M. Ali BEREKETOĞLU, Ayhan BÖLÜK, Sinem KALI

Atipik özellikli santro-temporal dikenli benign çocukluk epilepsili bir vaka

Lokalize idiopatik epilepsilerden Santro-Temporal Dikenli Benign Çocukluk Epilepsisi (Benign Epilepsy with Centro-Temporal Spikes) ile ilgili tanımlar çok açık olmasına rağmen bazen klinik pratikte hastaya yaklaşımda zorluklarla karşılaşılır. Bunlar nöbet semptomatolojisi, fokal EEG paroksismlerinin lokalizasyonu, generalize EEG paroksismlerinin birlikte olması ve klinik seyir başlıkları altında toplanabilmektedir Ayrıca bu sendromun diğer epilepsilerle ortak yönleri, sentro-temporal dikenlerin . epilepsi ile birlikte olan veya olmayan diğer durumlarda görülmesi ve genetik predispozisyon yönü ile idiopatik jeneralize epilepsiler ile ilişkileri gibi konular da tam olarak netleşmemiştir1'. Makalede Santro-Temporal Dikenli Benign Çocukluk Epilepsisi ile uyumlu kliniği olan, anoksik doğum öykülü, lateralize nörolojik defisitli, tedaviye dirençli nöbetleri olan, fokal EEG paroksismleri uyku aktivasyonu ile farklı özellikler gösteren bir vaka değerlendirildi.

A case of benign epilepsy with centro-temporal spikes with atypical features

Benign epilepsy with centro-temporal spikes still remains the model of the benign localization-related epilepsies of childhood. However, its EEG and clinical features are variable and many of its aspects can be debated: manifestations of seizures, localization of the focal EEG paroksisms, existence of associated generalized abnormalities, long-term outcome. Other important problems consist of the relationship with other types of epilepsy and the significance of the EEG pattern when it is present in other situations. We disscussed this subject with a case of benign epilepsy with centro-temporal spikes, who had focal neurologic deficit and paroxysmal discharges that were highly activated during sleep.

PDF

___

1. Dravet C: Benign epilepsy with centrotemporal spikes: do we know all about it ? ImWolf P(ed), Epileptic Seizures and Syndromes. London: John Libbey&Company 1994, pp: 231-40
2. Commission on Classification and Terminology of the ILAE (1989): Proposal for revised classification of epilepsies and epileptic syndromes. Epilepsia 1989;30:389-99.
3. Loiseau P: Benign focal epilepsies of childood. imWylie E. (ed) The Treatment of Epilepsy, Principles and Practice, Philadelphia: Lea & Febiger 1993;503-512.
4. Blom S, Heijbel J, Bergfors PG: Benign epilepsy of children with centrotemporal EEG foci: prevalance and follow-study of 40 patients, Epilepsia 1972;13: 609-19.
5. Niedermeyer E, Naidu S. Further: EEG observations in children with the Rett syndrome, Brain Dev 1990; 12: 53-4.
6. Msumeci SA, Colognola RM, Ferri R, Gigli GL, et all: Fragile -X syndrome: a particular epileptogenic EEG pattern.Epilepsia 1988;29: 41-7.
7. Kraschnitz W, Scheer P, Korner K, Krakty-Dunitz M, et al: H. Rolandic spikes as an electroencephalographic manifestation of an oligodendroglioma, Pediatr Padol 1988;23:313-9
8. Kuzniecky P, Berkovic S, Andermann F, Melonson D, etal: Focal cortical myoclonus and rolandic cortical dysplasia: clarification by magnetic resonance imaging. Ann Neurol 1988;23: 317-25.
9. Pierre G, Guerrini R: Idiopathic localization-related epilepsies: the non-rolandic types. In:Wolf P (ed) Epileptic Seizures and Syndromes,London: John Libbey&Company 1994;241-56.
10. Tassinari CA, Bureau M, Dravet C, Dalla Bernardina B, et al: Epilepsy with contJnous spikes and waves during slow sleep-otherwise described as ESES (epilepsy with electrical status epilepticus during slow sleep).In: Roger J, Bureau M, Dravet C, Drefius FE, Wolf P and Perret A (eds), Epileptic syndromes in infancy, childood and adolesance. LondomJohn Libbey. 1992; 245-256.
11. Dalla Bernardina B, Fontana E, Michelliza B, Colamaria V, et al: Partial epilepsies of childood, bilateral synchronization, contJnous spike-waves during slow sleep. ImManelis J, Bental E, Loeber JN, Dreifus FE (eds), Advances in epileptology, New-York: Raven Press,1989, pp: 295-302.
12. Genton P, Guerrini R: What differentiates Landau-Kleffner syndrome from the syndrome of continous spikes and waves during slow sleep?, Arch Neurol 1993;50:1008-9.