Benign focal seizures of adolescence and neuropsychological findings in patients from community

RATIONALE: Benign focal seizures of adolescence (BFSA) described by Loiseau et al in 1972, is considered a rare entity, but maybe underdiagnosed. Although mild neuropsychological deficits have been reported in patients with benign epilepsies of childhood, these evaluations have not so far been described in BFSA. The aim of this study is to evaluate neuropsychological functions in BFSA with new onset seizures (<12 months). METHODS: Eight patients with BFSA (according to Loiseau et al, 1972, focal or secondarily tonic clonic generalized seizures between the ages of 10-18 yrs., normal neurologic examination, normal EEG or with mild focal abnormalities) initiated in the last 12 months were studied between July 2008 to May 2009. They were referred from the Pediatric Emergency Section of the Hospital Universitário of the University of Sao Paulo, a secondary care regionalized facility located in a district of middle-low income in Sao Paulo city, Brazil. The study was approved by the Ethics Committee of the Institution. All patients performed neurological, EEG, brain CT and neuropsychological evaluation which consisted of Raven's Special Progressive Matrices - General and Special Scale (according to different ages), Wechsler Children Intelligence Scale-WISC III with ACID Profile, Trail Making Test A/B, Stroop Test, Bender Visuo-Motor Test, Rey Complex Figure, Rey Auditory Verbal Learning Test-RAVLT, Boston Naming Test, Fluency Verbal for phonological and also conceptual patterns - FAS/Animals and Hooper Visual Organization Test. For academic achievement, we used a Brazilian test for named "Teste do Desempenho Escolar", which evaluates abilities to read, write and calculate according to school grade. RESULTS: There were 2 boys and 6 girls, with ages ranging from 10 yrs. 9 m to 14 yrs. 3 m. Most (7/8) of the patients presented one to two seizures and only three of them received antiepileptic drugs (AEDs). Six had mild EEG focal abnormalities and all had normal brain CT. All were literate, attended regular public schools and scored in a median range for IQ, and seven showed discrete higher scores for the verbal subtests. There were low scores for attention in different modalities in six patients, mainly in alternated attention as well as inhibitory subtests (Stroop test and Trail Making Test part B). Four of the latter cases who showed impairment both in alternated and inhibitory attention were not taking AEDs. Visual memory was impaired in five patients (Rey Complex Figure). Executive functions analysis showed deficits in working memory in five, mostly observed in Digits Indirect Order and Arithmetic tests (WISC III). Reading and writing skills were below the expected average for school grade in six patients according to the achievement scholar performance test utilized. One patient of this series who had the best scores in all tests was taking phenobarbital. CONCLUSIONS: Neuropsychological imbalance between normal IQ and mild dysfunctions such as in attention domain and in some executive abilities like working memory and planning, as well as difficulties in visual memory and in reading and writing, were described in this group of patients with BFSA from community. This may reflect mild higher level neurological dysfunctions in adolescence idiopathic focal seizures probably caused by an underlying dysmaturative epileptogenic process. Although academic problems often have multiple causes, a specific educational approach may be necessary in these adolescents, in order to improve their scholastic achievements, helping in this way, to decrease the stigma associated to epileptic seizures in the community.

Children`s oral production of narratives and constructive memory using prior reading of pictures

With the purpose of approximating two issues, oral narrative and constructive memory, we assume that children, as well as adults, have a constructive memory. Accordingly, researchers of the constructive memory share with piagetians the vision that memory is an applied cognition. Under this perspective, understanding and coding into memory constitute a process which is considered similar to the piagetian assimilation of building an internal conceptual representation of the information (hence the term constructive memory. The objective of this study is to examine and illustrate, through examples drawn from a research about oral narrative with 5, 8 and 10 years old children, the extent to which the constructive memory is stimulated by the acquisition of the structures of knowledge or ""mental models"" (schemes of stories and scenes, scripts), and if they automatically employ them to process constructively the information in storage and rebuild them in the recovery. A sequence of five pictures from a book without text was transformed into computerized program, and the pictures were thus presented to the children. The story focuses on a misunderstanding of two characters on a different assessment about a key event. In data collection, the demands of memory were preserved, since children narrate their stories when the images were no longer viewed on the computer screen. Each narrative was produced as a monologue. The results show that this story can be told either in a descriptive level or in a more elaborated level, where intentions and beliefs are attributed to the characters. Although this study allows an assessment of the development of children`s capabilities (both cognitive and linguistic) to narrate a story, there are for sure other issues that could be exploited.

Characterization of language and reading skills in familial polymicrogyria

Polymicrogyria (PMG) is a malformation of cortical development characterized by an excessive number of small gyri and abnormal cortical lamination, giving the cortical surface an irregular and gross appearance. The severity of clinical manifestations correlates with the extent of cortical involvement. The objective of the present study was to describe three families with linguistic features of developmental language disorder and reading impairment, and to establish a neuroanatomic correlation through neuroimaging. Subjects have been submitted to a comprehensive protocol including psychological assessment, language evaluation, neurological examination, and neuroimaging investigation. In our families, children usually had the diagnosis of developmental language disorder while adults had the diagnosis of reading impairment. MRI showed perisylvian polymicrogyria in several subjects of each family. Our data support the idea that there is a co-occurrence of developmental language disorder and reading impairment and both conditions may be associated with polymicrogyria. (c) 2007 Elsevier B.V. All rights reserved.

Comparison of reading performance after bilateral implantation of multifocal intraocular lenses with+3.00 or+4.00 diopter addition

PURPOSE To compare reading ability after cataract surgery and bilateral implantation of multifocal intraocular lenses (IOLs) with a +3 00 diopter (D) addition (add) or a +4 00 D add SETTING Department of Ophthalmology, University of Sao Paulo, Sao Paulo, Brazil DESIGN Prospective comparative study METHODS Patients scheduled for cataract surgery were randomly assigned to bilateral implantation of an aspheric AcrySof ReSTOR multifocal IOL with a +3 00 diopter (D) addition (add) or a +4 00 D add The reading speed, critical print size, and reading acuity were measured binocularly with best correction using MNREAD acuity charts 6 months after surgery Patients were tested with the chart at the best patient-preferred reading distance and at 40 cm Binocular uncorrected and best distance-corrected visual acuities at far and near were also measured RESULTS The study enrolled 32 patients At the best reading distance the results were similar between the 2 IOL groups in all reading parameters When tested at 40 cm, reading speed at all print sizes from 03 to 00 (all P< 001), critical print size (P< 001) and reading acuity (P = 014) were statistically significantly better in the +3 00 D IOL group than in the +4 00 DIOL group Uncorrected and corrected visual acuities at far and near were similar between the 2 groups CONCLUSION Although the 2 IOL groups had similar performance in reading parameters, patients had to adjust to their best reading distance The +3 00 D IOL performed better than the +4 00 D IOL at 40 cm

Immunohistochemical detection of polyductin and co-localization with liver progenitor cell markers during normal and abnormal development of the intrahepatic biliary system and in adult hepatobiliary carcinomas

The longest open reading frame of PKHD1 (polycystic kidney and hepatic disease 1), the autosomal recessive polycystic kidney disease (ARPKD) gene, encodes a single-pass, integral membrane protein named polyductin or fibrocystin. A fusion protein comprising its intracellular C-terminus, FP2, was previously used to raise a polyclonal antiserum shown to detect polyductin in several human tissues, including liver. In the current study, we aimed to investigate by immunohistochemistry the detailed polyductin localization pattern in normal (ductal plate [DP], remodelling ductal plate [RDP], remodelled bile ducts) and abnormal development of the primitive intrahepatic biliary system, known as ductal plate malformation (DPM). This work also included the characterization of polyductin expression profile in various histological forms of neonatal and infantile cholestasis, and in cholangiocellular carcinoma (CCC) and hepatocellular carcinoma (HCC). We detected polyductin expression in the intrahepatic biliary system during the DP and the RDP stages as well as in DPM. No specific staining was found at the stage of remodelled bile ducts. Polyductin was also detected in liver biopsies with neonatal cholestasis, including mainly biliary atresia and neonatal hepatitis with ductular reaction as well as congenital hepatic fibrosis. In addition, polyductin was present in CCC, whereas it was absent in HCC. Polyductin was also co-localized in some DP cells together with oval stem cell markers. These results represent the first systematic study of polyductin expression in human pathologies associated with abnormal development of intrahepatic biliary tree, and support the following conclusions: (i) polyductin expression mirrors developmental properties of the primitive intrahepatic biliary system; (ii) polyductin is re-expressed in pathological conditions associated with DPM and (iii) polyductin might be a potential marker to distinguish CCC from HCC.