Atrofia cerebelar relacionada à exposição ocupacional crônica ao tolueno: relato de caso

A 31-year-old woman presented slowly progressing ataxia and neurasthenic symptoms after 14-year occupational exposure to low concentration toluene vapour. Examination disclosed only cerebellar signs. Cognitive functions were normal except moderate visuo-spatial and constructive deficit CT imaging showed severe pancerebellar atrophy without pathological signs in other brain structures. Two years after she was removed from workplace, CT imaging and ataxia showed no worsening, while visuo-constructive function improved. The authors warn against possible neurotoxic risk associated with this kind of exposure.Uma mulher de 31 anos desenvolveu síndrome cerebelar e neurastênica, de evolução lentamente progressiva, após 14 anos de exposição ocupacional a vapores de tolueno em baixas concentrações. Os únicos achados do exame neurológico e neuropsicológico foram sinais cerebelares (severos) e déficit visuo-espacial e práxico-construcional (moderado). A neuroimagem tomográfica mostrava acentuada atrofia pancerebelar, com preservação das outras estruturas cerebrais. Dois anos após interrompida a exposição, a ataxia e a neuroimagem (TC) estavam inalteradas, enquanto a função visuo-construcional havia parcialmente melhorado. Os autores chamam a atenção para o possível risco neurotóxico relacionado a esse tipo de exposição, numa tentativa de prevenção de casos semelhantes.909

Valor preditivo da punção liquórica (tap test) na hidrocefalia de pressão normal

Eighteen patients (mean age of 66.5 years) with normal pressure hydrocephalus (NPH) underwent a ventriculo-peritoneal shunt surgery. Prior to operation a cerebrospinal fluid tap-test (CSF-TT) was performed with measurements of gait pattern and psychometric functions (memory, visuo-motor speed and visuo-constructive skills) before and after the removal of 50 ml CSFby lumbar puncture (LP). Fifteen patients improved and 3 were unchanged after surgery. Short duration of disease, gait disturbance preceding mental deterioration, wide temporal horns and small sulci on CT-scan were associated with good outcome after shunting. There was a good correlation between the results of CSF-TT and shunt surgery (X² = 4,11 ,phi = 0.48, p < 0.05), with gait test showing highest correlation (r = 0.99, p = 0.01). In conclusion, this version of CSF-TT proved to be an effective test to predict improvement after shunting in patients with NPH.Este estudo visa melhorar o valor diagnóstico e preditivo da punção liquórica (tap test, TT) na hidrocefalia de pressão normal (HPN), particularmente nos casos em que os dados clinicos e de neuroimagem são inconclusivos e não permitem uma decisão cirúrgica segura. Dezoito pacientes (média de idade = 66.5 anos) com HPN foram submetidos a derivação (shunt) ventriculo-peritoneal. O TT era realizado antes da cirurgia, consistindo na análise quantitativa da marcha e funções cognitivas (memória, rapidez perceptivo-motora e habilidade vísuo-construtiva) antes e depois da retirada de 50 ml de liquor via punção lombar. Quinze pacientes melhoraram e 3 permaneceram inalterados após a cirurgia. Curta duração da doença, distúrbio da marcha precedendo a deterioração mental, dilatação dos cornos temporais e apagamento (ou redução) dos sulcos corticais na tomografia computadorizada cerebral estavam associados a bons resultados pós-operatórios. Houve boa correlação entre os resultados do TT e os do shunt (X² = 4.11, phi = 0.48, p < 0.05), principalmente no teste da marcha (r = 0.99, p = 0.01). Esta versão do TT liquórico mostrou-se efetiva na prediçâo da melhora pós-operatória em pacientes com HPN.17918

[validation Of The Brazilian Version Of Mini-test Casi-s].

To determine CASI-S accuracy in the diagnosis of dementia. The Cognitive Abilities Screening Instrument - Short Form (CASI-S) was applied in 43 Alzheimer's disease (AD) patients and 74 normal controls. AD diagnosis was based on DSM-IV, NINCDS-ADRDA, and CAMDEX. CASI-S includes: registration, temporal orientation, verbal fluency (4-legged animals in 30s), and recall (3 words). Its maximum score is 33 points. A copy of 2 pentagons was added. ROC curve showed an accuracy of 0.87, with standard error of 0.032, and 95% confidence intervall between 0.795 and 0.925. The cut-off score for cognitive deficit was 23, with sensitivity of 76.7%, specificity 86.5%, positive likelihood ratio (LR) 5.68, and negative LR 0.27. The cut-off score for subjects 70 years or older was 20, with sensitivity of 71.4% and specificity 97.1%. CASI-S is a practical test, with high specificity, particularly in individuals above 70 years of age. The adding of the drawing test did not improve its accuracy.63416-2

A comparative study of pelvic floor muscle training in women with multiple sclerosis: its impact on lower urinary tract symptoms and quality of life

OBJECTIVE: To compare pelvic floor muscle training and a sham procedure for the treatment of lower urinary tract symptoms and quality of life in women with multiple sclerosis. METHODS: Thirty-five female patients with multiple sclerosis were randomized into two groups: a treatment group (n = 18) and a sham group (n = 17). The evaluation included use of the Overactive Bladder Questionnaire, Medical Outcomes Study Short Form 36, International Consultation on Incontinence Questionnaire Short Form, and Qualiveen questionnaire. The intervention was performed twice per week for 12 weeks in both groups. The treatment group underwent pelvic floor muscle training with assistance from a vaginal perineometer and instructions to practice the exercises daily at home. The sham group received a treatment consisting of introducing a perineometer inside the vagina with no exercises required. Pre- and post-intervention data were recorded. RESULTS: The evaluation results of the two groups were similar at baseline. At the end of the treatment, the treatment group reported fewer storage and voiding symptoms than the sham group. Furthermore, the differences found between the groups were significant improvements in the following scores in the treatment group: Overactive Bladder Questionnaire, International Consultation on Incontinence Questionnaire Short Form, and the General Quality of Life, and Specific Impact of Urinary Problems domains of the Qualiveen questionnaire. CONCLUSIONS: The improvement of lower urinary tract symptoms had a positive effect on the quality of life of women with multiple sclerosis who underwent pelvic floor muscle training, as the disease-specific of quality of life questionnaires demonstrated. This study reinforces the importance of assessing quality of life to judge the effectiveness of a treatment intervention

Aspectos físicos e mentais na qualidade de vida de pacientes com doença de Parkinson idiopática

Parkinson disease (PD) is a chronic and progressive disorder characterized by degeneration of dopaminergic neurons in the mesencephalic substantia nigra. Lesions involving the subcortical pathways, especially the nigro-striato-pallidal path, are particularly common, and cause a series of disabling signs and symptoms. The aim of this study was to analyse the quality of life of individuals with PD and compare them to healthy controls. Forty subjects of both sexes were analysed, aged between 61 and 83, divided into two groups: Parkinson group (n=21), made up of individuals with idiopathic PD; and control group (n=19), of subjects with no neurodegenerative disorder. Data were collected by means of the Brazilian version of the Short Form-36 (SF-36) questionnaire. Data were statistically analysed and the significance level set at 1%. PD group subjects had SF-36 scores significantly lower than control ones, both in physical and mental health domains. Such low, perceived health-related quality of life among PD subjects is probably due to the co-occurrence of the neurodegenerative phenomena and the psychological distress provoked both by the disease itself and the stigma assigned to it. Interdisciplinary approaches should be encouraged to improve the quality of life of PD patients, aiming at postponing the physical and mental decline inherent to this degenerative condition.A doença de Parkinson (DP) é um distúrbio crônico e progressivo, caracterizado pela degeneração de neurônios dopaminérgicos da substantia nigra mesencefálica. A lesão das vias subcorticais, em especial a nigro-estriato-palidal, é particularmente comum e ocasiona uma série de sinais e sintomas incapacitantes. O objetivo deste trabalho foi analisar a qualidade de vida (QV) relacionada à saúde de sujeitos com DP e compará-la à de indivíduos controles saudáveis. Foram avaliados 40 sujeitos com idades entre 61 e 83 anos, de ambos os sexos, que foram divididos em dois grupos: grupo Parkinson (n=21), composto por sujeitos com DP idiopática, e grupo controle (n=19), de indivíduos sem distúrbios neurodegenerativos. Para a coleta de dados, foi utilizada a versão brasileira do questionário Brasil SF-36. Os resultados foram analisados estatisticamente, com nível de significância fixado em 1%. Os sujeitos do grupo Parkinson apresentaram escores significativamente mais baixos do que os do grupo controle, tanto no domínio físico quanto no mental, medidos pelo SF-36. Essa baixa QV provavelmente é explicada pela co-ocorrência de fenômenos neurodegenerativos e do sofrimento psíquico gerado pela doença e pelo estigma que ela produz. Abordagens interdisciplinares devem ser estimuladas a pacientes com DP, com o objetivo de melhorar sua qualidade de vida e postergar o declínio físico e mental inerente às condições degenerativas

Aquaporin-4 Antibodies Are Not Related to HTLV-1\ud Associated Myelopathy

Introduction: The seroprevalence of human T-cell leukemia virus type 1 (HTLV-1) is very high among Brazilians (,1:200).\ud HTLV-1 associated myelopathy or tropical spastic paraparesis (HAM/TSP) is the most common neurological complication of\ud HTLV-1 infection. HAM/TSP can present with an acute/subacute form of longitudinally extensive myelitis, which can be\ud confused with lesions seen in aquaporin-4 antibody (AQP4-Ab) positive neuromyelitis optica spectrum disorders (NMOSD)\ud on MRI. Moreover, clinical attacks in patients with NMOSD have been shown to be preceded by viral infections in around\ud 30% of cases.\ud Objective: To evaluate the frequency of AQP4-Ab in patients with HAM/TSP. To evaluate the frequency of HTLV-1 infection\ud in patients with NMOSD.\ud Patients and Methods: 23 Brazilian patients with HAM/TSP, 20 asymptomatic HTLV-1+ serostatus patients, and 34 with\ud NMOSD were tested for AQP4-Ab using a standardized recombinant cell based assay. In addition, all patients were tested for\ud HTLV-1 by ELISA and Western blotting.\ud Results: 20/34 NMOSD patients were positive for AQP4-Ab but none of the HAM/TSP patients and none of the\ud asymptomatic HTLV-1 infected individuals. Conversely, all AQP4-Ab-positive NMOSD patients were negative for HTLV-1\ud antibodies. One patient with HAM/TSP developed optic neuritis in addition to subacute LETM; this patient was AQP4-Ab\ud negative as well. Patients were found to be predominantly female and of African descent both in the NMOSD and in the\ud HAM/TSP group; Osame scale and expanded disability status scale scores did not differ significantly between the two\ud groups.\ud Conclusions: Our results argue both against a role of antibodies to AQP4 in the pathogenesis of HAM/TSP and against an\ud association between HTLV-1 infection and the development of AQP4-Ab. Moreover, the absence of HTLV-1 in all patients\ud with NMOSD suggests that HTLV-1 is not a common trigger of acute attacks in patients with AQP4-Ab positive NMOSD in\ud populations with high HTLV-1 seroprevalence.This study received financial support from the Brazilian government agencies FAPESP (Fundação de Amparo à Pesquisa do Estado de São Paulo - www. fapesp.br/en) and CAPES (Coordenação de Aperfeiçoamento de Pessoal de Nível Superior - www.capes.gov.br). The work of S.J. and B.W. was supported by research grants from Bayer Schering Healthcare and from Merck Serono. The funders had no role in study design, data collection and analysis, decision to publish, or preparation of the manuscript

Plasmacytoid dendritic cells are increased in cerebrospinal fluid of untreated patients during multiple sclerosis relapse

The plasmacytoid dendritic cells (pDCs) express a high level of Toll-like receptor 9 (TLR-9), which recognizes viral DNA. Activated via TLR-9, pDCs also secrete large amounts of type I interferon which are involved either in stimulation or down regulation of immune response in multiple sclerosis (MS). In the present study, we determinate pDCs levels by flow cytometry in Cerebrospinal Fluid (CSF) and Peripheral Blood from MS patients in relapsing and in remitting phases of the disease, comparing with other non-inflammatory diseases (OND). We provide evidence that MS patients in relapse without any treatment have a significantly (p < 0.01) higher percentage of pDCs in CSF than do patients in remission or those with OND. No change in the percentage of pDCs was observed in the peripheral blood of any of these patients. The increase of pDCs in central nervous system during relapse may be explained either by a virus infection or a down regulatory process

Asymmetrical hippocampal connectivity in mesial temporal lobe epilepsy: evidence from resting state fMRI

<p>Abstract</p> <p>Background</p> <p>Mesial temporal lobe epilepsy (MTLE), the most common type of focal epilepsy in adults, is often caused by hippocampal sclerosis (HS). Patients with HS usually present memory dysfunction, which is material-specific according to the hemisphere involved and has been correlated to the degree of HS as measured by postoperative histopathology as well as by the degree of hippocampal atrophy on magnetic resonance imaging (MRI). Verbal memory is mostly affected by left-sided HS, whereas visuo-spatial memory is more affected by right HS. Some of these impairments may be related to abnormalities of the network in which individual hippocampus takes part. Functional connectivity can play an important role to understand how the hippocampi interact with other brain areas. It can be estimated via functional Magnetic Resonance Imaging (fMRI) resting state experiments by evaluating patterns of functional networks. In this study, we investigated the functional connectivity patterns of 9 control subjects, 9 patients with right MTLE and 9 patients with left MTLE.</p> <p>Results</p> <p>We detected differences in functional connectivity within and between hippocampi in patients with unilateral MTLE associated with ipsilateral HS by resting state fMRI. Functional connectivity resulted to be more impaired ipsilateral to the seizure focus in both patient groups when compared to control subjects. This effect was even more pronounced for the left MTLE group.</p> <p>Conclusions</p> <p>The findings presented here suggest that left HS causes more reduction of functional connectivity than right HS in subjects with left hemisphere dominance for language.</p

Whipple's Disease With Neurological Manifestations: Case Report.

Whipple's disease (WD) is an uncommon multisystem condition caused by the bacillus Tropheryma whipplei. Central nervous system involvement is a classical feature of the disease observed in 20 to 40% of the patients. We report the case of a 62 year old man with WD that developed neurological manifestations during its course, and discuss the most usual signs and symptoms focusing on recent diagnostic criteria and novel treatment regimens.62342-

Clinical treatment of resistant epilepsy

The authors analysed 34 cases of resistant epilepsy (20 males and 14 females, mean age 23 years), treated clinically between February/1984 and May/1986. The patients underwent neurological, neuropsychological, psychological, psychiatric, cerebrospinal fluid, electroencephalographic, tomographic and/or angiographic examination. Most of the patients had complex partial seizures. The etiology was unknown in 19 patients (55.8%), probable neurocysticercosis in 6, perinatal hypoxia in 5, delivery trauma in 3 and probable sequelae of encephalitis in 2 patients. There was a clear past history of infantile febrile convulsion in 2 patients. Most patients received carbamazepine (mean dose 24.5 mg/kg/day), phenytoin (5 mg/kg and valproic acid (28 mg/kg) as monotherapy or in association. Twenty-two patients (64.7%) had more than 80% decrease of the seizure frequency. Nine resistant epilepsy-cases (24.5%) were evaluated as candidates for surgical therapy. The authors concluded that the resistant epilepsy is best managed by a specialised, multidisciplinary team, and pointed out the need of a correct diagnosis of the seizure type, an adequate drug therapy and a good engagement of the patient and his family in the treatment.Foram estudados 34 casos de epilepsia de difícil controle, encaminhados ao Grupo de Trabalho de Epilepsia (GTE) do Instituto de Neurologia de Goiânia de 1984 a 1986. Todos eram submetidos a reavaliação neurológica. Vinte e três dos 34 pacientes tinham crises parciais complexas. A maioria foi tratada com carbamazepina, difenil-hidantoína e ácido valpróico, em forma monoterápica ou associada entre si. Obteve-se redução de mais de 80% da freqüência das crises em 22 pacientes (64,7%), além de leve melhora da qualidade de vida. Os autores concluem sobre as vantagens de uma equipe especializada, multidisciplinar, na abordagem da epilepsia rebelde e ressaltam a necessidade de: a. um correto diagnóstico das crises, seguido do uso da medicação apropriada, por tempo suficiente (mínimo de 0 meses) e em dose adequada (até a dose máxima tolerável), abandonando-a só depois do controle de sua concentração sérica; b. um bom engajamento do paciente e de sua família no tratamento.35135