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.

Discectomía, artrodesis intersomática y estabilización en mielopatía espondilótica cervical. Presentación de un caso / Discectomy, artrodesis and stabilization in cervical spondylotic myelopathy. Presentation of a case

RESUMEN Las alteraciones degenerativas de la columna se engloban en el término de espondilosis cervical. La mielopatía espondilótica cervical (MEC) es la forma más común de disfunción del cordón espinal en mayores de 55 años. Se considera la intervención quirúrgica en la mayoría de los casos de mielopatía cervical espondilótica evidente desde el punto de vista clínico, dado el riesgo de deterioro neurológico. En la mayoría de los casos de mielopatía cervical, la descompresión de la médula espinal genera estabilización o mejoría de la función de los haces largos medulares. La función es mejor cuando se restablecen bien las dimensiones del conducto vertebral después de la descompresión, cuando la descompresión es más precoz y cuando no hay comorbilidad considerable.

ABSTRACT The degenerative alterations of the column are included in the term of cervical espondilosis. The cervical spondylotic myelopathy it is the form more common of disfuntion of the spinal cord in bigger than 55 years. It is considered the surgical intervention in most of the cases of cervical spondylotic myelopathy evident from the clinical, given point of view the risk of neurological deterioration. In most of the cases of cervical myelopathy, the decompression of the spinal marrow generates stabilization or improvement of the function of the medullary long sheaves. The function is better when they recover well the dimensions of the vertebral conduit after the decompression, when the decompression is more precocious and when there is not considerable comorbility.

Schistosomal myeloradiculopathy - A case report

Abstract The most common neurological impairments related to schistosomiasis involve the lower portions of the medulla and the cauda equina. A 22-year-old woman, with no history, signs, or symptoms of hepatointestinal schistosomiasis, presented with lumbar pain associated with acute paresthesia and paresis of the right lower limb. Spinal schistosomiasis was suspected based on the disease progression and radiological findings, and the diagnosis was confirmed after cerebrospinal fluid analysis. The authors emphasize this pathology as important as a differential diagnosis in similar clinical scenarios, especially in endemic areas, because both early diagnosis and treatment are essential to avoid permanent sequelae.

Absceso epidural espinal: caso clínico pediátrico / Pediatric case report: Spinal epidural abscess

El absceso epidural espinal, una patología poco frecuente, presenta una incidencia de un caso cada 100 000 individuos, y se observa un aumento debido al incremento de factores de riesgo, tales como diabetes mellitus, anomalías espinales, tatuajes, acupuntura, analgesia epidural, sumado a una mayor disponibilidad de métodos de imágenes. Es una colección purulenta localizada entre la duramadre y el canal medular. Los gérmenes más comunes son Staphylococcus aureus y bacterias Gram-negativas. Sin tratamiento oportuno, evoluciona a la compresión medular y secuelas neurológicas permanentes. Una niña de 11 años se presentó con fiebre de 48 horas de evolución, dolor lumbar izquierdo, marcha antálgica con envaramiento lumbar. El examen neurológico era normal. Sobre los miembros inferiores, se observaban lesiones ampollares destechadas. La resonancia nuclear magnética mostró una imagen compatible con absceso epidural espinal. Evolucionó favorablemente. El tratamiento consistió en drenaje quirúrgico y antibióticos por 6 semanas. Del cultivo del material obtenido, creció Staphylococcus aureus meticilino sensible.

Spinal epidural abscess is an uncommon pathology. It has an incidence of one case per 100 000 individuals. An increase is observed due to the raise of risk factors such as diabetes mellitus, spinal abnormalities, tattoos, acupuncture, epidural analgesia, and a greater availability of imaging methods. It is a purulent collection located between the dura and the medullary canal. The most common germs are Staphylococcus aureus and Gram-negative bacteria. Without timely treatment, it evolves to medullary compression and permanent neurological sequelae. An 11-year-old girl was admitted with fever of 48 hs evolution, left lower back pain, antalgic gait with lumbar stiffness. Neurological examination was normal. Blunt blistering lesions were observed on lower limbs. Magnetic resonance imaging showed an image compatible with spinal epidural abscess. The evolutionwas favorable. Treatment consisted of surgical drainage and antibiotics for 6 weeks. From the culture of the material obtained, methidllin-sensitive Staphylococcus aureus was isolated.

A Brazilian Portuguese cross-cultural adaptation of the modified JOA scale for myelopathy

OBJECTIVES: To develop a version of the modified Japanese Orthopaedic Association (mJOA) scale that had been translated into Portuguese and cross-culturally adapted for the Brazilian population. METHODS: The well-established process of forward-backward translation was employed along with cross-cultural adaptation. RESULTS: Three bilingual translators (English and native Portuguese) performed the forward translation of the mJOA scale from English to Portuguese based on iterative discussions used to reach a consensus translation. The translated version of the mJOA scale was then back-translated into English by a native English-speaking translator unaware of the concepts involved with the mJOA scale. The original mJOA scale and the back-translated version were compared by a native North American neurosurgeon, and as they were considered equivalent, the final version of the mJOA scale that had been translated into Portuguese and cross-culturally adapted was defined. CONCLUSION: To facilitate global and cross-cultural comparisons of the severity of cervical myelopathy, this study presents a version of the mJOA scale that was translated into Portuguese and cross-culturally adapted for the Brazilian population.

Adrenoleucomielodistrofia como causa de paraparesia espástica en adulto joven. Reporte de caso / Adrenoleukomelodystrophy as a cause of spastic paraparesis in young adult. Case report

La adrenoleucodistrofia ligada al X (X-ALD), trastorno genético progresivo que afecta la sustancia blanca del SNC y la corteza suprarrenal, causa desde insuficiencia adrenal aislada y mielopatia lentamente progresiva hasta desmielinización cerebral devastadora. Presentamos un paciente masculino, 21 años de edad, tabaquista, con trastorno de la marcha de un año de evolución, paraparesia espástica e hiperreflexia en miembros inferiores. El análisis del líquido cefalorraquídeo (LCR) reveló proteinorraquia elevada, resultados negativos de bandas oligoclonales y virus Epstein Barr. Niveles de cortisol, ACTH, ácidos grasos de cadena muy larga en suero, fueron anormales. La RNM cerebral evidenció lesiones en sustancia blanca en región parietooccipital bilateral, comprometiendo el esplenio del cuerpo calloso, que realzaban con gadolinio. En RNM de columna cervical se observó lesión hiperintensa en secuencia T2 a nivel C7. Fue tratado con reemplazo adrenal. Presentamos un caso de X-ALD de inicio en adulto, con retraso en el diagnóstico debido a recursos limitados. Palabras claves: adrenoleucodistrofia ligada al X, paraparesia espástica, ácidos grasos de cadena muy larga, adrenomieloneuropatia

X- Linked adrenoleukodystrophy (X-ALD) is a progressive genetic disorder that affects CNS white matter and adrenal gland cortex, and causes from isolated adrenocortical insufficiency and slowly progressive myelopathy to devastating cerebral demyelination. We present a 21 years old male patient, smoker, with one year history of gradually progressive trouble walking, unsteady gait, asymmetric spastic paraparesis, lower extremity deep tendon reflexes were increased. Cerebrospinal fluid (CSF) analysis revealed elevated CSF protein, CSF oligoclonal bands and Epstein Barr virus negative results. Basal cortisol, ACTH and very-long- chain fatty acids in plasma with abnormal results. All other laboratory tests were normal. Cerebral MRI showed parietooccipital white matter abnormalities involving the splenium of the callosum that enhanced with gadolinium. Cervical spinal cord MRI showed a short-segment T2 hyperintense lesion at C7. He was treated with adrenal replacement. We present a case of adult onset X-ALD and diagnostic delay owed to limited resources

Management of degenerative cervical myelopathy - An update / Manejo da mielopatia cervical degeneratiiva - Uma atualização

SUMMARY Introduction Degenerative cervical myelopathy (DCM) is the most common cause of spinal cord dysfunction in adult patients. Patients generally present with a slow, progressive neurological decline or a stepwise deterioration pattern. In this paper, we discuss the most important factors involved in the management of DCM, including a discussion about the surgical approaches. Method The authors performed an extensive review of the peer-reviewed literature addressing the aforementioned objectives. Results Although the diagnosis is clinical, magnetic resonance imaging (MRI) is the study of choice to confirm stenosis and also to exclude the differential diagnosis. The severity the clinical symptoms of DCM are evaluated by different scales, but the modified Japanese Orthopedic Association (mJOA) and the Nürick scale are probably the most commonly used. Spontaneous clinical improvement is rare and surgery is the main treatment form in an attempt to prevent further neurological deterioration and, potentially, to provide some improvement in symptoms and function. Anterior, posterior or combined cervical approaches are used to decompress the spinal cord, with adjunctive fusion being commonly performed. The choice of one approach over the other depends on patient characteristics (such as number of involved levels, site of compression, cervical alignment, previous surgeries, bone quality, presence of instability, among others) as well as surgeon preference and experience. Conclusion Spine surgeons must understand the advantages and disadvantages of all surgical techniques to choose the best procedure for their patients. Further comparative studies are necessary to establish the superiority of one approach over the other when multiple options are available.

RESUMO Introdução a mielopatia cervical degenerativa (MCD) é uma das causas mais comuns de disfunção medular em adultos. Os pacientes em geral apresentam declínio neurológico lento e progressivo, ou deterioração escalonada. No presente artigo, discutimos os mais importantes fatores envolvidos no manejo da MCD, incluindo considerações sobre os aspectos relacionados à escolha da abordagem cirúrgica. Método realizou-se extensa revisão da literatura de artigos peer-reviewed relacionados ao tema. Resultados embora o diagnóstico seja realizado clinicamente, a ressonância magnética (RM) é o estudo de imagem de escolha para confirmá-lo e excluir eventuais diagnósticos diferenciais. A gravidade do quadro clínico pode ser avaliado utilizando-se diferentes escalas, como a modified Japanese Orthopedic Association (mJOA) ou a de Nürick, provavelmente as mais comuns. Uma vez que a melhora clínica espontânea é rara, a cirurgia é a principal forma de tratamento, em uma tentativa de evitar dano neurológico adicional ou deterioração e, potencialmente, aliviar alguns sintomas e melhorar a função dos pacientes. Abordagens cirúrgicas por via anterior, posterior ou combinada podem ser usadas para descomprimir o canal, concomitantemente a técnicas de fusão. A escolha da abordagem depende das características dos pacientes (número de segmentos envolvidos, local de compressão, alinhamento cervical, cirurgias prévias, qualidade óssea, presença de instabilidade, entre outras), além da preferência e experiência do cirurgião. Conclusão os cirurgiões de coluna devem compreender as vantagens e desvantagens de todas as técnicas cirúrgicas para escolher o melhor procedimento para seus pacientes. Estudos futuros comparando as abordagens são necessários para orientar o cirurgião quando múltiplas opções forem possíveis.

Myelopathy in systemic lupus erythematosus: clinical, laboratory, radiological and progression findings in a cohort of 1,193 patients / Mielopatia no lúpus eritematoso sistêmico: achados clínicos, laboratoriais, radiológicos e evolutivos em uma coorte de 1. 193 pacientes

Abstract Objective To describe clinical, laboratory, radiological and progression characteristics of myelopathy in systemic lupus erythematosus (SLE). Patients and methods A retrospective analysis was performed on a cohort of 1193 patients with SLE (ACR criteria) in order to identify patients with myelopathy (neuropsychiatric ACR). Disease activity was assessed by the SLE activity index (SLEDAI) on the date of the event and functional capacity was assessed by the Expanded Disability Status Scale (EDSS) at the last visit. Results We identified 14 (1.2%) patients with myelopathy. All were women with a mean age of 30 ± 11.5 years. Myelopathy occurred at the diagnosis of SLE in four (28%) patients; and nine (64%) patients had another type of neuropsychiatric manifestation associated. Neurological recurrence was observed in one (7%) patient. Disease activity was observed in 2 (14%) patients. Cerebrospinal fluid presented pleocytosis on 7 (53%) patients; antiphospholipid antibodies were positive in 5 (45%). Magnetic resonance imaging (MRI) showed T2 hyperintensity with a predominance of longitudinal involvement in 6 (86%) patients. Most were treated with intravenous corticosteroids and cyclophosphamide. No patient had full recovery and four (36%) had high EDSS scores. Three (21%) patients died from sepsis early in the course of their myelopathy, during or after immunosuppressive therapy. Conclusions Myelopathy occurred in 14 (1.2%) of the patients in our cohort and this may be the first manifestation of the disease occurring independently of systemic disease activity. Although rare, myelopathy shows great morbidity and mortality, can be recurrent and MRI is critical for diagnosis.

Resumo Objetivo Descrever características clínicas, laboratoriais, radiológicas e evolutivas de mielopatia no lúpus eritematoso sistêmico (LES). Pacientes e métodos Foi feita análise retrospectiva de uma coorte de 1.193 pacientes com LES (critérios ACR) para identificar os pacientes com mielopatia (ACR neuropsiquiátrico). A atividade de doença foi analisada pelo Índice de Atividade do LES (Sledai) na data do evento e a capacidade funcional pela Escala Expandida do Estado de Incapacidade (EDSS) na última consulta. Resultados Foram identificados 14 (1,2%) pacientes com mielopatia. Todas eram mulheres com média de 30 anos (DP ± 11,5 anos). A mielopatia ocorreu no diagnóstico do LES em quatro (28%) e em nove (64%) havia outro tipo de manifestação neuropsiquiátrica associada. Recorrência do quadro neurológico foi observado em uma (7%) paciente. Atividade de doença foi observada em dois (14%) pacientes. O líquido cefalorraquidiano apresentava pleocitose em sete (53%) pacientes anticorpos antifosfolípides eram positivos em cinco (45%). A ressonância magnética (RM) demonstrou hipersinal em T2 com predomínio do comprometimento longitudinal em seis (86%) pacientes. A maioria foi tratada com corticosteroides e ciclofosfamida endovenosos. Nenhuma paciente teve completa recuperação e quatro (36%) tinham escores altos da EDSS. Óbito foi observado em três (21%) durante episódio de mielopatia, por septicemia durante ou após terapia imunossupressora. Conclusões A mielopatia ocorreu em 14 (1,2%) dos pacientes da nossa coorte e pode ser a primeira manifestação da doença e ocorrer independentemente de atividade sistêmica da doença. Embora rara, é de grande morbimortalidade, pode ser recorrente e a RM é fundamental para o diagnóstico.

Clinical and epidemiological profiles of non-traumatic myelopathies / Perfil clínico e epidemiológico das mielopatias não-traumáticas

ABSTRACT Non-traumatic myelopathies represent a heterogeneous group of neurological conditions. Few studies report clinical and epidemiological profiles regarding the experience of referral services. Objective To describe clinical characteristics of a non-traumatic myelopathy cohort. Method Epidemiological, clinical, and radiological variables from 166 charts of patients assisted between 2001 and 2012 were compiled. Results The most prevalent diagnosis was subacute combined degeneration (11.4%), followed by cervical spondylotic myelopathy (9.6%), demyelinating disease (9%), tropical spastic paraparesis (8.4%) and hereditary spastic paraparesis (8.4%). Up to 20% of the patients presented non-traumatic myelopathy of undetermined etiology, despite the broad clinical, neuroimaging and laboratorial investigations. Conclusion Regardless an extensive evaluation, many patients with non-traumatic myelopathy of uncertain etiology. Compressive causes and nutritional deficiencies are important etiologies of non-traumatic myelopathies in our population.

RESUMO As mielopatias não-traumáticas representam um grupo heterogêneo de doenças neurológicas. Poucos estudos descrevem os perfis clínico e epidemiológico sobre a experiência de serviços de referência. Objetivo Descrever as características clínicas de uma coorte de mielopatia não-traumática. Método Os registros clínicos de 166 pacientes atendidos entre 2001 e 2012 foram revisados e variáveis epidemiológicas, clínicas e radiológicas foram compiladas. Resultados O diagnóstico mais prevalente foi o de degeneração combinada subaguda (11,4%), seguido por mielopatia espondilótica cervical (9,6%), doenças desmielinizantes (9%), paraparesia espástica tropical (8,4%) e paraparesia espástica hereditária (8.4%). Aproximadamente 20% dos pacientes apresentaram mielopatia não-traumática de etiologia indeterminada, apesar da ampla investigação clínica, laboratorial e de neuroimagem. Conclusão Independentemente da ampla avaliação, muitos pacientes com mielopatia não-traumática apresentam etiologia incerta. Causas compressivas e deficiências nutricionais permanecem como etiologias importantes de mielopatias não-traumáticas.

Espondilodiscitis con absceso intrarraquídeo y paraespinal causada por bacilo de Calmette-Guérin (BCG) / Spondilodyscitis with medullary and spinal abscess caused by Bacillus Calmette-Guérin (BCG)

Intravesical therapy with live-attenuated Mycobacterium bovis strain have demonstrated to be effective in the treatment of recurrent and high-grade superficial bladder tumors. The use of this therapy is widely extended; however spreading of bacillus from the injection site could be one rare complication that may cause infection in different locations. An appropriate anamnesis is very important to establish an etiological diagnostic of possible infections caused by M. bovis BCG. Laboratory diagnosis at species level is difficult because of the high genetic similarity (99.9%) with the other member of Mycobacterium tuberculosis complex. We present a case report who developed tuberculous spondylodiscitis by M. bovis BCG, which had a history of intravesical instillation for treatment of bladder cancer.

Las instilaciones intravesicales con la cepa viva atenuada de Mycobacterium bovis han demostrado su eficacia en el tratamiento de cáncer urotelial de vejiga. Su uso está ampliamente difundido; sin embargo, una reacción adversa infrecuente es la extravasación del bacilo del lugar de acción pudiendo causar infecciones en otras localizaciones. Una correcta anamnesis del paciente ayuda a orientar la etiología de posibles infecciones relacionadas con éste microorganismo. El diagnóstico de laboratorio a nivel de especie es dificultoso ya que comparte un 99,9% de identidad genética con los otros miembros del complejo Mycobacterium tuberculosis. Se presenta el caso de un paciente que desarrolló una espondilodiscitis tuberculosa por M. bovis BCG, el cual tenía antecedentes de instilaciones intravesicales para el tratamiento del cáncer de vejiga.

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.

Spinal Dural Arteriovenous Fistula: Imaging Features and Its Mimics

Spinal dural arteriovenous fistula (SDAVF) is the most common spinal vascular malformation, however it is still rare and underdiagnosed. Magnetic resonance imaging findings such as spinal cord edema and dilated and tortuous perimedullary veins play a pivotal role in the confirmation of the diagnosis. However, spinal angiography remains the gold standard in the diagnosis of SDAVF. Classic angiographic findings of SDAVF are early filling of radicular veins, delayed venous return, and an extensive network of dilated perimedullary venous plexus. A series of angiograms of SDAVF at different locations along the spinal column, and mimics of serpentine perimedullary venous plexus on MR images, are demonstrated. Thorough knowledge of SDAVF aids correct diagnosis and prevents irreversible complications.

Accuracy of Diffusion Tensor Imaging for Diagnosing Cervical Spondylotic Myelopathy in Patients Showing Spinal Cord Compression

OBJECTIVE: To assess the performance of diffusion tensor imaging (DTI) for the diagnosis of cervical spondylotic myelopathy (CSM) in patients with deformed spinal cord but otherwise unremarkable conventional magnetic resonance imaging (MRI) findings. MATERIALS AND METHODS: A total of 33 patients who underwent MRI of the cervical spine including DTI using two-dimensional single-shot interleaved multi-section inner volume diffusion-weighted echo-planar imaging and whose spinal cords were deformed but showed no signal changes on conventional MRI were the subjects of this study. Mean diffusivity (MD), longitudinal diffusivity (LD), radial diffusivity (RD), and fractional anisotropy (FA) were measured at the most stenotic level. The calculated performance of MD, FA, MD∩FA (considered positive when both the MD and FA results were positive), LD∩FA (considered positive when both the LD and FA results were positive), and RD∩FA (considered positive when both the RD and FA results were positive) in diagnosing CSM were compared with each other based on the estimated cut-off values of MD, LD, RD, and FA from receiver operating characteristic curve analysis with the clinical diagnosis of CSM from medical records as the reference standard. RESULTS: The MD, LD, and RD cut-off values were 1.079 × 10⁻³, 1.719 × 10⁻³, and 0.749 × 10⁻³ mm²/sec, respectively, and that of FA was 0.475. Sensitivity, specificity, positive predictive value and negative predictive value were: 100 (4/4), 44.8 (13/29), 20 (4/20), and 100 (13/13) for MD; 100 (4/4), 27.6 (8/29), 16 (4/25), and 100 (8/8) for FA; 100 (4/4), 58.6 (17/29), 25 (4/16), and 100 (17/17) for MD∩FA; 100 (4/4), 68.9 (20/29), 30.8 (4/13), and 100 (20/20) for LD∩FA; and 75 (3/4), 68.9 (20/29), 25 (3/12), and 95.2 (20/21) for RD∩FA in percentage value. Diagnostic performance comparisons revealed significant differences only in specificity between FA and MD∩FA (p = 0.003), FA and LD∩FA (p < 0.001), FA and RD∩FA (p < 0.001), MD and LD∩FA (p = 0.024) and MD and RD∩FA (p = 0.024). CONCLUSION: Fractional anisotropy combined with MD, RD, or LD is expected to be more useful than FA and MD for diagnosing CSM in patients who show deformed spinal cords without signal changes on MRI.

Mielopatia por deficiência de cobalamina: resolução clínica e de imagem / Cobalamin deficiency myelopathy: clinical and imaging resolution

Embora o quadro clássico de mielopatia por deficiência de vitaminaB12 seja a degeneração subaguda combinada da medula, a manifestaçãoclínica pode ser variável. Homem branco de 36 anos de idade com hipotireoidismo e vitiligo apresentou dormência nas mãos de início súbito. Exame físico: sinal de Lhermitte e hipoestesia nas palmas. Evidenciada alteração de sinal na ressonância magnética (RM) da medula cervical. Foram evidenciados nível sérico de vitamina B12 de 150 pg/mL, gastrite atrófica e hemograma normal. Paciente foi tratado com reposição intramuscular de vitamina B12. Após seis meses, houve remissão completa dos sintomas com normalização do exame de imagem em um ano. O presente caso ilustra discreta alteração clínica e lesão extensa na RM (dissociação entre a clínica e o exame de imagem) na deficiência de B12. A melhora dos sintomas precedeu a resolução da alteração no exame de imagem, no presente caso.

Although the classic manifestation of myelopathy due to vitamin B12deficiency is a subacute combined degeneration of the spinal cord, the clinical manifestation may be varied. A 36-year-old white man with hypothyroidism and vitiligo presented sudden onset of numbness in hands. Physical examination: Lhermitte's sign and hypoesthesia in palms. Signal change on magnetic resonance image (MRI) of the cervical spinal cord was evidenced. Serum vitamin B12 of 150 pg/mL, gastric atrophy and normal hemogram were shown. The patient was treated with intramuscular vitamin B12 replacement. After six months there was complete remission of the symptoms, and within one year the MRI was normal. This case illustrates mild clinical signs and extensive changes on MRI (dissociation between clinic and image) in B12 deficiency. Resolution of MRI was observed after the clinical signs, in the present case.

Mansonic neuroschistosomiasis / Neuroesquistossome mansonica

Mansonic neuroschistosomiasis (MN) is not only the most common but also the most serious ectopic presentation of the infection by Schistosoma mansoni. Both, brain and spinal cord can be independently affected by the infection, but the later is more frequently affected. Brain MN by itself is due to the presence of eggs and/or adult worms in situ and can be symptomatic or asymptomatic. Unlike the brain MN, spinal cord mansonic neuroschistosomiasis is more frequently symptomatic. In both forms the intensity, the seriousness and also the clinical characteristics of signs and symptoms depend on the amount of eggs in the compromised region and on the intensity of the inflammatory reaction surrounding the eggs. Cerebrospinal fluid examination and magnetic resonance imaging are important diagnostic tools. Both corticosteroids and drugs against S. mansoni are used in the treatment. The outcome may largely depend upon the prompt use of these drugs.

A neuroesquistossome mansônica (NM) é não apenas a mais comum, mas também a mais grave apresentação da infecção pelo Schistosoma mansoni. Tanto o encéfalo quanto a medula podem ser independentemente afetadas pela doença, embora a última o seja de forma mais frequente. A NM encefálica é secundária à presença dos ovos e/ou da forma adulta do verme in situ, e pode ser sintomática ou não. Ao contrário da forma encefálica, a NM medular é mais frequentemente sintomática. Em ambas as formas a gravidade dos sintomas dependerá na quantidade de ovos na região comprometida e na intensidade da reação inflamatória ao seu redor. Os exames do líquido cefalorraquiano e de imagem por ressonância magnética são importantes ferramentas diagnósticas. Corticosteróides e drogas parasiticidas são usadas no tratamento desta doença, e seu prognóstico dependerá diretamente do rápido uso destas drogas.

Perfil de ativação dos linfócitos T de pacientes com mielopatia associada ao vírus linfotrópico das células T humanas do tipo 1 (HTLV-1) / paraparesia tropical espástica (HAM/TSP) possível, provável e definido / Activation profile of T lymphocytes of patients with myelopathy associated with human T-lymphotropic virus type 1 cells (HTLV-1) tropical spastic paraparesis (HAM / TSP) possible, probable and definite

O vírus linfotrópico das células T humanas do tipo 1 (HTLV-1) é o agente etiológico da mielopatia associada ao HTLV / paraparesia espástica tropical (HAM / TSP ), que ocorre em menos de 5 % dos indivíduos infectados. A resposta imune controla parcialmente a infecção, porém pode estar ligada a patogênese da doença. O objetivo deste estudo foi caracterizar fenotipicamente as subpopulações de linfócitos T, em pacientes assintomáticos e com diagnóstico de HAM/TSP. Foram avaliados 103 pacientes acompanhados no Centro de HTLV da Escola Bahiana de Medicina e Saúde Pública (EBMSP) e 19 controles não infectados. Os pacientes foram categorizados de acordo com o grau de certeza do diagnóstico de HAM/TSP: possível (Ps), provável (Pb) e definido (D), além de pacientes assintomáticos (ASS). O perfil fenotípico (CD25, CD45RA, CD45RO, HLA-DR, CD25, CCR-7, CD62L)...

The human T-cell lymphotropic vírus type 1(HTLV-1) is the etiological agent of HTLV- associated myelopathy/ Tropical spastic paraparesis(HAM/TSP), wich occurs in less then 5% of the infected individuals. The immune response partially controls the infection, but may be linked to the pathogenesis of disease. The aim of this study was to characterize phenotipically T lymphocyte subpopulations in asymptomatic and in patients diagnosed with HAM/TSP. We evaluated 103 patients treated at the center for HTLV of Bahia School of Medicine and Public Health (EBMSP) and 19 uninfected controls. Patients were categorized as asymptomatic and according to the degree of certainty of the diagnosis of HAM/TSP: Possible(Ps), Probable(Pb) and Definite(D). The phenotypic profile (CD25, CD45RA, CD45RO, HLA-DR, CCR-7, CD62L)...

Mielopatia sinalizando o diagnóstico tardio da infecçãopor HtlV: um relato de caso / Myelopathy signaling the late diagnosis of HIV infection: a case report

A infecção pelo vírus T-linfotrópico humano (HTLV) caracteriza-se como uma doença sexualmente transmissível (DST), que pode também ser adquirida pelas vias parenteral e vertical. Subdivide-se em dois tipos: o HTLV-I, relacionado com doenças como mielopatia associada a HTLV/paraparesia espástica tropical (HAM/TSP) e a leucemia/linfoma de células T do adulto (ATL). Já o HTLV-II ainda não foi correlacionado cientificamente com nenhuma patologia na atualidade. Seu diagnóstico é realizado pela triagem sorológica para a detecção de anticorpo anti-HTLV-I/II, sendo o exame confirmatório o western blot. Neste contexto, o objetivo do presente estudo foi descrever um relato de caso em que a mielopatia foi a manifestação clínica sinalizadora da infecção pelo HTLV, em consequência do diagnóstico tardio da infecção por este patógeno, na qual a paciente apresentou os sintomas, progrediu lentamente e recebeu o diagnóstico apenas no último estágio da patologia (HAM/TSP), quando se tornou cadeirante. Embora a paciente realize na atualidade a terapêutica proposta e o acompanhamento ambulatorial segundo o protocolo estabelecido para o manejo desta infecção viral, membros de sua família também foram avaliados e diagnosticados e apenas um se apresentou positivo para a infecção. Este estudo visa demonstrar a importância do rastreio laboratorial para a infecção pelo HTLV, na mesma dimensão do diagnóstico da sífilis e do HIV, de modo que o mesmo não ocorra de forma tardia, quando associado a suas manifestações clínicas nos pacientes ou a infecções oportunistas relacionadas.

The human T-lymphotropic virus (HTLV) is characterized as a sexually transmitted disease (STD), it can also be transmitted by parenteral and vertical routes. It is subdivided into two types: the HTLV-I related diseases such as myelopathy/tropical spastic paraparesis (HAM/TSP) and adult T-cell leukemia/lymphoma (ATL). HTLV-II has not been scientifically correlated with pathology yet. This diagnosis is made by serological screening for detection of HTLV antibody, and the western blot confirmatory test. In this context, the objective of this study was to describe a case in which myelopathy was signaling to the clinical manifestation of HTLV, as a result of delayed diagnosis of infection by this pathogen in which the patient had symptoms progressed slowly and received diagnosis only in the last stage of pathology (HAM/TSP), becoming a wheelchair user. Although nowadays the patient performs the therapeutic proposed and outpatient treatment according to the established protocol for the management of this viral infection, members of her family were also diagnosed and only one had a positive diagnosis of infection. This study aims to demonstrate the importance of laboratory screening for HTLV infection, in the same dimension of the diagnosis of syphilis and HIV, so that it does not occur so late, when it is associated to clinical manifestations in patients or related opportunistic infections

Severity score system for progressive myelopathy: development and validation of a new clinical scale

Progressive myelopathies can be secondary to inborn errors of metabolism (IEM) such as mucopolysaccharidosis, mucolipidosis, and adrenomyeloneuropathy. The available scale, Japanese Orthopaedic Association (JOA) score, was validated only for degenerative vertebral diseases. Our objective is to propose and validate a new scale addressing progressive myelopathies and to present validating data for JOA in these diseases. A new scale, Severity Score System for Progressive Myelopathy (SSPROM), covering motor disability, sphincter dysfunction, spasticity, and sensory losses. Inter- and intra-rater reliabilities were measured. External validation was tested by applying JOA, the Expanded Disability Status Scale (EDSS), the Barthel index, and the Osame Motor Disability Score. Thirty-eight patients, 17 with adrenomyeloneuropathy, 3 with mucopolysaccharidosis I, 3 with mucopolysaccharidosis IV, 2 with mucopolysaccharidosis VI, 2 with mucolipidosis, and 11 with human T-cell lymphotropic virus type-1 (HTLV-1)-associated myelopathy participated in the study. The mean ± SD SSPROM and JOA scores were 74.6 ± 11.4 and 12.4 ± 2.3, respectively. Construct validity for SSPROM (JOA: r = 0.84, P < 0.0001; EDSS: r = -0.83, P < 0.0001; Barthel: r = 0.56, P < 0.002; Osame: r = -0.94, P < 0.0001) and reliability (intra-rater: r = 0.83, P < 0.0001; inter-rater: r = 0.94, P < 0.0001) were demonstrated. The metric properties of JOA were similar to those found in SSPROM. Several clinimetric requirements were met for both SSPROM and JOA scales. Since SSPROM has a wider range, it should be useful for follow-up studies on IEM myelopathies.

Ventriculus Terminalis in Adults: Unusual Magnetic Resonance Imaging Features and Review of the Literature

OBJECTIVE: The ventriculus terminalis (VT) in adults is a rare pathology. We report various MR imaging features of the adult VT. MATERIALS AND METHODS: Ten patients were included in this retrospective review.. All patients had undergone magnetic resonance (MR imaging with a surface coil that used two different 1.5T MR systems. All patients had undergone initial and follow-up MR imaging with contrast enhancement using gadopentate dimeglumine. Three patients underwent additional MR imaging using the echocardiogram-gated spatial modulation of magnetization (SPAMM) technique. If a shift in tagging band during the systolic phase was less than half of the band space, it was defined as a "non-pulsatile fluid". Two neuroradiologists independently reviewed these images, while clinical symptoms and outcomes were statistically analyzed between the treated and non-treated group. RESULTS: All cases presented an intramedullary cystic lesion in the conus medullaris and showed the same signal intensity as CSF. Three VTs had intracystic septation and cord edema, which were pathologically confirmed after surgery; two of these were associated with kyphotic deformity and spinal arteriovenous malformation. SPAMM-MRI of 3 patients demonstrated non-pulsatile fluid motion within the VT. In the treated group, clinical symptoms improved better than the non-treated group. CONCLUSION: The adult VT shows some unusual imaging features, including septation, cord edema, and coexistence of a spinal AVM, as well as the typical findings. Surgical maneuvers may be considered as a treatment option in adult VT with progressive neurological symptoms.