Mielopatía cervical degenerativa: una patología cada vez más frecuente y que requiere diagnóstico y manejo precoz / Degenerative cervical myelopathy

Degenerative cervical myelopathy (DCM) is the most common cause of spinal cord dysfunction in adults. Its prevalence is increasing as a result of population aging. The diagnosis of DCM is often delayed or overlooked, resulting in secondary neurologic morbidity. The natural course of DCM typically presents as a gradual neurological deterioration, with symptoms ranging from muscle weakness to complete paralysis, with variable degrees of sensory deficits and sphincter dysfunction. Magnetic resonance imaging (MRI) and electrophysiological studies allow the assessment of spinal cord function and its structural damage to determine treatment and clinical outcomes. All patients with signs and symptoms consistent with DCM should be referred to a spine surgeon for assessment and tailored treatment. Those patients with mild DCM can be managed non-operatively but require close monitoring and education about potentially alarming signs and symptoms. Surgery is not currently recommended for asymptomatic patients with evidence of spinal cord compression or cervical spinal stenosis on MRI, but they require a structured follow-up. Patients with moderate or severe DCM require surgical decompression to avoid further progression. The objective of this review is to raise awareness of degenerative cervical myelopathy and its increasing prevalence as well as to aid non-surgical healthcare workers for a timely diagnosis and management of this disabling condition.

Expert consensus on clinical diagnosis and treatment of bone metastases and bone-related diseases of prostate cancer (2021 edition) / 中华肿瘤杂志

The morbidity and mortality of prostate cancer ascend yearly, which seriously threatens the health of the male population. Bone is the main metastasis site of prostate cancer, with bone metastases and skeletal-related events (SREs) occuring in more than 70% of patients with advanced prostate cancer. Bone metastases and the resulting SREs, such as spinal cord compression and pathological fracture, seriously affect the life quality of the patients. Although the active treatments of the primary disease is important, the application of bone-modifying agents to prevent SREs cannot be overlooked. However, there is no standard treatment procedure for the bone metastasis of prostate cancer at present. According to the clinical status, the Genitourinary Oncology Committee of Chinese Anti-cancer Association formulated this consensus, which integrated clinical evidence and real world clinical practices in China and abroad, to help clinicians make more accurate diagnosis and treatment for the bone metastases of prostate cancer with multidisciplinary strategies.

High cervical spine spondylodiscitis management and literature review / Espondilodiscite da coluna cervical alta: manejo e revisão da literatura

Summary Spondylodiscitis affecting the cervical spine is the most unusual type. Disease progression can be dramatic, even causing quadriplegia and death. We present an unusual case that progressed with osteolytic lesions between C2 and C3, causing cord compression and epidural abscess. The patient was treated surgically by a double approach and improved without neurological deficits and with better inflammatory markers. We reviewed the current literature on the subject.

Resumo A espondilodiscite, que acomete a coluna cervical, é a de localização mais rara. Pode ter uma evolução dramática, inclusive causando tetraplegia e óbito. Apresentamos um caso atípico que evoluiu com lesões osteolíticas entre C2 e C3, causando compressão medular e abscesso epidural. O paciente foi submetido a tratamento cirúrgico por dupla abordagem e evoluiu bem, sem déficits neurológicos e com melhora dos marcadores inflamatórios. Revisamos a literatura vigente sobre o assunto.

Survival score scales of patients operated with spinal metastases: retrospective application in a Brazilian population / Escalas de prognóstico em pacientes operados com metástases vertebrais: aplicação retrospectiva em população brasileira

ABSTRACT Spinal cord epidural metastasis (SEM) is a common complication of systemic cancer. Predicting these patient’s survival is a key factor to select the proper treatment modality, but the three most used score scales to predict their survival (Tokuhashi revised score, Tomita score and Bauer modified score) were designed in single institutions and their reliability to predict correctly the patient’s survival were first tested only in those specific populations. This prognostication issue is addressed in this article, evaluating retrospectively the survival of 17 patients with SEM from a Brazilian general hospital with these score scales. Our results show that the actual survival of those patients were worse than the predicted of all three score scales, suggesting that differences between the different populations might have affected their reliability and alert that their usage as a major factor to select the most appropriate treatment have to be done with caution.

RESUMO Metástases vertebrais são uma complicação comum em pacientes com câncer sistêmico. Avaliar o prognóstico e a sobrevida desses pacientes é um fator de grande importância para escolher o tratamento mais adequado, porém as três escalas mais usadas atualmente para prever a sobrevida deles (Tokuhashi revisada, Tomita e Bauer modificada) foram desenhadas em instituições isoladas, e sua habilidade em estimar corretamente a sobrevida desses pacientes foram testadas primeiramente apenas nessas populações específicas. Essa questão de estimar o prognóstico é abordada nesse artigo, analisando retrospectivamente a sobrevida de 17 pacientes com metástase vertebral provenientes de um hospital geral no Brasil com essas escalas. Nossos resultados apontam que a sobrevida real desses pacientes foi menor que a prevista pelas três escalas, sugerindo que as diferenças entres as diferentes populações podem ter afetado a aplicabilidade delas. Assim, alertamos que o uso dessas escalas em populações diferentes das estudadas originalmente deve ser feito com cuidado.

The negative prognostic impact of bone metastasis with a tumor mass

OBJECTIVE: Typically, bone metastasis causes osteolytic and osteoblastic lesions resulting from the interactions of tumor cells with osteoclasts and osteoblasts. In addition to these interactions, tumor tissues may grow inside bones and cause mass lesions. In the present study, we aimed to demonstrate the negative impact of a tumor mass in a large cohort of patients with bone metastatic cancer. METHODS: Data from 335 patients with bone metastases were retrospectively reviewed. For the analysis, all patients were divided into three subgroups with respect to the type of bone metastasis: osteolytic, osteoblastic, or mixed. The patients were subsequently categorized as having bone metastasis with or without a tumor mass, and statistically significant differences in median survival and 2-year overall survival were observed between these patients (the median survival and 2-year overall survival were respectively 3 months and 16% in patients with a tumor mass and 11 months and 26% in patients without a tumor mass; p<0.001). RESULTS: According to multivariate analysis, the presence of bone metastasis with a tumor mass was found to be an independent prognostic factor (p=0.011, hazard ratio: 1.62, 95% confidence interval: 1.11–1.76). Bone metastasis with a tumor mass was more strongly associated with osteolytic lesions, other primary diseases (except for primary breast and prostate cancers), and spinal cord compression. CONCLUSION: Bone metastasis with a tumor mass is a strong and independent negative prognostic factor for survival in cancer patients. .

Spinal cord compression after radiolabeled metaiodobenzylguanidine analogue therapy in advanced malignant insulinoma

Malignant insulinomas are frequently diagnosed at a late stage. Medical management is necessary to slow progression of the disease and control of hypoglycemic symptoms when cure by surgical treatment is not possible. Multimodal treatment, in these cases, has been used with variable clinical response. We describe a 68-yr-old woman who presented response failure to usual treatment and was alternatively treated with radiolabeled metaiodobenzylguanidine ([131I]-MIBG) analogue therapy with development of neurologic complications. We also present a review of the current role of [131I]-MIBG treatment in insulinomas.

Compressions médullaires lentes : prévalence et étiologies, expérience du service de rhumatologie du CHU de Cocody à Abidjan

Objectif : Déterminer la prévalence du syndrome de compression médullaire lente et en identifier les étiologies en pratique rhumatologique à Abidjan.Patients et méthodes : Notre étude rétrospective et descriptive, a été menée au sein du service de rhumatologie du CHU de Cocody à Abidjan de Janvier 2004 à Décembre 2013. Nous avons sélectionné 121 patients souffrant de syndrome de compression médullaire clinique. Nous nous sommes intéressés aux données sociodémographiques et étiologiques.Résultats : La prévalence hospitalière était de 2,6% soit 121 patients sur 4531 pathologies rhumatologiques. Les hommes prédominaient (56,1%) avec un sex-ratio de 1,3. L'âge moyen était de 48,8 ans. Les patients se plaignaient d'un déficit moteur isolé (42,2%) ou associé à une cervicalgie (16,5%), à une dorsalgie (23,1%) et à une dorsolombalgie (18,2%). La durée moyenne d'évolution du syndrome de compression médullaire était de 1,2 mois. Les principaux signes cliniques étaient une parésie ou paralysie (98,3%) et une radiculalgie (93,4%). La tomodensitométrie constituait l'imagerie majeure (80,1%). Les étiologies étaient les spondylodiscites bactériennes (55,3%) dominées par le mal de Pott (52% des cas), les affections rachidiennes dégénératives (23,3% des cas) et les causes tumorales malignes (21,4%).Conclusion : Le syndrome de compression médullaire lente est rare en pratique rhumatologique à Abidjan. Les étiologies sont dominées par les spondylodiscites bactériennes principalement le mal de Pott

Mutation c.1190-1delG/N in intron 8 and c.1708G>C/N in exon 12 not reported in the IDUA gene developed a clinical phenotype of Scheie syndrome / Mutación c.1190-1delG/N en el intrón 8 y c.1708G>C/N en el exón 12 no reportada en el gen de IDUA desarrolló un fenotipo clínico de síndrome de Scheie

Mucopolysaccharidoses are a group of lysosomal storage disorders caused by deficiency of enzymes catalyzing the degradation of glycosaminoglycans. Mucopoly-saccharidosis I can present a wide range of phenotypic characteristics with three major recognized clinical entities: Hurler and Scheie syndromes represent phenotypes at the severe and mild ends of the clinical spectrum, respectively, and the Hurler-Scheie syndrome is intermediate in phenotypic expression. These are caused by the deficiency or absence of α-L-iduronidase, essential to the metabolism of both dermatan and heparan sulfate, and it is encoded by the IDUA gene. We report the case of a 34-year-old male patient with enzymatic deficiency of α-L-iduronidase, accumulation of its substrate and a previously unreported mutation in the IDUA gene that developed a phenotype of Scheie syndrome.

Las mucopolisacaridosis son un grupo de trastornos de almacenamiento lisosomal causada por la deficiencia de enzimas que catalizan la degradación de glicosaminoglicanos. La mucopolisacaridosis tipo I puede presentar un amplio rango de características fenotípicas englobadas en tres entidades clínicas reconocidas: los síndromes de Hurler y Scheie representan los fenotipos graves y leves del espectro clínico, respectivamente y el síndrome de Hurler-Scheie intermedio en la expresión fenotípica. Estos son causados por la deficiencia o ausencia de la α-L-iduronidasa esencial para el metabolismo del dermatán y el heparán sulfato y es codificada por el gen IDUA. Se presenta el caso de paciente masculino de 34 años de edad con deficiencia enzimática de α-L-iduronidasa, acumulación de su sustrato y una mutación en el gen IDUA, no reportada previamente, que desarrolló un fenotipo del síndrome de Scheie.

Tokuhashi Scoring System has limited applicability in the majority of patients with spinal cord compression secondary to vertebral metastasis / A Escala de Tokuhashi possui aplicabilidade limitada na maioria dos pacientes com compressao medular secundaria a metastase vertebral

Spine is the primary bone site affected by systemic metastasis. Although there are scales that attempt to manage these patients, their real applicability is unknown. The Tokuhashi Scoring System (TSS) is a widely used prognostic tool. At the time of treatment, the data necessary to complete TSS may be incomplete, making its application impossible. Objective To evaluate the number of TSS scores completed by the time the clinical therapeutic decision was made. Methods From July 2010 to January 2012, we selected patients who were diagnosed with spinal metastases. Results Sixty spinal metastasis patients (21 female, 39 male) were evaluated between July 2010 and January 2012. At the time of the treatment decision, only 25% of the patients had completed the TSS items. Conclusion In the majority of patients with vertebral metastasis, TSS variables cannot be applied. .

A coluna vertebral é o sítio ósseo mais acometido na doença neoplásica metastática. Embora haja escalas que buscam normatizar o tratamento destes pacientes, sua real aplicabilidade é incerta. A Escala de Tokuhashi (TSS) é uma ferramenta prognóstica vastamente empregada. No momento do tratamento, os dados necessários ao preenchimento da escala podem estar incompletos, tornando sua aplicação inviável. Objetivo Avaliar o número de TSS completos até a tomada de decisão terapêutica. Métodos De Julho de 2010 a Janeiro de 2012, selecionamos pacientes diagnosticados com metástases espinhais. Resultados Sessenta pacientes foram avaliados durante o período; destes, 21 eram mulheres e 39, homens. Até a tomada de decisão, foi possível completar os itens da TSS em apenas 25% dos pacientes. Conclusão Na maioria dos pacientes com metástases espinhais, a TSS não pôde ser aplicada. .

Paraplejia secundaria a quiste óseo aneurismático de columna torácica / Paraplegia due to aneurismatic bone cyst of the thoracic spine

Objectives: Case report of progressive paraparesis secondary to Aneurysmal Bone Cyst of thoracic spine presenting over 40 years. Describe diagnostic triad: clinical, imaging and anatomopathologic examination. Describe the choice of surgical treatment and clinical outcomes. Performed literature review of Spine Aneurysmal Bone Cyst.

Objetivo: Dar a conocer el caso de un paciente que presenta una paraparesia progresiva secundaria a quiste óseo aneurismático (QOA) de columna torácica de presentación sobre los 40 años. Describir la triada diagnóstica en patología tumoral: clínica, imágenes y estudio anatomopatológico. Describir la elección del tratamiento quirúrgico y los resultados clínicos del caso. Se realiza revisión de la literatura publicada de QOA de columna.

Estenosis acueductal en acondroplasia: a propósito de un caso / Aqueductal stenosis in achondroplasia: report of a case

Achondroplasia is the most frequent cause of disproportionate short stature. Characterized by abnormal growth of long bones, it renders a short-limbed individual of normal intelligence. A serious potential complication is spinal compression, which can happen at any level but is particularly common at the craniocervical junction. It can cause important morbility during the first few years of life, including sudden death. We present a 22-month-old patient diagnosed with achondroplasia, who developed aqueductal stenosis with symptomatic spinal cord compression, diagnosed during a routine consultation, requiring decompressive surgery with excellent results.

La acondroplasia es la condición asociada a talla baja desproporcionada más frecuente, caracterizada por un crecimiento óseo anormal, que resulta en talla baja con extremidades cortas e inteligencia normal. Una de las complicaciones más habituales es la compresión medular, que puede ocurrir a cualquier nivel, siendo más frecuente en la unión cráneo cervical, generando alta morbimortalidad en los primeros años de vida, principalmente por muerte súbita. Presentamos una paciente de 1 año 10 meses con diagnóstico precoz de acondroplasia, que presentó en su evolución estenosis acueductal con compresión medular, sintomática, pesquisada en control rutinario, que requirió cirugía descompresiva con buena evolución posterior.

Neuroblastoma in an adult: Case report / Neuroblastoma en un adulto: Informe de un caso

El neuroblastoma es el tumor maligno sólido extracraneal más común en niños. Sólo el 10 por ciento de los casos se diagnostican después de la primera década de vida. Presentamos una paciente afroamericana de 23 años, con una masa paravertebral en T3-T5, múltiples lesiones en los cuerpos vertebrales y una lesión expansiva en la región parietal derecha. El estudio inmmunohistoquímico (negativo para CD99, CD20, CD3 y desmina y positivo para cromogranina, sinaptofisina y NB84), confirmó el diagnóstico de neuroblastoma. La paciente fue sometida a 12 ciclos de quimioterapia recibiendo VAC (vincristina / doxorubicina/ cyclofosfamida) intercalada con ICE (ifosfamida/ mesna/ etoposido). La doxorubicina fue reemplazada por actinomicina en el séptimo ciclo. La paciente toleró bien la quimioterapia y está clínicamente estable.

Neuroblastoma is the most common extracranial solid malignancy in children but rarely described in adults, being 10 percent of all cases diagnosed after the first decade of life. We report a 23 year-old black woman with a mass at paravertebral region of T3-T5, multiple lesions in vertebral bodies and expanding skull-brain lesion at the right parietal region. Immunohistochemical analysis (negative for CD99, CD20, CD3 and desmin; and positive chromogranin, synaptophysin and NB84) confi rmed the diagnosis of neuroblastoma. The patient was submitted to 12 cycles of chemotherapy receiving VAC (vincristine/doxorubicin/cyclophosphamide) interspersed with ICE (ifosfamide/mesna/etoposide) and doxorubicin was replaced by actinomycin in the 7th cycle. She had good tolerance to this therapy, and has been clinically stable.

Mielopatias agudas não compressivas: [revisão] / Acute non-compressive myelopathy: [review]

Mielopatias agudas são emergências neurológicas. Por serem pouco comuns, muitas vezes há dificuldade no diagnóstico e atraso no início do tratamento destes pacientes. Através de revisão de literatura, descrevemos as características das principais causas de mielopatias não compressivas: 1) mielopatias inflamatórias (doenças desmielinizantes, auto-imunes e para-infecciosas); 2) pós radiação tardia, 3) por alterações vasculares e 4) por deficiência de vitamina B12. Um algoritmo para auxílio na abordagem destes pacientes e posterior investigação etiológica é apresentado.

Formações císticas epidurais relacionadas a bursite interespinhosa, cisto sinovial e cisto discal / Epidural cystic masses associated with interspinous bursitis, synovial and discal cysts

Os autores apresentam casos de cistos epidurais, dentre eles os cistos sinoviais, discais, do ligamento amarelo e relacionados a bursite interespinhosa, todas essas condições determinando compressão radicular, do saco dural ou estenose do canal vertebral. Descrevem as características de imagem e localização na ressonância magnética nessas diferentes afecções.

The authors describe some cases of epidural cysts, namely synovial, discal, ligamentum flavum cysts, and cysts secondary to interspinous bursitis, all of these conditions determining radicular, dural sac compression or spinal canal stenosis. Magnetic resonance imaging findings and localization of these entities are described.

Mielopatía cervical por pseudogota, reporte de un caso clínico / Cervical myelopathy due to pseudogout: case report

Compressive myelopathy due to calcium pyrophosphate crystals deposit is an uncommon pathology. This disease can be isolated or associated with other articular deposits. We present a 78 year-old women with a story of lower limbs weakness and limitation of motion. Myelopathic signs where found on physical examination, so a cervical spine MRI was performed showing C6-C7 thickening and calcification of yellow ligament with compression of the spinal cord from the back. A surgical procedure with decompressive laminectomy and posterior instrumentation from C5 to C7 was performed The patient evolved favorable without postoperative complications and with neurological recovery from M3 to M4 in lower limbs. Histological study of the yellow ligament showed deposits of calcium pyrophosphate crystals. Calcium pyrophosphate crystals deposit is uncommon in the cervical spine, but it must be considered in the differential diagnosis of posterior cervical cord compression.

La mielopatía cervical por depósito de cristales de pirofosfato de calcio es una patología poco frecuente. Ésta puede presentarse aislada o asociada a otros depósitos articulares. Presentamos el caso clínico de una mujer de 78 años con historia de debilidad de extremidades inferiores y limitación de la marcha. Al ingreso la paciente presentaba signos clínicos de mielopatía. La resonancia nuclear magnética de columna cervical evidenció a nivel C6-C7 engrosamiento y calcificación del ligamento amarillo con compresión del cordón medular de atrás adelante. La paciente fue sometida a una laminectomía descompresiva y fusión posterior instrumentada C5-C7 No hubo complicaciones post operatorias y se observó recuperación neurológica de M3 a M4 en el post operatorio. El estudio anatomopatológico del ligamento amarillo mostró reacción inflamatoria crónica y depósitos de cristales de pirofosfato de calcio. El depósito de cristales de pirofosfato de calcio es poco frecuente en la columna cervical, pero debe considerarse en el diagnóstico diferencial de la mielopatía cervical por compresión medular posterior.

Sagittal diameter of the lumbar spinal canal and canal/body ratio in normal adult Saudis

The determination of normal sagittal diameter of the lumbar spinal canal in normal adult Saudis, and as to whether there are any racial difference in the morphometry of the lumbar spinal canal which are essential in a reliable evaluation of patients with symptoms of lumbar canal stenosis or low back pain. A retrospective study over a period of 5 years [June 2001 - May 2006] utilizing the computer system [magic web] which saves all x-ray images where computed tomography measurements of the mid-sagittal diameter of the lower three lumbar vertebral canal were made in 170 adults. For the sake of consistency, all measurements were taken by one observer and results were recorded as the mean of two measurements. To evaluate the significance obtained, Student t-test were carried out. The measurements showed that the mean mid-sagittal diameter of the lumbar spinal canal in the Saudi population was smilar to Caucasian and wider than the Far Eastern Asian or African. The mean male mid-sagittal diameters were slightly wider than those of the female but the differences were not statistically significant. The ratio is increased steadily as we go from L3 - L5, especially in the females. The mid-sagittal body/canal ratio is higher in the female in this population, which indicates that the lumbar canal is more capacious in females than that of the males. In age group over 60 years, the mid-sagittal diameters were smaller than those of younger generations at all levels. The mid-sagittal diameter of the lumbar spinal canal among Saudis is similar to the measurement in the Caucasian population; the radiological criteria of spinal canal stenosis should be identical between these two populations

Synovial cyst of the thoracic spine: case report

Spinal cord compressing syndrome due to synovial cyst (SC) of the thoracic spine is a rare clinic condition. We report a case of SC located in the thoracic spine causing spastic paraparesis in a 14 year-old female patient. The SC was removed thoroughly by laminectomy. The patient had an excellent recovery. The etiological and therapeutic aspects are discussed.

Síndrome de compressão medular causada por cisto sinovial (CS) da coluna torácica é patologia rara e pouco descrita na literatura. Descrevemos um caso de CS da coluna torácica causando paraparesia espástica em uma paciente de 14 anos de idade. O cisto foi removido através de laminectomia e a paciente apresentou uma excelente recuperação. Discutimos os aspectos etiológicos e terapêuticos.