Vol. 19/010, nr 7
p R A C A O R Y G I N A L N A / O R I G I N A L A R T I C L E
padaczki z międzynapadowymi wyładowaniami w okolicach potylicznych
u dzieci
Epilepsies with interictal occipital epileptiform discharges in children
Maria Mazurkiewicz-Bełdzińska, Marta Szmuda, Agnieszka Matheisel
Klinika Neurologii Rozwojowej Gdańskiego Uniwersytetu Medycznego
SUMMARY
Introduction: Although occipital discharges (OED) are
rela-tively common finding in the EEG especially in children, there is still not enough data about the correlation of their presence and the clinical status of the patients. The presented work was undertaken in order to estimate such a correlation. Materials
and Methods: 31 children, aged from 12 months to 10 years
with the interictal occipital discharges were included in the study. Results: In most of them (71%) symptomatic etiology of epilepsy were identified. We found differences in morphology of seizures and in the age of onset, types of EEG changes, mode of treatment between the groups of idiopathic and symptomatic epilepsies. Conclusions: This study proves that detailed corre-lation between the clinical and EEG features is needed to make correct diagnosis and to imply therapeutic procedures in this very complex and difficult to treat form of epilepsy in children.
Key words: epilepsy, children, occipital epileptiform discharges
STRESZCZENIE
Wstęp i cel pracy: Mimo że międzynapadowe wyładowania w
okolicach potylicznych są dość częstym znaleziskiem w bada-niach EEG, ciągle brak jest danych o korelacji występowania tych zmian ze stanem klinicznym pacjentów. W prezentowa-nej pracy podjęto próbę określenia takiej zależności. Materiał i
metody: Do prezentowanego badania włączono 31 pacjentów z
padaczką, w wieku od 12 miesięcy do 10 lat, u których wystę-powały w zapisie międzynapadowym wyładowania w okolicach potylicznych. Wyniki: Większość z pacjentów miała napady o podłożu objawowym (71%). Wykazano, że obraz kliniczny napa-dów, rodzaj zmian w EEG oraz typ stosowanej terapii przeciw-padaczkowej różnią się znacznie pomiędzy grupami pacjentów z etiologią objawową a idiopatyczną napadów. Wnioski: Uzy-skane wyniki wydają się mieć dość istotne znaczenie, zwłasz-cza w tych typach napadów, które stanowią często problem diagnostyczny, gdzie przewidywanie przebiegu klinicznego na podstawie rodzaju zmian międzynapadowych może znacznie poprawić jakość i szybkość zastosowania procedur diagno-stycznych i terapeutycznych.
Słowa kluczowe: padaczka, dzieci, wyładowania w okolicach
potylicznych
Occ�p��al ep�lep��form d�scharges �OED� are rela��vely commo� f��d��g �� �he EEG, espec�ally �� ch�ldre�. They ch�ldre�. They still remain underestimated as little attention has been paid �o �he�r rela��o� �o �he cl���cal s�a�us of �hese pa��e��s, ��clud-��g se��ure e��ology, sem�ology or prog�os�s.
OED appears �� bo�h �d�opa�h�c a�d symp�oma��c ep�-lep��c sy�dromes. Amo�g �d�opa�h�c group, I��er�a��o�al League Aga��s� Ep�lepsy �� 1989 d�s���gu�shed early o�se� be��g� ch�ldhood occ�p��al se��ures �E�O�, �a�ay�-o�opoulos sy�drome, ��� a�d la�e o�se� �d�opa�h�c ch�ld-hood occ�p��al ep�lepsy of Gas�au� �ype �LIO��. Desp��e �he ev�de�ce �ha� �� �s �o� “pure” occ�p��al ep�lepsy, �he �ew ILAE repor� ma���a��s �he same descr�p��ve �ame: Early O�se� �e��g� Ch�ldhood Occ�p��al Ep�lepsy ��a�ay�-o�opoulos Type�, w��h �he remark �ha� “�he co�s�s�e�cy of local��a��o� rema��s co��rovers�al �� �h�s sy�drome”[1].
E�O� �s co��ec�ed w��h be��g� course a�d ma��ly au�o�om�c symp�oms: �c�al �ausea, re�ch��g a�d vom����g.
V�sual symp�oms are excep��o�al a�d co�vuls�o�s ex�s�s �� abou� half of �he cases. Au�o�om�c se��ures occur ma��ly dur��g ��gh� sleep. �revale�ce may be h�gh, affec���g eve� 13% of ch�ldre� 3 �o 6 years of age w��h o�e or more �o�-febr�le se��ures. �rog�os�s �s excelle�� [2].
LIO� symp�oms ��clude freque�� a�d br�ef v�sual se��ures, dev�a��o� of �he eyes a�d head, eyel�d flu��er-��g, complex v�sual halluc��a��o�s, �llus�o�s, ��freque��ly e�d��g w��h co�vuls�o�s. �rog�os�s of �h�s �ype of ep�lepsy �s worse �ha� �� E�O�[3,4].
Charac�er�s��c occ�p��al EEG f��d��gs a�d cl���cal s�a�us, es��ma�ed o� �he base of �he �eurolog�cal exam��a-��o� a�d �eurorad�olog�cal ��ves��gaexam��a-��o�s, e�able �o d�ag-�ose symp�oma��c ep�lepsy sy�dromes a�d recog���e �he e��ology. Major d�ag�os��c f��d��gs ��d�ca�e d�ffere�� �ypes of cor��cal dysplas�as, occ�p��al calc�f�ca��o�s of�e� asso-c�a�ed w��h coel�ac d�sease, vascular or me�abol�c or�g��n [5,6]. �rese�ce of psychomo�or re�arda��o� ��d�ca�es
symp-
M. Mazurkiewicz-Bełdzińska, M. Szmuda, A. Matheisel
Neurologia Dziecięca p R A C A O R Y G I N A L N A / O R I G I N A L A R T I C L E
�oma��c e��ology of �he ep�lepsy �oo. There �s s��ll l���le k�o-wledge abou� �he cl���cal p�c�ure of ep�leps�es w��h OED �� ch�ldhood, espec�ally �� symp�oma��c group [7]. The a�m of our s�udy was �o de�erm��e �he cl���cal p�c�ure �se��ure sem�ology� a�d o�her aspec�s �e��ology, �rea�me�� ou�come a�d prog�os�s� �� a group of ep�lep��c ch�ldre� w��h OED in order to find possible predictable features for seizures outcome.
MATERIALS AND METHODS
We �de���f�ed ch�ldre� w��h OEDs us��g a da�abase of EEG collec�ed a� ped�a�r�c v�deo-mo���or��g u��� a� Depar�me�� of Developme��al Neurology a� Med�cal U��vers��y of Gda�sk. There were �h�r�y o�e ch�ldre� �18 boys, 13 g�rls� ��cluded �� �he s�udy. All pa��e��s who prese��ed cl���cal se��ures a�d ���er�c�al EEGs w��h occ�p��al ep�lep��form d�scharges were ��cluded, pa��e��s who had d�scharges �� o�her loca��o�s �� add���o� �o OED’s were excluded from �he s�udy. The age of ch�ldre� osc�lla�ed be�wee� 12 mo��hs a�d 10 years. Ep�lepsy was co�s�dered symp�oma��c whe� psychomo�or re�arda��o� a�d/or ab�ormal���es a� �eurolog�cal as well as �eurorad�olog�cal exam��a��o�s were fou�d. �e��ure sem�-ology was assessed mos�ly by ���erv�ew��g �he pare��s or careg�vers, some��mes also by ch�ldre�, espec�ally whe� �he v�sual symp�oms were prese��.
RESULTS
F�rs� se��ures �� �he ��ves��ga�ed group occurred be�wee� 12 mo��hs a�d 6 years of age. Age a� se��ure o�se� �� a�aly�ed group of ch�ldre� w��h �d�opa�h�c a�d symp�oma��c se��ures �s prese��ed �� �he f�gure 1.
Fig. 1. Age at seizure onset in children with occipital
epilepti-form discharges.
9 ch�ldre� were class�f�ed as hav��g �d�opa�h�c ep�lepsyclass�f�ed as hav��g �d�opa�h�c ep�lepsy �29%� a�d 22 ch�ldre� were class�f�ed as symp�oma��c �71%�. I� �he symp�oma��c group �he e��olog�es ��cluded: cerebral palsy, focal cor��cal dysplas�as, age�es�s of corpus callosum, �umors �DNET, as�rocy�oma, ga�gl�ogl�oma�, ��fec��o�s, u�d�ag�osed me��al re�arda��o� a�d occ�p��al calcifications. Precise numbers of these cases are presented �� �he �able I.
Table I. Etiology of the symptomatic seizures in the group of
children included in a study.
Etiology Number
Cerebral palsy 8
Focal cortical dysplasias 5
Agenesis of corpus callosum 1
Tumours: - DNET - Astrocytoma - Ganglioglioma 3 1 1 1 Infection 2
Undiagnosed mental retardation 2 Occipital calcifications: - Coeliac disease - Other 2 1 1 �em�ology of se��ures �s prese��ed �� �he �able II.
Table II. Semiology of seizures in a group of children included
in the study.
Seizures semiology Idiopathic Symptomatic
Elementary visual hallucinations 2 7 Complex visual hallucinations 2 3
Blindness 0 3
Ictal/postictal headaches 4 7
Ictal vomiting 7 2
Tonic deviation of the eyes 8 6 Oculoclonic movements or
nystagmus 0 5
Repetitive eyelid closure or
eyelid fluttering 2 6
Tonic-clonic seizures 4 11
Myoclonic seizures 0 3
Absence seizures 0 2
�ymp�oma��c se��ures usually las�ed less �ha� 5 m��-u�es a�d �hese w��h v�sual symp�oms were eve� shor�er. Id�opa�h�c se��ures usually las�ed lo�ger �ha� 5 m��u�es. �e��ure freque�cy from �he o�se� �o �he ��me whe� a���ep�-lep��c �herapy was ���roduced �s demo�s�ra�ed �� �he f�gure 2.
padaczki z międzynapadowymi wyładowaniami w okolicach potylicznych u dzieci
Vol. 19/010, nr 7
Fig. 2. Seizure frequency before introducing antiepileptic
therapy in analyzed group of patients.
Major��y of ��cluded ch�ldre� rece�ved mo�o�herapy: carbama�ep��e or oxcarba�ep��e. The res� were �rea�ed w��h poly�herapy – a comb��a��o� of: carbama�ep��e, valpro�c ac�d, �op�rama�e, lamo�r�g��e a�d leve��race�am. Modes of �rea�me�� are prese��ed �� �he �able III.
Table III. Modes of treatment in the group of children included
in the study Rodzaj terapii w grupie badanej
Treatment Idiopathic Symptomatic
Monotherapy 8 10
Polytherapy 1 12
The EEG fea�ure wh�ch s�g��f�ca��ly sugges�ed symp-�oma��c form was �he prese�ce of ab�ormal backgrou�d ac��v��y �pos�er�or la�eral��ed slow-waves�. De�a�ls regard-��g backgrou�d ac��v��y �� �d�opa�h�c a�d symp�oma��c se�-�ures are show� �� �he f�gure 3. �pec�f�c EEG f��d��gs �� occ�p��al reg�o�s are prese��ed �� a �able IV.
Fig. 3. Background activity in EEG recordings in patients with
idiopathic and symptomatic seizures.
Table IV. Specific epileptiform discharges in occipital regions
in patients with idiopathic and symptomatic seizures.
Epileptiform discharges Idiopathic Symptomatic
Occipital spike or polyspike 8 17 Sharp wave slow wave or
spike-wave complexes
locali-zed in occipital regions 1 11
Occipital photosensivity 5 14
DISCUSSION
Our goal was �o f��d charac�er�s��c fea�ures for �d�opa�h�c a�d symp�oma��c form of ep�leps�es w��h occ�p��al d�scharges, as well as �o compare cl���cal p�c�ure, e��ology, �rea�me�� ou�-come a�d prog�os�s be�wee� �hese �wo forms. I� mos� of �he cases of our s�udy ep�lepsy proved �o be symp�oma��c. I� some of �he cases �he s�ruc�ural ab�ormal��y of �he bra�� was proved by �euro�mag��g ��umors, malforma��o�s, e�c.�, in some of them we found the coincidence between neuro-log�cal a�d me��al s�a�e �cerebral palsy, me��al re�arda��o�� and epileptic seizures. As it is mentioned in special report of ILAE [8] �he �erm “symp�oma��c” �s a �ru�sm – all ep�lepsy �s symp�oma��c of some�h��g. Hav��g d�v�ded our pa��e��s ���o �wo groups, we excluded from �he symp�oma��c group �hese pa��e��s who suffer from ep�lepsy o�ly. ��m�lar da�a may be also fou�d �� �he l��era�ure [5]. �em�ology of se�-�ures s�g��f�ca��ly var�ed �� �d�opa�h�c a�d symp�oma��c group: major��y of ch�ldre� from symp�oma��c group expe-r�e�ced eleme��ary v�sual halluc��a��o�s a�d �o��c-clo��c se��ures, whereas ch�ldre� from �d�opa�h�c group prese��ed more of�e� au�o�om�c symp�oms a�d �o��c dev�a��o� of �he eyes. I��eres���gly, we fou�d �ha� �� early-school ch�ldre� �he v�sual symp�oms seem �o� �o be as rare as prev�ously repor�ed [9]. Moreover, we fou�d �ha� �d�opa�h�c se��ures las�ed lo�ger �ha� symp�oma��c o�es. U�ders�a�d��gly poly-�herapy was ���roduced s�g��f�ca��ly more of�e� �� a group of symp�oma��c se��ures.
There were also d�ffere�ces �� �ypes of ep�lep��form d�s-charges �� �d�opa�h�c a�d symp�oma��c group. �rese�ce of sp�ke-wave or sharp wave - slow wave complexes s�g��f�-ca��ly ��d�ca�ed �he symp�oma��c or�g�� of se��ures, also �here were s�g��f�ca��ly more pa��e��s w��h occ�p��al pho-�ose�s���v��y �� symp�oma��c group. There were �o� e�ough pa��e��s, �� order �o perform �he correla��o�, be�wee� �he ��flue�ce of �he s�de of cha�ges �la�eral��a��o�� o� prog-nosis.
There are o�ly few s�ud�es descr�b��g pa��e��s w��h OED �� a v�ew of �he he�eroge�eous group of ch�ldre� a�d a�aly���g correla��o� be�wee� OED a�d spec�f�c fea�ures, such as d�ffere�� e��ology, cl���cal ma��fes�a��o�s, �rea�-me�� ou�come a�d prog�os�s [5,7,9-11]. V�sual ��c�de��s a�d �c�al or pos�-�c�al headache, �ha� are freque��ly prese�� �� ch�ldre� w��h OED, lead �o �he co�clus�o� �ha� �he majorusion that the major d�ffere���al d�ag�os�s �s a m�gra��e. �a�ay�o�opoulos
��d�-
M. Mazurkiewicz-Bełdzińska, M. Szmuda, A. Matheisel
Neurologia Dziecięca p R A C A O R Y G I N A L N A / O R I G I N A L A R T I C L E
ca�es [12] �ha� �h�s �s o�e of �he reaso�s why phys�c�a�s of�e� erro�eously d�ag�ose m�gra��e w��h aura, bas�lar aura, bas�lar or acephal�c m�gra��e �� pa��e��s w��h OED. Trea�me�� w��h a���ep�lep��c drugs �AEDs� �s of�e� delayed or �ever adm���s�ered before f�rs� �yp�cal ep�lep��c se��ure. I� some cases �LIOE, bra�� �umor, ��fec��o� of �he ce��ral �ervous sys�em� �he delay �� �rea�me�� s�g��f�ca��ly de�er�ora�es �he prog�os�s.
CONCLUSIONS
Desp��e �he d�ffere���al d�ag�os�s, �he key f��d��g of �he s�udy �s �ha� �he �ype of OED prese�� �� ch�ldre� w��h se�-�ures could pred�c� �he symp�omab�l��y of ep�lepsy a�d �mply more aggress�ve d�ag�os��c a�d �rea�me�� procedures.
Fur�her prospec��ve cl���cal s�ud�es are �eeded, espe-c�ally �o prov�de �he de�a�led correla��o� be�wee� �he cl���-cal a�d EEG fea�ures a�d prog�os�s of �h�s very complex a�d some��mes d�ff�cul� �o �rea� form of ep�lepsy �� ch�l-dren.
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Correspondence:
Maria Mazurkiewicz-Bełdzińska, Klinika Neurologii Rozwojowej Gdański Uniwersytet Medyczny, ul. Dębinki 7, 80-211 Gdańsk mmazur@amg.gda.pl