Acute neuromuscular manifestations in a patient associated with ingesting octopus (Octopus sp.)

Os autores relatam um quadro manifestado por sintomas neurológicos e musculares em uma mulher de 45 anos, que surgiu após o consumo da carne de polvo comum (Octopus sp.). A paciente apresentou intenso mal estar, parestesias em extremidades e área perioral, fraqueza muscular intensa e hipotensão arterial, seguidos de prurido importante e uma erupção eritêmato-descamativa disseminada tardia. Não foram observadas manifestações gastrintestinais ou febre, o que reduziu a probabilidade de uma intoxicação alimentar por conservação inadequada do molusco. A presença de sintomas neuro-musculares é sugestiva de ação de neurotoxinas, comprovadamente existentes em muitos gêneros de polvos e que podem ter sido ingeridas através do consumo das glândulas salivares ou acúmulo das toxinas na carne, por algum mecanismo ainda desconhecido. As toxinas dos polvos do gênero Octopus são pouco estudadas e julgamos esta comunicação importante por alertar para a possibilidade do envenenamento nos seres humanos que consomem carne de polvos e ainda sua diferenciação das intoxicações alimentares que ocorrem por conservação inadequada do animal.

Atypical manifestations in Brazilian patients with neuro-Behcet's disease

Type and frequency of systemic and neurologic manifestations of Beh double dagger et's disease (BD) vary with ethnicity. In Brazil, BD occurs as sporadic cases. We describe clinical and radiological features of 36 Brazilian patients of mixed ethnicity with neuro-Beh double dagger et's disease (NBD). Medical records of 178 BD patients were reviewed and 36 (20%) NBD patients were identified. Twenty-one NBD patients (58.3%) were female and 27 (75%) presented with parenchymal manifestations. Brainstem involvement was the most common neurologic syndrome (41.7%). Seizures (27.8%), isolated aseptic meningitis (16.7%), optic neuropathy (ON) (16.7%), cerebral venous thrombosis (CVT) (8.3%), peripheral neuropathy (2.8%), and spinal cord involvement (5.6%) were other neurologic manifestations observed among Brazilian NBD patients. Eighteen (50%) had at least one relapse, and isolated aseptic meningitis was the most common relapsing manifestation. No significant differences concerning the number of relapses between parenchymal and non-parenchymal groups were found. A multivariate model including disease duration, cell count in spinal fluid, cyclosporine use, immunosuppressive use at disease onset, age at NBD onset, and ON did not reveal any significant associations with NBD relapse. There was a low frequency of CVT and an unexpected higher number of isolated aseptic meningitis. Brazilian NBD patients present more parenchymal and atypical manifestations, and relapse more often than NBD patients from other populations.

Psychiatric manifestations of sporadic Creutzfeldt-Jakob disease simulate depressive disorder with psychotic symptoms

Creutzfeldt–Jakob disease (CJD) is a rare and fatal neurodegenerative disorder with a broad spectrum of early clinical manifestations, comprising neurological and psychiatric symptoms. The authors report the case of a patient admitted with a diagnosis of depressive disorder with psychotic symptoms, with post-mortem confirmation of CJD and discuss how CJD’s clinical heterogeneity can lead to misdiagnosis of the disease. Despite CJD’s unique pathogenesis, its kaleidoscopic presentation justifies the integrated investigation of patients with psychiatric symptoms, avoiding restrictive diagnosis.

Role Of Astrocytes In Thiamine Deficiency.

Thiamine deficiency (TD) is the underlying cause of Wernicke's encephalopathy (WE), an acute neurological disorder characterized by structural damage to key periventricular structures in the brain. Increasing evidence suggests these focal histological lesions may be representative of a gliopathy in which astrocyte-related changes are a major feature of the disorder. These changes include a loss of the glutamate transporters GLT-1 and GLAST concomitant with elevated interstitial glutamate levels, lowered brain pH associated with increased lactate production, decreased levels of GFAP, reduction in the levels of glutamine synthetase, swelling, alterations in levels of aquaporin-4, and disruption of the blood-brain barrier. This review focusses on how these manifestations contribute to the pathophysiology of TD and possibly WE.

Cerebral Cortex Involvement In Machado-joseph Disease.

Machado-Joseph disease (MJD/SCA3) is the most frequent spinocerebellar ataxia, characterized by brainstem, basal ganglia and cerebellar damage. Few magnetic resonance imaging based studies have investigated damage in the cerebral cortex. The objective was to determine whether patients with MJD/SCA3 have cerebral cortex atrophy, to identify regions more susceptible to damage and to look for the clinical and neuropsychological correlates of such lesions. Forty-nine patients with MJD/SCA3 (mean age 47.7 ± 13.0 years, 27 men) and 49 matched healthy controls were enrolled. All subjects underwent magnetic resonance imaging scans in a 3 T device, and three-dimensional T1 images were used for volumetric analyses. Measurement of cortical thickness and volume was performed using the FreeSurfer software. Groups were compared using ancova with age, gender and estimated intracranial volume as covariates, and a general linear model was used to assess correlations between atrophy and clinical variables. Mean CAG expansion, Scale for Assessment and Rating of Ataxia (SARA) score and age at onset were 72.1 ± 4.2, 14.7 ± 7.3 and 37.5 ± 12.5 years, respectively. The main findings were (i) bilateral paracentral cortex atrophy, as well as the caudal middle frontal gyrus, superior and transverse temporal gyri, and lateral occipital cortex in the left hemisphere and supramarginal gyrus in the right hemisphere; (ii) volumetric reduction of basal ganglia and hippocampi; (iii) a significant correlation between SARA and brainstem and precentral gyrus atrophy. Furthermore, some of the affected cortical regions showed significant correlations with neuropsychological data. Patients with MJD/SCA3 have widespread cortical and subcortical atrophy. These structural findings correlate with clinical manifestations of the disease, which support the concept that cognitive/motor impairment and cerebral damage are related in disease.

Neurological evaluation of neonates with intraventricular and periventricular hemorrhage

We studied the clinical aspects of 100 consecutive premature newborns with and without intraventricular and periventricular hemorrhage (IPVH).The diagnosis of IPVH was obtained by ultrasonic scans of the skull during the first week of life and at the age of one month. Forty eight percent of newborns with IPVH had abnormal results, and there was a significant correlation with the neurological evaluation in 85% of the infants. The probability of normality for a child with no associated brain abnormalities was 72%, whereas for a child of the same gestational age with associated brain abnormalities was 48.7%.

Neuronal Ceroid-lipofuscinosis with prominent chorea and without visual manifestations: a case report

A case of neuronal ceroid-lipofuscinosis (NCL) is reported in a 11-year-old girl, whose main symptoms were progressive dementia since the age of 4 years and choreic movements since age 10. Seizures, myoclonus and visual deterioration were absent and optic fundi were normal. A cerebral biopsy disclosed two basic types of stored substance in the cytoplasm of neurons: a) severely balloned nerve cells in cortical layers HI and V contained a non-autofluorescent material, which stained with PAS and Sudan Black B in frozen, but not in paraffin sections; ultrastructurally, these neurons showed abundant corpuscles similar to the membranous cytoplasmic bodies of Tay-Sachs disease and, in smaller amounts, also zebra bodies; b) slightly distended or non-distended neurons in all layers contained lipopigment granules, which were autofluorescent, PAS-positive and sudanophil in both frozen and paraffin sections; their ultrastructure was closely comparable to that of lipofuscin. Similar bodies were found in the swollen segments of axons and in a few astrocytes and endothelial cells. The histochemical and ultrastructural demonstration of large amounts of lipopigments allows a presumptive classification of the case as NCL. However, the presence of involuntary movements, the absence of visual disturbances and the unusual ultrastructural features place the patient into a small heterogeneous group within the NCL. A better classification of such unique instances of the disease must await elucidation of the basic enzymatic defects.

Características clínicas da fase aguda da infecção experimental de felinos pelo vírus da imunodeficiência felina

A infecção dos felinos pelo Vírus da Imunodeficiência Felina (FIV) resulta no desenvolvimento da síndrome de imunodeficiência dos felinos. Gengivite, perda de peso, linfadenomegalia generalizada, anemia, insuficiência renal crônica, complicações neurológicas, diarréia crônica e infecções bacterianas são encontradas frequentemente. A fase aguda da infecção pode ser assintomática, retardando o estabelecimento do diagnóstico e a implantação de medidas profiláticas para restringir o contágio e a transmissão do agente aos felinos suscetíveis. Com a finalidade de estudar as características clínicas da fase aguda da infecção, dez felinos jovens, sem definição racial, com oito meses de idade foram inoculados por via endovenosa com 1mL de sangue venoso de um gato portador do FIV subtipo B. A confirmação da infecção foi obtida através de teste sorológico em quatro e oito semanas pós-inoculação (p.i.) e por nested-PCR. Foram realizados hemogramas semanais, exame ultrassonográfico do abdômen quinzenais e exame oftalmológico mensal, durante doze semanas p.i. Discreta tendência a linfopenia na segunda semana p.i. e a neutropenia entre a quinta e sétima semana p.i., febre intermitente em alguns gatos, linfadenomegalia e hepato-esplenomegalia entre a quarta e a 12ª semana p.i. foram as alterações clínicas observadas. Apenas um gato apresentou uveíte unilateral direita. A fase aguda da infecção transcorreu com alterações clínicas inespecíficas. A linfadenomegalia e a hepato-esplenomegalia observadas no decorrer da infecção, refletindo hiperplasia linfóide, sugerem a necessidade de se realizar o teste sorológico para o FIV, em todos os gatos que se apresentarem com essas alterações, o que permitirá o diagnóstico precoce da infecção e a adoção de medidas profiláticas no sentido de minimizar a propagação da infecção.

HTLV-1 Tax Specific CD8+ T Cells Express Low Levels of Tim-3 in HTLV-1 Infection: Implications for Progression to Neurological Complications

The T cell immunoglobulin mucin 3 (Tim-3) receptor is highly expressed on HIV-1-specific T cells, rendering them partially ""exhausted'' and unable to contribute to the effective immune mediated control of viral replication. To elucidate novel mechanisms contributing to the HTLV-1 neurological complex and its classic neurological presentation called HAM/TSP (HTLV-1 associated myelopathy/tropical spastic paraparesis), we investigated the expression of the Tim-3 receptor on CD8(+) T cells from a cohort of HTLV-1 seropositive asymptomatic and symptomatic patients. Patients diagnosed with HAM/TSP down-regulated Tim-3 expression on both CD8(+) and CD4(+) T cells compared to asymptomatic patients and HTLV-1 seronegative controls. HTLV-1 Tax-specific, HLA-A*02 restricted CD8(+) T cells among HAM/TSP individuals expressed markedly lower levels of Tim-3. We observed Tax expressing cells in both Tim-3(+) and Tim-3(-) fractions. Taken together, these data indicate that there is a systematic downregulation of Tim-3 levels on T cells in HTLV-1 infection, sustaining a profoundly highly active population of potentially pathogenic T cells that may allow for the development of HTLV-1 complications.

Chagas Cardiomyopathy Manifestations and Trypanosoma cruzi Genotypes Circulating in Chronic Chagasic Patients

Chagas disease caused by Trypanosoma cruzi is a complex disease that is endemic and an important problem in public health in Latin America. The T. cruzi parasite is classified into six discrete taxonomic units (DTUs) based on the recently proposed nomenclature (TcI, TcII, TcIII, TcIV, TcV and TcVI). The discovery of genetic variability within TcI showed the presence of five genotypes (Ia, Ib, Ic, Id and Ie) related to the transmission cycle of Chagas disease. In Colombia, TcI is more prevalent but TcII has also been reported, as has mixed infection by both TcI and TcII in the same Chagasic patient. The objectives of this study were to determine the T. cruzi DTUs that are circulating in Colombian chronic Chagasic patients and to obtain more information about the molecular epidemiology of Chagas disease in Colombia. We also assessed the presence of electrocardiographic, radiologic and echocardiographic abnormalities with the purpose of correlating T. cruzi genetic variability and cardiac disease. Molecular characterization was performed in Colombian adult chronic Chagasic patients based on the intergenic region of the mini-exon gene, the 24S alpha and 18S regions of rDNA and the variable region of satellite DNA, whereby the presence of T. cruzi I, II, III and IV was detected. In our population, mixed infections also occurred, with TcI-TcII, TcI-TcIII and TcI-TcIV, as well as the existence of the TcI genotypes showing the presence of genotypes Ia and Id. Patients infected with TcI demonstrated a higher prevalence of cardiac alterations than those infected with TcII. These results corroborate the predominance of TcI in Colombia and show the first report of TcIII and TcIV in Colombian Chagasic patients. Findings also indicate that Chagas cardiomyopathy manifestations are more correlated with TcI than with TcII in Colombia.

Autoimmune Neurological Disease

This book provides a comprehensive and critical overview of the immunological aspects of autoimmune neurological disease. These diseases include common conditions such as multiple sclerosis, the Guillain–Barré syndrome and myasthenia gravis. The introductory chapters on antigen recognition and self–non-self discrimination, and on neuroimmunology, are followed by chapters on specific diseases. These are presented in a standardized format with sections on clinical features, genetics, neuropathology, pathophysiology, immunology and therapy. Each chapter has a concluding section which summarizes key points and suggests directions for future research. Animal models of autoimmune neurological disease are also covered in detail because of their importance in understanding the human diseases. The book is suitable for clinicians and neurologists managing patients with these diseases, and for immunologists, neuroscientists and neurologists investigating the pathogenesis and pathophysiology of these disorders.

Dysregulated hematopoiesis and a progressive neurological disorder induced by expression of an activated form of the human common beta chain in transgenic mice

Previously we described activating mutations of h beta(c), the common signaling subunit of the receptors for the hematopoietic and inflammatory cytokines, GM-CSF, IL-3, and IL-5. The activated mutant, h beta(c)FI Delta, is able to confer growth factor-independent proliferation on the murine myeloid cell line FDC-P1, and on primary committed myeloid progenitors. We have used this activating mutation to study the effects of chronic cytokine receptor stimulation. Transgenic mice were produced carrying the h beta(c)FI Delta cDNA linked to the constitutive promoter derived from the phosphoglycerate kinase gene, PGK-1. Transgene expression was demonstrated in several tissues and functional activity of the mutant receptor was confirmed in hematopoietic tissues by the presence of granulocyte macrophage and macrophage colony-forming cells (CFU-GM and CFU-M) in the absence of added cytokines. All transgenic mice display a myeloproliferative disorder characterized by splenomegaly, erythrocytosis, and granulocytic and megakaryocytic hyperplasia. This disorder resembles the human disease polycythemia vera, suggesting that activating mutations in h beta(c) may play a role in the pathogenesis of this myeloproliferative disorder. In addition, these transgenic mice develop a sporadic, progressive neurological disease and display bilateral, symmetrical foci of necrosis in the white matter of brain stem associated with an accumulation of macrophages. Thus, chronic h beta(c) activation has the potential to contribute to pathological events in the central nervous system.

Cisplatin-based therapy: A neurological and neuropsychological review

The present paper reviews research in the area of the broad-spectrum chemotherapeutic agent cisplatin (cis-diamminedichloro-platinum II) and examines the implications for clinical neuropsychology arising from the neurological disruption associated with cisplatin-based therapy. The paper begins with a brief review of cisplatin treatment in terms other than survival alone, and examines the side-effects and the potential central nervous system (CNS) dysfunction in terms of neurological symptoms and concomitant implications for neuropsychology. Two main implications for clinical neuropsychology arising from cisplatin therapy are identified. First, cisplatin therapy impacts upon the psychological well-being of the patient, particularly during and in the months following treatment. It is suggested that during this time, a primary role for neuropsychology is to focus upon the monitoring and the active enhancement of the patient's social, psychological and spiritual resources. Second, with regard to neurocognitive changes, the review suggests that (1) neurocognitive assessment may not yield stable results within 8 months following treatment and (2) while perceptual, memory, attentional and executive dysfunction may be predicted following cisplatin treatment, little systematic research has been carried out to investigate such a possibility. Future research might profitably address this issue and also specifically examine the effects of low dosage cisplatin-based therapy and the effects of recently developed neuroprotective agents. Finally, there is some evidence to suggest that women may be more susceptible to neurotoxicity during cisplatin therapy, but no gender-related cognitive effects are reported in the cisplatin literature. Future research could usefully investigate gender differences in association with cisplatin chemotherapy. Copyright (C) 2000 John Wiley & Sons, Ltd.

Unusual manifestations of tegumentary leishmaniasis in AIDS patients from the New World

Comorbidity from tegumentary leishmaniasis and AIDS is poorly characterized. To describe a series of patients coinfected with Leishmania and human immunodeficiency virus (HIV). Clinical records from patients were analysed by demographic data, clinical manifestations, diagnoses, treatments and outcomes. Fifteen cases of AIDS/tegumentary leishmaniasis were found. The diagnosis of leishmaniasis was confirmed by the detection of Leishmania amastigotes or antigens from the cutaneous or mucosal lesions. The mean CD4+ T-cell count was 84 cells mm(-3) (range 8-258) and all patients were classified as having AIDS according to the Centers for Disease Control and Prevention. A wide range of manifestations was found, varying from a single ulcer to multiple and polymorphic lesions. Mucosal lesions were present in 80% and cutaneous lesions in 73% of patients (53% with mucocutaneous form), disseminated lesions in 60% and genital lesions in 27% of patients. All patients received anti-Leishmania therapy and 53% showed relapses. Sixty-seven per cent received highly active antiretroviral therapy but showed no difference in outcomes and relapses compared with those not using medication. Forty per cent died during the study period. In these patients, the anti-Leishmania antibody and Montenegro skin test were useful in the diagnosis of leishmaniasis, probably because leishmaniasis preceded immunosuppression due to HIV infection. Clinical manifestations of tegumentary leishmaniasis in HIV-infected patients are diverse. Our data emphasize possible unusual manifestations of this disease in HIV-infected patients, particularly in severely immunosuppressed cases (< 200 CD4+ cells mm(-3)).

Neurological Patients Need Evidence-Based Urological Care

Aims: To report the conclusion of the Think Thank on Neurourology discussions during the first ICI-RS meeting in 2009. Methods: During a 3-day meeting a group of specialists discussed evidence-based medicine in neurourology and made suggestions for future research. Results: In the vast majority of patients with neurological disease bladder dysfunction occurs. The actual rules of diagnosis and treatment lack a study related evidence base. From a long list of possible research subjects, prevalence, detrusor pressure, imaging, catheterization and surgery have been first discussed. Conclusion: In each of these subjects, research items are suggested which can help to improve the care in this patient group. Neurourol. Urodynam. 29:662-669, 2010. (C) 2010 Wiley-Liss, Inc.