OBJECTIVE: To characterize neurological involvement in juvenile systemic lupus erythe-matosus. METHOD: The charts of all patients with the diagnosis of systemic lupus erythematosus before the age of 16 years, followed at the Rheumatology Unit of Pequeno Príncipe Hospital, from January 1992 to January 2006, were retrospectively reviewed, highlighting neuropsychiatric aspects. RESULTS: Forty-seven patients were included. Neuropsychiatric syndromes were found 29 (61.7%): seizures (17 / 36.2%), intractable headache (7 / 14.9%), mood disorders (5 / 10.6%), cerebrovascular disease (4 / 8.5%), acute confusional state (3 / 6.4%), aseptic meningitis (3 / 6.4%), psychosis (3 / 6.4%), chorea (3 / 6.4%), Guillain-Barré syndrome (2 / 4.3%) and cranial neuropathy (1 / 2.1%). Morbidity indexes (SLEDAI and SLICC) were higher among patients with neuropsychiatric manifestations (p<0.05). CONCLUSION: Neuropsychiatric syndromes are frequent, and add significant morbidity to juvenile systemic lupus erythematosus.

-Introduction: X-linked lissencephaly with ambiguous genitalia (XLAG) is a recently described genetic disorder caused by mutation in the aristaless-related homeobox (ARX) gene (Xp22.13). Patients p resent with lissencephaly, agenesis of the corpus callosum, re f r a c t o ry epilepsy of neonatal onset, acquire d m i c rocephaly and male genotype with ambiguous genitalia. Case re p o rt: Second child born to healthy nonconsanguineous parents, presented with seizures within the first hour of life that remained refractory to phenobarbital, phenytoin and midazolam. Examination identified micro c e p h a l y, axial hypotonia, pyramidal signs and ambiguous genitalia. EEG showed disorganized background activity and seizures starting at the right midtemporal, central and occipital regions. MRI showed diffuse pachygyria, moderate thickening of the cortex, enlarged ventricles, agenesis of the corpus callosum and septum pellucidum. K a ryotype showed a 46,XY genotype. Additional findings were hypercalciuria, vesicoureteral reflux, patent ductus arteriosus and chronic diarrhea.KEY WORDS: corpus callosum, ambiguous genitalia, epilepsy, ARX gene.Lisencefalia, genitália ambígua e epilepsia refratária: relato de caso da síndrome XLAG RESUMO -I n t rodução: Lisencefalia com genitália ambígua ligada ao X (XLAG) é doença genética re c e n t emente descrita, causada por mutação no genea r i s t a l e s s -related homeobox (ARX) (Xp22.13). Os pacientes a p resentam lisencefalia, agenesia de corpo caloso, epilepsia refratária com início no período neonatal, m i c rocefalia adquirida e genótipo masculino com genitália ambígua. Relato de caso: Segundo filho de pais não-consangüíneos, apresentou crises na primeira hora de vida que permaneceram refratárias a fenobarbital, fenitoína e midazolam. Apresentava microcefalia, hipotonia axial, sinais de liberação piramidal e genitália ambígua. EEG demonstrou atividade de base desorganizada, crises convulsivas com início nas regiões temporal-média, central e occipital direitas. RNM demonstrou paquigiria difusa, moderado espessamento do córtex, ventrículos aumentados, agenesia de corpo caloso e septo pelúcido. Cariótipo evidenciou genótipo 46,XY. Achados adicionais foram: hipercalciúria, refluxo vésico-ureteral, ducto arterioso persistente e diarréia crônica.

-Introduction: septo-optic dysplasia (De Morsier syndrome) is defined as the association between optic nerve hypoplasia, midline central nervous system malformations and pituitary dysfunction. Case re�� port: third child born to nonconsanguineous parents, female, adequate pre-natal medical care, cesarean term delivery due to breech presentation, Apgar score 3 at the first minute and 8 at 5 minutes, symptomatic hypoglycemia at 18 hours. Neurological follow-up identified a delay in acquisition of motor and language developmental milestones. Epileptic generalized seizures began at 12 months and were controlled with phenobarbital. EEG was normal. MrI revealed agenesis of the pituitary stalk, hypoplasia of the optic chiasm and periventricular nodular heterotopia. Ophthalmologic evaluation showed bilateral optic disk hypoplasia. Endocrine function laboratory tests revealed primary hypothyroidism and hyperprolactinemia. Con�� clusion: the relevance of this case report relies on its uniqueness, since periventricular heterotopia had not been described in association with septo-optic dysplasia until 2006.KEy wOrDs: De Morsier syndrome, septo-optic dysplasia, periventricular nodular heterotopia, primary hypothyroidism.síndrome de De Morsier associada a heterotopia nodular periventricular: relato de caso rEsuMO -Introdução: Displasia septo-óptica (síndrome de De Morsier) é definida como a associação entre hipoplasia do nervo óptico, malformações de linha média do sistema nervoso central e disfunção pituitária. Relato de caso: terceiro filho, pais não consangüíneos, sexo feminino, pré-natal adequado, parto cesário a termo por apresentação pélvica, Apgar 3 no primeiro minuto e 8 no quinto minuto, hipoglicemia sintomática com 18 horas de vida. Durante o acompanhamento neurológico identificou-se atraso na aquisição dos marcos de desenvolvimento motor e linguagem. Crises epilépticas generalizadas iniciaram com 12 meses de vida sendo controladas com fenobarbital. EEG era normal. ressonância magnéti-ca revelou agenesia de haste pituitária, hipoplasia de quiasma óptico e heterotopia nodular periventricular. Avaliação oftalmológica demonstrou hipoplasia bilateral de disco óptico. Investigação da função endócrina revelou hipotireoidismo primário e hiperprolactinemia. Conclusão: A relevância deste relato reside em seu ineditismo, já que heterotopia periventricular não havia sido descrita em associação com displasia septo-óptica até 2006.PAlAvrAs-ChAvE: síndrome de De Morsier, displasia septo-óptica, heterotopia nodular periventricular, hipotireoidismo primário.unidade de Neurologia Infantil Pequeno Príncipe, Curitiba Pr, brasil (uNIPP):

INTRODUCTION: Sleep spindles, the hallmark of sleep synchronization in stage 2 non-REM sleep, are rhythmic and monomorphic waves, between 10 and 14 Hz, with a maximum amplitude in the vertex (Cz), with bilateral irradiation to central regions (C3 and C4). OBJECTIVE: In this article we present an overview of sleep spindles ranging from well established concepts of morphology, generation mechanisms, maturational features, abnormal patterns and pathological aspects since the knowledge is important to identify the normal and abnormal patterns. CONCLUSION: Centro-parietal and frontal spindle are normal pattern and extreme spindle is abnormal, mostly found in children mentally retarded up to 5 years-old. In this review research boundaries of memory consolidation, synaptic potentiation and brain plasticity were also presented.

Detailed knowledge of electroencephalographic patterns accompanying epileptic seizures in children is paramount to the correct identification of epileptic seizures and syndromes. In this article, we present a review of ictal patterns of different seizure types in children, illustrating with examples collected in our video-EEG laboratory at Pequeno Príncipe Hospital.Keywords: electroencephalography; epilepsy; ictal pattern. RESUMO Padrão ictal em crianças: uma revisão ilustradaO conhecimento detalhado dos padrões eletrencefalográficos que acompanham crises epilépticas em crianças é essencial para a correta identificação de crises e síndromes epilépticas. Neste artigo apresentamos uma revisão dos padrões ictais de diferentes crises em crianças, ilustrando com exemplos coletados na nossa unidade de vídeo-eletrencefalograma no Hospital Pequeno Príncipe.Unitermos: eletrencefalograma; epilepsia; padrão ictal.