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This report aims to describe the identification of porcine astrovirus 3 (PAstV3) RNA in the central nervous system (CNS) of weaned pigs with clinical signs of neurological disease associated with polioencephalomyelitis in southeastern Brazil. Three, 20 -35 days-old piglets that died after clinical manifestations of a neurological syndrome were submitted to post-mortem evaluations. Tissue samples were examined by histopathology, bacteriology, and molecular assays (RT-PCR, nested-PCR, RT-qPCR, and Sanger sequencing) to detect the primary infectious disease agents associated with neurological disease in pigs. The principal neuropathological alterations occurred in the grey matter of the spinal cord and brainstem resulting in nonsuppurative poliomyelitis and rhombencephalitis. PAstV3 RNA was detected in the CNS samples of all piglets with histopathological evidence of disease and was confirmed by nucleotide sequencing. Nucleic acids from pathogens commonly associated with neurological diseases in pigs, such as porcine teschovirus, porcine sapelovirus, porcine enterovirus G, atypical porcine pestivirus, senecavirus A, and encephalomyocarditis virus was not detected by molecular assays in the three piglets. This is the first report of PAstV3 in piglets with neurological disease and lesions consistent with polioencephalomyelitis in Brazil. This report highlights the importance of monitoring health events that could compromise pig farming productivity and animal welfare., Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024 Elsevier Ltd. All rights reserved.)

Meningioangiomatosis (MA) is a rare proliferative meningovascular disorder that affects mainly the cerebral cortex, brainstem and spinal cord of humans and animals and can coexist with other proliferative disorders. A 7.5-year-old male Brazilian Campeiro Bulldog died after a convulsive crisis and cardiorespiratory arrest. At necropsy, a firm, white mass involving the piriform and right occipital lobes was seen. Histologically, the mass consisted of two morphologically distinct entities that collided: a congenital malformation characterized by a proliferation of meningothelial cells around blood vessels, within the perivascular spaces; and a neoplasm composed of two cell populations with astrocytic and oligodendrocytic differentiation. Meningothelial cells and neoplastic glial cells immunolabelled for vimentin. This first reported case of encephalic MA with a high-grade undefined glioma in a dog was confirmed through clinical signs, pathological and immunohistochemical findings., (Copyright © 2023 Elsevier Ltd. All rights reserved.)

Background: Neurodegenerative diseases are sporadic hereditary conditions characterized by progressive dysfunction of the nervous system. Among the symptoms, vestibulopathy is one of the causes of discomfort and a decrease in quality of life. Hereditary spastic paraplegia is a heterogeneous group of hereditary degenerative diseases involving the disorder of a single gene and is characterized by the progressive retrograde degeneration of fibers in the spinal cord., Objective: To determine the benefits of vestibular rehabilitation involving virtual reality by comparing pre intervention and post intervention assessments in individuals with hereditary spastic paraplegia., Methods: In this randomized controlled clinical trial from the Rebec platform RBR-3jmx67 in which allocation concealment was performed and the evaluators be blinded will be included. The participants will include 40 patients diagnosed with hereditary spastic paraplegia. The interventions will include vestibular rehabilitation with virtual reality using the Wii® console, Wii-Remote and Wii Balance Board (Nintendo), and the studies will include pre- and post intervention assessments. Group I will include twenty volunteers who performed balance games. Group II will include twenty volunteers who performed balance games and muscle strength games. The games lasted from 30 minutes to an hour, and the sessions were performed twice a week for 10 weeks (total: 20 sessions)., Results: This study provides a definitive assessment of the effectiveness of a virtual reality vestibular rehabilitation program in halting the progression of hereditary spastic paraplegia, and this treatment can be personalized and affordable., Conclusion: The study will determine whether a vestibular rehabilitation program with the Nintendo Wii® involving virtual reality can reduce the progressive effect of hereditary spastic paraplegia and serve as an alternative treatment option that is accessible and inexpensive. Rebec platform trial: RBR-3JMX67., Competing Interests: The authors have declared that no competing interests exist.

Canine Degenerative Myelopathy is a late onset recessive autosomal disease characterized by a progressive ascending degeneration of the spinal cord. Two causal mutations are associated with this disease: a transition (c.118G>A) in exon 2 of the SOD1 that was described in several breeds and a transversion (c.52A>T) in exon 1 of the same gene described in Bernese Mountain dogs. The aim of this study was to understand the impact of the SOD1:c.118G > A mutation by genotyping a population of German Shepherd dogs in Brazil. A PCR-RFLP approach was used to genotype 97 healthy individuals belonging from the Northeast (Bahia and Pernambuco states) and South (Santa Catarina state) regions of Brazil. A total of 95 individuals were successfully genotyped resulting in an observed genotype frequency (with 95% confidence interval) of: 0.758 (0.672-0.844), 0.242 (0.156-0.328) and 0.000 (0.000-0.000) for "GG", "AG" and "AA" genotypes, respectively. To our knowledge, this is the first attempt to describe the presence of the "A" allele associated with CDM (SOD1:c.118G > A) in German Shepherd dogs in Brazil and, as such, these results contribute toward important epidemiological data in this country and to the knowledge of the distribution of the aforementioned mutation worldwide., Competing Interests: The authors have declared that no competing interests exist.

Objective: We aimed to evaluate magnetic resonance imaging (MRI) previously used criteria (Matthews's criteria, MC) for differentiating multiple sclerosis (MS) from neuromyelitis optica spectrum disorders (NMOSD) in Caucasian and non-Caucasian populations (Argentina, Brazil and Venezuela) with positive (P-NMOSD), negative (N-NMOSD), and unknown (U-NMOSD) aquaporin-4 antibody serostatus at disease onset and to assess the added diagnostic value of spinal cord MRI in these populations., Methods: We reviewed medical records, and MRIs were assessed by two blinded evaluators and were scored using MC. Short-segment transverse myelitis (STM) was added as a new criterion. MC sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV) were determined., Results: We included 282 patients (MS = 188 and NMOSD = 94). MC applied to the entire cohort showed 97.8% sensitivity, 82.9% specificity, 92.0% PPV, and 95.1% NPV for differentiating MS from NMOSD. A subanalysis applied only to non-Caucasian (MS = 89 and NMOSD = 47) showed 100% sensitivity, 80.8% specificity, 90.8% PPV, and 100% NPV. Similar sensitivity, specificity, PPV, and NPV of MC for MS versus P-NMOSD ( n  = 55), N-NMOSD ( n  = 28), and U-NMOSD ( n  = 21) were observed., Conclusion: MC distinguished MS from NMOSD of all serostatus in a Latin American cohort that included non-Caucasian populations. Addition of STM to MC did not raise the accuracy significantly.

Background: HTLV-1 infection is endemic in Brazil. About 1 to 2% of the Brazilian population is estimated to be infected, but most infected HTLV-1 individuals do not know about their own infection, which favors the continuity of sexual and vertical virus transmission. In addition, HTLV-1 associated central nervous system diseases and their pathophysiologic mechanisms are not fully understood. This study aimed to evaluate the correlation of spinal cord metabolism, viral and inflammatory profiles with features of neurological presentation in HTLV-1 infected individuals., Methodology: This is a cross-sectional study of a cohort including 48 HTLV-1 infected individuals clinically classified as asymptomatic-AG (N = 21), symptomatic-SG (N = 11) and HAM/TSP-HG (N = 16) and a nested case-control study with HTLV-1 infected individuals-HIG (N = 48) and HTLV-1 non infected controls-CG (N = 30) that had their spinal cord analysed by Positron Emission Tomography with 18F-Fluordeoxyglucose (18F-FDG PET/CT). HTLV-1 infected individuals had 18F-FDG PET/CT results analyzed with clinical and demographic data, proviral load, cytokines and chemokines in the blood and cerebrospinal fluid (CSF)., Principal Findings: 18F-FDG PET/CT showed hypometabolism in the thoracic spinal cord in HTLV-1 infected individuals. The method had an accuracy of 94.4% to identify HAM/TSP. A greater involvement of the thoracic spinal cord was observed, although hypometabolism was also observed in the cervical spinal cord segment in HTLV-1 infected individuals. Individuals with HAM/TSP showed a pro-inflammatory profile in comparison to asymptomatic and symptomatic groups, with a higher level of Interferon-inducible T-cell alpha chemoattractant (ITAC/CXCL11), IL-6, IL-12p70 in the plasma; and ITAC, IL-4, IL-5, IL-8 (CXCL8) and TNF-alpha in the CSF. Using regression, thoracic spinal cord SUV (standardized uptake value) and CSF ITAC level were identified as the HAM/TSP predictors in the multivariate model., Conclusions: 18F-FDG PET/CT imaging showed spinal cord hypometabolism in most HTLV-1 infected individuals, even in the asymptomatic HTLV-1 group. Thoracic spinal cord hypometabolism and CSF-ITAC levels were identified predictors of HAM/TSP., Significance: Our findings suggested that in most HTLV-1 infected individuals there was compromise of central nervous system (CNS) structures despite of the lack of clinical symptoms. To explain the found hypometabolism, the role of microcirculatory and metabolic factors in the pathogenesis of neurological diseases associated with HTLV-1 infection must be further investigated. It is paramount to evaluate the central nervous function and to compare the performance among HTLV-1 infected individuals considered asymptomatic to the uninfected controls., Competing Interests: The authors have declared that no competing interests exist.

Introduction: Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids (CLIPPERS Syndrome) was first described in 2010. Since then, about 50 cases have been reported around the world, but none in Portuguese-speaking countries. We report a case of patient of the Neurological Rehabilitation ward of Sarah Hospital/Brasilia, with clinical and radiologic features compatible with CLIPPERS Syndrome. The diagnosis was made only after fourteen years of symptoms onset., Case Report: 49-year-old male, presenting with progressive pancerebellar syndrome followed by spastic paraplegia with neurogenic bladder and progressive worsening over 14 years. The radiological response to steroid was suggestive of CLIPPERS Syndrome, however it was not accompanied by significant clinical improvement., Conclusion: This is the first described case in Brazil,and this pattern of progression suggests that CLIPPERS is a degenerative disease. Its relevance must be acknowledged for being an important differential diagnosis of multiple sclerosis and other demyelinating diseases. Early diagnosis may be critical to halt the progression and affect outcome of the disease., (Copyright © 2015 Elsevier B.V. All rights reserved.)

This report describes the first detection of an equine herpesvirus 1 (EHV-1) neuropathogenic variant (G2254/D752) in Brazil from a case of fatal equine herpesvirus myeloencephalopathy (EHM) in a mare. The results of nucleotide sequencing of the EHV-1 ORF30 gene showed that two other Brazilian EHV-1 isolates from EHM cases are representatives of the non-neuropathogenic variant (A2254/N752), suggesting that other unidentified factors are probably also involved in the neuropathogenicity of EHV-1 in horses. These findings will contribute to the epidemiological knowledge of EHV-1 infection in Brazil.

The aim of this study was to describe the demographic, clinical and laboratory features of idiopathic acute transverse myelitis (IATM). Patients with non-compressive ATM receiving care at Hospital da Lagoa, Rio de Janeiro (Brazil) between 2000 and 2008 were selected. Of the 70 cases of acute myelopathies, the idiopathic form was identified in 41 following exclusion of the cases associated with systemic lupus erythematosus (n = 1), Sjogren's syndrome (n = 1), herpes zoster (n = 1), cytomegalovirus in an HIV-positive patient (n = 1), Schistosoma mansoni (n = 1), actinic myelitis (n = 1), infectious myelitis of unknown etiology (n = 2) and those that, following the first attack of myelitis, converted to NMO (n = 19) or to clinically defined MS (n = 2). Of the 41 cases of IATM, the majority of patients were female (68.3%) and white (65.9%). Median age at first myelitis was 37.0 +/- 11.8 years. Over a median observation time of 36 months, 39.0% of patients remained monophasic, while recurrences occurred in 61.0% of cases. The number of ATM/patient ranged from one to seven. Among the recurrent cases the median time between the first and the second ATM was 12 months (range 1-150 months).The first myelitis was characterized mainly by partial myelitis with motor and sensorial dysfunction (63.4%). Complete and severe myelitis occurred more frequently among monophasic patients and partial myelitis with moderate dysfunction at onset in recurrent cases; however, over the long-term, dysfunction and disability were mild in both groups. Serial spine MRI confirmed spinal cord inflammation in 92.0% of cases and extensive spinal cord lesion was identified in 61.0%. Brain MRI was normal or not suggestive of MS in 94.4% of cases. CSF showed pleocytosis in 41.2%, increased IgG index in 24.0% and oligoclonal bands in 38.0% of 34 patients tested. Abnormal visual evoked potentials occurred in 11.5% of 26 patients. Positivity for anti-AQP4 was found in 23.5% of the 17 cases tested, suggesting limited forms of NMO. This study suggests some new aspects of the clinical course of IATM such as the high conversion rate to NMO, the predominance of women and a higher frequency of recurrent forms.

The authors describe the histopathological necropsy findings of 45 human immunodeficiency virus (HIV)-infected patients with neurocryptococcosis. Systemic cryptococcosis with involvement of multiple organs such as spleen, liver, and lungs was present in all patients. Predominant diffuse meningoencephalitis predominantly in the basal ganglia, thalamus, and mid-brain, with minimal inflammatory infiltrate was seen in 30 AIDS patients (70%). We frequently observed in those patients the presence of multiple gelatinous pseudocysts with abundant Cryptococcus neoformans in the Virchow-Robin spaces and adjacent brain caused by the dissemination of the meningeal infection along the perivascular spaces. Isolated meningeal and cerebral involvement with minimal inflammatory infiltrate with numerous fungal organisms, and occasionally with granulomatous reaction and necrosis, was seen in 15 patients (30%). In addition to involvement of multiple organs by C. neoformans, HIV-infected patients with clinical manifestations of neurocryptococcosis frequently present a widespread involvement of the brain.

Object: The purpose of this study is to determine the epidemiology of tumors of the nervous system diagnosed according to the WHO 2000 classification in a single Brazilian institution., Patients and Method: One thousand one hundred ninety-five tumors in children between 0 and 21 years of age diagnosed between 1974 and 2003 were classified according the sex, topography, and age distribution., Results: In all ages, males were slightly more affected. In the first 2 years, the prevalence for boys was higher (68.3%). In the whole series, 58.7% were supratentorial, 31.4% infratentorial, and 9.9% spinal (44% intra- and 56% extramedullary). Among these latter, ependymomas and schwannomas were the most frequent. In the cerebral compartment, pilocytic astrocytomas were the single most frequent tumors (18%), followed by diffuse astrocytomas (14%), medulloblastomas (11%), and craniopharyngiomas (11%). In the posterior fossa, there was an even distribution among medulloblastomas and pilocytic astrocytomas, but the former was much more frequent in the first 2 years of age. High-grade (III and IV) diffuse astrocytomas were slightly more frequent than low grades (II), and this difference becomes more evident as the child grows older. Due to the new development of the surgery of epilepsy, the frequency of neuronal and mixed neuronal-glial tumors is increasing (8%)., Conclusion: Classified according to the latest WHO classification, by a single neuropathologist in a single institution, this large series of pediatric neurological tumors may reflect fairly well their real incidence. Our results obtained in a developing country do not differ substantially from other similar series reported in the literature from the First World.

Introduction: Schistosomiasis is a parasitic infection caused by Schistosoma sp that affects over 200 million people worldwide. The neurological manifestations, caused by the eggs or by anomalous migration of the parasite, can be epileptic seizures and myeloradicular syndromes. S. mansoni is endemic in Brazil and is an underdiagnosed cause of transverse myelitis and myeloradiculopathy., Case Reports: We present the cases of two Brazilian women, aged 28 and 32, who suffered from conus medullaris and cauda equina syndrome, and a thoracolumbar myelopathy, respectively. Diagnosis was based on the epidemiological exposure, suggestive neurological syndrome and positive serological studies in blood or cerebrospinal fluid (CSF) by means of indirect immunofluorescence (IFI) for S. mansoni. Other causes of myeloradiculopathy were dismissed. The first patient presented progressive sphincteric, motor and dysesthetic symptoms that suggested cauda equina and conus medullaris syndrome. Examination of the CSF revealed pleocytosis, eosinophils and high protein levels; (IgG) IFI was positive for S. mansoni. Spinal cord magnetic resonance imaging showed an increased volume in the conus and epiconus, areas of hypersignal in T2 and heterogeneous contrast enhancement. Case 2 presented an acute picture of paresthesias and myoclonias in the lower limbs. Serological analysis for S. mansoni in blood was positive. S. mansoni eggs were found in faeces. Results of the CSF study were normal. Both patients were treated with praziquantel and prednisone and responded well both clinically and radiologically., Conclusions: In patients with epidemiological evidence of exposure to S. mansoni, lumbosacral myeloradiculopathy is suggestive of neuroschistosomiasis.

Objectives: To report the clinical features and outcome of 24 Brazilian patients with optic neuromyelitis syndrome (ONM); discuss the underlying pathological events associated with the ONM syndrome; review the nosological situation of ONM in the group of inflammatory and demyelinating diseases of the central nervous system., Patients and Methods: Patients with ONM treated at the Hospital da Lagoa, Rio de Janeiro were studied. Demographic, clinical, magnetic resonance imaging, cerebrospinal fluid, and pathological data were analysed., Results: The study consisted of 20 women, four men of whom 10 were white and 14 Afro-Brazilians. Clinical course was recurrent in 22 cases and monophasic in two. Neurological manifestations at inclusion were: sensory impairment (66%), bilateral (41.6%) or unilateral blindness (20.8%), paraplegia or quadriplegia (37.5%). The EDSS was moderate/severe in 70.8%. The underlying pathological events were respectively pulmonary tuberculosis and upper respiratory infection in the two monophasic cases; in the 22 recurrent ONM patients: pulmonary tuberculosis (3), neurocysticercosis (1), polyarteritis nodosa (1), antinuclear antibody and rheumatoid factor (1), antiphospholipid antibody primary syndrome (1), diabetes mellitus (1), hypothyroidism (1), and amenorrhea-galactorrhea (4). Normal cerebrospinal fluid was found in 52% and an inflammatory profile in 48%. Only four recurrent ONM white patients had brain and spinal cord magnetic resonance imaging and cerebrospinal fluid findings compatible with the diagnosis of multiple sclerosis. Large lesions were seen in 62% of spinal magnetic resonance images. Six of 12 recurrent ONM Afro-Brazilian died. There were no statistical differences in the demographic data of the two ethnic groups. Afro-Brazilians were significantly more severely impaired and had a higher mortality rate than the white patients., Conclusion: These cases were classified as follows: two monophasic acute disseminated encephalomyelitis; one recurrent disseminated encephalomyelitis; three recurrent ONM associated with Hughes syndrome, autoantibodies and polyarteritis nodosa; six recurrent ONM with endocrinopathies; and finally, four multiple sclerosis cases. The remaining cases were not associated with any other condition. It would seem clear that ONM is a syndrome rather than a single disease.

We report epidemiologic and clinical features of human T cell lymphotropic virus type 1 (HTLV-1)-associated myelopathy/tropical spastic paraparesis in a Brazilian cohort of 86 patients from a university hospital. Cerebrospinal fluid (CSF) abnormalities and magnetic resonance imaging (MRI) findings were correlated with neurologic signs and symptoms. The patients' mean age at disease onset was 43.2 years and the female to male ratio 1.5:1. Risk factors for retroviral exposure included blood transfusions, sexual transmission and intravenous drug use. Neurologic manifestations consisted mostly of spastic paraparesis, of gradual and asymmetric onset, with increased deep-tendon reflexes. Disability was associated with disease duration. CSF findings included mild mononuclear pleocytosis and elevated protein levels. MRI showed hemispheric areas of high signal intensity in 32.7% and spinal cord atrophy in 5.6% of cases, particularly in patients with longer duration of disease. A high frequency of past blood transfusion (32.6%) suggests that blood-borne infection was an important means of HTLV infection before implementation of screening of blood donors in Brazil., (Copyright 2002 S. Karger AG, Basel)

Sarcocystis neurona-like schizonts were found in sections of brain and spinal cord of two ataxic horses from Brazil. The diagnosis was supported by staining with anti-Sarcocystis serum in an immunohistochemical test. One of the affected horses was born in Argentina and raised in Brazil, and the other horse was born and raised in Brazil.