Epidural spinal cord compression in a patient with blue rubber bleb nevus syndrome

2014 ◽  
Vol 14 (5) ◽  
pp. 486-489 ◽  
Author(s):  
Gary Rajah ◽  
Chiu Yuen To ◽  
Sandeep Sood ◽  
Steven Ham ◽  
Deniz Altinok ◽  
...  

Blue rubber bleb nevus syndrome (BRBNS) can present with vascular malformations throughout the body, especially in the gastrointestinal tract. Spinal cord compression from these lesions is rare, particularly in the pediatric population. The authors report a case of BRBNS involving an 18-year-old female patient who presented with back pain and an epidural thoracic mass with cord compression. She underwent an uncomplicated thoracic laminectomy and decompression, with removal of what appeared to be a venous malformation. Postoperatively her pain improved, and imaging revealed resolution of cord compression. Pathological analysis highlighted dilated venous channels with myxoid degeneration in the wall with clot, characteristic of BRBNS. The early age of presentation and location are unique based on the literature search of BRBNS. The present report highlights the multiplicity of venous malformations in BRBNS, and the management of this case.

2020 ◽  
Vol 2020 ◽  
pp. 1-5
Author(s):  
Albert Jang ◽  
Kallie N. Kram ◽  
Scott N. Berger ◽  
Mahmoud R. Gaballa ◽  
Lee B. Lu ◽  
...  

Spinal cord compression (SCC) is a rare initial presentation and complication of acute lymphoblastic leukemia (ALL) with nearly all reported cases occurring in the pediatric population. We report a 38-year-old previously healthy man who presented with acute on chronic lower back pain, gait instability, urinary retention, and severe thrombocytopenia. Radiologic examination revealed two soft tissue masses of the thoracic spine associated with compression fractures causing spinal canal narrowing and cord compression. Bone marrow biopsy confirmed the diagnosis of ALL. Immediate initiation of high-dose corticosteroids and systemic chemotherapy resolved the patient’s symptoms without radiation therapy or surgical intervention. After two courses of chemotherapy, the patient achieved complete remission in the bone marrow. Rapid administration of chemotherapy alone in this case resulted in a complete resolution of SCC. Given the rarity of this complication in adults, no standardized treatment has been established. The success of this case recommends chemotherapy as the initial management of SCC in chemotherapy-naïve ALL.


1977 ◽  
Vol 47 (4) ◽  
pp. 626-629 ◽  
Author(s):  
Raymond A. Martin ◽  
Frank M. Howard ◽  
Charles R. Salamone ◽  
David F. Reese

✓ Two patients had the initial complaint of fluctuating paraparesis, which was most evident at menstruation. One patient had a semimonthly fluctuating deficit. Spinal cord compression and ischemia, secondary to the vascular mass, were considered the most likely mechanisms. Blood levels of estrogen and progesterone during the menstrual cycle may have had a contributory effect. Fluctuating spinal cord deficits associated with a consistent portion of the menstrual cycle should alert the physician to the possibility of an arteriovenous malformation of the spinal cord.


2020 ◽  
Vol 48 ◽  
Author(s):  
Vanessa Martins Fayad Milken ◽  
Lilian Hilario Cursino ◽  
Fernanda Gabriela Menegon ◽  
Gustavo Gonçalves Parisi ◽  
Karina Michele Braga ◽  
...  

Background: Plasmacytoma is a neoplasm originating in plasma cells, derived from B lymphocytes. Extramedullary presentation is the most common form of plasmacytoma, mainly in the skin and rarely in other tissues, including the vertebral canal. Extramedullary plasmacytoma (EMP) occurs more frequently in senile dogs and rarely in cats and some dog breeds have predisposition for this type of neoplasm. The aim of this study was to report plasmacytoma in a dog located adjacent to the thoracic vertebrae.Case: An approximately 5-year-old mongrel male with sudden paraplegia resulting from upper motor neuron injury was admitted to a University Hospital. The dog presented a six-centimeter diameter mass in the dorsal region, adjacent to the thoracic vertebrae. The leucogram presented mild leukopenia by eosinopenia and lymphopenia. Myelogram associated with epidurography showed a fill failure in the contrast column between the seventh and ninth thoracic vertebrae, and there were no osteolysis points in the thoracic vertebrae, adjacent to the neoplastic mass. The evaluation of the intervertebral spaces between the fourth to ninth thoracic vertebrae presented no increase in radiopacity, nor alterations in the size of intervertebral spaces, indicating intervertebral disc extrusion. The laterolateral radiographs showed an alignment of the vertebrae and vertebral canal, without presence of bone neoformations on the ventral face of the vertebral bodies. The animal was euthanized and fragments of mass were collected for histological analysis. Macroscopic findings presented a non-encapsulated well-delimited mass, with color ranging from white to reddish. Microscopically, there was proliferation of moderately differentiated round cells in bone and muscle tissues, arranged in mantle, with moderately eosinophilic cytoplasm, and rounded nucleus with eccentric location, coarse chromatin sometimes indistinct. These cells presented moderate anisocytosis and, in general, one mitosis per field. Intradural extramedullary plasmacytoma was diagnosed.Discussion: The present report presented a diagnosis of intradural EMP, generating compression of the spinal cord because of its invasive growth, reaching the canal and, thus, reducing its lumen. The clinical status of the dog was compatible with thoracolumbar lesion of upper motor neuron, which causes paresis and ataxia in pelvic limbs. In this case, it was not possible to establish early diagnosis because the dog had been rescued from the streets recently. Radiographic examination was important to identify the real size of the mass, and the myelography confirmed spinal cord compression. The definitive diagnosis was obtained by histological examination. Although extramedullary plasmacytoma is a low-frequency neoplasm, in the present report, its manifestation was atypical, more aggressive, invading the medullary canal and compressing the spinal cord. Spinal cord compression may lead to degeneration of axons because of alterations in the vascular permeability associated with local inflammatory process, reducing the oxygen supply to nervous tissues, resulting in cellular degeneration. The occurrence of atypical neoplastic growth should be included as a differential diagnosis for lesions in upper motor neuron in cases of spinal cord compression. This is an atypical location for this kind of neoplasia, and cases of medullary compression of neoplastic masses should be included as a differential diagnosis.


BMC Neurology ◽  
2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Michael Axenhus ◽  
Nenad Bogdanovic

Abstract Background Plasmacytomas are rare tumors comprised of neoplastic monoclonal plasma cells and can be found anywhere in the body. Plasmacytomas that involve the nervous system can give rise to diffuse symptoms depending on their location. Patients with confusion or dementia might be difficult to neurologically assess in an acute setting and the subtle symptoms of neurological pathology caused by rare malignancies might go undiagnosed. Case presentation The patient is an 80 year old man presenting to the ER with walking difficulties, pain, and confusion. He underwent neurological evaluation for dementia and was eventually diagnosed with possible Alzheimer’s disease and a malignant plasmacytoma causing spinal cord compression. His CSF sample showed normal amyloid rate and very low Aβ. Following rehabilitation and oncological treatment, his walking ability and confusion improved. Conclusion This case is unique as we demonstrate that spinal cord compression by plasmacytoma can lead to abnormal CSF levels of several known pathology markers for Alzheimer’s disease and neuronal damage. We suggest that highly divergent amyloid CSF levels could be indicative of spinal pathologies affecting CSF circulation. We also suggest closer assessment of elderly confusion patients in ER settings by consultants specialized in neurological disorders.


2020 ◽  
Vol 78 (10) ◽  
pp. 663-664
Author(s):  
Renan Ramon Souza LOPES ◽  
Larissa Soares CARDOSO ◽  
Franz ONISHI

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