REEP1 Preserves Motor Function in SOD1G93A Mice by Improving Mitochondrial Function via Interaction with NDUFA4 / 神经科学通报·英文版

A decline in the activities of oxidative phosphorylation (OXPHOS) complexes has been consistently reported in amyotrophic lateral sclerosis (ALS) patients and animal models of ALS, although the underlying molecular mechanisms are still elusive. Here, we report that receptor expression enhancing protein 1 (REEP1) acts as an important regulator of complex IV assembly, which is pivotal to preserving motor neurons in SOD1G93A mice. We found the expression of REEP1 was greatly reduced in transgenic SOD1G93A mice with ALS. Moreover, forced expression of REEP1 in the spinal cord extended the lifespan, decelerated symptom progression, and improved the motor performance of SOD1G93A mice. The neuromuscular synaptic loss, gliosis, and even motor neuron loss in SOD1G93A mice were alleviated by increased REEP1 through augmentation of mitochondrial function. Mechanistically, REEP1 associates with NDUFA4, and plays an important role in preserving the integrity of mitochondrial complex IV. Our findings offer insights into the pathogenic mechanism of REEP1 deficiency in neurodegenerative diseases and suggest a new therapeutic target for ALS.

Clinical characteristics and treatment of spinal cord injury in children and adolescents / 中华创伤杂志(英文版)

Pediatric and adult spinal cord injuries (SCI) are distinct entities. Children and adolescents with SCI must suffer from lifelong disabilities, which is a heavy burden on patients, their families and the society. There are differences in Chinese and foreign literature reports on the incidence, injury mechanism and prognosis of SCI in children and adolescents. In addition to traumatic injuries such as car accidents and falls, the proportion of sports injuries is increasing. The most common sports injury is the backbend during dance practice. Compared with adults, children and adolescents are considered to have a greater potential for neurological improvement. The pathogenesis and treatment of pediatric SCI remains unclear. The mainstream view is that the mechanism of nerve damage in pediatric SCI include flexion, hyperextension, longitudinal distraction and ischemia. We also discuss the advantages and disadvantages of drugs such as methylprednisolone in the treatment of pediatric SCI and the indications and timing of surgery. In addition, the complications of pediatric SCI are also worthy of attention. New imaging techniques such as diffusion tensor imaging and diffusion tensor tractography may be used for diagnosis and assessment of prognosis. This article reviews the epidemiology, pathogenesis, imaging, clinical characteristics, treatment and complications of SCI in children and adolescents. Although current treatment cannot completely restore neurological function, patient quality of life can be enhanced. Continued developments and advances in the research of SCI may eventually provide a cure for children and adolescents with this kind of injury.

Glia Connect Inflammation and Neurodegeneration in Multiple Sclerosis / 神经科学通报·英文版

Multiple sclerosis (MS) is regarded as a chronic inflammatory disease that leads to demyelination and eventually to neurodegeneration. Activation of innate immune cells and other inflammatory cells in the brain and spinal cord of people with MS has been well described. However, with the innovation of technology in glial cell research, we have a deep understanding of the mechanisms of glial cells connecting inflammation and neurodegeneration in MS. In this review, we focus on the role of glial cells, including microglia, astrocytes, and oligodendrocytes, in the pathogenesis of MS. We mainly focus on the connection between glial cells and immune cells in the process of axonal damage and demyelinating neuron loss.

Spatiotemporal Dynamics of the Molecular Expression Pattern and Intercellular Interactions in the Glial Scar Response to Spinal Cord Injury / 神经科学通报·英文版

Nerve regeneration in adult mammalian spinal cord is poor because of the lack of intrinsic regeneration of neurons and extrinsic factors - the glial scar is triggered by injury and inhibits or promotes regeneration. Recent technological advances in spatial transcriptomics (ST) provide a unique opportunity to decipher most genes systematically throughout scar formation, which remains poorly understood. Here, we first constructed the tissue-wide gene expression patterns of mouse spinal cords over the course of scar formation using ST after spinal cord injury from 32 samples. Locally, we profiled gene expression gradients from the leading edge to the core of the scar areas to further understand the scar microenvironment, such as neurotransmitter disorders, activation of the pro-inflammatory response, neurotoxic saturated lipids, angiogenesis, obstructed axon extension, and extracellular structure re-organization. In addition, we described 21 cell transcriptional states during scar formation and delineated the origins, functional diversity, and possible trajectories of subpopulations of fibroblasts, glia, and immune cells. Specifically, we found some regulators in special cell types, such as Thbs1 and Col1a2 in macrophages, CD36 and Postn in fibroblasts, Plxnb2 and Nxpe3 in microglia, Clu in astrocytes, and CD74 in oligodendrocytes. Furthermore, salvianolic acid B, a blood-brain barrier permeation and CD36 inhibitor, was administered after surgery and found to remedy fibrosis. Subsequently, we described the extent of the scar boundary and profiled the bidirectional ligand-receptor interactions at the neighboring cluster boundary, contributing to maintain scar architecture during gliosis and fibrosis, and found that GPR37L1_PSAP, and GPR37_PSAP were the most significant gene-pairs among microglia, fibroblasts, and astrocytes. Last, we quantified the fraction of scar-resident cells and proposed four possible phases of scar formation: macrophage infiltration, proliferation and differentiation of scar-resident cells, scar emergence, and scar stationary. Together, these profiles delineated the spatial heterogeneity of the scar, confirmed the previous concepts about scar architecture, provided some new clues for scar formation, and served as a valuable resource for the treatment of central nervous system injury.

Hirayama Disease: Case Report / Doença de Hirayama: Relato de caso

Abstract A 26-year-old previously healthy patient who, at the age of 18 years, began progressive loss of distal strength, rest tremor, and muscle atrophy in the left upper limb. Upon examination, the patient presented moderate distal atrophy, degree 4 in muscular strength, and minipolymioclonus. Electromyoneurography revealed (EMNG) chronic preganglionic bilateral involvement of bilateral C7/C8/T1, worse on the left, with signs of active C8/T1 denervation. A cervical spine magnetic resonance imaging (MRI) scan showed spondylodiscal degenerative changes with central protrusions in C4-C5, C6-C7, and right central in C5-C6, which touched the dural sac. The anteroposterior diameter of the medulla in neutral position, in the C5-C6 plane, was of 5.1 mm. There was a reduction of the spinal cord caliber to 4.0 mm after the dynamic maneuver of forced flexion of the spine, as well as signal increase in the anterior horns. The clinical findings and those of the complementary tests were compatible with Hirayama disease (HD), a rare benign motor neuron disease that affects cervical spinal segments and is most prevalent in men, with onset in the early 20s. Unilateral and slowly progressive weakness is typical, but self-limited. Sensory disturbances, and autonomic and upper motor neuron signals are rare. Management is usually conservative, with the use of a soft cervical collar. Although rare, HD should be considered in young patients with focal asymmetric atrophy in the upper limbs. The early diagnosis of HD depends on the degree of suspicion, as well as on the cooperation and communication among the various specialties involved in the investigation.

Resumo Paciente de 26 anos, previamente hígido, que, aos 18 anos, iniciou perda progressiva de força distal, tremor de repouso, e atrofia muscular no membro superior esquerdo. Ao exame, apresentou atrofia moderada, distal, força muscular de grau 4, e minipolimioclonus. A eletroneuromiografia (ENMG) revelou comprometimento pré-ganglionar crônico de C7/C8/T1 bilateral pior à esquerda, com sinais de desnervação ativa em C8/T1. A ressonância magnética (RM) de coluna cervical mostrou alterações degenerativas espondilodiscais com protrusões centrais em C4-C5, C6-C7, e central direita em C5-C6, que tocavam o saco dural. O diâmetro anteroposterior da medula na posição neutra, no plano de C5-C6, era de 5,1 mm. Houve redução do calibre da medula para 4,0 mm após a manobra dinâmica de flexão forçada da coluna, e aumento de sinal nos cornos anteriores. Os achados clínicos e os dos exames complementares eram compatíveis com doença de Hirayama (DH), uma doença benigna rara dos neurônios motores, que afeta os segmentos espinhais cervicais e é mais prevalente em homens e de início próximo aos 20 anos. É típica a fraqueza unilateral e lentamente progressiva, porém autolimitada. Perturbações sensoriais, sinais autonômicos e do neurônio motor superior são raras. O manejo geralmente é conservador, com uso de colar cervical macio. Apesar de rara, a DH deve ser considerada em pacientes jovens que apresentam atrofias assimétricas focais de membros superiores. O diagnóstico precoce de DH depende do grau de suspeição, e da cooperação e comunicação entre as diversas especialidades envolvidas na investigação.

Idiopathic Hypertrophic Spinal Pachymeningitis: A Case Report / Paquimeningite hipertrófica idiopática espinhal: Relato de caso

Abstract Idiopathic hypertrophic pachymeningitis is rare cause of neurological symptoms with myelopathy due to spinal cord compression. We report a case of pachymeningitis, which was manifested primarily by tetraparesis after low-energy trauma and recurrence the myelopathy symptoms after 5 years of surgery. The patient, a 19-year-old woman, was subjected to extensive investigation without evidence of any underlying disease. A meningeal biopsy was performed and showed an unspecific inflammatory process with extensive fibrosis of the dura mater. These findings, associated with the exclusion of other causes, suggest idiopathic hypertrophic pachymeningitis.

Resumo A paquimeningite hipertrófica idiopática é uma causa rara de sintomas neurológicos apresentando mielopatia por compressão da medula espinhal. Relatamos um caso de paquimeningite com manifestação primária de tetraparesia após trauma de baixa energia e recorrência dos sintomas de mielopatia 5 anos após a cirurgia. A paciente, uma mulher de 19 anos, foi submetida a extensa investigação sem evidências de qualquer doença de base. Uma biópsia da meninge revelou processo inflamatório inespecífico com extensa fibrose da dura máter, também visualizado no período perioperatório. Esses achados, associados à exclusão de outras causas, sugerem o diagnóstico de paquimeningite hipertrófica idiopática.

Solitary Dorsal Intramedullary Schwanomma ­ A Rare Lesion

Intramedullary schwanommas are rare, and most cases are reported in cervical region. Less than 20 dorsal intramedullary schwanommas have been reported till date in literature. This is due to their cell of origin, the Schwann cell, which is not normally found within the parenchyma of the brain and spinal cord; therefore it is not surprising that these lesions are rare. We report a rare solitary dorsal intramedullary schwanomma in a young adult patient who presented with paraplegia.

Cornuside ameliorated experimental autoimmune encephalomyelitis by limiting the recruitment of CD4+ T lymphocytes in the spinal cord

We conducted this study to determine whether cornuside could improve the neurological deficit symptoms of experimental autoimmune encephalomyelitis (EAE) rats, as well as determine the potential involvement of CD4+ T lymphocytes, vascular cell adhesion molecule-1 (VCAM-1), intercellular adhesion molecule-1 (ICAM-1), and tumor necrosis factor-α (TNF-α). Altogether, 32 Lewis rats were randomly divided into control, EAE, EAE/prednisolone, and EAE/cornuside, wherein their neurological function was assessed every day. CD4+ T lymphocyte recruitment into the spinal cord (SC) was evaluated using immunohistochemistry. The VCAM-1, ICAM-1 and TNF-α mRNA expressions in the SC were determined by real-time quantitative PCR, and the VCAM-1 and ICAM-1 proteins were determined by western blotting. Compared to the control group, the EAE group rats with neurological deficits had enhanced CD4+ T lymphocyte infiltration and higher expression levels of VCAM-1, ICAM-1, and TNF-α in the SC. Meanwhile, compared with the EAE group, the EAE/cornuside and EAE/prednisolone groups had lower neurological scores, less CD4+ T lymphocyte infiltrations, and lower expression levels of VCAM-1, ICAM-1, and TNF-α in the SC. Thus, cornuside ameliorated EAE, which could be owed to the inhibition of CD4+ T lymphocyte recruitment and VCAM-1, ICAM-1, and TNF-α expressions in the SC

Altered oligodendrocytes in spinal enlargements of streptozotocin diabetic rats / Oligodendrocitos alterados en la intumescencia espinal de ratas diabéticas con estreptozotocina

SUMMARY: Disturbances of sensory and motor nerve conduction velocity in the spinal cord as well as degenerated myelin sheaths are observed in diabetic patients and animal models. Indeed, oligodendrocytes (OLs), which are important neuroglial cells, generate myelin in the central nervous system. Spinal enlargement, including cervical and lumbar enlargements, innervates all limbs. Thus, the purposes of this study were to examine and compare the ultrastructural alterations of OLs in spinal enlargements of streptozotocin (STZ)- induced diabetic rats and controls. Thirteen male Sprague-Dawley rats were induced with STZ in citrate buffer and six control rats were injected with the same buffer solution. All rats were sacrificed after inductions at four (short-term DM) and twenty-four weeks (long-term DM). The selected spinal enlargements were processed for transmission electron microscopy. The OL alterations in both the cervical and lumbar enlargements were apparently the same. In short-term DM, the nuclei of OLs became swelled with chromatin clumping. Cytoplasmic organelles were moderately damaged. In long-term DM, OLs contained shrinkage nuclei with thick heterochromatin clumping. Severely degenerated mitochondria with disrupted cristae and broken membranes were observed. Moreover, distended and fragmented rough endoplasmic reticulum were observed, and large clear areas were present in the cytoplasm. Additionally, the loosening, splitting, and destruction of myelin lamellae were found. This study can provide important preliminary information about the alteration of OLs in the spinal cords of diabetic patients, which might be involve in the impairments of sensory and motor conduction velocities in these individuals.

RESUMEN: En pacientes diabéticos y modelos animales se observan alteraciones de la velocidad de conducción nerviosa sensorial y motora en la médula espinal, así como vainas de mielina degeneradas. De hecho, los oligodendrocitos (OL), que son importantes células neurogliales, generan mielina en el sistema nervioso central. La intumescencia espinal, a nivel cervical y lumbar, inerva los miembros. Por lo tanto, los propósitos de este estudio fueron examinar y comparar las alteraciones ultraestructurales de los OL en la intumescencia espinal de ratas diabéticas inducidas por estreptozotocina (STZ) y controles. Se indujeron trece ratas macho Sprague-Dawley con STZ en tampón citrato y se inyectaron seis ratas de control con la misma solución tampón. Todas las ratas se sacrificaron después de la inducción a las cuatro (DM a corto plazo) y a las veinticuatro semanas (DM a largo plazo). Las ampliaciones de la columna seleccionadas se procesaron para microscopía electrónica de transmisión. Las alteraciones de OL en las intumescencias cervical y lumbar eran aparentemente las mismas. En la DM a corto plazo, los núcleos de los OL se hincharon con la acumulación de cromatina. Los orgánulos citoplasmáticos sufrieron daños moderados. En la DM a largo plazo, los OL contenían núcleos de contracción con aglutinación de heterocromatina gruesa. Se observaron mitocondrias severamente degeneradas con crestas y membranas rotas. Además, se observó un retículo endoplásmico rugoso distendido y fragmentado, y estaban presentes grandes áreas claras en el citoplasma. Además, se encontraron el aflojamiento, la división y la destrucción de las laminillas de mielina. Este estudio puede proporcionar información preliminar importante sobre la alteración de los OL en la médula espinal de los pacientes diabéticos, que podría estar involucrada en las alteraciones de las velocidades de conducción sensorial y motora en estos individuos.

Gunshot injury to spine: An institutional experience of management and complications from a developing country / 中华创伤杂志(英文版)

PURPOSE@#Gunshot wounds are the second leading cause of spinal cord injuries. Surgical intervention for gunshot injury to the spine carries a high rate of complications. There is a scarcity of data on civilian gunshot injuries to the spine in Pakistan. Approximately 60 cases over the last 10 years have been recoded, with unusual presentation and neurological recovery. Thus it is imperative to fill this gap in data, by reviewing cases of civilian gunshot injuries to spine presenting at a tertiary care hospital (Aga Khan University Hospital, Karachi).@*METHODS@#This is a retrospective cohort study. Patients of all ages who presented to the emergency department of Aga Khan University Hospital, with gunshot injuries to spine between January 2005 and December 2016 were included in the study. Data were collected on neurological status (American Spinal Injury Association score was used for the initial and follow-up neurological assessment), extent of cord transection, motor and sensory deficits. The patients were further grouped into those with cord transection, and those with fractures of the bony spine but an intact spinal cord. These patients were then followed and the outcomes were recorded.@*RESULTS@#A total of 40 patients were identified. The mean ± SD of patients age was (30.9 ± 9.5) years. Of the 40 patients with gunshot wounds, 31 had the medical imaging performed at the facility, and hence they were included in this categorization. The remaining 9 patients were excluded from this additional grouping. Thirteen patients were managed surgically and 27 patients underwent the conservative management. The mean ± SD of follow-up was (8.7 ± 7.2) months. In our study, the thoracic spine was the most commonly injured region in gunshot injuries. Of the 31 patients with medical imaging performed at our institute, 17 (54.8%) had cord transection, of whom 8 (47%) ultimately developed paraplegia.@*CONCLUSION@#The prognosis of gunshot injuries to the spine can be varied depending on whether the spinal cord is intact or transected. This will help healthcare providers to plan the further management of the patient and counsel them accordingly.

Influence of age, sex, height and lumber stenosis on the position of the conus medullaris in adults / Influencia de la edad, sexo, altura y estenosis lumbar en la posición del cono medular en adultos

Anatomical orientation of the termination level of Conus Medullaris (CM) has imperative role clinically for anesthetists and neurosurgeons which is considered as an objective guide to perform spinal anesthesia and spinal punctures circumspectly with less chance to have serious injuries. The current retrospective study was carried on to determine the location of the CM and how its termination level can be influenced by age, sex, height and lumber stenosis spine disease. The study included 462 participants that consisted of 199 men and 263 women range from 21 to 80 years of age and height range between 150-190 cm. Also, it included 150 lumber stenosis patients. The location of the CM was imaged using a series of magnetic resonance images (MRI) for the lumbosacral spine at different levels including, T12, T12-L1, L1, L1-L2, and L2. The measurements revealed inconsiderable differences in the CM termination level in relation to age, sex and height in a healthy study population. Moreover, the results showed insignificant differences in the CM termination level between men and women whether they are healthy or having lumbar spinal stenosis. In conclusion, the most common level of CM termination is at L1 followed by L1-L2. It is safe to perform a lumbar neuraxial procedures at the level of L3-L4.

La orientación anatómica del nivel de terminación del Conus medullaris (CM) tiene un importante papel clínico para los anestesistas y neurocirujanos, que se considera una guía objetiva para realizar la anestesia espinal y las punciones de la columna circunspectivamente con menos posibilidades de provocar lesiones graves. Se llevó a cabo un estudio retrospectivo para determinar la ubicación del CM y cómo su nivel de terminación puede verse afectado por la edad, el sexo, la altura y una patología de la columna vertebral, la estenosis lumbar. El estudio incluyó 462 participantes que consistían en 199 hombres y 263 mujeres de 21 a 80 años de edad y una altura de entre 150-190 cm. Además, se incluyeron 150 pacientes con estenosis lumbar. La ubicación del CM se determinó mediante una serie de imágenes de resonancia magnética (RM) de la columna lumbosacra a diferentes niveles, incluidos T12, T12-L1, L1, L1-L2 y L2. Las mediciones revelaron diferencias considerables en el nivel de terminación de CM en relación con la edad, el sexo o la altura en una población de estudio saludable. Además, los resultados mostraron diferencias poco significativas en el nivel de terminación de CM entre hombres y mujeres, sanos o con estenosis espinal lumbar. En conclusión, se encontró que el nivel más común de terminación del CM es L1 seguido de L1-L2, considerándose seguro realizar procedimientos neuroaxiales lumbares a nivel de L3-L4.

Impairment induction by axotomy in motor functional and histological anterior horn of spinal cord structure / Inducción del deterioro por axotomía en la estructura motora funcional e histológica del asta anterior de la médula espinal

We have surveyed the motor changes in rats subjected to sciatic nerve axotomy. The rats were divided into two groups, each one consisting of ten animals, which underwent the following intervention: The first group (control): healthy rats without any injuries and experimental group: rats with injured sciatic nerve without treatment. at 12 weeks, the L4 and L5 spinal cord segments were removed. We evaluated nerve function using muscle electromyography (EMG) activity and sciatic function index (SFI) simultaneously with histological spinal cord analyses by stereological methods at 12 week. After nerve injury presented gross locomotor deficits at week 12. We also found that the volume of the anterior horn of spinal cord and total number of motor neurons were decreased after nerve axotomy (p<0.05). In conjunction, these results indicate that peripheral nerve injuries have more severe consequences on hind limb motor output.

En este estudio se examinaron los cambios motores en ratas sometidas a axotomía del nervio ciático. Las ratas se dividieron en dos grupos diez animales. El primer grupo (control) eran ratas sanas sin lesiones, y el grupo experimental consistió en ratas con nervio ciático lesionado sin tratamiento. A las 12 semanas, los segmentos de la médula espinal L4 y L5 fueron removidos. Se evaluó la función nerviosa mediante electromiografía muscular (EMG) y el índice de función ciática (IFC), simultáneamente con análisis histológicos de la médula espinal mediante métodos estereológicos. A las 12 semanas de la lesión nerviosa presentó déficit locomotor grueso. Además, se observó que el volumen del asta anterior y el número total de neuronas motoras disminuyeron después de la axotomía nerviosa (P <0,05). En conjunto, estos resultados indican que las lesiones de los nervios periféricos determinan graves consecuencias de la función motora de los miembros posteriores.

Protective effect of combined melatonin and alpha-tocopherol administration in spinal cord ischemia-reperfusion injury in rat / Efecto protector de la administración combinada de melatonina y alfa-tocoferol en la lesión por isquemia-reperfusión de la médula espinal en ratas

Oxidative stress and inflammation are the key players in the development of motor dysfunction post-spinal cord ischemic reperfusion injury (SC-IRI). This study investigated the protective effect of concomitant pre-administration of melatonin and alpha-tocopherol on the early complications (after 48 hours) of spinal cord IRI injury in rats. Melatonin or α-tocopherol were preadministered either individually or in combination for 2 weeks, then rats were exposed SC-IRI. Neurological examinations of the hind limbs and various biochemical markers of oxidative stress and inflammation in the SC tissue were assessed. Solely pre-administration of either melanin or α-tocopherol significantly but partially improved motor and sensory function of the hind limbs mediated by partial decreases in SC levels of MDA, AOPP and PGE2 levels and activities of SOD, partial significant decreases in plasma levels of total nitrate/nitrite and significant increases in AC activity of GSH-Px. However, combination therapy of both drugs resulted in the maximum improvements in all neurological assessments tested and biochemical endpoints. In conclusion, by their synergistic antioxidant and antiinflammatory actions, the combination therapy of melatonin and α-tocopherol alleviates SC-IRI induced paraplegia.

El estrés oxidativo y la inflamación son claves en el desarrollo de la disfunción motora posterior a lesión isquémica de la médula espinal (SC-IRI). Este estudio investigó acerca del efecto protector de la administración previa concomitante de la melatonina y alfa-tocoferol en las complicaciones tempranas (después de 48 horas) de la lesión de IRI de la médula espinal en ratas. La melatonina o el α-tocoferol se administraron individualmente o en combinación durante 2 semanas, luego las ratas fueron expuestas a SC-IRI. Se evaluaron los exámenes neurológicos de las miembros pélvicos y diversos marcadores bioquímicos de estrés oxidativo e inflamación en el tejido subcutáneo. Solo la administración previa de melatonina o α-tocoferol mejoró parcial y significativamente la función motora y sensorial de los miembros pélvicos mediadas por disminuciones parciales en los niveles de SC de los niveles de MDA, AOPP y PGE2 y las actividades de la SOD, disminuciones significativas parciales en los niveles plasmáticos del total nitrato / nitrito y aumentos significativos en la actividad de AC de GSH-Px. Sin embargo, se observaron los mejores resultados durante la combinación de ambos fármacos en todas las evaluaciones neurológicas y en los puntos finales bioquímicos. En conclusión, debido a sus acciones antioxidantes y antiinflamatorias sinérgicas, la terapia de melatonina y α-tocoferol alivia la paraplejía inducida por SC-IRI.

Post mortem evaluation of spinal cord and protruded intervertebral discs in dogs without neurological abnormalities / Avaliação post mortem da medula espinhal e de discos intervertebrais protrusos em cães sem alterações neurológicas

The aim of this study was to verify the presence of protrusion of the intervertebral disc (IVD) causing compression of the spinal cord and/or roots of cauda equina in 30 dogs above seven years of age with no evidence of previous neurological abnormalities. After the occurrence of death or euthanasia, a laminectomy was performed from C2 to the sacral vertebra to verify the presence of IVD protrusions. The protruded IVD were macroscopically graded according to the Thompson scale, and the corresponding spinal cord segment was histologically analyzed for nervous tissue compression. Of the 30 dogs, twelve (40%) presented disc protrusion, and of these 12 dogs, seven (58%) presented more than one protruded disc. Disc protrusion was observed in 3.2% (25/780) of all IVD evaluated. Of the six chondrodystrophic dogs, five (83%) presented disc protrusion. Of the 24 nonchondrodystrophic dogs, seven (29%) presented IVD protrusion. The site that showed the highest frequency of protrusion was L4-L5 (6/25) followed by the L6-L7 region (3/25) and C6-C7 (3/25). Only two of the medullary segments evaluated showed histological changes, with presence of focal lymphocytic infiltration and multifocal mild hemorrhage in the gray matter. Under the conditions of this study, 40% of dogs older than seven years old showed IVD protrusions without showing neurological signs, with no abnormality of the nervous tissue in 92% of the evaluated segments. Thus, the protrusion of the IVD in some dogs, can only be a finding of no clinical relevance.(AU)

O objetivo do presente estudo foi verificar a presença de protrusão do disco intervertebral (DIV) causando compressão da medula espinhal e/ou raízes da cauda equina em cadáveres de cães, sem histórico de alterações neurológicas. Para isso, após a ocorrência de óbito ou realização de eutanásia em 30 cães com mais de sete anos de idade, sem histórico de alterações neurológicas, foi realizada a laminectomia entre C2 até a vértebra sacral para verificar a presença de protrusões do DIV. Os DIV protrusos foram graduados macroscopicamente quanto à degeneração segundo a escala de Thompson, e o segmentos medular correspondente à localização da compressão foi analisado microscopicamente em busca de lesões compressivas ao tecido nervoso. Quarenta por cento dos cães (12/30) apresentaram protrusão do DIV, e destes, 58% (7/12) apresentavam mais de um DIV protruso. A protrusão estava presente em 3,2% (25/780) de todos os DIV avaliados. Cães de raças condrodistróficas apresentaram protrusões em 83% dos casos (5/6) e raças não condrodistróficas em 29% (7/24). O local que apresentou maior frequência de protrusão foi L4-L5 (6/25), seguido da região L6-L7 (3/25) e C6-C7 (3/25). Apenas dois segmentos medulares avaliados na histopatologia apresentaram alterações, com presença de infiltrado linfoplasmocitário focal e hemorragia discreta multifocal na substância cinzenta. Nas condições do presente estudo, 40% dos cães com mais de sete anos de idade apresentaram protrusões do DIV sem que houvesse sinais neurológicos, com normalidade do tecido nervoso em 92% dos segmentos avaliados. Assim, a protrusão do DIV, em alguns cães, pode ser apenas um achado sem relevância clínica.(AU)

Foix-Alajouanine syndrome mimicking a spinal cord tumor / Síndrome de Foix-Alajouanine simulando um tumor intramedular espinal

Summary Subacute necrotizing myelopathy (SNM) or Foix-Alajouanine syndrome is a rare disease characterized by progressive neurological dysfunction caused by a spinal dural arteriovenous fistula (AVF). Radiological diagnosis is usually suspected when there is intramedullary nonspecific enhancement and perimedullary flow voids. Ring-enhancement is rarely reported in the scope of AVF, which poses a diagnostic challenge and raises the suspicion of a spinal cord tumor. In such situations, biopsy can be required and delay proper diagnosis. We report the case of a patient with SNM, who underwent biopsy on the assumption of it being a spinal cord tumor.

Resumo Mielopatia necrotizante subaguda (MNS) ou síndrome de Foix-Alajouanine é uma doença rara que se caracteriza por disfunção neurológica progressiva causada por uma fístula arteriovenosa espinal dural. O diagnóstico radiológico é comumente suspeitado quando aparece captação não específica de contraste e de artefatos de fluxo (flow voids) perimedulares. Raramente, a captação de contraste exibe o aspecto em anel, constituindo um grande desafio diagnóstico. Nesses casos, o principal diagnóstico diferencial é um tumor intramedular, e os pacientes são encaminhados para biópsia da lesão, atrasando o diagnóstico definitivo. Relatamos o caso de uma paciente com MNS, a qual foi submetida à biópsia da lesão em virtude de suspeita de tumor intramedular.

Efectos del tratamiento con vitamina E en el tubo neural y médula espinal en embriones y fetos de ratones Mus musculus expuestos al uso de ácido valproico / Effects of treatment with vitamin E in the neural tube and spinal cord Mus musculus mouse embryos exposed to the use of valproic acid

El ácido valproico (VPA) es el principal anticonvulsivante utilizado contra la epilepsia durante la gestación. Sin embargo, en etapas iniciales del embarazo actúa como teratógeno y ocasiona malformaciones como fisura labio-palatina, alteraciones en el desarrollo genital y espina bífida, siendo esta última la más frecuente. Esto se produce debido al aumento de especies reactivas de oxígeno, pudiendo contrarrestarse administrando vitamina E. El objetivo fue determinar si la vitamina E disminuye el daño en tubo neural y médula espinal de embriones y fetos de ratonas expuestas a VPA. Se conformaron 8 grupos de animales. A los 8 días post-fecundación se les administró a los grupos 1 y 5 suero fisiológico 0,3 mL; grupos 2 y 6 VPA 600 mg/Kg; grupos 3 y 7 VPA 600 mg/Kg y vitamina E 200 UI/Kg; grupos 4 y 8 vitamina E 200 UI/kg. A los 12 días post-fecundación, se sacrificaron los grupos 1, 2, 3 y 4, y a los 17 días los restantes grupos. Los embriones fueron procesados y teñidos con cresil violeta, observándose cortes histológicos a nivel cervical, torácico y lumbar. Los grupos tratados con vitamina E presentaron menor cantidad de neuroblastos y motoneuronas, pero de tamaño mayor en comparación al grupo tratado con VPA (p<0,05), siendo similares a los grupos controles. Al comparar el tubo neural y médula espinal en los distintos niveles (cervical, torácico y lumbar), no hubo diferencias estadísticamente significativas. La administración prenatal de vitamina E disminuye los defectos en tubo neural y médula espinal de embriones de 12 y 17 días de gestación sometidos a VPA.

Valproic Acid (VPA) is the main anticonvulsant used for epilepsy throughout the gestation period. However, when used at early stages of pregnancy, it acts as a tetarogenic agent, causing congenital malformations such as cleft-lip and/or cleft palate, abnormal genital development and spina bifida, being the latter the most frequent. This is the result of the increase of reactive oxygen species, which can be countered with the supplementation of vitamin E. The aim was determine if vitamin E minimizes the damage to the neural tube and spinal cord of mice embryos and fetuses previously exposed to VPA. Eight groups of mice were constituted. Eight days post fertilization, groups 1 and 5 were administered 0,3 ml of saline solution; groups 2 and 6 600mg/Kg of VPA, groups 3 and 7 600mg/Kg of VPA and 200UI/Kg of Vitamin E; groups 4 and 8 200 UI/Kg of Vitamin E. 12 days after fertilization, groups 1, 2, 3 and 4 were euthanized, whereas in the case of the remaining groups, the same process was performed 17 days after fertilization. The embryos were stained with cresyl violet, thus enabling the observation of histological sections at cervical, thoracic and lumbar levels. Groups supplied with vitamin E presented a lower amount of neuroblasts and motoneurons. However, these elements were bigger in size compared to the group treated with VPA (p<0,05), being these results similar to those obtained with the control groups. When comparing the neural tube and spinal cord at different levels (cervical, thoracic and lumbar), no statistically significant differences were found. It was determined that prenatal administration of vitamin E lessens the damage to the neural tube and spinal cord of mice embryos of 12 and 17 days of gestation previously exposed to VPA.

Síndrome de encefalopatía posterior reversible con afectación del tronco cerebral y médula espinal y retinopatía hipertensiva grado IV / Posterior reversible encephalopathy syndrome with brain stem and spinal cord involvement and grade iv hypertensive retinopathy

El síndrome de leucoencefalopatía posterior reversible se presenta con síntomas de edema cerebral e imágenes hiperintensas en las secuencias T2 y FLAIR en la resonancia magnética (RM) de cerebro. Las lesiones típicamente comprometen la región parietooccipital, aunque también pueden tener localizaciones atípicas. Presentamos el caso de una mujer de 33 años que consultó por trastornos visuales asociados con dolor de cabeza intenso e hipertensión grave (220/140 mmHg). El examen del fondo de ojo reveló retinopatía hipertensiva grado IV y la RM mostró lesiones hiperintensas en T2 y FLAIR a nivel centropontino, bulbo y médula espinal, las cuales regresaron tras el control sostenido de la presión arterial. (AU)

The posterior reversible leukoencephalopathy syndrome presents with symptoms of cerebral edema. On magnetic resonance imaging (MRI) of the brain, hyperintensities on T2 -weighted and FLAIR sequences typically affect the parietal-occipital region; however it may also have atypical locations. We report the case of a 33 year old patient with visual disturbances associated with intense headache and severe hypertension (220/140 mmHg). The Fundus examination revealed grade IV hypertensive retinopathy, and MRI showed hyperintense lesions in T2 and FLAIR affecting the pontine center, medulla and spinal cord, which regressed after sustain blood pressure control. (AU)

Consumption and expenditure on food prepared away from home among Mexican adults in 2006 / Consumo y gasto en alimentos preparados fuera de casa en adultos mexicanos en 2006

Objective. To describe food expenditure and consumption of foods prepared away from home among Mexican adults. Materials and methods. Data were from 45 241 adult participants in the National Health and Nutrition Survey 2006, a nationally-representative, cross-sectional survey of Mexican households. Descriptive statistics and multivariable linear and logistic regression were used to assess the relationship between location of residence, educational attainment, socioeconomic status and the following: 1) expenditure on all food and at restaurants, and 2) frequency of consumption of comida corrida or restaurant food and street food. Results. Food expenditure and consumption of food prepared away from home were positively associated with socioeconomic status, educational attainment, and urban vs. rural residence (p<0.001 for all relationships in bivariate analyses). Conclusions. Consumption of food prepared outside home may be an important part of the diet among urban Mexican adults and those with high socioeconomic status and educational attainment.

Objetivo. Describir los gastos en alimentos y el consumo de alimentos preparados fuera de casa en población mexicana. Material y métodos. Los datos fueron de 45 241 adultos mexicanos en la Encuesta Nacional de Salud y Nutrición de 2006, representativa al nivel nacional. Se utilizaron estadísticas descriptivas y regresión linear y logística para estimar la relación entre el lugar de residencia, el nivel educativo y el nivel socioeconómico, con el gasto en todos los alimentos y en restaurantes, y con la frecuencia de consumo de comida corrida, en restaurantes y de la calle. Resultados. El gasto en alimentos y el consumo de alimentos preparados se asociaron positivamente con el nivel socioeconómico, el nivel educativo y la residencia rural (p<0,001 para todas las relaciones). Conclusiones. El consumo de alimentos preparados puede ser una parte importante de la dieta de los adultos urbanos y de aquéllos con altos niveles socioeconómicos y educativos.

Neumomediastino y necrosis cutánea en asociación a dermatomiositis: presentación de un caso clínico y revisión de la literatura / Pneumomediastinum and cutaneous necrosis in dermatomyositis: Report of one case

We report a 37 years old male with a dermatomyositis treated with oral cyclophosphamide. He was admitted to the hospital due to a zone of skin necrosis with purulent exudate, located in the second left toe. A complete blood count showed a leukocyte count of 2,600 cells/mm³. A Chest CAT scan showed a pneumomediastinum with emphysema of adjacent soft tissue. Cyclophosphamide was discontinued and leukocyte count improved. The affected toe was amputated and a chest CAT scan showed a partial resolution of the pneumomediastinum. We discuss and review the pathogenesis, clinical presentation and management of pneumomediastinum and cutaneous necrosis in association with dermatomyositis.

Unique Imaging Features of Spinal Neurenteric Cyst

A 50-year-old male presented with acutely progressed paraplegia. His magnetic resonance imaging demonstrated two well-demarcated components with opposite signals in one cystic lesion between the T1- and T2-weighted images at the T1 spine level. The patient showed immediately improved neurological symptoms after surgical intervention and the histopathological exam was compatible with a neurenteric cyst. On operation, two different viscous drainages from the cyst were confirmed. A unique similarity of image findings was found from a review of the pertinent literature. The common findings of spinal neurenteric cyst include an isointense or mildly hyperintense signal relative to cerebrospinal fluid for both T1- and T2-weighted images. However, albeit rarer, the signals of some part of the cyst could change into brightly hyperintensity on T1-weighted images and hypointensity on T2-weighted images due to the differing sedimentation of the more viscous contents in the cyst.