bluuebunny/crossref_metadata_2025 huggingface dataset:
Myasthenic crisis: report of 24 cases
Authors: Werneck Lineu Cesar , Scola Rosana Herminia , Germiniani Francisco Manoel Branco , Comerlato Enio A. , Cunha Francisco Marcos Bezerra
Published: April 2005

Myasthenic crisis (MC) is a life-threatening complication of myasthenia gravis (MG) with a high mortality rate. The aim of our study was to review the different therapeutics approaches in the treatment of MC and their impact in the final outcome. We reviewed the medical files of patients diagnosed with MG admitted between February 1993 and October 1997, who developed MC. Sex, mean age, disease's duration, functional scale, symptoms preceding the crisis, crisis therapy in each set and mortality were then analysed. There were 24 patients who developed MC, 21 females and 3 males, with 1 neonatal, 1 congenital sporadic, 17 juvenile/adult, 3 over 50 years and 2 with thymoma. Dysphagia, dysphonia and dysartria were the most common symptoms preceding the crisis. A precipitating factor was elicited in 8 cases and the most common was infection (upper airway infection, urinary tract infection and pneumonia). 16 patients needed a nasogastric tube and 9 had a tracheostomy performed. 24 patients used anticholinesterase drugs, 21 prednisone, 7 immunosuppressive agents, 5 plasmapheresis, 3 human hyperimune gamma immunoglobulin and 12 had thymectomy. A good response was obtained in 13, satisfactory in 7 and there were 4 deaths. We concluded that in spite of all the therapeutics options, there were non statistically significant differences in the outcome of patients that underwent thymectomy and those who did not.
Movement disorders in 28 HIV-infected patients
Authors: Mattos James Pitágoras de , Rosso Ana Lúcia Zuma de , Corrêa Rosalie Branco , Novis Sérgio A.P.
Published: April 2005

From 1986 to 1999, 2460 HIV-positive inpatients were seen in our Hospital. Neurological abnormalities were detected in 1053 (42.8%) patients. In this group, 28 (2.7%) had involuntary movements, 14 (50%) with secondary parkinsonism, six (21.4%) with hemichorea/hemiballismus, four (14.2%) with myoclonus, two (7.2%) with painful legs and moving toes, one (3.6%) with hemidystonia and one (3.6%) with Holmes' tremor. The HIV itself (12 patients), toxoplasmosis of the midbrain (1) and metoclopramide-related symptoms (1) were the most probable causes for the parkinsonism. All patients with hemichorea/hemiballismus were men and in all of them toxoplasmosis of the basal ganglia, mostly on the right side, was the cause of the involuntary movements. Generalized myoclonus was seen in two patients and they were due to toxoplasmosis and HIV-encephalopathy respectively; two others presented with spinal myoclonus. The two patients with painful legs and moving toes had an axonal neuropathy. The patient with hemidystonia suffered from toxoplasmosis in the basal ganglia and the patient with Holmes' tremor had co-infection with tuberculosis and toxoplasmosis affecting the midbrain and cerebellum. We conclude that HIV-infected patients can present almost any movement disorder. They can be related to opportunistic infections, medications, mass lesions and possibly to a direct or indirect effect of the HIV itself.
Neuropathology of two Brazilian autopsied cases of tropical spastic paraparesis / HTLV-I associated myelopathy (TSP/HAM) of long evolution
Authors: Castro-Costa Carlos Maurício de , Dom René , Carton Herwig , Goubau Patrick , Santos Terezinha de Jesus Teixeira , Ferreira Márcia Valéria Pitombeira , Silva Neto Francisco Ursino da
Published: April 2005

We report on a neuropathological analysis of two cases of TSP/HAM originating from Brazil. These two cases had, respectively, an evolution of 13 and 40 years. The main neuropathological findings consisted of spinal cord atrophy, mainly the lower thoracic cord, diffuse degeneration of the white and grey matter, rare foci of mononuclear and perivascular cuffs, and hyaline hardening of arteriolae. The supraspinal structures were normal, excepting for a slight gliosis in the cerebellum. An analysis on the long evolutive cases as described in the literature is outlined in this study.
Computed tomography scan of the head in patients with migraine or tension-type headache
Authors: Valença Marcelo Moraes , Valença Luciana Patrízia A. Andrade , Menezes Terce Liana
Published: April 2005

A retrospective study was performed in order to evaluate the frequency of abnormalities found by computed tomography (CT) scan of the head in 78 patients with migraine or tension-type headache. In the present study CT scan was normal in 61.5% of the patients with migraine or tension-type headache. A number of abnormalities were encountered in more than one third of the patients studied, including inflammatory sinus disease (19.2%), cysticercosis (3.9%), unruptuted cerebral aneurysm (2.6%), basilar impression (2.6%), intracranial lipoma (2.6%), arachnoid cyst (2.6%), empty sella (2.6%), intracranial neoplasm (2.6%), and others (2.6%). None of these lesions were symptomatic or responsible by the headache picture, therefore, considered incidental findings. In conclusion, the fortuitous encounter of some abnormalities on CT scan of the head is often higher than what we could predict in patients suffering migraine or tension-type headache. We briefly discuss clinical, epidemiologic, and practical management of some of the abnormalities detected by CT scan as well as the indication to request a neuroimaging investigation.
Clinical and demographic features of patients with dementia attended in a tertiary outpatient clinic
Authors: Vale Francisco A.C. , Miranda Stênio J.C.
Published: April 2005

We describe clinical and socio-demographic features of patients with dementia attended in a tertiary outpatient clinic during a three years period (56.9% of the total attendance). Most of them were men, white, from the local community, urban district. Nobody had a job at the moment, two thirds of them got social welfare benefit. They lived with their family, the caregiver being the spouse or a daughter. The education level was very low, a quarter of them being illiterate. They were referred mostly from the public health care service, by neurologists or psychiatrists due to cognitive disorders. Family history as well as individual history of previous neurological/psychiatric disorders were frequent, especially alcoholism, stroke, head trauma and dementia. The neurological exam showed abnormalities in two thirds of cases, chiefly extra-pyramidal and pyramidal signs. Alzheimer's disease was the most frequent cause, followed by cerebrovascular disorder; alcoholism and normal pressure hydrocephalus were also frequent causes. Most patients presented concomitant non-etiological neurological/psychiatric disorders, mainly alcoholism and depression, and non-neurological/psychiatric diseases, predominantly hypertension, cardiopathy and diabetes. Most patients had been referred under medication, frequently politherapy, including psychotropics.
Psychological features in panic disorder: a comparison with major depression
Authors: Almeida Yasmin A. , Nardi Antonio E.
Published: April 2005

OBJECTIVE: We aim to evaluate the psychodymanic model for panic disorder (PD) formulated by Shear et al. (1993), comparing PD patients and major depression (MD) patients. METHOD: We evaluated these parameters in open interviews in 10 PD patients and 10 patients with MD (DSM-IV). The data were recorded on videotape and were examined by 5 diagnostic blind appraisers. RESULTS: The data allowed a comparative analysis that underscores the existence of a psychological model for PD vs MD: 1) the protracted symbiotic phase of development and the existence of problems with separation in PD patients; 2) patients with MD tended to have a particularly negative impression of relationship with the first objects; furthermore, they had remarkable experiences of loss; and 3) while the PD patients tended to be shy and inhibited in childhood, especially showing a clear difficulty in expressing aggressiveness, the depressed patients tended to disclose an impulsive aggressiveness from infancy to adulthood. CONCLUSION: Exposure to parental behaviours that augment fearfulness may result in disturbances in object relations and persistence of conflicts between dependence and independence may predispose to anxiety symptoms and fears of PD.
Excessive daytime sleepiness in Campo Grande general population, Brazil
Authors: Souza José Carlos , Magna Luiz Alberto , Reimão Rubens
Published: April 2005

The prevalence of excessive daytime sleepiness (EDS) in general population was determined by means of 408 home interviews of adults, in a representative sample of Campo Grande city, Brazil. The random sample was stratified by sex, age and economic social status. EDS was considered in those with indexes 11 or more in the Epworth Sleepiness Scale. Statistics used chi-square, Fisher and Pearson tests; and inferences based on binomial distribution parameters; the significance level was 5% and confidence interval (CI) was 95%. The prevalence of EDS was 18.9% of the general population ( SD=1.9%; CI 15.1% to 22.7%). No significant association was found between EDS and the use of hypnotics, nor with insomnia, body mass index, sex, age, years of schooling, economic social status, marital status, occupation and the use of alternative means to improve sleep. When the sample was separated according to sex, only the male group showed significant association between EDS and actual insomnia (p=0.005).
Occipital lobe morphological anatomy: anatomical and surgical aspects
Authors: Flores Leandro Pretto
Published: April 2005

BACKGROUND: The occipital lobe is an important region of the central nervous system and site of a wide variety of lesions. Different from others regions of the brain, whose anatomy has been already meticulously detailed, controversies about occipital lobe morphology can occasionally hinder the surgical approaches to it. METHOD: Twenty-six hemispheres were dissected, examining the distances of the medial structures of the posterior interhemispheric fissure region; identifying the sulci and gyri more frequently found in the lateral surface; and detailing the anatomy of the occipital horn of the lateral ventricle. Also, anatomical details of structures such as the calcarine sulcus and preoccipital notch were evaluated. RESULTS: Twenty-four hemispheres (92%) showed sulci with evident transverse trajectories in the lateral surfarce, and it was possible to identify marked longitudinal sulci in 16 (61%). All of the specimen presented a transverse sulcus in the inferior third of the lobe -- labeled in this paper as Inferior Transverse Occipital -- and 69% of the cases showed a transverse sulcus in the superior third -- labeled as Superior Transverse Occipital. The occipital horn was present in 53% of the cases at level of the anterior boundary of the lobe. It was a half moon shaped cavity, whose the medial wall was formed by the calcar avis and the lateral wall, roof and floor formed by fibers of the tapetum. CONCLUSION: The occipital lobe presents parameters that are often recognized in most of the dissected brain and can be useful for the planning and performance of surgery in this area of the brain.
The contribution of the lateral posterior and anteroventral thalamic nuclei on spontaneous recurrent seizures in the pilocarpine model of epilepsy
Authors: Scorza Fulvio Alexandre , Arida Ricardo Mario , Priel Margareth , Calderazzo Lineu , Cavalheiro Esper Abrão
Published: April 2005

The pilocarpine model of epilepsy in rats is characterised by the occurrence of spontaneous seizures (SRSs) during the chronic period that recur 2-3 times per week during the whole animal life. In a previous study on brain metabolism during the chronic period of the pilocarpine model it was possible to observe that, among several brain structures, the lateral posterior thalamic nuclei (LP) showed a strikingly increased metabolism. Some evidences suggest that the LP can participate in an inhibitory control system involved in the propagation of the seizures. The aim of the present study was to verify the role of LP in the expression and frequency of spontaneous seizures observed in the pilocarpine model. Ten adult male rats presenting SRSs were monitored for behavioural events by video system one month before and one month after LP ibotenic acid lesion. Another group of chronic epileptic rats (n=10) had the anteroventral thalamic nuclei (AV) lesioned by ibotenic acid. After the surgical procedure, the animals were sacrified and the brains were processed for histological analysis by the Nissl method. The LP group seizure frequency was 3.1±1.9 before ibotenic acid injection and showed an increase (16.3±7.2 per week) after LP lesion. No changes in SRSs frequency were observed in the AV group after ibotenic lesion in these nuclei. These results seem to suggest that LP play a role in the seizure circuitry inhibiting the expression of spontaneous seizures in the pilocarpine model.
Morphoquantitative effects of acute diabetes on the myenteric neurons of the proximal colon of adult rats
Authors: Furlan Maria Montserrat D.P. , Molinari Sônia L. , Miranda Neto Marcílio H. de
Published: April 2005

The effects of acute diabetes on the density and size of the myenteric neurons of the proximal colon of adult rats were investigated. The injection of streptozotocin was followed by a period of observation of seven days, during which the diabetic animals showed weight loss, excessive food and water intake, large urinary debt and hyperglicemia. The whole-mounts from the proximal colon were stained with the techniques of Giemsa and of the NADH-diaphorase, and the employment of these techniques made it possible to verify a decrease on the neuronal density and on the cell body size of the myenteric neurons in the colon of the diabetic rats. These observations were discussed in terms of the pathophysiology of the diabetes and the experimental protocol.
Influência dos níveis de prolactina e tamanho tumoral na função hipofisária pós-operatória em macroadenomas hipofisários clinicamente não-funcionantes
Authors: Fonseca Ana Luiza Vidal , Chimelli Leila , Santos Mario José C. Felippe , Santos Alair Augusto S.M. Damas dos , Violante Alice Helena Dutra
Published: April 2005

OBJETIVO: Estudar a influência da hiperprolactinemia e de tamanho tumoral na função hipofisária em macroadenomas hipofisários clinicamente não funcionantes. MÉTODOS: Foram analisados 23 pacientes com macroadenomas hipofisários clinicamente não funcionantes, com exames de imagem (tomografia computadorizada ou ressonância magnética) e dosagens hormonais basais; 16 tinham provas de função hipotálamo-hipofisária (megateste) pré-operatórios. Todos os tumores tiveram diagnóstico histológico e em 17 foi realizado também estudo imuno-histoquímico para os hormônios adeno-hipofisários. A análise estatística foi feita por meio dos testes t de Student, qui-quadrado, exato de Fisher e de Mc Neman. O nível de significância adotado foi 5% (p<0,05). RESULTADOS: O diâmetro tumoral variou de 1,1 a 4,7 cm (média=2,99 cm ± 1,04). No pré-operatório, 5 (21,7%) pacientes não apresentaram déficit hormonal laboratorial, 9 (39,1%) desenvolveram hiperprolactinemia, 13 (56,5%) níveis normais de prolactina (PRL) e 1 (4,3%) subnormal; 18 (78,3%) pacientes desenvolveram hipopituitarismo (4 pan-hipopituitarismo). Dezenove pacientes (82,6%) foram submetidos a via de acesso transesfenoidal, 3 (13%) a craniotomia e 1 (4,4%) ao acesso combinado. Somente 6 pacientes tiveram ressecção tumoral total. Dos 17 submetidos a estudo imuno-histoquímicos, 5 tumores eram imunonegativos, 1 misto, 1 LH+, 1 FSH +, 1 sub-unidade alfa e 8 apresentaram imunorreatividade focal ou em células isoladas para um dos hormônios adeno-hipofisários ou sub-unidades; dos outros seis tumores, 5 eram cromófobos e 1 cromófobo/acidófilo. Não houve correlação estatisticamente significativa entre o diâmetro tumoral e os níveis de PRL pré-operatória (p=0,82), nem entre tamanho tumoral e o estado hormonal pós-operatório, exceto nos eixos GH e gonadotrófico. Foi observada significância estatística entre o tamanho tumoral e o estado hormonal pré-operatório (exceto no eixo gonadal); entre níveis normais de PRL, associados a discreta ou a nenhuma disfunção hipofisária pré-operatória, e recuperação da função hipofisária pós-operatória. CONCLUSÃO: A hiperprolactinemia e tamanho tumoral pré-operatórios isolados não foram preditivos para a recuperação da função hipofisária pós-operatória.
Diagnóstico da esquistossomose medular: contribuição da ressonância magnética e eletroneuromiografia
Authors: Peregrino Alberto Jorge Pereira , Puglia Paula Marzorat Kuntz , Bacheschi Luiz Alberto , Hirata Maria Tereza Alves , Brotto Mario Wilson Iervolino , Nóbrega José Paulo Smith , Scaff Milberto
Published: April 2005

OBJETIVO: Analisar os resultados obtidos pela ressonância magnética (RM) e eletroneuromiografia (ENMG) em pacientes com diagnóstico de esquistossomose medular (EM). MÉTODO: Foram analisadas 18 RM da coluna vertebral tóraco-lombar e 24 ENMG dos membros superiores e inferiores de pacientes com diagnóstico definido ou altamente provável de EM nas suas formas clínicas mielorradiculítica e mielítica. RESULTADOS: Entre as 18 RM realizadas, 16 (88,8%), mostraram alterações caracterizadas por imagens hipointensas em aquisições pesadas em T1, hiperintensas em T2, com captação heterogênea do contraste gadolínico e/ou realce das meninges e cauda equina. Entre as 24 ENMG, 23 (95,8%) mostraram quadro de multirradiculopatia bilateral das raízes nervosas L2, L3, L4, L5, S1, S2, assimétrico em 10 casos (41,6%). CONCLUSÃO: A RM e a ENM demonstraram ser exames úteis para o diagnóstico da EM ao revelarem anormalidades que se repetiram sob um determinado padrão em 88,8% e 95,8% dos casos, respectivamente. Apesar de não terem especificidade para a EM, estes procedimentos devem ser realizados rotineiramente com o objetivo de fortalecer o diagnóstico presuntivo desta doença.
Esquistossomose medular: análise de 80 casos
Authors: Peregrino Alberto Jorge Pereira , Puglia Paula Marzorat Kuntz , Nóbrega José Paulo Smith , Livramento José Antonio , Marques-Dias Maria Joaquina , Scaff Milberto
Published: April 2005

O objetivo deste estudo é analisar dados clínicos e laboratoriais de 80 casos de esquistossomose medular (EM) que possam contribuir para melhorar o diagnóstico e tratamento dessa doença. Em 59 pacientes o estudo foi de maneira prospectiva, em 79 pacientes o diagnóstico foi catalogado como altamente provável, presuntivo, baseado em critérios clínico e laboratoriais. Em um paciente houve confirmação anatomo-patológica. Houve predominância do sexo masculino (63,7%), faixa etária entre 21 e 40 anos (63,7%), procedência nordestina (85%), trabalhador em construção civil (31,2%), esforço abdominal prévio (57,5%), início subagudo (61,2%), forma clínica mielorradiculítica e lesão no cone medular e cauda equina (72,5%). O estudo do líquido cefaloraquidiano mostrou pleocitose e taxas elevadas de proteínas em 100% dos casos, da gamaglobulina em 76.5%, positividade de reações imunológicas específicas (imunofluorescência e/ou ELISA) em 100% dos casos (titulo médio de 1/16 e 61u/dl, respectivamente). Costicosteróides e drogas esquistossomicidas foram administrados em todos os pacientes com evolução satisfatória em 80% dos casos.
Fatores desencadeantes de crises de migrânea em pacientes com migrânea sem aura
Authors: Ierusalimschy Ricardo , Moreira Filho Pedro Ferreira
Published: April 2005

Com o objetivo de estudar a distribuição de fatores desencadeantes de migrânea em uma população determinada, foram avaliados, através de entrevista pessoal, 100 pacientes que preenchiam os critérios diagnósticos para migrânea sem aura propostos pela Sociedade Internacional de Cefaléia. O estresse foi o desencadeante mais citado, respondendo pelo surgimento de crises de migrânea em 76% dos pacientes. Em seguida, em ordem de frequência decrescente, foram citados: estímulos sensoriais (75%), privação do sono (49%), jejum (48%), fatores ambientais (47%), alimentos (46%), menstruação (39%), fadiga (35%), bebidas alcoólicas (28%), sono prolongado (27%), cafeína (22%), esforço físico (20%), trauma craniano (20%), viagens (4%), atividade sexual (3%), medicamentos (2%), os movimentos do pescoço (2%), tabagismo (1%) e uso de travesseiro baixo (1%). Conclui-se que determinados fatores parecem desempenhar papel importante na precipitação da migrânea.
Avaliação fonoaudiológica na atrofia de múltiplos sistemas: estudo com cinco pacientes
Authors: Knopp Denise Botelho , Barsottini Orlando G. Povoas , Ferraz Henrique Ballalai
Published: April 2005

A atrofia de múltiplos sistemas (AMS) é caracterizada pela presença de sinais parkinsonianos, cerebelares, autonômicos e piramidais, em várias combinações. O aparecimento de disartria e disfagia no primeiro ano de manifestação de parkinsonismo, sugere o diagnóstico de AMS. O objetivo deste estudo foi o de caracterizar do ponto de vista fonoaudiológico os distúrbios da fala e da voz dos pacientes com AMS. Foram selecionados cinco pacientes, com idade média de 51,2 anos e com diagnóstico provável de AMS. Cada paciente foi submetido a avaliação neurológica e fonoaudiológica. Esta última foi composta dos seguintes itens: anamnese; avaliação miofuncional e avaliação perceptivo-auditiva da fala. Os sintomas de fala e voz apareceram 1,1 ano após o início dos sintomas motores e a disartrofonia apresentada por todos os pacientes foi a do tipo mista, mesclando os componentes hipocinético, atáxico e espástico, com predomínio do primeiro. Nossos achados são diferentes daqueles comumente vistos em pacientes com a doença de Parkinson, onde o componente hipocinético é o único achado. Os dados levantados indicam que a avaliação fonoaudiológica é importante no diagnóstico diferencial e no planejamento terapêutico da AMS.
Recurrent abdominal pain: when an epileptic seizure should be suspected?
Authors: Franzon Renata C. , Lopes Camila F. , Schmutzler Kátia M.R. , Morais Maria Isabel R. , Guerreiro Marilisa M.
Published: April 2005

Recurrent episodes of abdominal pain are common in childhood. Among the diagnostic possibilities are migraine and abdominal epilepsy (AE). AE is an infrequent syndrome with paroxystic episodes of abdominal pain, awareness disturbance, EEG abnormalities and positive results with the introduction of antiepileptic drugs. We present one 6 year-old girl who had short episodes of abdominal pain since the age of 4. The pain was followed by cry, fear and occasionally secondary generalization. MRI showed tumor in the left temporal region. As a differential diagnosis, we report a 10 year-old boy who had long episodes of abdominal pain accompanied by blurring of vision, vertigo, gait ataxia, dysarthria, acroparesthesias and vomiting. He received the diagnosis of basilar migraine. In our opinion, AE is part of a large group (partial epilepsies) and does not require a special classification. Pediatric neurologists must be aware of these two entities that may cause abdominal pain.
Epilepsy and ring chromosome 20: case report
Authors: Gomes Marleide da Mota , Lucca Irene , Bezerra Sonia Alonso Monteiro , Llerena Jr Juan , Moreira Denise Madeira
Published: April 2005

We present the clinical, electroencephalographic, neuroimaging (brain magnetic resonance image - MRI and spectroscopy by MRI) and cytogenetic findings of a young male patient with a rare cytogenetic anomaly characterised by a de novo 46,XY,r(20)(p13q13.3) karyotype. He presents with mental retardation, emotional liability, and strabismus, without any other significant dysmorphies. There are brain anomalies characterised by corpus callosum, uvula, nodule and cerebellum pyramid hypoplasias, besides arachnoid cysts in the occipital region. He had seizures refractory to pharmacotherapy and long period of confusional status with or without a motor component. The authors recognised that the EEG pattern was not fixed but changed over time, specially for bursts of slow waves with great amplitude accompanied or not by sharp components, and bursts of theta waves sharply contoured. Previously, epilepsy solely has been assigned to region 20q13. However, the important structural cerebral alterations present in our case has not been reported associated to such chromosomal abnormality and may indicate possible new chromosomal sites where such atypical neurological characteristics could be mapped.
Metástases intrarraquidianas de glioblastoma multiforme supratentorial da infância: relato de caso
Authors: Rondinelli Patricia Imperatriz Porto , Martinez Carlos Alberto Osório
Published: April 2005

Relatamos o caso de uma menina de onze anos de idade com glioblastoma multiforme na região têmporo-parietal direita, completamente ressecado cirurgicamente, submetida a radioterapia craniana pós-operatória. Houve recaída três meses após, em topografia distante do sítio primário, na porção caudal do canal raquidiano. Após, ocorreu evolução rápida para o óbito. A propósito desse caso, discutimos nossa experiência quanto à conduta nesses tumores e a literatura sobre o assunto.
Sonolência diurna excessiva pós-traumatismo de crânio: associação com movimentos periódicos de pernas e distúrbio de comportamento do sono REM: relato de caso
Authors: Rodrigues Raimundo Nonato D. , Silva Aída A.A. Abreu e
Published: April 2005

Um homem de 52 anos, procurou o Hospital. Universitário de Brasília com queixa de sono agitado. Sua esposa relatava, desde há cerca de 10 anos, intensa movimentação de membros e agressividade em meio a sonhos violentos. Desde então apresentava sonolência diurna excessiva. Havia relato de traumatismo de crânio há 34 anos e coma de 2 meses de duração. A vídeo-polissonografia revelou comportamento agressivo e agitado durante o sono REM, e movimentos periódicos de pernas. Havia importante sonolência diurna no teste de latências de sono. Foi instituído tratamento com levodopa-benzerazida 100/25 mg à noite. Após 10 semanas de evolução, houve melhora da movimentação noturna global, e desaparecimento dos episódios ligados a sonhos de conteúdo violento. Este caso nos permite analisar a associação entre trauma craniano e alterações nas vias dopaminérgicas (movimentos periódicos das pernas e distúrbio de comportamento do sono REM) e revisar a importância dos distúrbios na produção de hipocretina hipotalâmica na fisiopatologia desse quadro clínico.
Coleta do líquido cefalorraquidiano, termo de consentimento livre e esclarecido e aspectos éticos em pesquisa: recomendações do Departamento Científico de LCR da Academia Brasileira de Neurologia
Authors: Puccioni-Sohler Marzia , Machado Luís dos Ramos , Canuto Ricardo , Takayanagui Oswaldo Massaiti , Almeida Sérgio Monteiro de , Livramento José Antonio
Published: April 2005

O Departamento Científico de LCR (DC-LCR) da Academia Brasileira de Neurologia (ABN) recomenda a adoção do Termo de Consentimento Livre e Esclarecido (TCLE) como procedimento prévio à punção para coleta de LCR, tendo como finalidade o adequado esclarecimento dos pacientes quanto aos riscos do procedimento e às medidas de prevenção de complicações do exame. O documento final do TCLE foi resultado de consenso entre os membros de DC-LCR. O DC-LCR da ABN considerou também pertinente e importante a divulgação das normas legais relacionadas à pesquisas em seres humanos em nosso país.
Hemicrania continua: a report of ten new cases
Authors: Bigal Marcelo E. , Tepper Stewart J , Sheftell Fred D. , Rapoport Alan M.
Published: April 2005

Hemicrania continua (HC) is an uncommon primary headache first described as a syndrome in 1984. Being quite unusual, its clinical characterization still demands better description. The aim of this study is to present the main clinical characteristics of 10 patients with the diagnosis of HC seen in a tertiary center, critically discussing their main features. All subjects had strictly unilateral headache without side shift and absolute response to indomethacin. Seven patients (70%) presented autonomic features during pain exacerbations. Four (40%) had migrainous symptoms during the exacerbations and one presented partial relief with dihydroergotamine. One patient had pain excruciatingly severe during the exacerbations. Although the cardinal features of HC - continuous, unilateral, indomethacin responsive, remain strongly reliable, a refinement on the clinical characterization is needful and desired.
A comparison between averaged spikes and individual visually-analyzed spikes in rolandic epileptiform discharges
Authors: Braga Nadia I.O. , Manzano Gilberto M. , Nóbrega João Antonio M.
Published: April 2005

PURPOSE:This study compared some morphological features of individual rolandic epileptiform discharges, used to obtain an averaged estimate, with those of the resulting estimate. METHOD: Twenty-four averaged discharges from EEGs of 24 children showing rolandic spikes were compared with 480 individual discharges used in the averaging. The analysis was based on the occurrence of tangential dipole and "double spike" patterns. RESULTS: In 15 averaged discharges the tangential dipole pattern was found. Individual spikes used in the averaging process displayed the same pattern in 35-100% of them; in the remaining 9 averaged discharges, up to 20% of the individual spikes showed the same pattern, although this was not found in the averaged waveforms. "Double spike" pattern was found in 11 of the averaged waveforms and was recognized in 50-100% of its individual discharges, whereas up to 45% of individual spikes showed this pattern without expression in the averaged waveform. CONCLUSION: When visually analyzing an EEG with rolandic spikes, caution should be exercised in characterizing these patterns, since a few discharges showing them may not be expressed in the averaged waveform and the clinical correlations proposed for these patterns may not apply.
Insomnia and hypnotic use in Campo Grande general population, Brazil
Authors: Souza José Carlos , Magna Luiz Alberto , Reimão Rubens
Published: April 2005

The insomnia prevalence in general population was determined by means of 408 home interviews of adults, in a representative sample of Campo Grande city, Brazil. The random sample was stratified by sex, age and economic social status. Insomnia subtypes evaluated were the disorders of sleep initiation (DSI), sleep maintenance (DSM) and early awakening (DEA). A structured questionnaire was used with the consent from the interviewed subjects. Statistics used chi-square, and Fisher tests; and inferences based on binomial distribution parameters; the significance level was 5% and confidence interval (CI) was 95%.The general prevalence of insomnia was 19.1% (sd=2.0%), mostly women (p=0.0015), and people of less years of schooling (p=0.0317), subtype DSI (14.2%, p=0.0043), and chronic (p=0.7022). Hypnotic drugs were used by 6.9%(sd=1.3%) in the last month. Use in the last 2 years, 70.3% mostly insomniacs (p<0.0001), women (p=0.0372) and people over 30 years of age (p=0.0536).
Inferences from a community study about non-epileptic events
Authors: Gomes Marleide da Mota , Kropf Leandro Albuquerque Lemgruber , Beeck Erica da Silva van , Figueira Ivan Luiz de Vasconcellos
Published: April 2005

OBJECTIVE: To demonstrate the epidemiological importance of the different types of non-epileptic events (NEE) in a low-income urban community. METHOD: The patients suspected of having epilepsy, who were detected in the first phase (screening one) of this prevalence study, were interviewed by a neurologist in a non-structured neurological interview. These NEE were classified as physiological and psychogenic, subdivided by various types. The psychogenic NEE were classified according to the DSM-IV criteria. RESULTS: We compared the cases suspected of having epilepsy (n=176) with those not suspected (n=806) and discovered that those cases suspected of having epilepsy had a greater median age (<0.01) and female predominance (p<0.01). Among the cases suspected of having epilepsy there were different diagnosis: epileptic events without identifiable cause (n=20) or with identifiable causes (e.g., febrile convulsions and eclampsia). The most prevalent diagnosis for those suspected of having epilepsy was syncope (n=63; 35.8%). In terms of physiological events, the most frequent were: epileptic seizures, paroxysmal toxic phenomena (including alcoholism) and brain trauma, besides syncope; in terms of psychogenic events the most frequent were: dissociative and anxiety disorders. Regarding gender differences, paroxysmal toxic problems were significantly more prevalent in men (p= 0.02), and dissociative disorders (p=0.01) in women. CONCLUSION: This survey confirms the epidemiological importance of syncope in a populational sample with NEE. However, among the psychogenic disorders of this NEE sample, the most frequent were dissociative and anxiety phenomena. This finding contrasts with the literature based on samples from tertiary epileptic centers with video-EEG resources, which found somatoform disorders to be more prevalent than dissociative and anxiety phenomena.
Cerebellar liponeurocytoma: a newly recognized clinico-pathological entity
Authors: Montagna Nádia , Moreira Daniel , Vaz Luiz Carlos , Reis Marcelo
Published: April 2005

The term "cerebellar liponeurocytoma", recently adopted by the World Health Organization Working Group (WHO), replaced many other different terms used up to now to give name to this rare tumor. To our knowledge, less than 20 cases have been related up to now under different names like as "lipomatous medulloblastoma, lipidized medulloblastoma, neurolipocytoma, medullocytoma and lipomatous glioneurocytoma". The new nomenclature eliminates the word "medulloblastoma", reinforces its benign caracter, and includes it in the category of glioneuronal tumors. We describe an adictional case of this distinct clinico-pathological entity removed from the right cerebellar hemisphere of a 53-year-old woman. With the present case report, we hope to contribute to the knowledge on the diagnostic and prognostic implications derived from the finding of mature adipose-like tissue within a medulloblastomatous tumour.
Cerebral trypanosomiasis and AIDS
Authors: Antunes Apio Claudio Martins , Cecchini Felipe Martins de Lima , Bolli Fernando von Bock , Oliveira Patricia Polanczyk de , Rebouças Ricardo Gurgel , Monte Thais Lampert , Fricke Daniele
Published: April 2005

A 36 year-old black female, complaining of headache of one month's duration presented with nausea, vomiting, somnolence, short memory problems, loss of weight, and no fever history. Smoker, intravenous drugs abuser, promiscuous lifestyle. Physical examination: left homonimous hemianopsia, left hemiparesis, no papilledema, diffuse hyperreflexia, slowness of movements. Brain CT scan: tumor-like lesion in the splenium of the corpus calosum, measuring 3.5 x 1.4 cm, with heterogeneous enhancing pattern, sugesting a primary CNS tumor. Due to the possibility of CNS infection, a lumbar puncture disclosed an opening pressure of 380 mmH(2)0; 11 white cells (lymphocytes); glucose 18 mg/dl (serum glucose 73 mg/dl); proteins 139 mg/dl; presence of Trypanosoma parasites. Serum Elisa-HIV tests turned out to be positive. Treatment with benznidazole dramatically improved clinical and radiographic picture, but the patient died 6 weeks later because of respiratory failure. T. cruzi infection of the CNS is a rare disease, but we have an increasing number of cases in HIV immunecompromised patients. Diagnosis by direct observation of CSF is uncommon, and most of the cases are diagnosed by pathological examination. It is a highly lethal disease, even when properly diagnosed and treated. This article intends to include cerebral trypanosomiasis in the differential diagnosis of intracranial space-occupying lesions, especially in immunecompromised patients from endemic regions.
Schwartz-jampel syndrome: report of five cases
Authors: Reed Umbertina Conti , Reimão Rubens , Espíndola Adriana Ávila , Kok Fernando , Ferreira Lúcio Gobbo , Resende Maria Bernardete Dutra , Messias Thelma Correia , Carvalho Mary Souza , Diament Aron , Scaff Milberto , Marie Suely Kazue Nagahashi
Published: April 2005

We describe five patients with Schwartz-Jampel syndrome (SJS) examined at the outpatient service for neuromuscular disorders at our Institution from 1996 to 1999 with the objective of emphasizing the characteristic dysmorphic phenotype of SJS and its different clinical forms. Two cases presented SJS-type 1A, two had SJS-type 1B and one manifested SJS-type 2. Two boys with 3 and 13 years of age had generalized stiffness and the characteristic facial as well as osteoarticular changes from birth. Other two boys with 11 and 7 years had less marked dysmorphic changes at birth and manifested myotonia, as a limiting factor, during the second year of age. A girl with two months of age had severe myotonia from birth leading to feeding diffuculties. In all cases the diagnosis was based on dysmorphic features, and on electromyographic changes showing continuous electrical activity of muscle fibers. All were treated with carbamazepine, 20-30 mg/Kg since diagnosis. The four boys (all with normal intelligence) improved of myotonia in daily activities, markedly in three, and moderately in one. The girl did not improve and showed global development delay: by the last follow-up (at 20 months of age) she did not sit unsupported, and had mental retardation. Carbamazepine in SJS-type 1 improves general daily performance and psychological status of the patients.
King-Denborough Syndrome: report of two Brazilian cases
Authors: Reed Umbertina Conti , Resende Maria Bernardete Dutra , Ferreira Lúcio Gobbo , Carvalho Mary Souza , Diament Aron , Scaff Milberto , Marie Suely Kazue Nagahashi
Published: April 2005

We report on two boys aged 2 and 6 years-old respectively with dysmorphic face, ptosis, down-slanting palpebral fissures, hypertelorism, epicanthic folds, low-set ears, malar hypoplasia, micrognathia, high-arched palate, clinodactyly, palmar simian line, pectus excavatum, winging of the scapulae, lumbar lordosis and mild thoracic scoliosis who present congenital hypotonia, slightly delayed motor development, diffuse joint hyperextensibility and mild proximal weakness. The muscle biopsy revealed minimal but identifiable changes represented by size fiber variability, type I fiber predominance and atrophy, perimysial fibrous infiltration and some disarray of the intermyofibrillary network. These cases correspond to the first Brazilian reports of the King-Denborough syndrome and our objective is increasing the awareness of this disorder as these patients are predisposed to developing malignant hyperthermia.
Cefaléia associada a indicadores de transtornos de ansiedade em uma amostra de escolares de Ribeirão Preto, SP
Authors: Gorayeb Maria Angela Marchini , Gorayeb Ricardo
Published: April 2005

Avaliaram-se 374 crianças de 8 a 13 anos de idade em escolas públicas de Ribeirão Preto, objetivando averiguar a prevalência de queixas de cefaléia e sua associação com indicadores de transtornos de ansiedade. Os pais responderam um questionário sobre a presença e frequência de cefaléia e de comportamentos/sintomas associados a transtornos de ansiedade. As crianças foram avaliadas através da escala de ansiedade "O que Penso e Sinto". Das 374 crianças, 45,4% nunca se queixaram de cefaléia; 41,2%, ocasionalmente e 13,5%, frequentemente. Entre aquelas que se queixam frequentemente de cefaléia, encontrou-se maior número de meninas, índice de ansiedade mais elevado e maior frequência de bruxismo e agitação. A alta prevalência de cefaléia na amostra e sua associação com maior frequência de bruxismo é compatível com dados epidemiológicos norte-americanos e brasileiros. Os resultados apontam para uma associação entre queixas frequentes de cefaléia e elevado índice de ansiedade.
[NO TITLE AVAILABLE]
Authors: Andraus Maria Emilia Cosenza , Cosenza Carlos Alberto Nunes , Fonseca Léa Mirian Barbosa da , Andraus Cesar Fantezia , D'Andrea Isabela , Alves-Leon Soniza Vieira
Published: April 2005

O objetivo desse estudo foi avaliar a utilidade do SPECT cerebral interictal na localização do foco epileptogênico em 23 pacientes do Ambulatório de Epilepsias do Hospital Universitário Clementino Fraga Filho (HUCFF/UFRJ), com epilepsia do lobo temporal (ELT) de difícil controle e tomografia computadorizada (TC) do crânio normal, estudando a correlação entre SPECT interictal, eletrencefalograma (EEG) e, em 11 casos, ressonância magnética (RM) do crânio, e comparar os resultados com os de outras seis séries da literatura. Doze (52,2%) pacientes apresentaram SPECT anormal. Entre esses, cinco (41,6% dos SPECTs anormais) apresentaram alteração unilateral ao SPECT do mesmo lado do EEG (hipoperfusão em 4 e hiperperfusão em 1), três (25% dos SPECTs anormais) apresentaram hipoperfusão bilateral ao SPECT e alterações também bilaterais ao EEG e 4 (33,3% dos SPECTs anormais) apresentaram hipoperfusão unilateral ao SPECT e EEG bilateral. A análise estatística fundamentou-se na lógica fuzzy. Os índices de correlação entre SPECT X EEG, SPECT X RM e SPECT X EEG X RM foram altamente significativos, com níveis de significância de 0,01, p < 0,0005 e intervalo de 99% de confiança em todas as correlações. Os estudos de correlação entre as séries estudadas apresentaram resultados semelhantes entre si.
Epilepsia parcial associada a tumores cerebrais primários
Authors: Brainer-Lima Paulo Thadeu , Brainer-Lima Alessandra Mendes , Azevedo Filho Hildo , Cukiert Arthur
Published: April 2005

OBJETIVO: Estudar as características clínicas da epilepsia em pacientes com tumores cerebrais primários de crescimento lento. MÉTODO: Quarenta e dois pacientes com epilepsia refratária ao tratamento medicamentoso associada a tumor cerebral de crescimento lento foram avaliados entre junho de 1992 e setembro de 1999. RESULTADOS: Setenta e cinco por cento dos pacientes tinha menos de 15 anos quando iniciaram-se as crises convulsivas. O exame neurológico foi normal em 67,7% dos pacientes. Predominaram as crises epilépticas do tipo parcial complexa (90,4%), com até 16 crises mensais. As crises foram do tipo parcial simples (PS) evoluindo para parcial complexa (PC) em 10 pacientes, PC isoladas em 11 e PC evoluindo para generalizada em 11. CONCLUSÃO: Noventa e cinco por cento dos pacientes com tumores cerebrais de crescimento lento apresentaram crises parciais, mesmo que com baixa frequência. Estas últimas podem ser uma manifestação precoce de tumor de crescimento lento nas crianças, mesmo sem nenhuma alteração no exame neurológico.
Fatores prognósticos no tratamento dos tumores intramedulares
Authors: Koerbel Andrei , Tatsui Claudio Esteves , Prevedello Daniel Monte-Serrat , Hanel Ricardo Alexandre , Grande César Vinícius , Ditzel Léo Fernando da Silva , Araújo João Cândido , Antoniuk Affonso
Published: April 2005

Entre 1993 e 1999, foram analisados 35 pacientes submetidos a microcirurgia para remoção de tumor intramedular nos Serviços de Neurocirurgia do Hospital Nossa Senhora das Graças e do Hospital de Clínicas de Curitiba - PR. A população foi composta por 68,6% de pacientes do sexo masculino; a média de idade era de 32,9 anos. A localização tumoral mais frequente foi torácica em 40% dos casos, seguida pela região cervical e cervicotorácica em 25,7%. Ao exame neurológico realizado entre 6 a 12 meses do pós-operatório, 42,9% dos pacientes demonstraram melhora, 34,3% permaneceram estáveis e 22,9% dos casos apresentaram piora. A ressecção foi total em 57,2% dos casos e subtotal em 37,1%. Em 5,7% dos pacientes, foi realizada apenas biópsia para diagnóstico. O grau de ressecção completa foi maior nos ependimomas (13 de 17 casos) e menor nos astrocitomas (5 de 12 casos). Entretanto, o grau de ressecção e o tipo histológico não interferiram na morbidade pós-operatória. Fatores como sexo, idade e tamanho do tumor também não demonstraram importância na evolução após a cirurgia. A localização torácica do tumor foi associada a maior morbidade cirúrgica (p=0,021).
Síndrome de Landau-Kleffner e regressão autística: a importância do diagnóstico diferencial
Authors: Ribeiro Karla M.N. , Assumpção Jr. Francisco B. , Valente Kelte D.R.
Published: April 2005

Algumas doenças neurológicas podem apresentar sinais e sintomas psiquiátricos, portanto a exploração do diagnóstico etiológico é crucial. O objetivo deste estudo é relatar o caso de um paciente com um distúrbio neurológico, diagnosticado durante internação psiquiátrica. Um menino com desenvolvimento neuropsicomotor normal até 3 anos, quando começou a apresentar crises epilépticas, seguidas por distúrbio de comportamento e deterioração da linguagem. Durante o acompanhamento neurológico, o paciente foi encaminhado ao Departamento de Psiquiatria com a suspeita de autismo, regressão autística (RA). Durante internação, o diagnóstico de síndrome de Landau-Kleffner (SLK) foi estabelecido em bases clínicas e eletrencefalográficas. A SLK é caracterizada por afasia adquirida, epilepsia, anormalidades eletrencefalográficas e distúrbios de comportamento, incluindo traços autísticos. A regressão da linguagem é observada na SLK e na RA. Enfatizamos as principais diferenças entre estas entidades, pois o diagnóstico errôneo adia a intervenção precoce e benefícios, como observado em nosso caso.
Calcificação intracraniana occipital bilateral, epilepsia e doença celíaca: relato de caso
Authors: Santos Carlos Henrique Souza , Almeida Iara Leda Brandão de , Gomes Maria Durce Costa , Serafim Alexandre , Pereira Mariana Machado , Muszkat Mauro , Rizzutti Sueli , Patrício Francy Reis da Silva , Miranda Mônica Carolina , Vilanova Luiz Celso Pereira
Published: April 2005

Relatamos o caso de uma menina com 6 anos de idade que apresentava episódios recorrentes de diarréia desde os 6 meses de vida associada a anemia ferropriva com desenvolvimento neuromotor normal. Aos 3 anos de idade começou a apresentar crises parciais que foram controladas com carbamazepina. Tomografia computadorizada de crânio aos 5 anos demonstrou calcificações girais grosseiras nas regiões occipital e parietal posterior bilateralmente. A ressonância magnética de crânio evidenciou áreas de hipossinal em T2 na região parieto-occipital bilateralmente. Realizou investigação para síndrome de mal absorção incluindo estudo da função digestivo/absortiva (teste D-xilose), avaliação sorológica (anticorpos antigliadina, antiendomísio e antitransglutaminase) e biopsia de intestino delgado que demonstrou intensa atrofia de vilosidades com infiltrado linfoplasmocitário no córion compatível com doença celíaca.
Adenocarcinoma do saco endolinfático: relato de caso
Authors: Silveira Roberto Leal , Gusmão Sebastião Silva , Pittella José Eymard H. , Santos Sinval Pereira
Published: April 2005

Apresenta-se um caso de adenocarcinoma do saco endolinfático e revê-se a literatura. O quadro clínico manifestou-se por tonteira e perda progressiva da audição consequentes a tumor do saco endolinfático. A remoção cirúrgica foi completa e realizada por abordagem retro e translabiríntica. Os tumores do saco endolinfático são raros, localmente invasivos e comprometem a mastóide e parte petrosa do osso temporal e o ângulo pontocerebelar. A remoção cirúrgica completa apresenta bom prognóstico.
Sarcoma granulocítico de sistema nervoso central pós transplante de medula óssea: relato de caso
Authors: Tatsui Cláudio Esteves , Koerbel Andrei , Prevedello Daniel Monte-Serrat , Araújo João Candido , Ditzel Léo Fernando da Silva , Bleggi-Torres Luis Fernando
Published: April 2005

Sarcoma granulocítico é tumor sólido, constituído por células precursoras de granulócitos, localizado em sítio extra medular. Geralmente surge precedendo uma leucemia mielóide aguda ou concomitantemente a ela, sendo considerado fator de mau prognóstico. Tem como principais modalidades terapêuticas a radioterapia e a quimioterapia. Um caso de sarcoma granulocítico intracraniano ocorrido seis meses após transplante de medula óssea por leucemia mielóide aguda é relatado. A paciente apresentava cefaléia e hemiplegia esquerda, causada por extensa lesão fronto-parietal direita. Após a ressecção completa do tumor, houve total recuperação do déficit neurológico. A paciente completou o tratamento radio e quimioterápico, estando livre de doença após três meses de acompanhamento. O manejo cirúrgico do sarcoma granulocítico é modalidade adjuvante, indicada quando o efeito compressivo tumoral determina déficit neurológico. É o meio mais rápido de descompressão do tecido nervoso, possibilitando a chance de recuperação funcional, melhorando a qualidade de vida do paciente.