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https://www.readbyqxmd.com/read/27930548/primary-central-nervous-system-alk-positive-anaplastic-large-cell-lymphoma-in-an-adult-a-rare-case-report
#1
Xiaoqin Dong, Jun Li, Na Huo, Yan Wang, Zhao Wu, Xiaohong Lin, Hong Zhao
RATIONALE: Anaplastic large cell lymphoma (ALCL) is an aggressive non-Hodgkin lymphoma. It mostly invades lymph nodes with extranodal involvement observed in the soft tissue, bone, and skin. PATIENT CONCERNS: We report a 34-year-old Chinese male patient who presented with headache, diplopia, and vomit. Cerebrospinal fluid (CSF) analysis via lumbar puncture showed elevated CSF pressure, elevated CSF protein concentrations, decreased CSF glucose and chloride concentration significantly, and pleocytosis of 68 to 350 × 10/L, in which lymphocytes and monocytes were predominant...
December 2016: Medicine (Baltimore)
https://www.readbyqxmd.com/read/27923222/pediatric-primary-diffuse-leptomeningeal-primitive-neuroectodermal-tumor-a-case-report-and-literature-review
#2
Jesna Mathew Sublett, Caitlin Davenport, Howard Eisenbrock, Shamsher Dalal, Syed A Jaffar Kazmi, Amir Kershenovich
BACKGROUND: Primary diffuse leptomeningeal primitive neuroectodermal tumor (PDL PNET) is extremely rare, with only 19 cases reported in the literature to date. We present a case of a child with rapidly progressive PDL PNET and a literature review. A 10-year-old boy presented with mood lability, hallucinations, generalized pain, enuresis, and headaches. Initial investigation failed to produce a diagnosis. The symptoms progressed to seizure, back pain, and papilledema. Imaging showed acute hydrocephalus and mild diffuse leptomeningeal enhancement without an identifiable primary lesion...
December 7, 2016: Pediatric Neurosurgery
https://www.readbyqxmd.com/read/27916758/-hydrocephalus-mimicking-idiopathic-normal-pressure-hydrocephalus-as-the-first-manifestation-of-neurosarcoidosis
#3
Atsuhiko Sugiyama, Makoto Kobayashi, Kumiko Agatsuma, Takeshi Bo, Toshiaki Shiojiri, Hidetoshi Mochida, Yoshio Suzuki, Takashi Matsunaga, Satoshi Kuwabara
A 61-year-old woman presented with a 10-month history of gait disturbance and a 7-month history of urinary incontinence. The Hasegawa dementia scale-revised score indicated cognitive impairment. Brain magnetic resonance imaging (MRI) indicated hydrocephalus with disproportionately enlarged subarachnoid space. This is usually considered a characteristic finding in idiopathic normal pressure hydrocephalus (iNPH). Ventriculo-peritoneal shunting improved the patient's symptoms. Neurosarcoidosis was suspected as a cause of the hydrocephalus because of the abnormalities in the cerebrospinal fluid and the abnormal enhancement of the cauda equina, the leptomeninges of the brainstem, and the spinal cord, as seen on MRI with gadolinium enhancement...
December 2016: Brain and Nerve, Shinkei Kenkyū No Shinpo
https://www.readbyqxmd.com/read/27913626/ring-enhancing-spinal-cord-lesions-in-neuromyelitis-optica-spectrum-disorders
#4
Nicholas L Zalewski, Padraig P Morris, Brian G Weinshenker, Claudia F Lucchinetti, Yong Guo, Sean J Pittock, Karl N Krecke, Timothy J Kaufmann, Dean M Wingerchuk, Neeraj Kumar, Eoin P Flanagan
OBJECTIVE: We assessed the frequency and characteristics of ring-enhancing spinal cord lesions in neuromyelitis optica spectrum disorder (NMOSD) myelitis and myelitis of other cause. METHODS: We reviewed spinal cord MRIs for ring-enhancing lesions from 284 aquaporin-4 (AQP4)-IgG seropositive patients at Mayo Clinic from 1996 to 2014. Inclusion criteria were as follows: (1) AQP4-IgG seropositivity, (2) myelitis attack and (3) MRI spinal cord demonstrating ring-enhancement...
December 2, 2016: Journal of Neurology, Neurosurgery, and Psychiatry
https://www.readbyqxmd.com/read/27905158/metastatic-spinal-cord-compression-from-basal-cell-carcinoma-of-the-skin-treated-with-surgical-decompression-and-vismodegib-case-report-and-review-of-hedgehog-signalling-pathway-inhibition-in-advanced-basal-cell-carcinoma
#5
J McGrane, S Carswell, T Talbot
We report a case of a 66-year-old man with locally advanced and metastatic basal cell carcinoma (BCC) causing spinal cord compression, which was treated with spinal surgery and subsequent vismodegib. The patient presented with a large fungating chest wall lesion and a metastasis in T8 that was causing cord compression. He had neurosurgical decompression of the T8 lesion and fixation of the spine. Punch biopsy from the fungating chest wall lesion showed a BCC with some malignant squamous differentiation (basosquamous)...
November 30, 2016: Clinical and Experimental Dermatology
https://www.readbyqxmd.com/read/27895949/primary-ewing-s-sarcoma-of-the-spine-in-a-two-year-old-boy
#6
Ali J Electricwala, Jaffer T Electricwala
Ewing's Sarcoma (ES) is a highly malignant bone tumour. It may involve any part of the skeleton but the most frequent parts are the ilium and diaphysis of femur and tibia (Alfeeli et al., 2005; Zhu et al., 2012). Primary ES of the spine is extremely rare (Yan et al., 2011). It accounts for only 3.5 to 14.9 percent of all primary bone sarcomas. The age of presentation ranges from 12 to 24 years (median 21 years) (Ferguson, 1999; Sharafuddin et al., 1992; Klimo Jr. et al., 2009). We report an unusual case of primary ES of the spine in a two-year-old boy, who presented to us with paraparesis and features of cauda equina syndrome...
2016: Case Reports in Orthopedics
https://www.readbyqxmd.com/read/27863379/targeted-next-generation-sequencing-identifies-two-novel-mutations-in-sepn1-in-rigid-spine-muscular-dystrophy-1
#7
Yi Dai, Shengran Liang, Yan Huang, Lin Chen, Santasree Banerjee
Rigid spine muscular dystrophy 1 (RSMD1) is a neuromuscular disorder, manifested with poor axial muscle strength, scoliosis and neck weakness, and a variable degree of spinal rigidity with an early ventilatory insufficiency which can lead to death by respiratory failure. Mutations of SEPN1 gene are associated with autosomal recessive RSMD1. Here, we present a clinical molecular study of a Chinese proband with RSMD1. The proband is a 17 years old male, showing difficulty in feeding, delayed motor response, problem in running with frequent fall down, early onset respiratory insufficiency, general muscle weakness and rigid cervical spine...
November 14, 2016: Oncotarget
https://www.readbyqxmd.com/read/27861371/chronic-lymphocytic-inflammation-with-pontine-perivascular-enhancement-responsive-to-steroids-clippers-with-intracranial-epstein-barr-virus-infection-a-case-report
#8
Yue Ma, Xiaolong Sun, Wen Li, Yi Li, Tao Kang, Xiai Yang, Wen Jiang
BACKGROUND: Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is an inflammatory disorder in the central nervous system (CNS) with distinct clinical, radiological, and pathological features. The pathophysiology of CLIPPERS still remains unclear and the reports are quite few. Although the radiological lesions were reported to be located predominantly in the pons, brachium pontis, and cerebellum, other adjacent structures such as the white matter and spinal cord were very recently reported as involved regions in CLIPPERS...
November 2016: Medicine (Baltimore)
https://www.readbyqxmd.com/read/27857933/retro-odontoid-pseudotumor-without-atlantoaxial-subluxation-or-rheumatic-arthritis
#9
Seung Han Yu, Hyuk Jin Choi, Won Ho Cho, Seung Heon Cha, In Ho Han
We present a case of retro-odontoid pseudotumor (ROP) without rheumatoid arthritis (RA) or atlantoaxial subluxation (AAS). A 76-year-old woman presented with paresthesia and weakness of both lower and upper extremities. She had no laboratory or physical findings of RA. Dynamic X-ray showed no AAS and magnetic resonance imaging (MRI) showed a retro-odontoid mass compressing the spinal cord. Transdural mass debulking and biopsy were performed via minimal left suboccipital craniectomy and C1 hemilaminectomy. Two months after surgery, her symptoms were aggravated...
October 2016: Korean Journal of Neurotrauma
https://www.readbyqxmd.com/read/27843691/co-existing-spinal-intradural-ependymal-cyst-and-sacral-tarlov-cyst-in-adult-onset-tethered-cord-syndrome-with-syringomyelia-case-report-and-literature-review
#10
Hamid H Rai, Muhammad F Khan, Syed Ather Enam, Imtiaz Hashmi
BACKGROUND: Synchronous spinal intradural ependymal cysts and sacral Tarlov cysts in adult onset tethered cord syndrome are extremely rare. CASE DESCRIPTION: A 23-year-old male presented with back pain radiating into both lower extremities, accompanied by acute onset of gait difficulty and sphincter dysfunction. Magnetic resonance imaging identified a low lying conus medullaris, syringomyelia with septations extending from T12 to S1, a tethered cord, and a thickened filum terminale with a sacral Tarlov cyst...
2016: Surgical Neurology International
https://www.readbyqxmd.com/read/27842677/spinal-diffusion-tensor-tractography-for-differentiation-of-intramedullary-tumor-suspected-lesions
#11
K Egger, M Hohenhaus, V Van Velthoven, S Heil, H Urbach
BACKGROUND AND PURPOSE: Primary MRI diagnosis of spinal intramedullary tumor-suspected lesions can be challenging and often requires spinal biopsy or resection with a substantial risk of neurological deficits. We evaluated whether Diffusion Tensor Imaging (DTI) tractography can facilitate the differential diagnosis. MATERIALS AND METHODS: Twenty-five consecutive patients with an intramedullary tumor-suspected lesion considered for spinal surgery were studied with a Diffusion-weighted multi-shot read out segmented EPI sequence (RESOLVE)...
December 2016: European Journal of Radiology
https://www.readbyqxmd.com/read/27819134/fungal-spondylodiscitis-in-a-patient-recovered-from-h7n9-virus-infection-a-case-study-and-a-literature-review-of-the-differences-between-candida-and-aspergillus-spondylodiscitis
#12
Lie-Dao Yu, Zhi-Yun Feng, Xuan-Wei Wang, Zhi-Heng Ling, Xiang-Jin Lin
To report a rare case of fungal spondylodiscitis in a patient recovered from H7N9 virus infection and perform a literature review of the different characteristics of Candida and Aspergillus spondylodiscitis, we reviewed cases of spondylodiscitis caused by Candida and Aspergillus species. Data, including patients' information, pathogenic species, treatment strategy, outcomes, and relapses, were collected and summarized. The characteristics of Candida and Aspergillus spondylodiscitis were compared to see if any differences in clinical features, management, or consequences could be detected...
2016: Journal of Zhejiang University. Science. B
https://www.readbyqxmd.com/read/27818821/disseminated-cerebrospinal-embryonal-tumor-in-the-adult
#13
Alessandro Caporlingua, Daniele Armocida, Federico Caporlingua, Gennaro Lapadula, Grazia Maria Elefante, Manila Antonelli, Maurizio Salvati
Introduction. According to the 2016 World Health Organization classification of Tumors of the Central Nervous System, the term Primitive Neuroectodermal Tumor has been replaced by the term Embryonal Tumor (ET). We present a case of disseminated cerebrospinal ET presenting in an adult patient. Illustrative Case. A 49-year-old male presenting with low back pain, dysuria, and hypoesthesia of the lower extremities referred to our emergency department. Brain and whole spine contrast-enhanced MRI documented a diffusively disseminated heterogeneous neoplasm with intradural extra- and intramedullary involvement of the cervicothoracic tract and cauda equina...
2016: Case Reports in Pathology
https://www.readbyqxmd.com/read/27807335/-clinical-features-of-a-genetically-identified-spinal-and-%C3%A2-bulbar-muscular-atrophy-pedigree
#14
Zhe Wang, Qihua Chen, Qiuxiang Li, Fangfang Bi
Spinal and bulbar muscular atrophy (SBMA) is a rare X-linked motor neuron disease with significant phenotypic viability. Here, we present a genetically identified SBMA family without bulbar paralysis or androgen insensitivity. All four male patients presented with progressive lower motor neuron paralysis in all limbs, with distal extremities more dominant. None of them had bulbar palsy or androgen insensitivity. A consistently mild elevated blood creatine phosphokinase (CPK) levels were detected in all patients and the EMG showed a chronic neurogenic damage...
October 28, 2016: Zhong Nan da Xue Xue Bao. Yi Xue Ban, Journal of Central South University. Medical Sciences
https://www.readbyqxmd.com/read/27790322/role-of-transpedicular-percutaneous-vertebral-biopsy-for-diagnosis-of-pathology-in-vertebral-compression-fractures
#15
Saurabh Shrinivas Pagdal, Sunil Nadkarni, Sharad Moreshwar Hardikar, Madan Sharad Hardikar
STUDY DESIGN: Retrospective observational study. PURPOSE: To identify the role of percutaneous vertebral biopsy in histopathological diagnosis of vertebral compression fractures and to identify the frequency of unexpected malignancy in vertebral compression fractures. OVERVIEW OF LITERATURE: Vertebral compression fractures are common in the Indian population. Magnetic resonance imaging and nuclear imaging have some limitations in the diagnosis of definitive pathology of vertebral compression fractures...
October 2016: Asian Spine Journal
https://www.readbyqxmd.com/read/27790312/magnetic-resonance-imaging-and-genexpert-a-rapid-and-accurate-diagnostic-tool-for-the-management-of-tuberculosis-of-the-spine
#16
Ayush Sharma, Harvinder Singh Chhabra, Rajat Mahajan, Tarun Chabra, Sahil Batra
STUDY DESIGN: Retrospective study. PURPOSE: The aim of this study was to analyze various diagnostic tools, including GeneXpert, for the management of tuberculosis of the spine. OVERVIEW OF LITERATURE: Traditional diagnostic methods of microscopy, histology, and culture have low sensitivity and specificity for the management of tuberculosis of the spine. METHODS: Of the 262 treated cases of spinal tuberculosis, data on 1 year follow-up was available for 217 cases...
October 2016: Asian Spine Journal
https://www.readbyqxmd.com/read/27777151/management-of-patients-with-primary-intramedullary-spinal-cord-glioblastoma
#17
Bedjan Behmanesh, Matthias Setzer, Juergen Konczalla, Patrick Harter, Johanna Quick-Weller, Lioba Imoehl, Kea Franz, Florian Gessler, Volker Seifert, Gerhard Marquardt
BACKGROUND: Primary intramedullary spinal cord glioblastoma are very rare tumors of the spinal cord. They imply a very poor prognosis since complete surgical resection is not possible due to the infiltrative growth of these tumors. The aim of this study is to present our data achieved with an aggressive multimodality treatment. METHODS: We retrospectively reviewed our clinical database. All patients with histologically proven intramedullary spinal cord glioblastoma treated in our department were included in this study...
October 21, 2016: World Neurosurgery
https://www.readbyqxmd.com/read/27776947/-paradoxical-reaction-following-antituberculosis-therapy-in-immunocompetent-patient
#18
S Bacha, M Khemiri, H Racil, S Hantous, N Chaouch, S Cheikhrouhou, A Chabbou, M L Megdiche
INTRODUCTION: The features of paradoxical reactions (PR) that occurred in non-HIV infected patients are rare and not well known. CASE REPORT: The authors reported the case of a 21years old, non-immunocompromised, and HIV negative patient treated for disseminated tuberculosis. PR occurred after 8months after initiation of antituberculous treatment. PR presented as left cervical lymphadenopathy, pulmonary, pleural, costal and spinal location of the tuberculosis. The antituberculous drugs were prolonged...
October 21, 2016: Revue de Pneumologie Clinique
https://www.readbyqxmd.com/read/27776522/inositol-1-4-5-trisphosphate-receptor-type-1-autoantibodies-in-paraneoplastic-and-non-paraneoplastic-peripheral-neuropathy
#19
Sven Jarius, Marius Ringelstein, Jürgen Haas, Irina I Serysheva, Lars Komorowski, Kai Fechner, Klaus-Peter Wandinger, Philipp Albrecht, Harald Hefter, Andreas Moser, Eva Neuen-Jacob, Hans-Peter Hartung, Brigitte Wildemann, Orhan Aktas
BACKGROUND: Recently, we described a novel autoantibody, anti-Sj/ITPR1-IgG, that targets the inositol 1,4,5-trisphosphate receptor type 1 (ITPR1) in patients with cerebellar ataxia. However, ITPR1 is expressed not only by Purkinje cells but also in the anterior horn of the spinal cord, in the substantia gelatinosa and in the motor, sensory (including the dorsal root ganglia) and autonomic peripheral nervous system, suggesting that the clinical spectrum associated with autoimmunity to ITPR1 may be broader than initially thought...
October 24, 2016: Journal of Neuroinflammation
https://www.readbyqxmd.com/read/27771479/surgical-management-for-destructive-atlantoaxial-spondyloarthropathy-in-long-term-hemodialysis-patients
#20
Je Hoon Jeong, Hee Kyung Kim, Soo Bin Im
BACKGROUND: Atlantoaxial spondyloarthropathy is most often resulted from rheumatoid arthritis, cancer metastasis and basilar invagination. Dialysis-related spondyloarthropathy is a rare cause of spinal deformity and cervical myelopathy at the atlantoaxial joint. We report two long-term hemodialysis patients who presented with atlantoaxial spondyloarthropathy. CASE DESCRIPTION: Two patients with end-stage renal failure (ESRD) presented with history of progressively worsening neck pain and motion limitation, and gait disturbance...
October 19, 2016: World Neurosurgery
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