Mielopatía aguda compresiva por absceso pulmonar. Presentación de un caso / Compressive acute myelopathy by pulmonary abscess. A case report

Las mielopatías constituyen un conjunto heterogéneo de procesos patológicos que directa o indirectamente afectan a la médula espinal. Son poco frecuentes y pueden ser producidos por variadas causas y vías de afectación, que conllevan a grandes secuelas y elevada discapacidad. Se describe un caso de mielopatía aguda compresiva desencadenada por un absceso pulmonar como causa y mecanismo de producción inusual. Hombre de 48 años, con antecedentes de salud y cuadro clínico de inicio brusco de paraparesia fláccida e hiporreflexia osteotendinosa de miembros inferiores asimétrica, con predominio derecho, nivel sensitivo dorsal a nivel de D4 y dolor a la percusión en procesos espinosos dorsales en D3-D6. Los estudios sanguíneos, inmunológicos y de líquido cefalorraquídeo resultaron irrelevantes, excepto muestra de signos indirectos de infección. La radiografía de tórax mostró la presencia del absceso pulmonar primario derecho y la afectación medular se corroboró a través de la realización de la resonancia magnética medular dorsal. Presentó pobre respuesta ante los esteroides parenteral, hasta que se inició la terapia antibiótica, de forma paulatina y con apoyo rehabilitador. Transcurridos tres meses se investigaron y fueron descartadas otras etiologías no compresivas. Las mielopatías agudas representan un grupo heterogéneo de trastornos con distintas etiologías y mecanismos de producción, a través de su estudio se evidenció como causa un absceso pulmonar con afectación medular, por lo que debe ser considerado en la extensa lista de diagnósticos diferenciales (AU).

Myelopathies are a heterogeneous group of pathological processes that directly or indirectly affect the spinal cord. They are rare and can be produced by different causes and affectation pathways leading to major consequences and high disability. A case of acute compressive myelopathy triggered by a lung abscess as unusual cause and production mechanism is described. It is the case of a man, aged 48 years, with a clinical history and symptoms of acute onset of flaccid paraparesis and asymmetric osteotendinous hyporeflexia of lower limbs, with right predominance, dorsal sensitive level at D4, and pain at percussion in D3-D6 dorsal spinal processes. Blood, immune and cerebrospinal fluid (CSF) studies were irrelevant, but showing indirect signs of infection. Chest radiography showed the presence of the right primary lung abscess and spinal cord involvement was confirmed by performing dorsal medullar magnetic resonance (MRI). The patient had poor response to parenteral steroids until antibiotic therapy began, gradually and with rehabilitation support. After three months, other non-comprehensive etiologies were studied and ruled out. Acute myelopathies represent a heterogeneous group of disorders with different etiologies and production mechanisms. The study evidenced a lung abscess with spinal cord involvement as a cause, so it should be considered in the extensive list of differential diagnoses (AU).

Cervical Myelopathy Caused by Engorgement of the Epidural Venous Plexus due to Cerebrospinal Fluid Overdrainage: Case Report and Review of the Literature

This is a case report of a 33-year-old woman with cervical myelopathy caused by an enlargement of the cervical venous plexus, after she was submitted to a ventriculoperitoneal (VP) shunt that evolved to overdrainage. Magnetic Resonance Imaging (MRI) revealed an epidural venous enlargement within the spinal channel, with a 50% narrowing from C2 to C5, and spinal cord compression. A shunt revision was performed using a programmable drainage system, and a second MRI revealed the absence of the venous enlargement, resulting in cervical spinal cord decompression and remission of neurological symptoms. Compressive myelopathy consequent to the enlargement of the epidural venous plexus related to the overdrainage of the ventriculoperitoneal shunt system without typical signs of intracranial hypotension may result in misleading etiological diagnoses. Acknowledging this disorder is important to distinguish it from neoplastic processes or hematomas, for which surgical intervention may be needed.

Este é um relato de caso de uma paciente de 33 anos de idade que apresentou mielopatia cervical devido a alargamento do plexo venoso cervical, após ser submetida a uma derivação ventriculoperitoneal que evoluiu com hiperdrenagem. Exame de Ressonância Nuclear Magnética (RNM) revelou alargamento do plexo venoso epidural no canal espinhal, com estreitamento de 50% em C2 a C5, e compressão da medula espinhal. Uma revisão da derivação foi feita com um sistema de drenagem programável, e uma segunda RNM revelou ausência de ingurgitamento venoso, resultando em descompressão medular e remissão dos sintomas neurológicos. Mielopatia compressiva consequente ao alargamento do plexo venoso epidural relacionado à hiperdrenagem do sistema de derivação ventriculoperitoneal sem sinais típicos de hipotensão intracraniana pode resultar em diagnósticos incorretos. O conhecimento dessa patologia é importante para distingui-la de processos neoplásicos ou hematomas, nos quais intervenções cirúrgicas podem ser necessárias.

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

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

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

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

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

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

Effect of melatonin on the functional recovery from experimental traumatic compression of the spinal cord

Spinal cord injury is an extremely severe condition with no available effective therapies. We examined the effect of melatonin on traumatic compression of the spinal cord. Sixty male adult Wistar rats were divided into three groups: sham-operated animals and animals with 35 and 50% spinal cord compression with a polycarbonate rod spacer. Each group was divided into two subgroups, each receiving an injection of vehicle or melatonin (2.5 mg/kg, intraperitoneal) 5 min prior to and 1, 2, 3, and 4 h after injury. Functional recovery was monitored weekly by the open-field test, the Basso, Beattie and Bresnahan locomotor scale and the inclined plane test. Histological changes of the spinal cord were examined 35 days after injury. Motor scores were progressively lower as spacer size increased according to the motor scale and inclined plane test evaluation at all times of assessment. The results of the two tests were correlated. The open-field test presented similar results with a less pronounced difference between the 35 and 50% compression groups. The injured groups presented functional recovery that was more evident in the first and second weeks. Animals receiving melatonin treatment presented more pronounced functional recovery than vehicle-treated animals as measured by the motor scale or inclined plane. NADPH-d histochemistry revealed integrity of the spinal cord thoracic segment in sham-operated animals and confirmed the severity of the lesion after spinal cord narrowing. The results obtained after experimental compression of the spinal cord support the hypothesis that melatonin may be considered for use in clinical practice because of its protective effect on the secondary wave of neuronal death following the primary wave after spinal cord injury.

Mal de Pott: uma doença milenar em um novo século / Pott's disease: an ancient disease in a new century

A tuberculose espinhal, também conhecida como Mal de Pott ou Doença de Pott, caracteriza-se como a forma mais frequente de tuberculose extrapulmonar. Apresentamos aqui o caso de uma paciente encaminhada ao Serviço de Neurologia do Hospital de Clínicas de Porto Alegre com um quadro de dois meses de evolução, apresentando sintomas de compressão medular. O diagnóstico de tuberculose espinhal foi realizado por punção guiada por tomografia computadorizada e a paciente foi submetida à drenagem do abscesso. Concomitantemente, foi iniciado o tratamento com RHZ e a paciente evoluiu com melhora dos sintomas neurológicos. Esse caso ilustra que mesmo pacientes com alterações neurológicas importantes devido à tuberculose medular podem apresentar melhora significativa com tratamento.

Spinal tuberculosis, also known as Pott's disease, is the most common form of extra-pulmonary tuberculosis. We report on a patient referred to the Division of Neurology of Hospital de Clínicas de Porto Alegre presenting with spinal cord compression symptoms for two months. The diagnosis of spinal tuberculosis was rapidly done by a computerized tomography guided biopsy. The patient was submitted to abscess surgical draining and complementary RHZ treatment, with recovery of neurological symptoms. This case illustrates that even patients with severe neurological deficits due to spinal tuberculosis may have a good outcome with the appropriate treatment.

Spinal Cord Tumors of the Thoracolumbar Junction Requiring Surgery: A Retrospective Review of Clinical Features and Surgical Outcome

PURPOSE: A retrospective review of medical records and imaging studies. To investigate characteristic clinical features and surgical outcomes of spinal cord tumors (SCTs) of the thoracolumbar junction (TLJ). The spinal cord transitions to the cauda equina in the TLJ. The TLJ contains the upper and lower motor neurons of the spinal cord and cauda equina. As a result, the clinical features of lesions in the TLJ vary, and these anatomical characteristics may affect surgical outcome. MATERIALS AND METHODS: Pathological diagnosis, clinical features, neurological signs, and surgical outcomes were investigated in 76 patients surgically treated at our institute for SCTs arising from T11 to L2. The patients were divided into epiconus (T11-12, n=18) and conus groups (L1-2, n=58). RESULTS: Patients in the epiconus group had hyperactive deep tendon reflexes (DTRs), while those in the conus group had hypoactive DTRs (p < 0.05). Nine patients were misdiagnosed with intervertebral disc diseases (IVDs) before correct diagnoses were made. It was impossible to definitively determine the exact cause of symptoms in four patients who had both SCTs and IVDs. CONCLUSION: Among SCTs of the TLJ, the epiconus group displayed upper motor neuron syndrome and the conus group displayed lower motor neuron syndrome. SCTs of the TLJ were frequently misdiagnosed as IVDs due to symptomatic similarities. SCTs of the TLJ should be included in differential diagnosis of back and leg pain, and it is highly recommended that routine lumbar magnetic resonance imaging include the TLJ.

Hemodynamic parameters and neurogenic pulmonary edema following spinal cord injury: an experimental model

Edema pulmonar neurogênico é complicação séria e aumenta o risco de vida em pacientes com várias lesões do sistema nervoso central. Apresentamos uma experiência com 58 ratos Wistar machos e adultos. Foram formados dois grupos: controle (n=4) e experimental (n=54). O grupo experimental sofreu trauma raquimedular torácico médio com o cateter-balão de Fogarty contendo 20µL de salina por 5, 15, 30 ou 60 segundos de compressão. Os ratos foram anestesiados com pentobarbital sódico (p.s.), 60 mg/Kg intraperitoneal (i.p.). Foi investigada a relação entre a lesão medular e o tempo de compressão. A evolução neurológica foi quantificada e apresentada com 4, 24 e 48 horas da compressão para caracterizar a graduação da lesão nos diferentes grupos. A pior evolução ocorreu com 60 segundos de compressão. Seis animais morreram subitamente com edema pulmonar. Vinte ratos foram randomicamente distribuídos em um dos seguintes grupos: controle (1, n=4, anestesiados com p.s. i.p., 60 mg/Kg, mas sem compressão) e experimental (2, n=7, anestesiados com xilasina 10 mg/Kg e ketamina 75 mg/Kg) e (3, n=9, anestesiados com p.s. i.p., 60 mg/Kg). O índice pulmonar (100 x peso pulmonar / peso corporal) foi 0,395 ± 0,018 no grupo controle, 0,499 ± 0,060 no grupo 2, e 0,639 ± 0,14 no grupo 3. O exame histológico da medula espinhal mostrou rupturas no parênquima e hemorragia aguda. Comparando-se o índice pulmonar com o índice morfométrico através da microscopia óptica, evidenciou-se que ocorreu edema intra-alveolar relevante para índices acima de 0,55. A presente experiência sugere que o edema pulmonar induzido pela compressão medular é de natureza neurogênica e que o tipo de anestesia utilizada no experimento poderia ter participação na gênesis do edema pulmonar.

Ventral extradural spinal meningeal cyst causing cord compression: neurosurgical treatment

Spinal extradural meningeal cysts are typically formed by a thin fibrotic membranous capsule, macroscopically similar that of an arachnoid membrane, filled by cerebro spinal fluid and related to a nerve root or to the posterior midline. Ventral location is extremely rare and when it occurs they usually cause spinal cord herniation through the ventral dural gap. A 61 year-old man who began with a two years long history of insidious tetraparesis, spasticity and hyperreflexia in lower extremities, and flaccid atrophy of upper limbs, without sensory manifestations, is presented. Investigation through magnetic resonance imaging demonstrated an extensive spinal ventral extradural cystic collection from C6 to T11. The lesion was approached through a laminectomy and a cyst-peritoneal shunt was introduced. The cyst reduced in size significantly and the patient is asymptomatic over a 48 months follow-up. This is the first reported case of a spontaneous ventral extradural spinal meningeal cyst causing cord compression. Cyst-peritoneal shunt was effective in the treatment of the case and it should be considered in cases in which complete resection of the cyst is made more difficult or risky by the need of more aggressive surgical maneuvers.

Cistos meníngeos extradurais espinhais são formados tipicamente por estreita cápsula membranosa fibrótica, macroscopicamente semelhante a uma membrana de aracnóide, repleta de líquor e relacionada com uma raiz nervosa ou com a linha média posterior. Eles são extremamente raros em posição anterior e, quando ocorrem, habitualmente causam herniação da medula espinhal pela falha dural ventral. O caso de um homem de 61 anos de idade que iniciou com tetraparesia, espasticidade e hiperreflexia em membros inferiores, e flacidez com hipotrofia nos membros superiores, sem manifestação sensitiva, é apresentado. A investigação com ressonância magnética demonstrou extensa coleção cística extradural ventral à medula de C6 a T11. A lesão foi abordada diretamente via laminectomia com introdução de derivação cisto-peritoneal, reduzindo o cisto e tornando o paciente assintomático com um seguimento de 48 meses. Este é o primeiro caso relatado de cisto meníngeo extradural ventral espontâneo causando compressão medular. A derivação cisto-peritoneal se mostrou eficaz no tratamento do caso e deve ser considerada em situações em que a ressecção completa do cisto esteja impossibilitada, ou dificultada pela necessidade de manobras cirúrgicas mais agressivas e arriscadas.

Lift-up laminoplasty for myelopathy caused by ossification of the posterior longitudinal ligament of the cervical spine.

BACKGROUND AND AIMS: We have utilized lift-up laminoplasty to treat patients with myelopathy caused by ossification of the posterior longitudinal ligament (OPLL) of the cervical spine. The preliminary surgical outcome with computer-assisted morphological assessment is presented. MATERIAL AND METHODS: The surgical technique of lift-up laminoplasty includes standard posterior exposure of the cervical spine, en-bloc laminectomy, and expansion of the cervical canal by lift-up of the laminae with custom-designed hydroxyapatite laminar spacers and stabilization of the laminae using titanium miniplates. From 1998 to 2003, 10 consecutive patients with cervical myelopathy secondary to OPLL have been treated with this method and comprehensively evaluated. Care was taken to tailor the treatment to individual patients by using different sizes of spacers to adjust the degree of expansion depending on the amount of stenosis of the cervical spine. The degree of expansion of the cervical canal was altered by design, based on the preoperative imaging simulation. RESULTS: Preliminary surgical outcome, evaluated at 6 months after surgery, revealed a significant improvement of neurological function. Image analysis revealed that the cervical canals were significantly expanded, with a mean reduction of 13.1% in the stenosis ratio. Lift-up laminoplasty was effective in the treatment of patients with myelopathy secondary to cervical OPLL, and the amount of expansion could be individually adjusted at the discretion of the surgeon. CONCLUSION: Although analysis with a larger population and a longer follow-up period needs to be undertaken, our method of lift-up laminoplasty appears to be a viable choice among standard posterior cervical approaches for cervical OPLL.

Sinus histiocytosis with nodal and extranodal involvement--a case report.

A two and half years old male child of sinus histiocytosis with massive lymphadenopathy, paraplegia and spinal cord involvement was treated with surgery and radiotherapy for the spinal cord compression and later with chemotherapy for his nodal disease in the neck. There was a significant improvement in his neurologic status as well as in his nodal status reiterating the role of combination therapy in this disease.

Compressão medular alta por cisto neuroentérico / High medular compression caused by neurenteric cyst

The authors report the case of a child with a neuroenteric cyst compressing the high cervical spinal cord at the level of C1 to C3. The tumor was completely excised through a laminotomy with good results. The literature is revised, and the embryology, symptomatology, treatment and results are discussed.

Spinal cord compression in HbE-thalassaemia.

Two cases of spinal cord compression due to extramedullary haemopoiesis in haemoglobine E thalassaemia are described. Possible mechanisms and treatment modes are discussed.

Compresión medular extradural por paracoccidioidomicosis :presentación de un caso / Dorsal extradural paracoccidioidomycosis: presentation of a case

Se presenta un caso de paracoccidioidomicosis extradural dorsal en un paciente masculino de 30 años quien fue diagnosticado y tratado en el Servicio de Neurocirugia de Hospital Universitario de Caracas.Se revisa la bibliografía, encontrándose que la pracoccidioidimicosis, aunque es una micosis frecuente en America Latina, su efección al sistema nervioso central es inusual, y la ubicación espinal es, todabia,más rara.Se presenta bajo la forma de lesión de ocupación de espacio (75