Ultraestrutura do nervo facial intratemporal em pacientes com paralisia facial idiopática: estudo de evidências de infecção viral

A etiologia da paralisia facial periférica idiopática (PFPI) ainda é uma incógnita, no entanto, alguns autores aventam a possibilidade de ser uma infecção viral. OBJETIVO: Analisar a ultraestrutura do nervo facial procurando evidências virais que possam nos fornecer dados etiológicos. MATERIAL E MÉTODO: Foram estudados 20 pacientes com PFP, com graus de moderado a severo, de ambos os sexos, entre 18-60 anos, provenientes de Ambulatório de Distúrbios do Nervo Facial. Os pacientes foram divididos em dois grupos: Estudo, onze pacientes com PFPI e Controle, nove pacientes com Paralisia Facial Periférica Traumática ou Tumoral. Foram estudados fragmentos de bainha do nervo facial ou fragmentos de seus cotos, que durante a cirurgia de reparação do nervo facial, seriam desprezados ou encaminhados para estudo anatomopatológico. O tecido foi fixado em glutaraldeído 2% e analisado em Microscopia Eletrônica de Transmissão. RESULTADO: Observamos no grupo estudo atividade celular intensa de reparação com aumento de fibras colágenas, fibroblastos com organelas desenvolvidas, isentos de partículas virais. No grupo controle esta atividade de reparação não foi evidente, mas também não foram observadas partículas virais. CONCLUSÃO: Não foram encontradas partículas virais, no entanto, houve evidências de intensa atividade de reparação ou infecção viral.

Doença de Kawasaki: experiência clínica em hospital universitário

OBJETIVO: A doença de Kawasaki é uma vasculite sistêmica aguda de etiologia desconhecida. Seu diagnóstico baseia-se em critérios clínicos. O objetivo deste estudo foi descrever os casos de pacientes com doença de Kawasaki internados no Hospital Universitário da Universidade de São Paulo entre janeiro/2000 e junho/2008. MÉTODOS: Dentre todos os pacientes internados na Enfermaria de Pediatria no período acima, foram selecionados aqueles cujo CID de alta foi doença de Kawasaki. Realizou-se estudo descritivo por meio da análise dos prontuários dessas crianças. RESULTADOS: Foram encontrados 18 casos. A média de internações foi de 2,1 casos/ano. A idade variou de três meses a nove anos. A proporção meninos:meninas foi 1:1,25. Receberam outros diagnósticos prévios 17 pacientes, sendo escarlatina em 2/3 dos casos. O tempo de febre antes do diagnóstico variou de cinco a 11 dias. Nove crianças apresentaram quatro sinais sugestivos de doença de Kawasaki; oito apresentaram cinco sinais e uma apresentou dois sinais, o que foi considerado doença de Kawasaki incompleta. Receberam gamaglobulina 15 crianças (entre o sexto e o décimo dias de evolução) e 11 (73%) ficaram afebris após infusão da medicação. Os demais tiveram febre até 24 horas após a administração. Todos os pacientes realizaram ecocardiograma e três apresentaram aneurisma leve da coronária. CONCLUSÕES: A doença de Kawasaki é habitualmente confundida com outras doenças, o que causa retardo no tratamento e aumento no risco de complicações cardíacas.

Linfoma ileal primário como uma causa de intussuscepção ileocecal recorrente

INTRODUÇÃO: A intussuscepção ocorre quando um segmento proximal do intestino invagina para dentro do lúmen do segmento distal adjacente. Esta patologia é relativamente comum em crianças, sendo geralmente idiopática, diferentemente do que é evidenciado em adolescentes e adultos, os quais apresentam uma causa orgânica comprovada na maioria dos casos. O linfoma intestinal como etiologia desta patologia é extremamente raro. RELATO DE CASO: Um paciente de 16 anos, masculino, referindo dor abdominal em quadrante inferior direito há 36 horas associada a vômitos e fezes com sangue vivo compareceu em nosso serviço. O exame físico se apresentava dentro da normalidade exceto por uma massa palpável no quadrante inferior direito. A ultra-sonografia abdominal revelou intussuscepção ileocecal. A colonoscopia demonstrou uma massa protuberante proveniente do orifício da válvula ileocecal que foi reduzida, tendo o paciente um alívio completo dos sintomas. Três semanas após, o paciente retornou ao nosso hospital com recorrência dos sintomas. Uma laparotomia exploradora foi realizada evidenciando uma massa polipóide no íleo terminal com intussuscepção para dentro do ceco. Uma colectomia direita ampliada foi realizada. Após exame patológico da peça e estadiamento tumoral, um linfoma de Burkitt primário foi diagnosticado. A recuperação pós-operatória não apresentou intercorrências e o paciente foi encaminhado para quimioterapia adjuvante.

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.

Categorization skills and recall in brain damaged children: a multiple case study

During development, children become capable of categorically associating stimuli and of using these relationships for memory recall. Brain damage in childhood can interfere with this development. This study investigated categorical association of stimuli and recall in four children with brain damages. The etiology, topography and timing of the lesions were diverse. Tasks included naming and immediate recall of 30 perceptually and semantically related figures, free sorting, delayed recall, and cued recall of the same material. Traditional neuropsychological tests were also employed. Two children with brain damage sustained in middle childhood relied on perceptual rather than on categorical associations in making associations between figures and showed deficits in delayed or cued recall, in contrast to those with perinatal lesions. One child exhibited normal performance in recall despite categorical association deficits. The present results suggest that brain damaged children show deficits in categorization and recall that are not usually identified in traditional neuropsychological tests.

Etiology of cutaneous leishmaniasis and anthropophilic vectors in Juruti, Pará State, Brazil

In a preliminary study in Juruti, a mining municipality in western Pará State, Brazil, 12 out of 21 patients suspected of presenting cutaneous leishmaniasis showed positive PCR (SSUrDNA and G6PD): Leishmania (Viannia) braziliensis (9/12; 75%) and L. (V.) sp. (3/12; 25%). Entomological studies in the same location revealed the presence of 12 different phlebotomine species (n =105). One of the most common species was Lutzomyia (Psychodopygus) complexa (17%) which is both highly anthropophilic and a known vector of L. (V.) braziliensis in other regions of Pará. These preliminary findings should serve to guide future epidemiological surveillance in Juruti.

O conceito de vivência em Freud e Husserl

Este artigo visa explicitar as concepções freudiana e husserliana de “vivência” (Erlebnis). Por “vivência” entende-se genericamente um tipo fundamental de experiência do mundo. Esse tema, ainda que não diretamente explorado por Freud, tornou-se necessário em sua teoria desde a descoberta da etiologia da histeria no início dos anos 1890 à luz do método catártico: a da vivência traumática. Por outro lado, Husserl, a partir do problema filosófico de garantir a possibilidade do conhecimento universal e necessário, se viu obrigado a combater o naturalismo das ideias, em 1900, e o naturalismo da consciência, em 1913, em ambos os casos partindo de uma análise das vivências (intencionais). Mostrarei que a abordagem freudiana (natural-científica) visa explicar metapsicologicamente a vivência, ao passo que a abordagem husserliana pretende descrever a estrutura da vivência (intencional). Por fim, apontarei para algumas das grandes diferenças entre ambas as abordagens desse tema.

New insights into Vogt-Koyanagi-Harada disease

Vogt-Koyanagi-Harada disease (VKH), a well-established multiorgan disorder affecting pigmented structures, is an autoimmune disorder of melanocyte proteins in genetically susceptible individuals. Several clinical and experimental data point to the importance of the effector role of CD4+ T cells and Th1 cytokines, the relevance of searching a target protein in the melanocyte, and the relevance of the HLA-DRB1*0405 in the pathogenesis of the disease. Vogt-Koyanagi-Harada disease has a benign course when early diagnosed and adequatey treated. Full-blown recurrences are rare after the acute stage of Vogt-Koyanagi-Harada disease is over. On the other hand, clinical findings, such as progressive tissue depigmentation (including sunset glow fundus) and uveitis recurrence, indicate that ocular inflammation may persist after the acute phase. Additionally, indocyanine green angiography findings suggest the presence of choroidal inflammation in eyes without clinically detectable inflammation. The aim of this paper is to review the latest research results on Vogt-Koyanagi-Harada disease pathogenesis and chronic/convalescent stages, which may help to better understand this potentially blinding disease and to improve its treatment.

Meningoencefalite necrotizante de cão Maltês

A Meningoencefalite Necrotizante (MEN) é uma encefalopatia causada por uma disfunção inflamatória de característica necrotizante. O objetivo deste relato é descrever os aspectos clínicos e anatomopatológicos da Meningoencefalite Necrotizante (MEN) em um cão Maltês. A doença tem um caráter necrótico único e está relacionada intimamente à Encefalite do Cão Pug (ECP) devido a suas semelhanças, bem como à Leucoencefalite Necrotizante (LEN). Embora o primeiro relato de caso de ECP tenha mais de 15 anos e o primeiro relato de caso de MEN em Maltês tenha 11 anos, há muito a ser revelado sobre a etiologia e os mecanismos imunopatológicos da doença. Neste trabalho, relata-se o caso de um cão Maltês com sinais que foram compatíveis com a MEN. Foram detectadas nas imagens macroscópicas, cavitação cerebral, e na microscopia, perda de células do parênquima em certas regiões do córtex cerebral. A partir dessas descobertas descreve-se o primeiro caso de MEN em cão Maltês no Brasil.

Etiologic diagnosis of bovine infectious abortion by PCR

Infectious abortion is a significant cause of reproductive failure and economic losses in cattle. The goal of this study was to detect nucleic acids of several infectious agents known to cause abortion including Arcanobacterium pyogenes, Bovine Herpesvirus 1, Brucella abortus, Campylobacter fetus subsp. venerealis, Chlamydophila abortus, Leptospira sp., Listeria monocytogenes, Salmonella sp., Mycoplasma bovis, Mycoplasma bovigenitalium, Neospora caninum, and Tritrichomonas foetus. Tissue homogenates from 42 fetuses and paraffin-embedded tissues from 28 fetuses and 14 placentas/endometrium were included in this study. Brucella abortus was detected in 14.2% (12/84) of the samples. Salmonella sp. DNA was amplified from 2 fetuses, and there was one positive for Neospora caninum, and another for Listeria monocytogenes. This PCR-based approach resulted in identification of the etiology in 19% of samples, or 20% if considered fetal tissues only.

What are the similarities between stress, sudden cardiac death in Gallus gallus and sudden unexpected death in people with epilepsy

Individuals with epilepsy are at higher risk of sudden unexpected death in epilepsy (SUDEP), responsible for 7.5% to 17% of all deaths in epilepsy. Many factors are current associated with SUDEP and possible effect of stress and cardiac arrhythmia are still not clear. Sudden death syndrome (SDS) in chickens is a disease characterized by an acute death of well-nourished and seeming healthy Gallus gallus after abrupt and brief flapping of their wings, similar to an epileptic seizure, with an incidence estimated as 0.5 to 5% in broiler chickens. A variety of nutritional and environmental factors have been included: but the exactly etiology of SDS is unknown. Studies had suggested that the hearts of broiler chickens are considerably more susceptible to arrhythmias and stress may induce ventricular arrhythmia and thus, sudden cardiac death. In this way, SDS in Gallus gallus could be an interesting model to study SUDEP.

O uso da acupuntura no auxílio à terapia da doença idiopática do trato urinário inferior dos felinos

Diferentes afecções podem acometer o trato urinário inferior dos felinos, acarretando sinais clínicos inespecíficos como: hematúria, disúria, polaquiúria, estrangúria, periúria ou obstrução, caracterizando a doença do trato urinário inferior dos felinos (DTUIF). Entretanto, em até 65% dos felinos acometidos, a etiologia é indeterminada, denominada de doença idiopática do trato urinário inferior dos felinos (DTUIF idiopática), que tem se mostrado como um desafio ao clínico veterinário, uma vez que não há diagnóstico específico ou terapia efetiva. Atualmente, tem sido estudado o papel da inflamação neurogênica da bexiga urinária secundária ao estresse, como etiologia da DTUIF idiopática. Há evidências científicas de que a acupuntura restaure a homeostase, reduza o estresse e, pela estimulação neural periférica, ative mecanismos endógenos de antinocicepção, regulando a liberação de mediadores dos mecanismos da dor e do processo inflamatório, como a substância P. O objetivo desta revisão bibliográfica foi descrever como a acupuntura pode ser um recurso na terapia da DTUIF idiopática, tendo como base a modulação da inflamação neurogênica da bexiga urinária e o controle do estresse desses gatos.

Two distinct regions in 2q24.2-q24.3 associated with idiopathic epilepsy

P>Approximately 50% of all carriers of 2q21-q31 deletions present epileptic seizures. The band 2q24 constitutes the smallest commonly deleted segment in these patients, and contains the voltage-gated sodium channel genes SCN1A and SCN2A, associated with Dravet syndrome and benign familial neonatal-infantile seizures, respectively. A further putative locus involving epilepsy in the region was previously identified through disruption of the SLC4A10 gene by translocation. In the course of performing high-resolution DNA copy number analyses on syndromic mentally impaired individuals, we encountered three patients with overlapping deletions in chromosome region 2q24. Two of these patients exhibited epileptic seizures in addition to mental deficiency. The deletion in one of the epileptic patients did not include the SCN cluster, demonstrating that a less severe form of epilepsy maps to an adjacent genomic region. This second region comprises about 3 Mb and contains the candidate gene SLC4A10, providing further support for the potential role of this gene in epilepsy.

From lateral to mesial: The quest for a surgical cure for temporal lobe epilepsy

Purpose: A gap of more than a hundred years occurred between the first accounts of mesial temporal sclerosis and recognition of its role in the pathogenesis of psychomotor seizures. This paper reviews how the understanding and surgical treatment of temporal lobe epilepsy developed, particularly from the work of Penfield, Jasper, and their associates at the Montreal Neurological Institute (MNI). Methods: Publications on EEG and surgery for temporal lobe seizures from 1935 to 1953 were reviewed and charts of selected patients operated on at the MNI in the same period were examined. Attention was focused on the evolution of surgical techniques for temporal lobe epilepsy. Results: In the late 1930s, some EEG findings suggested deep-lying disturbances originating in the temporal lobe. However, it took another two decades before the correlation of clinical, neurophysiological, and anatomical findings provided evidence for the involvement of the mesial structures in psychomotor or temporal lobe seizures. From 1949 and onward, Penfield and his associates applied this evidence to extend the surgical resections to include the uncus and the hippocampus. Conclusion: The collaborative work of a team led by Penfield and Jasper at the MNI helped to define the role of neurophysiological studies in epilepsy surgery. As a result, the importance of removing the mesial structures in order to obtain better seizure control in patients with temporal lobe epilepsy became firmly established.

Good surgical outcome in discordant ictal EEG-MRI unilateral mesial temporal sclerosis patients

Purpose:Video electroencephalography (vEEG) monitoring of patients with unilateral mesial temporal sclerosis (uMTS) may show concordant or discordant seizure onset in relation to magnetic resonance imaging (MRI) evidence of MTS. Contralateral seizure usually leads to an indication of invasive monitoring. Contralateral seizure onset on invasive monitoring may contraindicate surgery. We evaluated long-term outcome after anteromesial temporal lobectomy (AMTL) in a consecutive series of uMTS patients with concordant and discordant vEEG findings, uniformly submitted to AMTL on the MRI evidence of MTS side without invasive monitoring. Methods:We compared surgical outcome of all uMTS patients undergoing vEEG monitoring between January 1999 and April 2005 in our service. Discordant cases were defined by at least one seizure onset contralateral to the MRI evidence of MTS. Good surgical outcome was considered as Engel`s class I. We also evaluated ictal SPECT concordance to ictal EEG and surgical outcome. Results:Fifty-four patients had concordant (C) and 22 had discordant (D) scalp EEG and MRI. Surgical outcome was similar in both groups (C = 74% versus D = 86%). Duration of follow-up was comparable in both groups: C = 56.1 +/- 20.7 months versus D = 59.8 +/- 21.2 months (p = 0.83, nonsignificant). Discordant single-photon emission computed tomography (SPECT) results did not influence surgical outcome. Discussion:Surgical outcome was not influenced by contralateral vEEG seizure onset or contralateral increased flow on ictal SPECT. Although vEEG monitoring should still be performed in these patients, to rule out psychogenic seizures and extratemporal seizure onset, a potentially risky procedure such as invasive monitoring may not only not be indicated in this patient population, but may also lead to patients erroneously being denied surgery.