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.

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.

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.

Percutaneous transplantation of human umbilical cord-derived mesenchymal stem cells in a dog suspected to have fibrocartilaginous embolic myelopathy

The use of human umbilical cord blood-derived mesenchymal stem cells for cell transplantation therapy holds great promise for repairing spinal cord injury. Here we report the first clinical trial transplantation of human umbilical cord (hUCB)-derived mesenchymal stem cells (MSCs) into the spinal cord of a dog suspected to have fibrocartilaginous embolic myelopathy (FCEM) and that experienced a loss of deep pain sensation. Locomotor functions improved following transplantation in a dog. Based on our findings, we suggest that transplantation of hUCB-derived MSCs will have beneficial therapeutic effects on FCEM patients lacking deep pain sensation.

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.

Mielopatia torácica por calcificação do ligamento amarelo cursando com hiperproteinorraquia e resposta à corticoterapia: relato de caso / Thoracic myelopathy due to calcification of the ligamentum flavum with hyperproteinorachia and responsive to steroid therapy: case report

Calcificação e ossificação do ligamento amarelo ou do ligamento longitudinal posterior são causas de mielopatia compressiva, mais frequentes nos níveis torácicos inferiores e bastante raras em populações ocidentais. A descompressão cirúrgica é a única terapia proposta, mas a doença costuma ser progressiva e sua recorrência após a cirurgia não é incomum. Mediadores inflamatórios podem ter algum papel na progressão da mielopatia compressiva, mas não se tem notícia de qualquer proposta de abordagem terapêutica envolvendo agentes anti-inflamatórios. Neste contexto, relatamos um caso de mielopatia compressiva por calcificação do ligamento amarelo em que se observou hiperproteinorraquia e resposta à corticoterapia. Tais informações são inéditas e podem fornecer novas ideias para a compreensão da doença.

Calcification and ossification of the ligamentum flavum or of the posterior longitudinal ligament are causes of compressive myelopathy, more frequent in the lower thoracic levels, and extremely rare in Western populations. Surgical decompression is the only therapy, but the disease is usually progressive, and its recurrence after surgery is common. Inflammatory mediators might play a role in the progression of compressive myelopathy, but, to our knowledge, the therapeutic approach involving anti-inflammatory agents has never been tried before. We report a case of compressive myelopathy due to calcification of the ligamentum flavum, in which hyperproteinorachia and response to steroid therapy have been observed. Those data have not been published before and might provide new ideas for the disease understanding.

Lesiones medulares no traumáticas: etiología, demografía y clínica / Nontraumatic spinal cord injury: etiology, demography and clinics

Se realizó un estudio retrospectivo y descriptivo en 210 pacientes hospitalizados con lesión medular del Instituto Nacional de Rehabilitación (INR), Callao-Perú (2000-2006) para conocer la etiología, características clínicas y socio-demográficas de los pacientes con lesiones medulares no traumáticas (LMNT). Se encontró una prevalencia de 27 por ciento para LMNT siendo la edad promedio al inicio de la lesión 32,0 años; siendo varones el 50,5 por ciento, y el 41,9 por ciento tuvieron educación secundaría, el nivel de pobreza alcanzó el 90,5 por ciento. Predominó la etiología infecciosa (viral y bacteriana) en 37,6 por ciento, con un 11,9 por ciento de infección por HTLVI. No obstante ser el INR un centro de referencia, nuestros resultados son preliminares, siendo necesario realizar mayores estudios para proponer estrategias de prevención y control, dado el alto costo del tratamiento integral de rehabilitación en estos pacientes.

We performed a retrospective and descriptive cross-sectional; study in 210 hospitalized patients with spinal cord injury at the National Institute of Rehabilitation (INR), Callao, Peru from 2000-2006. The goal was to describe etiology, and clinical and socio-demographic characteristics of non traumatic spinal cord injuries (LMNT). We found a prevalence of 27 percent for LMNT, average age at onset of 32.0 years, male gender 50.5 percent, and secondary education completed in 41.9 percent, poverty 90.5 percent. The infectious etiology (viral and bacterial) was predominant in 37.6 percent, with 11.9 infected with HTLVI. Although the INR is a reference center, the findings can’t be generalized because it isn’t a representative sample of the Peruvian population, further studies are necessary to propose strategies for prevention and control, considering the high cost of integral rehabilitation treatment in these patients.

Hematoma neuroaxial após bloqueio peridural: é possível prevenir ou detectar?: relato de dois casos / Neuraxial hematoma after epidural anesthesia: is it possible to prevent or detect it?: report of two cases

JUSTIFICATIVA E OBJETIVOS: Os hematomas espinais são raros e acometem o sistema nervoso central. Podem causar sequelas neurológicas permanentes e morte se não tratados adequadamente. O diagnóstico e tratamento precoces são fundamentais para o bom prognóstico neurológico. O objetivo deste trabalho foi despertar no anestesiologista maior sensibilidade para o diagnóstico e o tratamento precoces dos hematomas espinais, além de aperfeiçoar sua prevenção. RELATO DOS CASOS: Caso 1: Paciente submetido à anestesia peridural lombar para a realização de revascularização fêmuro-poplítea. Estava em uso de ácido acetilsalicílico, clopidogrel e enoxaparina, medicamentos suspensos antes da operação. O paciente evoluiu com paraplegia no pós-operatório imediato. Realizou-se descompressão neurocirúrgica após o diagnóstico, porém sem recuperação do quadro em longo prazo. Caso 2: Paciente submetido à anestesia peridural lombar para osteotomia em joelho direito, sem intercorrências. O paciente permaneceu sem queixas neurológicas até aproximadamente 48 horas, quando iniciou quadro de retenção urinária, dor em membro inferior direito, parestesias e dificuldade de movimentação dos pés. Foi feita ressonância nuclear magnética, a qual evidenciou hematoma peridural lombar, sendo realizada a descompressão cirúrgica imediata. Após 10 meses de reabilitação, houve recuperação neurológica completa. CONCLUSÕES: Os casos clínicos apresentados mostraram desfechos diferentes, destacando a importância do diagnóstico e do tratamento precoces para a boa evolução do quadro clínico. O diagnóstico pela ressonância nuclear magnética, com a descompressão precoce logo após as primeiras manifestações clínicas, permanece como tratamento padrão. A identificação dos pacientes de risco para sangramentos neuroaxiais, a mudança de técnica anestésica, assim como o estabelecimento de protocolos de avaliação neurológica pós-operatória nos pacientes submetidos aos bloqueios de neuroeixo...

BACKGROUND AND OBJECTIVES: Spinal hematomas are rare and they affect the central nervous system. They can cause permanent neurologic sequelae and death if they are not treated properly. Early diagnosis and treatment are fundamental for a good neurologic prognostic. The objective of this report was to emphasize for anesthesiologists the importance of early diagnosis and treatment of spinal hematomas, besides improving their prevention. CASE REPORTS: Case 1: The patient underwent epidural lumbar anesthesia for femoropopliteal revascularization. He was being treated with acetylsalicylic acid, clopidogrel, and enoxaparin, which were discontinued before the surgery. The patient developed paraplegia in the immediate postoperative period. Neurosurgical decompression was performed after the diagnosis, but without recovery in the long run. Case 2: The patient underwent epidural lumbar anesthesia for right knee osteotomy, without intercurrences. The patient remained without neurological complaints until approximately 48 hours after the surgery when he developed urinary retention, pain in the right lower limb, paresthesias, and difficulty moving both feet. The MRI showed an epidural lumbar hematoma, and the patient underwent immediate surgical decompression. He showed complete neurological recovery after 10 months of rehabilitation. CONCLUSIONS: The clinical cases presented here showed different outcomes, indicating the importance of early diagnosis and treatment for a good evolution. Diagnosis by MRI with early decompression shortly after the development of the first clinical manifestations remains the standard treatment. Identification of patients at risk for neuraxial bleeding and change in anesthetic technique, as well as the establishment of postoperative neurologic evaluation protocols in patients undergoing neuraxial anesthesia can contribute for prevention of severe neurologic sequelae.

JUSTIFICATIVA Y OBJETIVOS: Los hematomas espinales son raros y acometen el sistema nervioso central. Pueden causar secuelas neurológicas permanentes e incluso la muerte si no se tratan adecuadamente. El diagnóstico y el tratamiento precoces son elementos fundamentales para el buen pronóstico neurológico. El objetivo de este trabajo fue despertar en el anestesiólogo una mayor sensibilidad hacia el diagnóstico y el tratamiento precoces de los hematomas espinales, además de perfeccionar su prevención. RELATO DE LOS CASOS: Caso 1: Paciente que fue sometido a la anestesia epidural lumbar para la realización de la revascularización femoro-poplítea. Estaba usando ácido acetilsalicílico, clopidogrel y enoxaparina, medicamentos que fueron suspendidos antes de la operación. El paciente evolucionó con paraplejia en el postoperatorio inmediato. Se realizó la descompresión neuro-quirúrgica posterior al diagnóstico, pero sin la recuperación del cuadro a largo plazo. Caso 2: Paciente que fue sometido a la anestesia epidural lumbar para la osteotomía en la rodilla derecha, sin intercurrencias. El paciente no presentó quejas neurológicas durante aproximadamente 48 horas, cuando inició el cuadro de retención urinaria, dolor en el miembro inferior derecho, parestesias y dificultad para mover los pies. Se realizó la resonancia nuclear magnética, que arrojó hematoma epidural lumbar, siendo realizada la descompresión quirúrgica inmediata. Después de 10 meses de rehabilitación, se verificó una recuperación neurológica completa. CONCLUSIONES: Los casos clínicos presentados aquí, arrojaron diferentes desenlaces, destacando la importancia del diagnóstico y del tratamiento precoces para una buena evolución del cuadro clínico. El diagnóstico por la resonancia nuclear magnética, con la rápida descompresión inmediatamente después de las primeras manifestaciones clínicas, permanece como un tratamiento estándar. La identificación de los pacientes de riesgo para sangramientos neuroaxiales...

Histiocitose de células de Langerhans como causa de mielopatia: relato de caso e revisão da literatura / Langerhans-cells histiocytosis as a cause of myelopathy: case report and literature review

Histiocitose de células de Langerhans é uma doença uni ou multifocal que acomete osso, tecido mole ou ambos. Relativamente incomum, abrange entidades clínicas anteriormente conhecidas como doença de Hand-Schüller-Christian, Abt-Letterer-Siwe, Hashimoto-Pritzker, granuloma eosinofílico e histiocitose X. Responsável por menos de 1% dos tumores ósseos, muito raramente leva a acometimento neurológico. Sua etiologia é desconhecida e seu tratamento, controverso. A ocorrência de um caso com comprometimento neurológico nos motivou a este relato. Paciente do sexo feminino, 4 anos deidade, com processo expansivo de C7 a T3 e paraparesia crural; após a cirurgia apresentou melhora importante já no segundo pós-operatório. Encontra-se em terapia complementar com corticoide e quimioterapia estando no nono mês de tratamento. Na revisão da literatura foram encontrados poucos relatos de manejo cirúrgico desse tipo de lesão, em razão de sua característica de doença autolimitada. O tratamento adequado pode variar dependendo das características do paciente e de seu comprometimento neurológico.

Langerhans cell histiocytosis is a uni- or multifocal disease that affects bone, soft tissue, or both. Relatively uncommon, covers clinical entities previously known as Hand-Schüller-Christian disease,Abt-Letterer-Siwe, Hashimoto-Pritzker, eosinophilic granuloma and histiocytosis X. Responsible forless than 1% of bone tumors, rarely causes neurological impairment. Its etiology is unknown and the treatment controversial. We report a rare case with neurological impairment. A 4 year-old female children, presented with paraparesis due to expansive process at C7 to T3; soon after surgery showed significant improvement in the second postoperative day; she is in complementary therapy with corticosteroids and chemotherapy in the ninth month of treatment. In the review of the literature we found few reports ofsurgical management of this type of injury, due to its characteristic of self-limited disease. The appropriate treatment may vary depending on the characteristics of patients and their neurological impairment.

Immune reconstitution inflammatory syndrome manifesting as disseminated tuberculosis, deep venous thrombosis, encephalopathy and myelopathy.

We describe an unusual case of a 25-year-old human immunodeficiency virus (HIV)-positive male with a pre-treatment CD4 count of 144 cells/microL, who had received highly active antiretroviral therapy (HAART) consisting of lamivudine, stavudine and nevirapine for three months, developing immune reconstitution inflammatory syndrome (IRIS) manifesting as disseminated tuberculosis (TB), myelopathy, encephalopathy and deep venous thrombosis (DVT). In addition to HAART and antituberculosis treatment (ATT), the patient was given non-steroidal anti-inflammatory drugs, oral vitamin B12 and heparin, which was later switched to oral warfarin.

Cytomegalovirus polyradiculomyelopathy in AIDS: a case report and review of the literature

Cytomegalovirus (CMV) neurologic disease is a serious complication of the acquired immunodeficiency virus (AIDS). We report the case of a 40 year-old woman with AIDS who presented paralysis of lower extremities, areflexia, sensory loss, and urinary retention. CMV polymerase chain reaction (PCR) of cerebrospinal fluid (CSF) allowed confirmation of CMV polyradiculomyelopathy (PRAM).

Hepatic myelopathy: a rare complication following extrahepatic portal vein occlusion and lienorenal shunt.

A 19-year-old gentleman presented with slowly progressive spastic paraparesis, 2 years after the therapeutic lienorenal shunt for portal hypertension secondary to cirrhosis and portal vein occlusion. After 2 years of initial evaluation, the motor functions had not worsened further. He did not have any obvious clinical or EEG features of hepatic encephalopathy. Other causes for myelopathy were ruled out. Contribution of portal vein occlusion to portosystemic shunting has not been reported previously in patients with 'hepatic myelopathy.' This uncommon complication needs to be considered in patients with shunt surgery for relieving portal hypertension.