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.

Fístula arteriovenosa dural espinal: a propósito de un caso / Spinal dural arteriovenous fistula: a case report

Las fístulas arteriovenosas durales medulares son malformaciones vasculares adquiridas que constituyen una causa muy infrecuente de mielopatía progresiva (5-10 casos por millón de habitantes por año). La resonancia magnética es el estudio por imágenes de elección para su diagnóstico. A continuación presentamos el caso de una paciente femenina de 89 años, que consultó a la guardia de nuestra institución por un cuadro de paraparesia moderada asociada a parestesias e incontinencia urinaria posterior a esfuerzo físico. Se le diagnosticó una fístula arteriovenosa dural medular como causante de su cuadro. (AU)

Spinal dural arteriovenous fistulas (SDAVF) are acquired spinal vascular malformations and a rare cause of progressive myelopathy (5-10 new cases per year and per 1 million inhabitants). Magnetic resonance imaging is the diagnosis modality of choice. We present a case of a 89-year-old female patient who consulted the emergency department of our institution because of paraparesis and lower extremities paresthesias associated with urinary incontinence post physical effort. With the final diagnosis of spinal dural arteriovenous fistula, as a cause of the clinical symptoms. (AU)

Enfermedad de motoneurona inferior asociada al signo ojos de serpiente: una nueva entidad clínico-radiológica / Lower motor neuron disease with snake eyes sign: a new clinical-radiological entity

Las enfermedades de la moto neurona inferior constituyen un grupo heterogéneo de entidades con pronósticos diferentes. El signo radiológico "ojos de serpiente" hace referencia a la hiperintensidad bilateral en el asta anterior de la médula espinal en los cortes axiales de las imágenes por resonancia magnética, generalmente asociada a afecciones como infartos espinales, amiotrofia espondilótica, enfermedad de Hirayama y esclerosis lateral amiotrófica. Se ha descripto recientemente un reducido número de casos de enfermedad de moto neurona inferior asociada a "ojos de serpiente" con características clínicas y pronóstico distintivos. Presentamos dos casos de enfermedad de moto neurona inferior asociada al signo "ojos de serpiente" en pacientes jóvenes. El curso clínico fue progresivo con períodos prolongados de estabilidad clínica y ausencia de compromiso de moto neurona superior durante la evolución. Los pacientes presentaron debilidad segmentaria y asimétrica de miembros superiores con predominio distal en el primer caso y proximal en el segundo. Los casos presentados se corresponden en gran parte con lo comunicado en la literatura, dando apoyo a la existencia de una nueva entidad con pronóstico relativamente benigno denominada enfermedad de moto neurona inferior esporádica con signo de ojos de serpiente en las imágenes por resonancia magnética y cuya identificación debiera obviar tratamientos innecesarios (AU)

Lower motor neuron diseases are a heterogeneous group of entities with different prognosis. The "snake eyes" sign refers to bilateral hyper intensity of the anterior horns on axial magnetic resonance imaging of the spinal cord. It has been associated with ischemia, cervical spondylosis, Hirayama disease, and amyotrophic lateral sclerosis. Recently, a small number of cases of lower motor neuron disease associated with the "snake eyes" sign have been described as having distinctive clinical manifestations and prognosis. Two young patients with "snake eyes" sign associated with lower motor neuron disease had a progressive initial course followed by a stabilization of symptoms without involvement of upper motor neuron. They presented with asymmetric segmental arm weakness with distal predominance in the first a case and proximal predominance in the second. These cases match with those reported in the literature giving support to lower motor neuron disease with "snake eyes" as a pathological entity with a relatively good prognosis. This diagnosis should avoid unnecessary treatments (AU)

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.

Idiopathic Spinal Cord Herniation­Case Report

Idiopathic spinal cord herniation is a rare cause of progressivemyelopathy, especially in the absence of a history of spinal or surgical trauma. The radiological diagnosis ismade through a myelography or an MRI exam. The spinal cord is pushed anteriorly, buffering the dural defect and leading inmost cases to Brown-Séquard syndrome. The present study describes the case of a male patient with a clinical picture of progressive thoracicmyelopathy. In the clinical and radiological investigation, an idiopathic spinal cord herniation on the chest level was identified. During the surgery, the spinal cord was reduced to the natural site, taking its usual elliptical shape, and the dural defect was repaired with a dural substitute. The numbness of the patient improved, and the shocks in the lower limbs disappeared. A postoperative MRI confirmed the surgical reduction of the herniation and the restoration of the anterior cerebrospinal fluid (CSF) column to the spinal cord. The authors describe the clinical, radiological, intraoperative, and postoperative evolution.

Foix-Alajouanine syndrome mimicking a spinal cord tumor / Síndrome de Foix-Alajouanine simulando um tumor intramedular espinal

Summary Subacute necrotizing myelopathy (SNM) or Foix-Alajouanine syndrome is a rare disease characterized by progressive neurological dysfunction caused by a spinal dural arteriovenous fistula (AVF). Radiological diagnosis is usually suspected when there is intramedullary nonspecific enhancement and perimedullary flow voids. Ring-enhancement is rarely reported in the scope of AVF, which poses a diagnostic challenge and raises the suspicion of a spinal cord tumor. In such situations, biopsy can be required and delay proper diagnosis. We report the case of a patient with SNM, who underwent biopsy on the assumption of it being a spinal cord tumor.

Resumo Mielopatia necrotizante subaguda (MNS) ou síndrome de Foix-Alajouanine é uma doença rara que se caracteriza por disfunção neurológica progressiva causada por uma fístula arteriovenosa espinal dural. O diagnóstico radiológico é comumente suspeitado quando aparece captação não específica de contraste e de artefatos de fluxo (flow voids) perimedulares. Raramente, a captação de contraste exibe o aspecto em anel, constituindo um grande desafio diagnóstico. Nesses casos, o principal diagnóstico diferencial é um tumor intramedular, e os pacientes são encaminhados para biópsia da lesão, atrasando o diagnóstico definitivo. Relatamos o caso de uma paciente com MNS, a qual foi submetida à biópsia da lesão em virtude de suspeita de tumor intramedular.

Toxoplasmosis intramedular en una paciente con coinfección por VIH y tuberculosis / Intramedullary toxoplasmosis in HIV-tuberculosis co-infected patient

The most common clinical presentation of Toxoplasma gondii in HIV patients is encephalitis; however, the intramedullary involvement has been reported in a few cases. We report a case of intramedullary toxoplasmosis in a female patient diagnosed with HIV/tuberculosis co-infection, and history of poor adherence to antiretroviral therapy. The patient developed subacute paraparesis with compromise of sensory function and urinary sphincter. The nuclear magnetic resonance evaluation showed a single intramedullary ring-enhanced lesion at the T-8 level which was solved after an anti-Toxoplasma therapy with trimethoprim/sulfamethoxazole.

El compromiso encefálico por Toxoplasma gondii en pacientes con VIH es la localización más frecuente, no obstante, la localización intramedular ha sido escasamente reportada. Comunicamos un caso de toxoplasmosis intramedular en una mujer con diagnóstico de coinfección por VIH y tuberculosis, con mala adherencia a la terapia antirretroviral, que desarrolló de forma subaguda un cuadro de paraparesia con compromiso sensitivo y de esfínteres. La resonancia magnética mostró una lesión única intramedular con captación de contraste periférico en anillo a nivel T-8, que se resolvió tras recibir tratamiento anti-toxoplasmosis con cotrimoxazol.

Paracoccidioidomycosis in the spine: case report and review of the literature / Paracoccidioidomicose na coluna vertebral: relato de caso e revisão da literatura

ABSTRACT: CONTEXT: Paracoccidioidomycosis is a systemic form of mycosis that spreads hematogenously, secondarily to reactivation of lung infection or infection at another site or to new exposure to the causative agent. Few cases of bone involvement have been reported in the literature and involvement of the spine is extremely rare. CASE REPORT: We describe a case of a 68-year-old male patient with spondylodiscitis at the levels L4-L5 caused by presence of the fungus Paracoccidioides brasiliensis, which was diagnosed through percutaneous biopsy. The patient was treated with sulfamethoxazole and trimethoprim for 36 months, with complete resolution of the symptoms. CONCLUSION: Spondylodiscitis caused by the fungus Paracoccidioides brasiliensis is uncommon. However, in patients with chronic low-back pain who live or used to live in endemic regions, this infection should be considered as a possible differential diagnosis.

RESUMO: CONTEXTO: Paracoccidioidomicose é uma micose sistêmica de disseminação hematogênica, secundária a reativação de uma infecção pulmonar ou de outro sítio, ou a uma nova exposição ao agente causador. Poucos casos de envolvimento ósseo são relatados na literatura, e o acometimento da coluna vertebral é extremamente raro. RELATO DE CASO: Descrevemos o caso de um paciente masculino de 68 anos, apresentando espondilodiscite no nível L4-L5, causada pela presença do fungo Paracoccidioides brasiliensis, diagnosticada após biópsia percutânea. O paciente foi tratado com sulfametoxazol e trimetoprim por 36 semanas, com resolução completa dos sintomas. CONCLUSÃO: A espondilodiscite causada pelo fungo Paracoccidioides brasiliensis é incomum, mas, em pacientes portadores de lombalgia crônica que viveram ou vivem em regiões endêmicas, deve ser considerada como um possível diagnóstico diferencial.

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.

Digital Subtraction Cystography for Detection of Communicating Holes of Spinal Extradural Arachnoid Cysts

OBJECTIVE: The purpose of this study was to demonstrate the usefulness of digital subtraction cystography to identify communicating holes between a spinal extradural arachnoid cyst (SEAC) and the subarachnoid space prior to cyst removal and hole closure. MATERIALS AND METHODS: Six patients with SEAC were enrolled in this retrospective study. Digital subtraction cystography and subsequent CT myelography were performed for every patient. The presence and location of the communicating holes on cystography were documented. We evaluated the MRI characteristics of the cysts, including location, size, and associated spinal cord compression; furthermore, we reviewed cystographic images, CT myelograms, procedural reports, and medical records for analysis. If surgery was performed after cystography, intraoperative findings were compared with preoperative cystography. RESULTS: The location of the communicating hole between the arachnoid cyst and the subarachnoid space was identified by digital subtraction cystography in all cases (n = 6). Surgical resection of SEAC was performed in 4 patients, and intraoperative location of the communicating hole exactly corresponded to the preoperative identification. CONCLUSION: Fluoroscopic-guided cystography for SEAC accurately demonstrates the presence and location of dural defects. Preoperative digital subtraction cystography is useful for detection of a communicating hole between a cyst and the subarachnoid space.

CT myelography of the thoraco-lumbar spine in 8 dogs with degenerative myelopathy

CT myelography of the T11-L2 region was performed in 8 large-breed dogs with a clinical diagnosis of degenerative myelopathy (DM) and 3 large-breed dogs that were clinically normal. CT myelographic characteristics were recorded for each dog, at each disc level. Area measurements of the spinal cord, dural sac, vertebral canal, and vertebral body were recorded at 4 slice locations for each disc level. Mean area ratios were calculated and graphically compared, by slice location and group. In all dogs, CT myelography identified morphologic abnormalities that were not suspected from conventional myelograms. Characteristics observed with higher frequency in DM versus normal dogs were: spinal stenosis, disc protrusion, focal attenuation of the subarachnoid space, spinal cord deformity, small spinal cord, and paraspinal muscle atrophy. Mean spinal cord: dural sac, spinal cord: vertebral canal, dural sac: vertebral canal, and vertebral canal:vertebral body ratios were smaller in DM versus normal dogs at more than one disc level. Some CT myelographic characteristics in DM dogs were similar to those previously reported in humans, dogs and horses with stenotic myelopathy.

Intradural extramedullary tuberculoma of the spinal cord: a case report

Intradural extramedullary (IDEM) tuberculoma of the spinal cord is uncommon entity and moreover, few reports have been documented on concurrent IDEM and intracranial tuberculomas. Authors report a case of IDEM spinal tuberculoma having intracranial lesion simultaneously. A 49-year-old woman suffered from paraparesis and urinary incontinence while being given medical treatment for tuberculous meningitis. Magnetic resonance imaging (MRI) revealed an IDEM mass lesion between the T1 and T2 spinal levels, and multiple intracranial tuberculous granulomas. Surgical resection of the IDEM tuberculoma followed by anti-tuberculous medication resulted in good outcome.