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Labrune syndrome is an uncommon central nervous system disorder characterized by leukoencephalopathy, cerebral calcifications, and cysts on brain imaging. The basic pathology is microangiopathy resulting from a mutation in the SNORD118 gene. Radiological imaging is the hallmark of the disease.
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Calcinose , Cistos , Leucoencefalopatias , Humanos , Calcinose/diagnóstico por imagem , Calcinose/complicações , Leucoencefalopatias/diagnóstico por imagem , Leucoencefalopatias/genética , Leucoencefalopatias/complicações , Cistos/diagnóstico por imagem , Cistos/complicações , Imageamento por Ressonância Magnética , Encéfalo/diagnóstico por imagem , Encéfalo/patologia , Masculino , Cistos do Sistema Nervoso Central/diagnóstico por imagem , Cistos do Sistema Nervoso Central/complicações , Cistos do Sistema Nervoso Central/genética , Feminino , Tomografia Computadorizada por Raios X , RNA Nucleolar Pequeno/genéticaRESUMO
The duplicated origin of the vertebral artery (VA) is an uncommon anatomical variant, which is generally identified incidentally during angiography and can be misdiagnosed as dissection in the setting of posterior circulation stroke. Here, we describe a case of the right V1 VA duplication with embryological aspects in a patient with Klippel-Feil anomaly, which was diagnosed during preoperative evaluation. Surgeons must be aware to avoid vascular injury from a duplicated VA before head-neck and spinal surgery.
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Síndrome de Klippel-Feil , Artéria Vertebral , Humanos , Síndrome de Klippel-Feil/complicações , Síndrome de Klippel-Feil/diagnóstico , Artéria Vertebral/anormalidades , Artéria Vertebral/diagnóstico por imagem , Masculino , Adulto , Angiografia por Tomografia Computadorizada , FemininoRESUMO
Background Moyamoya disease (MMD) can be a major cause of hemorrhagic stroke. Though extensive angiographic studies have been undertaken, the understanding of the association between aneurysms and MMD remains unanswered. In this study, we explore the association of the aneurysm with MMD and its management. We have also reviewed such associations described in the literature and how the present cases differ from those previously described. Materials and methods The clinical and radiologic data of moyamoya disease cases were accessed from medical and radiological records between January 2010 and July 2017. Two neuroradiologists independently analyzed the data and imaging details. Results Out of 103 patients with MMD, eight patients (7.77%) had associated intracranial aneurysms with eleven aneurysms. Out of the 11 aneurysms, five were the tip of the basilar artery aneurysms and were the most common location for aneurysm (45.5%), followed by lenticulostriate artery, PCA perforator, and distal ACA (DACA) in the P1 PCA, P2 PCA, and P3 PCA artery aneurysms. Out of eight patients, five (62.5%) had a hemorrhage on a non-contrast computed tomography (NCCT) scan of the brain, whereas three (37.5%) had an ischemic presentation. Out of 11 aneurysms, seven aneurysms, including three basilar tip aneurysms (unruptured) and one PCA perforator (ruptured), and three saccular PCA (P1, P2, and P3) (ruptured) were treated by endovascular coiling. Follow-up angiography showed stable aneurysmal occlusion except in one basilar tip, where recurrence was observed. Conclusions MMD-intracranial aneurysm is commonly observed in patients with intracranial hemorrhage and carries a higher risk of rupture. Therefore, identification of the aneurysm is essential for management. Endovascular treatment, either with coil or glue embolization, can be a safe and effective treatment method for such aneurysms with long-term good results.
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Objectives: Neural crest cells (NCCs) are transient structures in the fetal life in vertebrates, which develop at the junctional site of the non-neural and neural ectoderm, sharing a common developmental origin for diverse diseases. After Epithelio-mesenchymal (EMT) of the NCCs within the neural tube, delamination of NCCs occurs. After delamination, the transformation of these cells into various cell lineages produces melanocytes, bones, and cartilage of the skull, cells of the enteric and peripheral nervous system. After the conversion, these cells migrate into various locations of the entire body according to the cell lineage. Abnormalities in neural crest (NC) formation and migration result in various malformations and tumors, known as neurocristopathy. Material and Methods: Herein, this case series describes a single-center experience in cephalic NC disorders over the past 3 years, including 17 cases of varying composition (i.e., vascular, dysgenetic, mixed, and neoplastic forms) involving the brain and occasionally skin, eyes, and face of the patients. Results: In our study of 17 patients with cephalic NC disease, 6 (35.3%) patients had vascular form, 5 (29.4%) had dysgenetic form, 4 (23.5%) had mixed form, and 2 (11.7%) had neoplastic form. Brain involvement in the form of vascular or parenchyma or both vascular and parenchymal was seen in all of our patients (100%), skin in 6 (35.3%) patients, eye in 2 (11.7%), and face in 1 (5.9%) patient. Treatment was planned according to the various manifestations of the disease. Conclusion: Neural crest diseases (NCDs) are a rare and under-recognized group of disorders in the literature and may have been under-reported due to a lack of awareness regarding the same. More such reporting may increase the repertoire of these rare disorders such that clinicians can have a high degree of suspicion leading to early detection and timely counseling and also improve preventive strategies and help in developing new drugs for these disorders or prevent them.
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Background and purpose: Mechanical thrombectomy is the standard treatment modality for flow restoration in acute ischemic stroke. In cases of persistent occlusion, the optimal number of retrieval attempts before considering procedure termination is currently undetermined and is a topic for research. Therefore in this study, we studied the impact of the number of stent retrieval maneuvers on the recanalization of vessels. Methods: In this retrospective single-center observational study we included 52 patients with large vessel occlusion who underwent stent retriever mechanical thrombectomy. Successful recanalization rate was defined as modified TICI (Thrombolysis in Cerebral Infarction) 2b or 3. Result: The overall successful recanalization rate was 44.24%. The recanalization rate per stent retrieval attempt was the highest in 1st attempt (28.84%) and no recanalization was observed with the 3rd, 5th, and 6th attempts (p<0.001). At most 6 retrieval attempts were used. Conclusions: After two retrieval attempts, 91% of the patients were successfully recanalized and other after the 5th attempt could not result in recanalization.
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Background: Mechanical thrombectomy has been established as a safe, standard and effective treatment option for occlusions of the proximal segment of the middle cerebral artery (MCA), as demonstrated in numerous studies. However, performing thrombectomy in the M2 divisions of MCA presents inherent challenges. In this institutional experience, we aim to delineate the recanalisation rates achieved through mechanical thrombectomy in cases involving the M2 segment of the MCA. Methods: We conducted a retrospective analysis of patients who underwent thrombectomy due to M2 MCA occlusions in the period from January 2018 to December 2021. Various factors affecting recanalisation rates were assessed. Results: A total of 15 patients with M2 segment occlusions of the middle cerebral artery were included in the study, comprising 11 in the superior division and 4 in the inferior division. The successful recanalisation rate was 72.33%, with notably higher success observed in cases of inferior division occlusion. The primary outcome of our study was the mTICI recanalisation status, categorised as successful recanalisation (mTICI = 2b or mTICI = 3) and unsuccessful recanalisation (mTICI = 1 or mTICI = 2a) and mRS at 6 months. None of the predictors assessed reached statistical significance. Conclusions: Mechanical thrombectomy demonstrates favourable efficacy and recanalisation rates in cases of M2 MCA division occlusion. Notably, inferior division occlusions exhibit a higher likelihood of successful recanalisation.
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Introduction: Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) causes coronavirus disease 2019 (COVID-19), which not only produces respiratory symptoms but is known to involve almost every system, and its neuroinvasive properties have been well demonstrated throughout the pandemic. Also, to combat the pandemic, there was rapid development and induction of various vaccination drives, following which many adverse events following immunization (AEFIs) have been reported, which include neurological complications as well. Method: We present a series of three cases, post vaccination, with and without a history of COVID illness that showed remarkably similar findings on magnetic resonance imaging (MRI). Result: A 38-year-old male presented with complaints of weakness of the bilateral lower limbs with sensory loss and bladder disturbance a day after receiving his first dose of ChadOx1 nCoV-19 (COVISHIELD) vaccine. A 50-year-old male with hypothyroidism characterized by autoimmune thyroiditis and impaired glucose tolerance experienced difficulty in walking 11.5 weeks after being administered with COVID vaccine (COVAXIN). A 38-year-old male presented with subacute onset progressive symmetric quadriparesis 2 months after their first dose of a COVID vaccine. The patient also had sensory ataxia, and his vibration sensation was impaired below C7. All three patients had typical pattern of involvement of the brain and spine on MRI with signal changes in bilateral corticospinal tracts, trigeminal tracts in the brain, and both lateral and posterior columns in the spine. Conclusion: This pattern of brain and spine involvement on MRI is a novel finding and is likely a result of post-vaccination/post-COVID immune-mediated demyelination.
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Encéfalo , Vacinas contra COVID-19 , COVID-19 , Doenças Desmielinizantes , Adulto , Humanos , Masculino , Pessoa de Meia-Idade , Encéfalo/diagnóstico por imagem , Encéfalo/patologia , ChAdOx1 nCoV-19 , COVID-19/complicações , COVID-19/imunologia , Vacinas contra COVID-19/efeitos adversos , Doenças Desmielinizantes/induzido quimicamente , Neuroimagem , Tratos Piramidais , Vacinação/efeitos adversos , Medula Espinal/diagnóstico por imagem , Medula Espinal/patologiaRESUMO
We quantified and investigated multimodal brain MRI measures in the LoCARPoN Study due to lack of normative data among Indians. A total of 401 participants (aged 50-88 years) without stroke or dementia completed MRI investigation. We assessed 31 brain measures in total using four brain MRI modalities, including macrostructural (global & lobar volumes, white matter hyperintensities [WMHs]), microstructural (global and tract-specific white matter fractional anisotropy [WM-FA] and mean diffusivity [MD]) and perfusion measures (global and lobar cerebral blood flow [CBF]). The absolute brain volumes of males were significantly larger than those of females, but such differences were relatively small (<1.2% of intracranial volume). With increasing age, lower macrostructural brain volumes, lower WM-FA, greater WMHs, higher WM-MD were found (P = 0.00018, Bonferroni threshold). Perfusion measures did not show significant differences with increasing age. Hippocampal volume showed the greatest association with age, with a reduction of approximately 0.48%/year. This preliminary study augments and provides insight into multimodal brain measures during the nascent stages of aging among the Indian population (South Asian ethnicity). Our findings establish the groundwork for future hypothetical testing studies.
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Schimke immuno-osseous dysplasia (SIOD) is an uncommon autosomal recessive (AR) spondylo-epiphyseal dysplasia (SED) and its clinical course and phenotype are yet to be properly described. The phenotypic presentation is quite varied with involvement of the renal, skeletal, vascular, immune, and hematopoietic systems being the most common presentation. We describe a 19-year-old female who presented with adolescent-onset brain and skeletal involvement without renal manifestations. Based on imaging and clinical features, she was diagnosed with a case of SIOD. There is no definitive treatment yet for this disorder, however, clinicians should be aware of this disorder so that adequate counseling and symptomatic management, especially in controlling hypertension and dyslipidemia, can be provided to the affected patients.
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Megalencephaly-capillary malformation-polymicrogyria syndrome (MCAP) is an uncommon malformation syndrome, characterized by primary megalencephaly, capillary malformations of the midline face and body, or distal limb anomalies such as syndactyly and polymicrogyria. Herein, we report a young male child, who presented with complaints of increasing head size, delay in speech, and one episode of focal seizure with distinctive morphological and neuroradiological manifestations which led to the diagnosis of MCAP. We have also reviewed recently published literature and the various diagnostic criteria proposed by authors to achieve the early clinical diagnosis of these patients in the outpatient department.
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Craniopagus conjoined twins are extremely rare, reported 1 in 2.5 million live births. To date, 62 separation attempts in 69 well-documented cases of craniopagus twins have been made. Of these, 34 were performed in a single-stage approach, and 28 were attempted in a multistage approach. One or both twins died of massive intraoperative blood loss and cardiac arrest in 14 cases. We report our surgical experience with conjoined craniopagus twins (JB) with type III total vertical joining and shared circumferential/circular sinus with left-sided dominance. A brief review of the literature is also provided. In our twins, the meticulous preoperative study and planning by the multidisciplinary team consisting of 125-member, first-staged surgical separation consisted of creation of venous conduit to bypass part of shared circumferential sinus and partial hemispheric disconnection. Six weeks later, twin J manifested acute cardiac overload because of one-way fistula development from blocked venous bypass graft necessitating emergency final separation surgery. Unique perioperative issues were abnormal anatomy, hemodynamic sequelae from one-way fistula development after venous bypass graft thrombosis, cardiac arrest after massive venous air embolism requiring prolonged cardiopulmonary resuscitation, and return of spontaneous circulation at 15 minutes immediately after separation. This is the first Indian craniopagus separation surgery in a complex total vertical craniopagus twin reported by a single-center multidisciplinary team. Both twins could be sent home, but one remained severely handicapped. Adequate perioperative planning and multidisciplinary team approach are vital in craniopagus twin separation surgeries.
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Fístula , Parada Cardíaca , Procedimentos de Cirurgia Plástica , Gêmeos Unidos , Cavidades Cranianas/cirurgia , Parada Cardíaca/cirurgia , Humanos , Gêmeos Unidos/cirurgiaRESUMO
Basilar artery fenestration aneurysms are very rare and endovascular management of large and complex aneurysms is extremely challenging. Most of these type of cases are managed with stent assisted coiling, dual flow diverters (FD) and single FD with additional coiling of aneurysm and occlusion of one of the vertebral artery. Here, we report a case of large complex basilar artery fenestration aneurysm successfully treated with single FD using novel technique called "crossing flow diverter technique" without any additional coiling of aneurysm or occlusion of vertebral artery. Using this technique cost of procedure and procedural complexity inherent with other above mentioned techniques can be significantly reduced.
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Procedimentos Endovasculares , Aneurisma Intracraniano , Artéria Basilar/diagnóstico por imagem , Humanos , Aneurisma Intracraniano/diagnóstico por imagem , Aneurisma Intracraniano/terapia , Stents , Artéria Vertebral/diagnóstico por imagemRESUMO
BACKGROUND: As the health systems around the world struggled to meet the challenges of COVID-19 pandemic, care of many non-COVID emergencies was affected. AIMS: The present study examined differences in the diagnosis, evaluation and management of stroke patients during a defined period in the ongoing pandemic in 2020 when compared to a similar epoch in year 2019. METHODS: The COVID stroke study group (CSSG) India, included 18 stroke centres spread across the country. Data was collected prospectively between February and July 2020 and retrospectively for the same period in 2019. Details of demographics, stroke evaluation, treatment, in-hospital and three months outcomes were collected and compared between these two time points. RESULTS: A total of 2549 patients were seen in both study periods; 1237 patients (48.53%) in 2019 and 1312 (51.47%) in 2020. Although the overall number of stroke patients and rates of thrombolysis were comparable, a significant decline was observed in the month of April 2020, during the initial period of the pandemic and lockdown. Endovascular treatment reduced significantly and longer door to needle and CT to needle times were observed in 2020. Although mortality was higher in 2020, proportion of patients with good outcome were similar in both the study periods. CONCLUSIONS: Although stroke admissions and rates of thrombolysis were comparable, some work flow metrics were delayed, endovascular stroke treatment rates declined and mortality was higher during the pandemic study period. Reorganization of stroke treatment pathways during the pandemic has likely improved the stroke care delivery across the globe.
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COVID-19 , Acidente Vascular Cerebral , Controle de Doenças Transmissíveis , Humanos , Índia/epidemiologia , Pandemias , Estudos Retrospectivos , SARS-CoV-2 , Acidente Vascular Cerebral/epidemiologia , Acidente Vascular Cerebral/terapia , Tempo para o TratamentoRESUMO
Intracranial dermoid and epidermoid cysts are usually considered to be two different entities in the radiological and surgical literature. Epidermoid cysts are classically off midline in location, isointense to cerebrospinal fluid on T1 and T2-weighted images and have restricted diffusion, whereas dermoid cysts are classically midline in location, have T1-hyperintense regions due to the presence of fat and show facilitated diffusion. We report a case of radiological epidermoid cyst in baseline imaging, which evolved into a radiological dermoid cyst over time, and explain this unique occurrence with a review of the embryology and histopathogenesis of these cysts.
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Neoplasias Encefálicas/diagnóstico por imagem , Neoplasias Encefálicas/embriologia , Cisto Dermoide/diagnóstico por imagem , Cisto Dermoide/embriologia , Cisto Epidérmico/diagnóstico por imagem , Cisto Epidérmico/embriologia , Neoplasias Encefálicas/patologia , Neoplasias Encefálicas/cirurgia , Cisto Dermoide/patologia , Cisto Dermoide/cirurgia , Cisto Epidérmico/patologia , Cisto Epidérmico/cirurgia , Humanos , Imageamento por Ressonância Magnética , Masculino , Ruptura Espontânea , Tomografia Computadorizada por Raios X , Adulto JovemRESUMO
BACKGROUND AND PURPOSE: Differentiating between neurocysticercosis (NCC) and neurotuberculosis has serious therapeutic implications and this distinction relies heavily on neuroimaging. Few case reports discuss the conglomeration of ring-enhancing lesions (RELs) in patients with solitary NCC. The aim of our study is to describe the imaging findings of conglomerate RELs in a cohort of patients with solitary NCC, emphasizing the frequency of conglomeration. MATERIALS AND METHODS: This retrospective study included 100 patients with solitary NCC. Two neuroradiologists analyzed contrast-enhanced computed tomography (CT) images regarding morphology, enhancement pattern, location, number of lesions, and degree of perilesional edema. The solitary lesions were classified as solitary discrete RELs (SD-RELs) when a well-defined lesion was seen and solitary conglomerate RELs (SC-RELs) when two or more ring lesions or ring/rings plus disc lesions were present contiguously. Follow-up CT scans were evaluated for the resolution of lesions and surrounding edema. RESULTS: Out of 100 patients, 42 were SD-RELs and 58 were SC-RELs. No statistically significant difference was found between both groups in terms of age of presentation, clinical presentation, lesion size and location, and degree of perilesional edema. Larger lesions (>10 mm) were more likely to show scolex and were associated with greater degree of edema in both subgroups. During follow-up, 13 patients had new lesions (SD-RELs-5, SC-RELs-8). In SD-RELs, follow-up lesions were in the same location in four patients and new location in one; and in SC-RELs, lesions were in the same location in seven and in new location in one case. CONCLUSION: Conglomeration of RELs is a common finding in patients with solitary NCC.
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Posterior condylar canal dural arteriovenous fistula (PCC dAVF) is a rare entity with only three cases having been reported so far in the English literature. We describe the clinical presentation, imaging, and endovascular management of an elderly man with left PCC dAVF presenting with subarachnoid haemorrhage (SAH). Endovascular management of such cases requires thorough understanding of the vascular anatomy around the craniovertebral junction (CVJ) and variable bridging vein draining patterns. The fistula in our case was fed by the posterior meningeal branch of the left vertebral artery and was draining through a dilated and tortuous medullary bridging vein into the antero-lateral pontomedullary venous system. Transarterial glue embolisation was performed with complete exclusion of the fistula and venous pouches. The patient developed intractable hiccough and left-sided facial pain on the second post-procedural day, and MRI showed focal diffusion restriction in the left dorso-lateral medulla. He recovered completely after a short course of steroids.
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Malformações Vasculares do Sistema Nervoso Central/diagnóstico por imagem , Malformações Vasculares do Sistema Nervoso Central/cirurgia , Procedimentos Endovasculares/métodos , Hemorragia Subaracnóidea/diagnóstico por imagem , Hemorragia Subaracnóidea/cirurgia , Idoso , Malformações Vasculares do Sistema Nervoso Central/etiologia , Angiografia Cerebral , Humanos , Imageamento Tridimensional , Imageamento por Ressonância Magnética , Masculino , Hemorragia Subaracnóidea/etiologia , Tomografia Computadorizada por Raios XRESUMO
BACKGROUND AND PURPOSE: Vein of Galen aneurysmal malformation (VGAM) is a rare developmental intracranial vascular malformation. We analyzed the clinical presentations, imaging findings, angioarchitecture, management options, and outcome in a demographically heterogeneous set of VGAM patients. METHODS: We retrospectively analyzed cases of VGAM from our departmental archive collected between 1988 and January 2015. Demographic, clinical, therapeutic, and follow-up details were obtained for each patient from the available records. RESULTS: We identified 36 patients with VGAM including 6 neonates, 18 infants, 7 children aged 2-10â years, and 5 adults. Macrocrania was the commonest presenting feature. Type of fistulae was mural in 14 and choroidal in 18 patients while 4 had a thrombosed sac at presentation. In 3 cases the dilated venous sac had connection with the deep venous system. Bilateral jugular atresia and stenosis were seen in 9 and 6 patients, respectively. Giant venous sac (>4â cm) was significantly correlated with mural type (p=0.0001). Dural arterial recruitment was seen in 4 patients including 3 adults. Among the 23 patients treated by endovascular means, 14 had a good outcome, 5 had a poor outcome, and 4 died. A significant correlation was noted between jugular atresia and poor outcome (p=0.003). CONCLUSIONS: We encountered a wide range of demographic, clinical, and angiographic features in VGAM. Mural type malformations were associated with giant venous sacs. Good outcome after embolization was seen in selected neonates and in most of the infants, children, and adults. Jugular atresia was significantly associated with poor outcome.
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Procedimentos Endovasculares/métodos , Malformações da Veia de Galeno/diagnóstico por imagem , Malformações da Veia de Galeno/cirurgia , Adolescente , Adulto , Anatomia Transversal , Angiografia Cerebral , Artérias Cerebrais/diagnóstico por imagem , Criança , Pré-Escolar , Embolização Terapêutica , Feminino , Humanos , Lactente , Recém-Nascido , Imageamento por Ressonância Magnética , Masculino , Estudos Retrospectivos , Tomografia Computadorizada por Raios X , Resultado do Tratamento , Derivação Ventriculoperitoneal , Adulto JovemRESUMO
Spinal dural arteriovenous fistulas (DAVFs), the most common of spinal vascular malformations, are AVFs in the dura mater of the nerve root and/or adjacent spinal dura. These fistulas are most often solitary and are fed by a single radicular artery that primarily supplies the dura mater. Multiple spinal DAVFs are rarely reported in the literature. Those that have been documented have been synchronous in their presentation in that they were found during the same examination or were present at the initial examination but missed and only recognized at the second examination. The authors report the case of a patient with two spinal DAVFs occurring at different spinal levels at different points in time (metachronous).
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Fístula Arteriovenosa/diagnóstico por imagem , Fístula Arteriovenosa/cirurgia , Malformações Vasculares do Sistema Nervoso Central/diagnóstico por imagem , Malformações Vasculares do Sistema Nervoso Central/cirurgia , Fístula Arteriovenosa/etiologia , Malformações Vasculares do Sistema Nervoso Central/etiologia , Humanos , Vértebras Lombares , Masculino , Pessoa de Meia-Idade , Radiografia , Vértebras TorácicasRESUMO
Although posterior reversible encephalopathy syndrome (PRES) is a widely encountered clinicoradiological entity, spinal cord involvement on MRI is very rarely reported. We found only eight cases that have been reported so far. Reports of post-contrast meningeal or parenchymal enhancement in PRES are even rarer. Herein we report a case of PRES with extensive spinal cord signal abnormality with contrast enhancement. Familiarity with this rare imaging finding of PRES, in the appropriate clinical setting, will avoid unnecessary investigations and inappropriate treatment.