateral motor beha- viour. Some patients, especially children, show autonomic symptoms during the attacks.

Juvenile alternating hemiplegic migraine

L.A.E.M. Laan*, H.G.J. Krouwer* *, P.M. Stuurman* * *, W.F.M. Arts*,A.W. de Weerd* (Den Haag*, Vlaardingen* *, Gorinchem* * *)

A girl with normal development and intelli- gence is described, who suffered from an al- ternating hemiplegia. The symptoms followed by headaches started at the age of 13. The family history revealed no migraine. During an attack the clinical signs varied from hemiplegia and aphasia to vasomotor disturbances and dysfunc- tion of alternating parts of the brain stem. Laboratory tests of blood and cerebrospinal fluid, computerized tomography and four-

Seizure frequency often is high, up to 20 or more seizures a day, but may vary widely within one patient. The duration is short, usually less than a minute and conciousness and behaviour are completely normal again immediately fol- lowing the cessation of the seizure.

Seizures may occur at any time of the day but often are associated with sleep. The ictal EEG often shows unilateral or bilateral abnormalities over the frontal or central areas but may be negative or, unless adequate recording tech- niques are applied, even unreadable due to ma- jor movement artifact.

Due to these factors these seizures often are misinterpreted as hysterical seizures or, when occurring during sleep, as nightmares or other parasomnias.

In pediatric patients, as in adults, the inci- dence of this specific seizure syndrome is more common than is generally appreciated, especial- ly during puberty or adolescence but in some cases even during infancy or early childhood.

Child-neurologists, pediatricians and youth- psychiatrists should be aware of this type of epilepsy that in most cases responds well to treatment with Carbamazepine or Phenytoin, alone or combined with Valproate.

vessel-angiography were normal. EEG- examination showed generalized disturbances and F.I.R.D.A.; an interictal EEG was normal. Ergotamine, clonidine or propanolol had no ef- fects. The disease responded favourably to flu- narizine with a complete abolition of the at- tacks. The course of the disease, the EEG-disturbances, the normal findings at all other laboratory tests and the favourable reac- tion to flunarizine, all point to a juvenile form of alternating hemiplegic migraine. This entity has been described only a few times and can be considered as a variant of the also rare infantile alternating hemiplegia.

287