[Neurocysticercosis in children: clinical study and follow-up of 112 patients]. / Neurocisticercosis en la infancia: estudio clínico y seguimiento de 112 casos.

INTRODUCTION: Neurocysticercosis (NC) is the infection of the central nervous system caused by the Taenia solium larva. It is related to a wide variety of clinical symptoms and pathological findings. AIM: Clinical study, diagnosis, treatment and evolution of 112 patients with NC. PATIENTS AND METHODS: 112 patients with NC, between 1 and 14 years of age, were evaluated and followed from 18 months up to 13 years. RESULTS: The most common clinical symptoms were epileptic seizures and signs of intracranial hypertension. The disease progressed as follows: active forms were seen in around 39% of the cases (viable cysts in 3% and transitional/granulomatous form in 36%), encephalitic form in 22% and inactive form (calcifications) in 39%. In the great majority of the cases, a treatment with anti-helmitic was not used. The control of the crises was positive in 86% of the cases -94% in the transitional forms, 93% in the inactive forms and 68% in the encephalitic form-. Recurrence of crises happened after suspension of the medication in 12,5% of the granulomatous form and in 11,2% of the inactive form. Neurological sequelae occurred only in the encephalitic form (12/25 patients). CONCLUSIONS: Clinical findings and clinical evolution of neurocysticercosis in children is related to the evolutive form of the disease. The clinical evolution, including control of the crises and radiological control, is benign in the inactive and active forms, except in the encephalitic forms. The extraparenquimatous form is quite rare in the pediatric group.

Neurocisticercosis en la infancia: estudio clínico y seguimiento de 112 casos / Neurocysticercosis in children: clinical study and follow-up of 112 patients

Introducción. La neurocisticercosis (NC) es la infeccióndel sistema nervioso central causada por larva de la Taenia solium.Está relacionada con una amplia variedad de manifestaciones clínicasy hallazgos patológicos. Objetivo. Estudio clínico, diagnóstico,tratamiento y evolución de 112 pacientes con NC. Pacientes y métodos.112 pacientes con NC de 1 a 14 años de edad, seguidos entre 18meses y 13 años. Resultados. los síntomas clínicos más comunes fueroncrisis epilépticas y signos de hipertensión endocraneal. Conrelación a la evolución de la enfermedad se encontraron formas activasen el 39% de los casos (quiste viable en el 3% y forma de transición/granular en el 36%), forma encefalítica en el 22% e inactiva(calcificaciones) en el 39%. En la gran mayoría de los pacientes nose realizó tratamiento con antihelmínticos. El control de las crisis selogró en el 86% de los casos –94% en las formas de transición, 93%en las formas inactivas y 68% en la forma encefalítica–. La recidivade las crisis después de la suspensión de la medicación ocurrió en12,5% en la forma granular y 11,2% en la forma inactiva. Sólo en laforma encefalítica se vieron secuelas neurológicas (12/25 pacientes).Conclusiones. Los hallazgos y la evolución clínica de la neurocisticercosisen la infancia están asociados con la forma evolutiva de laenfermedad. La evolución clínica, incluido el control de las crisis eimágenes, es benigna en las formas inactivas y activas, excepto enlas formas encefalíticas. La forma extraparenquimatosa es rara enla edad pediátrica

Introduction. Neurocysticercosis (NC) is the infection of the central nervous system caused by the Taenia soliumlarva. It is related to a wide variety of clinical symptoms and pathological findings. Aim. Clinical study, diagnosis, treatmentand evolution of 112 patients with NC. Patients and methods. 112 patients with NC, between 1 and 14 years of age, wereevaluated and followed from 18 months up to 13 years. Results. The most common clinical symptoms were epileptic seizuresand signs of intracranial hypertension. The disease progressed as follows: active forms were seen in around 39% of the cases(viable cysts in 3% and transitional/granulomatous form in 36%), encephalitic form in 22% and inactive form (calcifications)in 39%. In the great majority of the cases, a treatment with anti-helmitic was not used. The control of the crises was positivein 86% of the cases –94% in the transitional forms, 93% in the inactive forms and 68% in the encephalitic form–. Recurrenceof crises happened after suspension of the medication in 12,5% of the granulomatous form and in 11,2% of the inactive form.Neurological sequelae occurred only in the encephalitic form (12/25 patients). Conclusions. Clinical findings and clinicalevolution of neurocysticercosis in children is related to the evolutive form of the disease. The clinical evolution, includingcontrol of the crises and radiological control, is benign in the inactive and active forms, except in the encephalitic forms. Theextraparenquimatous form is quite rare in the pediatric group

Seizures associated with calcifications and edema in neurocysticercosis.

We describe eight cases of pediatric patients whose neuroimages performed after seizures revealed abnormalities that were compatible with edema surrounding calcified lesions and which disappeared in subsequent examinations.

Developmental milestones of vertically HIV infected and seroreverters children: follow up of 83 children.

The aim of the study was to detect neurological abnormalities in human immunodeficiency virus (HIV) infected children. This was achieved by a prospective evaluation, from November/1995 to April/2000, of 43 HIV infected children (group I) and 40 HIV seroreverters children (group II) through neurological exam and neurodevelopmental tests: Denver Developmental Screening Test (DDST) and Clinical Adaptive Test/Clinical Linguistic and Auditory Milestone Scale (CAT/CLAMS). A control group (III), of 67 children, were evaluated by CAT/CLAMS. Hyperactivity, irritability and hypotonia were the findings on neurological examination, without statistical differences between group I and II. On CAT/CLAMS, the group I developmental quotient (DQ) was significantly lower than the other groups. The same occurred in DDST, with group I presenting significantly more failures than group II. Nineteen HIV children of group I had brain computed tomographic scan, with abnormalities in three of them (basal ganglia calcification, white matter hypodensity and asymmetry of lateral ventricles). We conclude that in HIV infected children a neurodevelopment delay occur early in the disease, and it can be detected by screening tests.

Learning disabilities: analysis of 69 children.

With this article we intend to demonstrate the importance of evaluation and follow up of children with learning disabilities, through a multidisciplinary team. As well as to establish the need of intervention. We evaluate 69 children, from Aline Picheth Public School, in Curitiba, attending first or second grade of elementary school, through general and evolutionary neurological examination, pediatric checklist symptoms, and social, linguistic and psychological (WISC-III, Bender Infantile and WPPSI-figures) evaluation. The incidence was higher in boys (84,1%), familiar history of learning disabilities was found in 42%, and writing abnormalities in 56,5%. The most frequent diagnosis was attention deficit and hyperactivity disorder, in 39,1%. With this program, we aimed to reduce the retention taxes and stress the importance of this evaluation, and, if necessary, multidisciplinary intervention in the cases of learning disabilities.

[Rett syndrome: retrospective and prospective study of 28 patients]. / Síndrome de Rett: estudo retrospectivo e prospectivo de 28 pacientes.

From November 1982 to May 1999, 28 children with Rett syndrome were followed-up for a medium period of 6 years and 2 months. Regression of developmental milestones started at the age between 5 and 20 months. Nineteen cases of typical Rett syndrome had uneventful pre and perinatal periods, loss of previously acquired purposeful hand skills, mental and motor regression and developed hand stereotypies; sixteen had head growth deceleration and 12 gait apraxia. Nine patients were atypical cases, 2 formes frustres, 2 congenital, 3 with early seizure onset, 1 preserved speech and 1 male. Epilepsy was present in 21 patients, predominantly partial seizures and the drug of choice was carbamazepine (15 patients). In the initial evaluation most patients were distributed on Stages II and III and on follow-up on Stages III and IV. Three children died.

[Cerebral arterial aneurysm in a child with acquired immunodeficiency syndrome: case report]. / Aneurisma de artéria cerebral em criança com síndrome da imunodeficiência adquirida: relato de caso.

Cerebral aneurysmal arteriopathy of the circle of Willis is an uncommon manifestation of acquired human immunodeficiency virus (HIV) infection and up to now only 15 cases have been published in the literature. For this reason we add our experience of this rare case, and review the most important aspects related to this entity. The patient is a 6 year old male with perinatal transmitted AIDS, tetraparethic, developed symptoms characterized by episodes of dystonic postures. The computed tomography of the brain showed aneurismal arteriopathy of the circle of Willis. He had a previous normal examination. The review of the literature shows the pathological abnormalities of the affected vessels are mainly medial fibrosis, with destruction of the internal elastic lamina and intimal hyperplasia. The etiology for the vasculitis is unknown. Varicela zoster virus, as well as HIV by itself, can be related to the physiopathology of the vasculitis. On conclusion, it can be said that although uncommon, such complications are of great importance by the fact that the patients with vascular aneurismal arteriopathy are in high risk for vascular accidents, and once a diagnosis was made, death occurs in less than 6 months, according to the literature.

[Schizencephaly: report of eleven cases]. / Esquizencafalia: relato de onze casos.

A retrospective study comparing clinical and computerized tomography (CT) in 11 patients diagnosed as having schizencephaly was conducted. Seven of these patients were girls and four boys. Six of them had tetraparesis, three hemiparesis and one no motor deficits. Six had epilepsy and ten developmental delay. On CT examinations, 7 patients were found as having bilateral clefts and four unilateral defect. Eight had opened lip clefts and four had a closed lip defect. The commonest associated anomaly was an absent septum pellucidum (n=9), followed by subependymal nodules (n=4), hydrocephalus (n=2) and microcephaly (n=1). Despite magnetic resonance image is the gold-standard to diagnose neuronal migration anomalies, CT can be useful in showing typical aspects of schizencephaly.

Sudden unexpected, unexplained death in epilepsy autopsied patients.

Sudden unexpected, unexplained death in epilepsy (SUDEP) has been reported to be responsible for 2 to 17% of all deaths in patients with epilepsy. This study was conducted to determine the circumstances of SUDEP and the autopsy findings in these patients. Fifty-three individuals whose cause of death was related to epilepsy were identified and in 30 cases relatives or friends were interviewed about the circumstances of death and other information which allowed to classify the patients as SUDEP or not. The death certificates were also reviewed. We found 20 cases of SUDEP. Most of them were found dead lying on the bed with no evidence of seizure event, and most of them had pulmonary and/or cerebral edema as the cause of death. The incidence and the risk of SUDEP can only be fully ascertained if all sudden deaths had postmortem examination. Consensus in certifying SUDEP cases would allow better accuracy in national mortality rate.

Epilepsy in children with cerebral palsy.

OBJECTIVE: To describe the prevalence and characteristics of epilepsy in patients with cerebral palsy in a tertiary center. METHODS: a total of 100 consecutive patients with cerebral palsy were retrospectively studied. Criteria for inclusion were follow-up period for at least 2 years. Types and incidence of epilepsy were correlated with the different forms of cerebral palsy. Other factors associated with epilepsy such as age of first seizure, neonatal seizures and family history of epilepsy were also analysed. RESULTS: follow-up ranged between 24 and 151 months (mean 57 months). The overall prevalence of epilepsy was 62%. Incidence of epilepsy was predominant in patients with hemiplegic and tetraplegic palsies: 70.6% and 66.1%, respectively. First seizure occurred during the first year of life in 74.2% of patients with epilepsy. Generalized and partial were the predominant types of epilepsy (61.3% and 27.4%, respectively). Thirty-three (53.2%) of 62 patients were seizure free for at least 1 year. Neonatal seizures and family history of epilepsy were associated with a higher incidence of epilepsy. CONCLUSIONS: epilepsy in cerebral palsy can be predicted if seizures occur in the first year of life, in neonatal period and if there is family history of epilepsy.

The ring-type polymerase sliding clamp family.

Ring-type polymerases consist of a DNA polymerase, a ring-shaped sliding clamp protein and a clamp-loading complex. Sliding clamp proteins are found in all organisms and are called proliferating cell nuclear antigen (PCNA) in eukaryotes and the beta clamp in prokaryotes. Both PCNA and beta form a ring around DNA, which is made up of two subunits of three domains each in beta but three subunits of two domains each in PCNA. Despite this difference and a lack of detectable sequence homology, the structures of the two rings are very similar. The sliding clamp slides along DNA and tethers the polymerase to the DNA, enabling rapid and processive DNA replication.

[Intravenous immunoglobulin in children with Guillain-Barré syndrome]. / Imunoglobulina endovenosa em crianças com síndrome de Guillain-Barré.

We report our experience with intravenous immunoglobulin (IVIG), plasmapheresis and supportive care in 13 patients with the Guillain-Barré syndrome. Seven of 13 patients received IVIG, 2 plasmapheresis and 4 supportive care. At 15th day after IVIG administration, all patients in this group had improved at least one disability grade. In the plasmapheresis group, 1 improved at 5th day after the procedure. Two of the 4 patients that received supportive care improved at 20th day of evaluation. In the IVIG group, the final scores were lower and had no relapses. These results suggest faster clinical improvement with IVIG when compared with supportive measures.

Cerebellar vermis hypoplasia - non progressive congenital ataxia: clinical and radiological findings in a pair of siblings.

We describe the clinical and radiological findings of a pair of siblings with cerebellar vermis hypoplasia and compare them with the literature. Both of them present pregnancies and deliveries uneventful and both presented some grade of hypotonia, ataxia, ocular motor abnormalities and mild motor delay and slurred speech. These siblings meet many of the criteria described in non-progressive congenital ataxia in which can occur familial cases with cerebellar atrophy, including vermis hypoplasia. As differential diagnosis we compare them with related syndromes and with Joubert's syndrome which main radiological finding on MRI is vermis hypoplasia associated with "molar tooth" appearance. The correct answer for these cases will only be possible by molecular genetics.

[West syndrome: clinical and electroencephalographic follow up of 70 patients and response to its treatment with adrenocorticotropic hormone, prednisone, vigabatrin, nitrazepam and valproate]. / Síndrome de West: evolução clínica e eletrencefalográfica de 70 pacientes e resposta ao tratamento com hormônio adrenocorticotrófico, prednisona, vigabatrina, nitrazepam e ácido valpróico.

In a retrospective study we assessed the outcome of the criptogenic and symptomatic forms of West syndrome and evaluated the efficacy of adrenocorticotropic hormone, vigabatrin, prednisone, valproate and nitrazepam in the spasms control. Seventy patients were follwed up by 2 years. Twelve (17%) were criptogenics and 58 (83%) symptomatics. In criptogenic group significantly more patients were in regular school classes and with normal motor development, better control of seizure, less tendency to evoluate to Lennox Gastaut syndrome and 83. 3% had control of spasms (72.4% of patients from symptomatic group had control of spasms). Adrenocorticotropic hormone and vigabatrin were the most efective drugs, with 68.75% and 60% of spasms control, respectivelly, when used as first line of therapy and 75% and 50%, respectivelly, as second line of therapy.

The DNA replication machine of a gram-positive organism.

This report outlines the protein requirements and subunit organization of the DNA replication apparatus of Streptococcus pyogenes, a Gram-positive organism. Five proteins coordinate their actions to achieve rapid and processive DNA synthesis. These proteins are: the PolC DNA polymerase, tau, delta, delta', and beta. S. pyogenes dnaX encodes only the full-length tau, unlike the Escherichia coli system in which dnaX encodes two proteins, tau and gamma. The S. pyogenes tau binds PolC, but the interaction is not as firm as the corresponding interaction in E. coli, underlying the inability to purify a PolC holoenzyme from Gram-positive cells. The tau also binds the delta and delta' subunits to form a taudeltadelta' "clamp loader." PolC can assemble with taudeltadelta' to form a PolC.taudeltadelta' complex. After PolC.taudeltadelta' clamps beta to a primed site, it extends DNA 700 nucleotides/second in a highly processive fashion. Gram-positive cells contain a second DNA polymerase, encoded by dnaE, that has homology to the E. coli alpha subunit of E. coli DNA polymerase III. We show here that the S. pyogenes DnaE polymerase also functions with the beta clamp.

Effect of administering a crude equine gonadotrophin preparation to mares on follicular development, oocyte recovery rate and oocyte maturation in vivo.

In mares, the shortage of oocytes and the variability in nuclear maturation at a certain time of the oestrous cycle hinders the optimization of methods for in vitro maturation and in vitro fertilization. Increasing the number of small-to-medium-sized follicles available for aspiration in vivo may increase the overall oocyte yield. The aims of the present study were to investigate whether administration of crude equine gonadotrophins affects follicular development, oocyte recovery rate, in vivo oocyte maturation and follicular concentrations of meiosis-activating sterols. During oestrus, all follicles >/= 4 mm were aspirated from 19 pony mares (first aspiration: A1). Over the next 8 days, the mares were treated daily with either 25 mg crude equine gonadotrophins (n = 10) or physiological saline (n = 9). Between day 1 and day 8, follicular growth was monitored by ultrasonography. On day 8, all follicles >/= 4 mm were evacuated (second aspiration: A2) and nuclear maturation of the recovered oocytes was assessed after orcein staining. Follicular growth between A1 and A2, as well as the number and size of follicles at A2 were similar for control mares and mares treated with crude equine gonadotrophins. The oocyte recovery rates at A1 and A2 were similar. At A2, the oocyte recovery rate and oocyte maturation in vivo were not affected by treatment with crude equine gonadotrophins. The number of expanded cumulus oophorus complexes recovered from follicles

[Hypertensive encephalopathy associated to repetitive seizures: case report]. / Encefalopatia hipertensiva associada a estado de mal epiléptico de repetição: relato de caso.

Five years old, female, who started with tonic-clonic seizures on the right side of the body, with vomits and unconsciousness. The patient had been hospitalized for eight times in the last sixty days because of seizures. At physical exam, she had a severe arterial hypertension (270/140 mmHg). The computerized tomographic scan and magnetic resonance imaging revealed hypodense areas, mainly on the right parietal-temporal region, suggesting presence of edema. The angiography showed stenosis of the right renal artery, that was the cause of arterial hypertension. After the control of arterial hypertension by nephrectomy, the patient had a complete remission of the symptoms, as well as the images anomalies.

Visualization of two binding sites for the Escherichia coli UmuD'(2)C complex (DNA pol V) on RecA-ssDNA filaments.

The heterotrimeric UmuD'(2)C complex of Escherichia coli has recently been shown to possess intrinsic DNA polymerase activity (DNA pol V) that facilitates error-prone translesion DNA synthesis (SOS mutagenesis). When overexpressed in vivo, UmuD'(2)C also inhibits homologous recombination. In both activities, UmuD'(2)C interacts with RecA nucleoprotein filaments. To examine the biochemical and structural basis of these reactions, we have analyzed the ability of the UmuD'(2)C complex to bind to RecA-ssDNA filaments in vitro. As estimated by a gel retardation assay, binding saturates at a stoichiometry of approximately one complex per two RecA monomers. Visualized by cryo-electron microscopy under these conditions, UmuD'(2)C is seen to bind uniformly along the filaments, such that the complexes are completely submerged in the deep helical groove. This mode of binding would impede access to DNA in a RecA filament, thus explaining the ability of UmuD'(2)C to inhibit homologous recombination. At sub-saturating binding, the distribution of UmuD'(2)C complexes along RecA-ssDNA filaments was characterized by immuno-gold labelling with anti-UmuC antibodies. These data revealed preferential binding at filament ends (most likely, at one end). End-specific binding is consistent with genetic models whereby such binding positions the UmuD'(2)C complex (pol V) appropriately for its role in SOS mutagenesis.

[Krabbe s disease - case report] / Doença de Krabbe - relato de caso.

OBJECTIVE: Report a case of Krabbés disease with necropsy. METHODS: Review of medical and necropsy records. RESULTS: An 8 months-old male patient developed tremors, swallowing difficulty and excessive salivation for 4 months prior to admission, evolving with vomiting and fever. Physical examination showed microcephaly and diffuse pigmentation of the retinae. Neurological examination showed flexion of upper limbs with spastic hyperthony, symmetrical global hyperreflexia, nystagmus and spontaneous spasms. EEG showed multifocal irritative activity. There was increase in both CSF protein and gamaglobulin. The patient evolved with transitory hyperthermia, vomiting and pneumopathy, dying on the 23rd day after admission. Post mortem studies revealed microcephaly with widening of brain sulci. Histological examination revealed several globoid cells in the deep portion of the white matter, reactive gliosis and demyelination. CONCLUSIONS: These findings were similar to those in the world literature, indicating a poor prognosis due to substantial brain damage.

Morphology of the oocyte-follicular connection in the mare.

The present study characterised the oocyte-follicular connection (i.e., oocyte fixation site) in Graafian follicles of the mare morphologically. Antral follicles were dissected in toto from ovaries obtained from oestrous, dioestrous and transitional mares after slaughter. The location of the cumulus oophorus complex in relation to the ovulation fossa, the width and density of the blood vessels surrounding the cumulus oophorus complex, the relative dimensions and histological aspects of the cumulus oophorus were investigated. For ultrastructural analysis of the junctional regions, cumulus-oocyte complexes were recovered in vivo by transvaginal ultrasound-guided follicle aspiration. The location of the oocyte fixation site was independent of mare, follicular size and stage of the oestrous cycle. In 82% of follicles, the oocytes were embedded in a broad based cell mount. The width and density of the blood vessels surrounding the oocyte fixation site were correlated to each other, but independent of follicular size and cyclic stage. The histological appearance of the cumulus oophorus varied, especially in respect to the compactness, and loosening of the cumulus cell population was observed in several medium-sized follicles from dioestrous mares. Loosening of the cumulus cell population was apparently associated with decreased interdigitation between adjacent corona radiata cells. It can be concluded that the fixation site of the equine cumulus oophorus complex represents a firm cellular anchorage between follicular wall and oocyte. Furthermore, the location of the cumulus oophorus complex in relation to the ovulation fossa and characteristics of the surrounding blood vessels is independent of follicular size and cyclic stage.