Mielopatía por déficit de cobre: serie de casos y revisión de la literatura / Myelopathy due to copper deficiency: A case series and review of the literature

El déficit de cobre puede presentarse como una mielopatía y manifestarse como una ataxia sensorial secundaria a una desmielinización de los cordones posteriores de la médula espinal. Puede acompañarse de citopenias, principalmente anemia y leucopenia. Se presenta una serie de casos de tres pacientes con mielopatía por déficit de cobre, diagnosticados y manejados desde el año 2020 al 2022 en un hospital universitario de alta complejidad en Colombia. Dos de los casos eran mujeres. El rango de edad fue entre 57 y 68 años. En los tres casos, los niveles séricos de cobre estaban disminuidos y en dos de ellos, se descartaron diferentes causas de mielopatía que afectan los cordones posteriores de la médula espinal como el déficit de vitamina B12, vitamina E y ácido fólico, tabes dorsal, mielopatía por virus de la inmunodeficiencia humana, esclerosis múltiple e infección por el virus linfotrópico humano de tipo I y II, entre otras. Sin embargo, un paciente tenía deficiencia de vitamina B12 asociada con de cobre en el momento del diagnóstico de la mielopatía. En los tres casos hubo ataxia sensitiva y en dos, la paraparesia fue el déficit motor inicial. Se deben incluir siempre la determinación de los niveles de cobre dentro del abordaje diagnóstico de todo paciente con enfermedad gastrointestinal crónica, con diarrea crónica, síndrome de mala absorción o reducción significativa de la ingestión en la dieta, y que desarrolle síntomas neurológicos sugestivos de compromiso de los cordones, ya que se ha reportado que el retraso en el diagnóstico de las mielopatías se asocia con pobres desenlaces neurológicos.

Copper deficiency can present as myelopathy by the manifestation of sensory ataxia, secondary to demyelination of the posterior cords of the spinal cord, accompanied by cytopenia, mainly anemia, and leukopenia. Case series study of three patients with myelopathy due to copper deficiency, diagnosed and managed from 2020 to 2022 in a highly complex university hospital in Colombia. Regarding gender, two cases were female patients. The age range was between 57 and 68 years. In all three cases serum copper levels were decreased, and in two of these, different causes of myelopathy affecting the posterior cords of the spinal cord were ruled out, such as vitamin B12, vitamin E and folic acid deficiency, tabes dorsalis, myelopathy due to human immunodeficiency virus, multiple sclerosis and infection by the human lymphotropic virus type I and II, among others. However, at the moment of the myelopathy diagnosis, one patient had vitamin B12 deficiency associated with copper insufficiency. All three cases presented sensory ataxia, and in two, paraparesis was the initial motor deficit. The diagnostic approach must include copper levels assessment in every case of patients with chronic gastrointestinal pathology, chronic diarrhea, malabsorption syndrome, or significant reduction in dietary intake; and the development of neurological symptoms that may suggest cord involvement. It has been reported that a delay in diagnosis can lead to poor neurological outcomes.

Mucopolisacaridosis tipo VI: a propósito de un caso / Mucopolysaccharidosis type VI. Case report

La mucopolisacaridosis tipo VI o síndrome de Maroteaux-Lamy se produce por la deficiencia de la enzima arilsulfatasa B que ocasiona la acumulación intracelular de dermatán sulfato. El riesgo de compresión medular es particularmente elevado y muy frecuente en la unión occipito-cervical. La terapia de reemplazo enzimático ha sido esencial para los pacientes con esta enfermedad; sin embargo, no tiene efecto sobre las alteraciones esqueléticas, y su impacto sobre la estabilidad espinal está aún en estudio. Se sugiere un examen anual (evaluación neurológica, radiografías, resonancia magnética y potenciales provocados somatosensitivos) y, en caso de anomalías, cada 6 meses. Pese al alto riesgo anestésico, la mielopatía y los síntomas progresivos indican la necesidad de una descompresión quirúrgica. Presentamos a una niña de 12 años con mucopolisacaridosis tipo VI tratada con terapia de reemplazo enzimático desde los 7 años, que acude a la consulta con síntomas compatibles con mielopatía cervical alta progresiva. Fue sometida a una descompresión y artrodesis occipito-cervical con ampliación del foramen magno. Esta enfermedad es infrecuente; por lo tanto, es imperativo el seguimiento multidisciplinario del paciente, así como conocer el riesgo de compresión medular y su oportuno tratamiento quirúrgico a cargo de cirujanos espinales. Nivel de Evidencia: IV

Mucopolysaccharidosis type VI, also known as Maroteaux-Lamy syndrome, is caused by a deficiency of the arylsulfatase B enzyme, which causes intracellular accumulation of dermatan sulfate. The risk of spinal cord compression is particularly high and frequent at the occipitocervical junction. Enzyme replacement therapy has been essential for patients with this disease; however, it has no effect on skeletal abnormalities, and its impact on spinal stability is still under study. An annual examination (neurological evaluation, radiography, magnetic resonance imaging, and somatosensory evoked potentials) is recommended. In case of anomalies, it should be repeated every 6 months. Despite the high anesthetic risk, myelopathy and progressive symptoms indicate the need for surgical decompression. We present the case of a 12-year-old girl with mucopolysaccharidosis type VI treated with enzyme replacement therapy since the age of 7, who came to the consultation with symptoms compatible with progressive high cervical myelopathy. She underwent occipitocervical decompression and fusion with enlargement of the foramen magnum. This disease is rare; therefore, multidisciplinary patient follow-up is imperative, as well as knowing the risk of spinal cord compression and its timely surgical treatment by spinal surgeons. Level of Evidence: IV

Intensive care unit deaths among neurosurgery patients in a tertiary hospital in South Western Nigeria

Neurosurgical patients are the most critical ICU admissions. While advancements in neurosurgical ICUs (NICU) have improved outcomes of care globally, ICU mortality remains a major clinical issue in developing nations. This study evaluates ICU mortalities of neurosurgical patients in a general ICU setting at the UNIOSUN Teaching Hospital, Osogbo, Nigeria. Method: Case records of neurosurgery patients who died in the ICU of UNIOSUN Teaching Hospital, Osogbo, South-Western, Nigeria from June 2012 to May 2022 were reviewed. Simple descriptive statistics of data on demographics, clinical diagnoses, management and outcome were done. Results: Mortality rate was 38.9% (84 of 216 admissions). Males were 67(79.8%) and the mean age was 41.5years (Range: 2-85years). The average duration of ICU stay was 3.5days (Range: 30minutes-20days). Most patients had severe traumatic brain injury (TBI) (62, 73.8%). This was followed by cerebrovascular diseases (12, 14.3%) and brain tumours (6, 7.1%). Two had brain abscess. One patient each had mixed subacute/chronic subdural haematoma and severe cervical spondylotic myelopathy. Of the 69 whose case files were found, 7(10.1%) had a diagnosis of brainstem death before eventual 'final' death after an average of 13.5 additional hours on mechanical life support. The identified secondary causes of death included raised ICP, sepsis, primary surgical haemorrhage, seizures, acute kidney injury, malignant hypertension, poor glycaemic control and aggressive blood pressure lowering. Only 1 patient had autopsy. Conclusion: Most ICU mortalities among neurosurgical patients were from severe TBI. The establishment of NICU is necessary to improve outcome of care of neurosurgical patients

Mielopatia ou paraparesia espástica tropical (HAM/TSP) e os domínios da SF-36 / Tropical spastic myelopathy/ paraparesis (HAM/TSP) and the SF-36 domains

Introdução: a paraparesia espástica tropical ou mielopatia associada ao HTLV (HAM/TSP) é umadoença infecciosa e inflamatória crônica, que pode interferir em vários aspectos da vida do indivíduo e, com isso, alterar sua qualidade de vida (QV). Objetivo: avaliar os domínios da escala SF-36 que mais contribuem para as alterações na qualidade de vida dos pacientes com HAM/TSP. Metodologia: nesse estudo observacional, transversal e quantitativo, realizado com 49 pacientes diagnosticados com HAM/TSP do setor de Neurociências do Ambulatório Professor Francisco Magalhães Neto, na cidade de Salvador, Bahia, Brasil, a qualidade de vida foi avaliada pelo questionário de saúde Short Form-36 (SF-36), no período de fevereiro de 2019 a julho de 2020, e de fevereiro de 2022 a abril de 2022. Os dados obtidos foram avaliados por análise estatística descritiva e testes de correlação de Pearson e Spearman. Resultados: foi observado que os menores escores do questionário SF-36, indicando pior qualidade de vida, foram relativos aos domínios vinculados às limitações físicas, capacidades funcionais e limitações emocionais, nessa ordem. Os melhores domínios, indicando melhor qualidade de vida, nessa população, foram saúde mental e aspectos sociais, demostrando que os pacientes com HAM/TSP relatam alterações físicas e emocionais em sua qualidade de vida. Conclusão:os domínios que mais alteraram a qualidade de vida dos pacientes com HAM/ TSP foram AF, CF e AE. Assim, utilizando-se da escala SF-36, profissionais de saúde podem identificar e intervir precocemente em domínios que comprometam a saúde física e emocional dos pacientes com HAM/TSP, alterando, consequentemente, sua qualidade de vida.

Introduction: tropical Spastic Paraparesis or HTLV-associated Myelopathy (HAM/TSP) is a chronic infectious and inflammatory disease that can interfere with various aspects of individuals life and, thereby alter their Quality of Life (QoL). Objective: to evaluate the domains of the sf-36 scale that most contribute to changes in the quality of life of patients with HAM/TSP. Methods: in this observational, cross-sectional and quantitative study carried out with 49 patients diagnosed with HAM/TSP from the Neuroscience sector of the Professor Francisco Magalhães Neto Ambulatory, in the city of Salvador, Bahia, Brazil, quality of life was assessed using the Short Form Health Questionnaire Form ­ 36 (SF-36), from February/19 to July/20 and from February/22 to April/22. Data obtained were evaluated by descriptive statistical analysis and Pearson and Spearman correlation tests. Results: it was observed that the lowest scores on the SF-36 questionnaire, indicating worse quality of life, were related to the domains linked to physical limitations, functional capacities and emotional limitations, in that order. The best domains, indicating better quality of life in this population, were mental health and social aspects, showing that patients with HAM/TSP report physical and emotional changes in their quality of life. Conclusion: the domains that most changed the quality of life of patients with HAM/TSP were PA, FC and EA. Thus using the SF-36 scale, health professionals can identify and intervene early in areas that compromise the physical and emotional health of patients with HAM/TSP, consequently altering their quality of life.

Mielopatía cervical secundaria al consumo recreativo de óxido nitroso una afección emergente / Cervical myelopathy secondary to recreational nitrous oxide use: an emergent pathology

Resumen Se describe el caso de una paciente de 27 años que se presentó al servicio de urgencias por hipoestesia y parestesia de dos meses de evolución. El cuadro se inició en ambos pies, progresó en pocos días hasta las rodillas sin trastornos de la marcha y se acompañó de distonías en pulgar e índice de ambas manos. La analítica sanguínea incluyendo tóxicos fue negativa. La resonancia magnética medular mostró una imagen sugestiva de mielopatía o mielitis (C3-C5) sin otras anomalías sugestivas de enfermedad sistémica. El análisis bioquímico y el bacteriológico del líquido cefalorraquídeo fueron normales. Ante estos elementos se re-interrogó a la paciente en busca de consumo de tóxicos inusuales con la confirmación de consumo de óxido nitroso. La paciente fue internada para la realización de otros estudios que confirmaron la hipótesis diagnóstica del servicio de urgencias.

Abstract We describe the case of a 27-year-old female patient who presented to the emergency ward with hypoesthesia and paresthesia developing over the last two months, initially in both feet and progressing to the knees in a few days without associated gait disorders. Dystonia in the thumb and index finger of both hands was noted. Blood tests including toxic drugs were negative. The spinal magnetic resonance imaging was consistent with (C3-C5) myelopathy or myelitis without other abnormalities suggestive of systemic diseases. The biochemi cal and bacteriological analysis of the cerebrospinal fluid was normal. Because of these findings, the patient was re-interviewed to determine the consumption of unusual drugs, and nitrous oxide consumption was referred. The patient was admitted for further studies, which confirmed the diagnosis.

Cervical spondylotic myelopathy: is a combined approach necessary? / Mielopatia cervical espondilótica: a abordagem combinada é necessária? / Mielopatía cervical espondilótica: ¿el abordaje combinado es necesario?

ABSTRACT Objective: Cervical spondylotic myelopathy (CSM) is the main cause of spinal dysfunction in adults. The type of surgical approach to treatment is not well defined in the literature. The objective is to report the results obtained through isolated posterior decompression in patients with a previous indication of the combined approach for the treatment of cervical spondylotic myelopathy. Methods: This is a therapeutic study with level of evidence II, according to the Oxford classification table. Ten patients who underwent isolated posterior approach surgery for the treatment of cervical spondylotic myelopathy were evaluated through imaging and questionnaires (visual analog scale, mJOA-Br scale - Brazilian Portuguese version of the Modified Japanese Orthopedic Association Scale, and Neck Disability Index (NDI)), comparing pre- and postoperative results. Results: Late evaluation of the 10 patients was performed in the period ranging from 24 to 36 months (mean of 30.3 months ± 7.25) following surgery. The comparison of the clinical and radiological parameters in all patients showed a statistical difference in relation to the preoperative scales applied and to the degree of cervical lordosis (p <0.05), evidencing improvement after decompression and posterior fixation of the cervical spine. Conclusions: The isolated posterior approach (decompression, fixation and arthrodesis) allowed the clinical and radiological improvement of patients with cervical spondylotic myelopathy and who had an indication of the complementary anterior approach. Level of evidence II; Retrospective study.

RESUMO Objetivo: A mielopatia cervical espondilótica (MCE) é a principal causa de disfunção medular nos adultos. O tipo de abordagem cirúrgica para o tratamento não é bem definido na literatura. O objetivo é relatar os resultados obtidos por meio da descompressão posterior isolada nos pacientes com indicação prévia da abordagem combinada para o tratamento da mielopatia cervical espondilótica. Métodos: Trata-se de um estudo terapêutico com nível de evidência II, conforme a tabela de classificação Oxford. Dez pacientes submetidos apenas à abordagem cirúrgica posterior para tratamento de mielopatia cervical espondilótica foram avaliados por meio de exames de imagem e de questionários (escala visual analógica, escala mJOA-Br - Versão em Português da Escala Modificada da Sociedade Japonesa de Ortopedia e escala de incapacidade cervical - Neck Disability Index - NDI), comparando os resultados pré e pós-operatórios. Resultados: A avaliação tardia dos 10 pacientes foi realizada no período que variou de 24 a 36 meses (média de 30,3 meses ± 7,25) de pós-operatório. A comparação dos parâmetros clínicos e radiológicos em todos os pacientes mostrou diferença estatística com relação ao pré-operatório para as escalas aplicadas e para o grau de lordose cervical (p < 0,05), evidenciando a melhora depois da descompressão e da fixação posterior da coluna cervical. Conclusões: A abordagem posterior isolada (descompressão, fixação e artrodese) permitiu a melhora clínica e radiológica de pacientes com mielopatia cervical espondilótica e que tinham indicação da abordagem anterior complementar. Nível de evidência II; Estudo retrospectivo.

RESUMEN Objetivo: La mielopatía cervical espondilótica (MCE) es la principal causa de disfunción medular en los adultos. El tipo de abordaje quirúrgico para el tratamiento no está bien definido en la literatura. El objetivo es relatar los resultados obtenidos por medio de la descompresión posterior aislada en los pacientes con indicación previa del abordaje combinado para el tratamiento de la mielopatía cervical espondilótica. Métodos: Se trata de un estudio terapéutico con nivel de evidencia II, conforme a la tabla de clasificación Oxford. Diez pacientes sometidos únicamente al abordaje quirúrgico posterior para el tratamiento de la mielopatía cervical espondilótica fueron evaluados mediante exámenes de imagen y cuestionarios (escala analógica visual, escala mJOA-Br - versión en portugués de la escala modificada de la Sociedad Japonesa de Ortopedia y escala de incapacidad cervical - Neck Disability Index - NDI), comparando los resultados pre y postoperatorios. Resultados: La evaluación tardía de los 10 pacientes fue realizada en el período que varió de 24 a 36 meses (promedio de 30,3 meses ± 7,25) de postoperatorio. La comparación de los parámetros clínicos y radiológicos en todos los pacientes mostró diferencia estadística con relación al preoperatorio para las escalas aplicadas y para el grado de lordosis cervical (p <0,05), evidenciando la mejora después de la descompresión y de la fijación posterior de la columna cervical. Conclusiones: El abordaje posterior aislado (descompresión, fijación y artrodesis) permitió la mejora clínica y radiológica de pacientes con mielopatía cervical espondilótica y que tenían indicación del abordaje anterior complementario. Nivel de evidencia II; Estudio retrospectivo.

Cervical kyphoscoliosis / Cifoescoliosis cervical

Cervical kyphoscoliosis is an uncommon spinal deformity. Kyphosis or outward curvature of cervical-spine (Image A) has led to a fixed flexion state resulting in suspension of patient's head in the air while lying on the imaging table. Additionally, dextroscoliosis or rightward convexity of the cervical vertebral axis has resulted in a persistent leftward head tilt (Image B). Head and neck radiation and trauma can lead to cervical kyphoscoliosis. In addition to the cosmetic deformity, patients present with myelopathic sensorimotor symptoms such as weakness and tingling of upper extremities. The Poisson effect states that flexion of the spine lengthens and stretches the spinal canal, reduces its area and narrows its lumen. This causes spinal cord impingement and myelopathy.

La cifoescoliosis cervical es una deformidad de la columna vertebral poco frecuente. La cifosis o la curvatura hacia fuera de la columna cervical (imagen A) ha dado lugar a un estado de flexión fija que provoca la suspensión de la cabeza del paciente en el aire mientras está tumbado en la mesa de diagnóstico por imagen. Además, la dextroscoliosis o convexidad hacia la derecha del eje vertebral cervical ha dado lugar a una inclinación persistente de la cabeza hacia la izquierda (Imagen B). La radiación de cabeza y cuello y los traumatismos pueden provocar cifoescoliosis cervical. Además de la deformidad estética, los pacientes presentan síntomas sensoriomotores mielopáticos como debilidad y hormigueo en las extremidades superiores. El efecto Poisson establece que la flexión de la columna vertebral alarga y estira el canal espinal, reduce su área y estrecha su lumen. Esto provoca el pinzamiento de la médula espinal y la mielopatía.

Microscope assisted anterior cervical discectomy and fusion for the treatment of single segment cervical spondylotic myelopathy / 中国骨伤

OBJECTIVE@#To compare the efficacy of microscope assisted anterior cervical discectomy and fusion with conventional surgical approach in the treatment of single-segment cervical spondylotic myelopathy.@*METHODS@#The clinical data of 89 patients with single-segment cervical spondylotic myelopathy treated from March 2015 to March 2019 were retrospectively analyzed. There were 55 males and 34 females, with an average of (52.00±11.36) years old. Among the patients, 34 cases were treated with conventional anterior cervical discectomy with fusion (conventional group), including C@*RESULTS@#Intraoperative blood loss and hospital stay in microscope group were less than those in conventional group (@*CONCLUSION@#Both methods can achieve satisfactory effect in treating single-segment cervical spondylotic myelopathy. However, microscope-assisted anterior cervical discectomy and fusion has advantages of clear vision, less bleeding and fewer intraoperative complications.

Imaging study of soft tissue swelling after anterior cervical corpectomy / 中国骨伤

OBJECTIVE@#To study the changes of anterior soft tissue swelling after anterior cervical subtotal corpectomy, titanium mesh fusion and internal fixation.@*METHODS@#From November 2015 to July 2018, 151 patients with cervical spondylotic myelopathy were treated with anterior single corpectomy, titanium mesh fusion and internal fixation, including 109 males and 42 females, aged 44 to 81 (59.77±8.34) years. Through postoperative follow up observation, the C@*RESULTS@#All patients were followed up for 15 to 40(28.00±3.52) months. One week after the operation, the swelling of anterior soft tissue reached the peak, and then decreased. At 8 months after the operation, the swelling of anterior soft tissue on C@*CONCLUSION@#Anterior subtotal cervical corpectomy, titanium mesh bone graft fusion and internal fixation can cause swelling of the anterior soft tissue. One week after operation, we should pay more attention to the aggravation of the swelling of the anterior soft tissue to avoid the occurrence of dysphagia, respiratory obstruction, asphyxia and other complications.

Microcirugía de las fístulas durales arteriovenosas espinales, más un repaso de la anatomía vascular vertebromedular / Microsurgery of spinal dural arteriovenous fistulas, plus a review of the vertebromedullary vascular anatomy

Introducción: la fístula dural arteriovenosa espinal (FDAVE) es una enfermedad vascular rara, de etiología desconocida y frecuentemente subdiagnosticada. El tratamiento puede ser microquirúrgico o endovascular. Material y Método: Análisis retrospectivo de una serie de 8 pacientes consecutivos con FDAVE tratados por microcirugía entre 2010 y 2020. Fueron evaluados parámetros como edad, sexo, cuadro clínico pre y postoperatorio medido con las escalas de Aminoff-Logue y Rankin modificada. Los estudios diagnósticos con RMN (Resonancia Magnética Nuclear), ARM (Angio Resonancia Magnética) y ADM (Angiografía Digital Medular) se utilizaron para determinar nivel lesional y resultados quirúrgicos. Resultados: Fueron operados 8 pacientes (7 masculinos y 1 femenino) con un promedio de edad de 58 años. El tiempo de evolución del cuadro clínico al diagnóstico fue menor a 12 meses salvo un caso de 32 meses. Las FDAVE fueron localizadas en: 6 a nivel dorsal entre D6 y D12, una en L2 y la última en S1 (5 derechas y 3 izquierdas). La arteria de Adamkiewicz se identificó en: 4 casos en L1, 2 en D12, 1 en D10 y un caso en D7 (6 izquierdas y 2 derechas). De los 8 pacientes operados, 3 fueron embolizados previamente. La evolución postoperatoria del cuadro neurológico fue: 2 de 8 permanecieron estables y 6 de 8 mejoraron uno o más puntos en la escala de Rankin modificada; no hubo complicaciones en el postoperatorio. Todos los pacientes mejoraron las imágenes en RMN diferida y la ADM luego de los 6 meses fue negativa. El seguimiento promedio fue de 48 meses con un rango de 11 a 116 meses, ningún paciente presentó recidiva de la FDAVE. Conclusiones: El tratamiento quirúrgico de las FDAVE es un método muy eficaz, de baja morbilidad y menor tasa de recurrencia comparado con el tratamiento endovascular.

Introduction: Spinal dural arteriovenous fistula (SDAVF) is a rare vascular disease, of unknown etiology and frequently underdiagnosed. Treatment can be microsurgical or endovascular. Material and Method: Retrospective analysis of a series of 8 SDAVF patients treated by microsurgery between 2010 and 2020. Parameters including age, sex, pre and postoperative clinical condition were analyzed according to modified Aminoff-Logue and modified Rankin scales. Diagnostic studies such as MRI (Magnetic Resonance Imaging), MRA (Magnetic Resonance Angiogram) and spinal DSA (Digital Subtraction Angiography), were evaluated for lesion level, as were surgical results. Results: Eight patients (7 male and 1 female), average age of 58 years were operated. The interval from symptom onset to diagnosis was less than 12 months in all cases except one (32 months). SDAVF locations were thoracic in 6 cases between T6 and T12, at L2 in one and at S1 in one case (5 on the right and 3 on the left). The Adamkiewicz artery was identified in: 4 cases at L1, 2 at D12, 1 at D10 and in one case at D7 (6 left-sided and 2 right-sided). Three of the 8 patients operated had undergone prior embolization. Postoperative neurological outcomes showed: 2 patients remained stable and 6 had improved one or more points on the modified Rankin scale; no postoperative complications were observed. Follow-up MRI images improved in all cases and spinal DSA was negative at six months. Average follow-up was 48 months (range 11 to 116 months), no patient presented recurrence. Conclusions: Microsurgical treatment of SDAVF proved to be efficient, with low morbidity and lower recurrence rates compared to endovascular results.

Cervical Myelopathy Secondary to Gout: Case Report / Mielopatia cervical secundária a gota: Relato de caso

Abstract Gout is a crystalline arthropathy frequent in the population, but gouty spondyloarthropathy, also called axial gout, is uncommon. The current case report presents a rare case of cervical myelopathy secondary to axial gout. A 50-year-old female patient, without previous pathologies, presented with loss of strength, altered sensitivity, and pyramidal release for 2 years. The computed tomography showed a lytic image in the spinous process of C7, and signs of myelopathy with myelomalacia on magnetic resonance imaging of the cervical spine. After the surgical procedure and biopsy of the material, the diagnosis was gout, and treatment for the pathology was started, with complete improvement of the condition. The diagnosis of axial gout should be included in the spectrum of the differential diagnosis of diseases that affect the spine. Although gouty spondyloarthritis (or spondylitis) is uncommon, there is an underestimated occurrence due to the lack of investigation of the cases. The early diagnosis and treatment of the pathology can prevent patients from presenting complications of the disease, as reported in the present study.

Resumo A gota é uma artropatia cristalina frequente na população; entretanto, a espondiloartropatia gotosa, também chamada de gota axial, é incomum. O presente relato de caso apresenta um caso raro de mielopatia cervical secundária a gota axial. Uma paciente de 50 anos de idade, sem patologias prévias, apresentou quadro de perda de força, alteração de sensibilidade e liberação piramidal há 2 anos. A tomografia computadorizada evidenciou imagem lítica no processo espinhoso de C7, e sinais de mielopatia com mielomalácia foram observados na ressonância magnética da coluna cervical. Após o procedimento cirúrgico e biópsia do material, o diagnóstico foi de gota, e o tratamento para a patologia foi iniciado, com melhora completa do quadro. O diagnóstico de gota axial deve ser incluído no espectro do diagnóstico diferencial das doenças que acometem a coluna vertebral. Apesar de a espondiloartrite gotosa ser incomum, há uma ocorrência subestimada devido a não investigação dos casos. O diagnóstico precoce e tratamento da patologia pode evitar que pacientes apresentem complicações da doença, como a relatada no presente estudo.

Achados clínicos, histopatológicos e moleculares da mielopatia degenerativa canina: relato de caso / Clinical, histopathological and molecular findings of canine degenerative myelopathy: case report

Objetivou-se descrever os achados clínicos, histopatológicos e moleculares associados à MDC em um cão da raça Pastor-Suiço. O cão possuía uma paraparesia progressiva em membros pélvicos e foi submetido a avaliações clínicas, pelas quais se obteve, entre outros diferenciais, o diagnóstico presuntivo de MDC. Com a evolução dos sinais, o tutor optou pela eutanásia. Os achados histopatológicos da medula espinhal foram compatíveis com uma degeneração segmentar axonal e mielínica. O diagnóstico molecular foi realizado por meio da extração do DNA obtido por swab oral. Uma PCR foi otimizada utilizando-se primers descritos em literatura para amplificar a região do gene SOD1. A amostra foi, então, submetida a sequenciamento unidirecional, que revelou que o animal em questão era homozigoto para o alelo A para a mutação c.118G>A no éxon 2 do gene SOD1. O diagnóstico clínico presuntivo da MDC no presente caso foi esclarecido por meio dos achados histopatológicos, associados aos achados clínicos, e da sua caracterização molecular. Ressalta-se a contribuição deste relato, que traz aspectos clínicos, histopatológicos e moleculares associados à MDC na raça Pastor-Suíço, para a qual, até o presente momento, na literatura consultada, não há relato dessa enfermidade.(AU)

The objective of this study was to describe the clinical, histopathological and molecular findings associated with MDC in a Swiss Shepherd dog. The dog had a progressive paraparesis in pelvic limbs and was submitted to clinical evaluations where, among other differentials, the presumptive diagnosis of MDC was obtained. With the progression of the nervous deficits tutor opted for euthanasia. The histopathological findings of the spinal cord were compatible with axonal and myelinic segmental degeneration. Molecular diagnosis was performed by extracting the DNA obtained by oral swab. PCR was optimized using primers described in the literature to amplify the SOD1 gene region. The sample was then subjected to one-way sequencing which revealed that the animal in question was homozygous for the A allele for the c.118G>A mutation in exon 2 of the SOD1 gene. The presumptive diagnosis of MDC in the present case was clarified by histopathological findings, as well as by its molecular characterization. The contribution of this report brings clinical, histopathological and molecular aspects associated with canine degenerative myelopathy in the Swiss Shepherd breed, that until this moment, in the literature consulted, there is no report of this disease in the breed mentioned.(AU)

Center of gravity oscillations in HTLV-1-associated myelopathy/tropical spastic paraparesis / Oscilações do centro de gravidade na mielopatia associada ao HTLV-1/ paraparesia espástica tropical / Oscilaciones del centro de gravedad en la mielopatía asociada al HTLV-1/paraparesia espástica tropical

Abstract Introduction: Postural control in individuals with HTLV-1-associated myelopathy or tropical spastic paraparesis (HAM/TSP) is usually compromised, which increases the risk of falls, makes it difficult to perform activities of daily living, and impairs the quality of life. The profile of the center of gravity oscillations in this population is unknown and may aid in clinical follow-up and research. Objective: To compare the stabilometric values between HAM/TSP and uninfected individuals and verify the existence of correlations between stabilometric variables and the Berg Balance Scale (BBS). Method: A cross-sectional observational study was performed with infected individuals, classified as defined and likely (WHO criteria), compared to accompanying persons and seronegative relatives. A baropodometry platform (Footwork®) was used to obtain the oscillation values of the body's center of gravity in total oscillation area (TOA), anterior-posterior oscillation (APO) and lateral oscillation (LO). Mean values were correlated with BBS by Spearman's Correlation (5% alpha). Approved by the ethical committee of Escola Bahiana de Medicina e Saúde Pública under Opinion 49634815.2.0000.5628. Results: An asymmetric distribution of all the stabilometric variables analyzed in the HAM/TSP population was found, different from the uninfected group (p < 0.05). It was also possible to verify strong to moderate and inverse correlations between the variables of center of gravity oscillation with the scores obtained in BBS, especially for TOA and LO. Conclusion: People with HAM/TSP presented higher values for the center of gravity oscillations and these were correlated with the BBS in the balance evaluation.

Resumo Introdução: o controle postural em indivíduos com mielopatia associada ao HTLV-1 ou paraparesia espástica tropical (HAM/TSP) é geralmente comprometido, o que aumenta o risco de quedas, dificulta a realização de atividades de vida diária e prejudica a qualidade de vida. O perfil das oscilações do centro de gravidade nesta população é desconhecido e pode auxiliar no acompanhamento clínico e na pesquisa. Objetivo: comparar os valores estabilométricos entre pessoas com HAM/TSP e não infectados, e verificar a existência de correlações entre variáveis estabilométricas e a Escala de Equilíbrio Berg (EEB). Método: foi realizado um estudo observacional transversal com indivíduos infectados, classificados como definidos e prováveis (critérios da OMS), comparados com acompanhantes e familiares soronegativos. Uma plataforma de baropodometria (Footwork®) foi utilizada para obter os valores de oscilação do centro de gravidade do corpo em área de oscilação total (AOT), oscilação anteroposterior (OAP) e oscilação laterolateral (OLL). Os valores médios foram correlacionados com a BBS pela Correlação de Spearman (alfa 5%). Aprovado pelo Comitê de Ética da Escola Bahiana de Medicina e Saúde Pública sob o CAAE 49634815.2.0000.5628. Resultados: encontrou-se distribuição assimétrica de todas as variáveis estabilométricas analisadas na população com HAM/TSP, diferentes do grupo de não infectados (p < 0,05). Também foi possível verificar correlações de forte a moderada e inversas entre as variáveis de oscilação do centro de gravidade com os escores obtidos na EEB, especialmente para AOT e OLL. Conclusão: Pessoas com HAM/TSP apresentaram valores maiores para as oscilações do centro de gravidade e estas foram correlacionadas com a EEB na avaliação do equilíbrio.

Resumen Introducción: El control postural en individuos con mielopatía asociada al HTLV-1 o paraparesia espástica tropical (HAM/TSP) suele estar comprometido, lo que aumenta el riesgo de caídas, les dificulta en las actividades de la vida diaria y perjudica su calidad de vida. Conocer el perfil de las oscilaciones del centro de gravedad en esta población puede ayudar en el seguimiento clínico y la investigación. Objetivo: Comparar los valores estabilométricos entre personas con HAM/TSP y personas no infectadas, y verificar la existencia de correlaciones entre las variables estabilométricas y la Escala de Equilibrio de Berg (BBS). Método: Se realizó un estudio observacional transversal con individuos infectados, clasificados como definidos y probables (criterios de la OMS), comparados a acompañantes y familiares seronegativos. Se utilizó una plataforma de baropodometría (Footwork®) para obtener los valores de oscilación del centro de gravedad del cuerpo en el área de oscilación total (AOT), oscilación antero-posterior (OAP) y oscilación lateral-lateral (OLL). Los valores medios se correlacionaron con la BBS por la correlación de Spearman (alfa 5%). Estudio aprobado por el Comité de Ética de la Escuela Bahiana de Medicina y Salud Pública bajo CAAE 49634815.2.0000.5628. Resultados: Se encontró una distribución asimétrica de todas las variables estabilométricas analizadas en la población HAM/TSP diferente en el grupo no infectado (p <0,05). También fue posible verificar correlaciones de fuertes a moderadas e inversas entre las variables de oscilación del centro de gravedad con las puntuaciones obtenidas en la BBS, especialmente para AOT y OLL. Conclusión: Las personas con HAM/TSP presentaron valores más altos en las oscilaciones del centro de gravedad, las cuales se correlacionaron con la BBS en la evaluación del equilibrio.

First case report of nematode parasitic myelopathy in a wild feline in Brazil / Primeiro relato de caso de mielopatia parasitária causada por nematódeo em um felídeo selvagem no Brasil

Abstract Parasitic myelopathy caused by Gurltia paralysans in domestic cats is a disease commonly reported in several South American countries. The adult parasite is lodged in the meningeal veins and spinal cord, often causing clinical manifestations of vascular proliferation, thrombophlebitis, and medullary compression. Wild felines are believed to be the definitive hosts of this parasite. The infection occurs through the ingestion of paratenic hosts, but the life cycle of G. paralysans is not yet clearly understood. In this paper, we discuss a case of parasitic myelopathy in a margay (Leopardus wiedii) that died during post-surgical care. Necropsy revealed focal hemorrhages in the thoracolumbar spinal cord. A microscopic examination revealed adult nematodes and eggs inside the veins of subarachnoid space in spinal cord, suggesting G. paralysans infection. This is first description of parasitic myelopathy in a margay in Brazil.

Resumo Mielopatia parasitária causada por Gurltia paralysans em gatos domésticos é uma doença comumente relatada em vários países da América do Sul. O parasita adulto aloja-se nas veias das meninges e da medula espinhal, muitas vezes causando proliferação vascular, tromboflebite e compressão medular, que se manifestam como sinais clínicos. Acredita-se que os felídeos selvagens sejam hospedeiros definitivos deste parasita e que a infecção ocorre por ingestão de hospedeiros paratênicos, entretanto seu ciclo de vida completo é desconhecido. Aqui, apresentamos um caso de um gato-maracajá (Leopardus wiedii) que morreu durante a assistência pós-cirúrgica. Na necropsia, foram observadas hemorragias focais na medula espinhal toracolombar. A microscopia revelou presença de nematódeos adultos e ovos, localizados dentro das veias do espaço subaracnoide da medula espinhal, o que sugeriu a infecção por G. paralysans. Esta é a primeira descrição de mielopatia parasitária em um gato-maracajá no Brasil.

Retro-odontoid pseudotumor: a poorly recognized alteration of the craniocervical junction

SUMMARY INTRODUCTION Retro-odontoid pseudotumor (ROP) is a rare disease that affects the atlantoaxial joint and, in general, is associated with local biomechanical alterations that may or may not cause instability. METHODS Descriptive study of the literature available in databases MEDLINE/PubMed, LILACS, and Scopus. The research was conducted in April 2019. DISCUSSION ROP is, possibly, a syndromic designation that encompasses a significant variety of diseases of the atlantoaxial joint. There are different pathophysiological mechanisms implicated in its genesis. The patients, almost in their entirety, present with severe myelopathy, and most of them are treated surgically, with the posterior decompression being the most commonly used method, with or without arthrodesis. Evolution is usually favorable. CONCLUSION The ROP is still poorly recognized as a differential diagnosis between the diseases of the cranial-cervical junction. The information available in the literature analyzed was based mainly on the study of reports or case series; therefore, it is insufficient to define conducts with a high level of scientific evidence.

RESUMO INTRODUÇÃO O pseudotumor retro-odontoide (PRO) é uma patologia rara que acomete a articulação atlantoaxial e, em geral, está associada a alterações biomecânicas locais que podem ou não causar instabilidade. METODOLOGIA Estudo descritivo da literatura disponível nas bases de dados Medline/PubMed, Lilacs e Scopus. A pesquisa foi realizada em abril de 2019. DISCUSSÃO O PRO é, possivelmente, uma designação sindrômica que abrange uma variedade significativa de doenças da articulação atlantoaxial. Existem diferentes mecanismos fisiopatológicos implicados em sua gênese. Os pacientes, quase em sua totalidade, apresentam quadro de mielopatia grave e a maioria deles é tratada cirurgicamente, sendo a descompressão posterior o método mais utilizado, com ou sem artrodese. A evolução costuma ser favorável. CONCLUSÃO O PRO ainda é pouco reconhecido como diagnóstico diferencial entre as doenças da junção crânio-cervical. As informações disponíveis na literatura analisada foram baseadas principalmente no estudo de relatos ou séries de casos, sendo, portanto, insuficientes para definir condutas com alto nível de evidência científica.

Intraoperative Motor Evoked Potential Improvement in Cervical Spondylotic Myelopathy: Comparison of Cortical Stimulation Parameters