R E S U M O -EscalasReliability of neurological assessment scales in patients with stroke ABSTRACT -Scales for the assessment of neurological impairment are employed in clinical trials, outcome evaluation and in therapeutic decisions. We evaluated the reliability of the Portuguese version of the Rankin Scale (RS), Barthel Index (BI) and the NIHSS (National Institute of Health Stroke Scale) in 51 stroke patients, estimating the agreement of the results for examiners. The interrater reliability was measure d using the Kappa Coefficient and Intraclass Correlation Coefficient. The RS showed moderate, substantial and excellent coefficients of agreement. The BI showed from substantial to excellent coefficients of a g reement as a whole and also for its constituents. This scale was stratified in prognostic and functional g roups, both presenting coefficients of agreement from substantial to excellent. The NIHSS pre s e n t e d excellent agreement on its total and substantial agreements on its individual items. The NIHSS was also stratified in groups of points, which presented moderate or excellent coefficient, and in groups of congru o u s items, wich showed a moderate to excellent agreement. These results suggest that the Portuguese version of the RS, BI and the NIHSS present adequate reliability.

-Hippocampal sclerosis (HS) is the commonest pathology in epileptic patients undergoing temporal lobe epilepsy surgery. Beside, there are an increased density of corpora amylacea (CA) founded in 6 to 63% of those cases. Objective: verify the presence of CA and the clinical correlates of their occurrence in a consective series of patients undergoing temporal surgery with diagnosis of HS. Method: We reviewed 72 hippocampus specimens from January 1997 to July 2000. Student's t test for independent, samples, ANOVA and Tukey test were performed for statistical analysis. Results: CA were found in 35 patients (49%), whose mean epilepsy duration (28.7 years) was significantly longer than that group of patients without CA (19.5 years, p= 0.001). Besides, when CA were found, duration was also significantly correlated with distribution within hippocampus: 28.7 years with diffuse distribution of CA, 15.4 with exclusively subpial and 17.4 years with distribution subpial plus perivascular (p= 0.001). Conclusion: Our findings corroborate the presence of CA in patients with HS and suggest that a longer duration of epilepsy correlate with a more distribution of CA in hippocampus.KEY WORDS: hippocampal sclerosis, corpora amylacea, temporal lobe epilepsy. Corpos amiláceos associados à esclerose hipocampal na epilepsia do lobo temporalRESUMO -A esclerose hipocampal (EH) é a patologia mais observada em lobectomias temporais de pacientes com epilepsia intratável. Além dos achados histopatológicos clássicos existe a presença de corpos amiláceos (CA) em até 60% dos hipocampos ressecados. Objetivo: Verificar a presença de corpos amiláceos em hipocampos de pacientes submetidos a cirurgia para tratamento de epilepsia e verificar se havia diferenças clínicas entre pacientes que apresentavam CA e aqueles em que eles não foram encontrados. Método: Foram revisados 80 hipocampos ressecados entre janeiro de 1997 a dezembro de 2000 e, posteriormente, os prontuários de 72 dos pacientes. Foi realizado o teste t de Student para amostras independentes, ANOVA e posteriormente o teste de Tukey. Resultados: Dos setenta e dois pacientes estudados, 40 eram homens (55,6%) e trinta e dois mulheres (44,4%). Trinta e cinco (48,6%) apresentavam CA e em trinta e sete (51,4%) não foram encontrados. Houve diferença estatisticamente significativa entre a média de tempo de evolução da epilepsia: o grupo com CA apresentava tempo maior de evolução (28,97 anos) em relação ao grupo que não apresentava CA (19,54 anos), com p= 0,001. Também observou-se diferença significativa quando foi comparada a localização de corpos amiláceos com o tempo de evolução da epilepsia: os pacientes com CA presentes difusamente no hipocampo apresentavam maior tempo de epilepsia (p= 0,001). Conclusão: Observamos que o nosso estudo confirma a presença de corpos amiláceos em hipocampos de pacientes com EH e sugerimos que quanto maior o período de epilepsia maior é a distribuição de CA no hipocampo PALAVRAS-CHAVE: esclerose hipocampal, corpora amylacea, epilepsia de lobo temporal.

Pyridoxine dependency and congenital hypophosphatasia are unusual metabolic disorders. We report a female infant born from healthy consanguineous parents with shortening of limbs, detected during pregnancy by ultrasonography. Immediately after delivery, the baby was admitted to the neonatal intensive care unit because of respiratory distress. A bone radiograph showed hypomineralization of all bones, and serum alkaline phosphatase was very low (10 U/L). Within the first day of life, seizures (focal clonic and tonic) started. The seizures were refractory to phenobarbital and other antiepileptic drugs. The first electroencephalogram (EEG) showed a burst-suppression pattern. Pyridoxine was administered (50 mg/kg) and completely controlled the seizures. Antiepileptic drugs were discontinued, and a maintenance dose of pyridoxine (10 mg/day) was established. A postpyridoxine EEG revealed the disappearance of the burst-suppression pattern. The patient died at age 26 days. Pyridoxine-dependent seizures, when recognized early and treated, have a more favorable prognosis. However, hypophosphatasia detected at birth almost always has a lethal outcome.

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