Differentiation between spinal cord diffuse midline glioma with histone H3 K27M mutation and wild type: comparative magnetic resonance imaging

2019 ◽  
Vol 61 (3) ◽  
pp. 313-322 ◽  
Author(s):  
Jo Sung Jung ◽  
Yoon Seong Choi ◽  
Sung Soo Ahn ◽  
Seong Yi ◽  
Se Hoon Kim ◽  
...  
2021 ◽  
pp. 231-232
Author(s):  
Ivan D. Carabenciov ◽  
Michael W. Ruff

A 48-year-old woman sought care for progressive right arm and hand pain with radial nerve–distribution sensory loss. She had a past history of multiple prior athletics-associated, musculoskeletal, upper cervical spine injuries. Her symptoms were initially attributed to a right C6 radiculopathy. Over the next several months, the sensory loss spread to involve the entire right hand and subsequently the entire left hand. She had development of diffuse right hand weakness and a sense of imbalance that was particularly prominent while in the dark. Finally, she experienced progressive constipation and urinary retention. Magnetic resonance imaging of the cervical spine showed an expanded cervical spinal cord from C3 through C7-T1 with diffuse T2-hyperintense changes and heterogeneous gadolinium enhancement most prominent at C5-6. In combination with a congenitally small central canal, severe central canal narrowing was seen at C5-6 and moderate narrowing at C4-5. Magnetic resonance imaging of the brain and thoracic spine were normal, and magnetic resonance imaging of the lumbar spine indicated only mild lumbar spondylosis. On suspicion of a spinal cord neoplasm with a secondary compressive myelopathy, C3 through C7 laminectomy and posterior instrumented fusion from C2 through T1 was performed, with a biopsy obtained at the C5-6 level. Postoperatively, her gait and right upper extremity pain improved. The biopsy showed atypical glial cells. Neurofilament staining demonstrated an infiltrative pattern. Atypical cells were positive for glial fibrillary acidic protein, oligodendrocyte transcription factor 2, and a Lys27Met sequence variation of histone H3, with overexpression of p53 on immunohistochemical staining. There was loss of H3 K27-trimethylation on the infiltrating cells, corresponding to the presence of Lys27Met sequence variation of histone H3. These findings were diagnostic for diffuse midline glioma with Lys27Met sequence variation of histone H3 (World Health Organization grade IV). A total of 5,400 cGy of photon radiation was delivered in 30 fractions over 42 days. She was subsequently treated with an oral histone deacetylase inhibitor, panobinostat, for 12 months. During this time, she had clinical response to treatment and reported improvement in balance and numbness. Follow-up magnetic resonance imaging at 3 months showed a slight decrease in the size of the mass, and this response was sustained 1 year post radiotherapy. Diffuse midline gliomas that contain Lys27Met sequence variation of histone H3are incurable, often inoperable, midline brain tumors that are most commonly seen in the pediatric population. These tumors can also occur in adult patients and are considered high grade, even in the absence of features such as necrosis or microvascular proliferation.


Animals ◽  
2021 ◽  
Vol 11 (6) ◽  
pp. 1702
Author(s):  
Eiji Naito ◽  
Kohei Nakata ◽  
Yukiko Nakano ◽  
Yuta Nozue ◽  
Shintaro Kimura ◽  
...  

Canine degenerative myelopathy (DM) is a progressive and fatal neurodegenerative disease. However, a definitive diagnosis of DM can only be achieved by postmortem histopathological examination of the spinal cord. The purpose of this study was to investigate whether the volumetry of DRG using the ability of water-excitation magnetic resonance imaging (MRI) to visualize the DRG in dogs has premortem diagnostic value for DM. Eight dogs with DM, twenty-four dogs with intervertebral disc herniation (IVDH), and eight control dogs were scanned using a 3.0-tesla MRI system, and water-excitation images were obtained to visualize and measure the volume of DRG, normalized by body surface area. The normalized mean DRG volume between each spinal cord segment and mean volume of all DRG between T8 and L2 in the DM group was significantly lower than that in the control and the IVDH groups (P = 0.011, P = 0.002, respectively). There were no correlations within the normalized mean DRG volume between DM stage 1 and stage 4 (rs = 0.312, P = 0.128, respectively). In conclusion, DRG volumetry by the water-excitation MRI provides a non-invasive and quantitative assessment of neurodegeneration in DRG and may have diagnostic potential for DM.


Neurosurgery ◽  
2006 ◽  
Vol 58 (6) ◽  
pp. 1081-1089 ◽  
Author(s):  
John Sinclair ◽  
Steven D. Chang ◽  
Iris C. Gibbs ◽  
John R. Adler

Abstract OBJECTIVE: Intramedullary spinal cord arteriovenous malformations (AVMs) have an unfavorable natural history that characteristically involves myelopathy secondary to progressive ischemia and/or recurrent hemorrhage. Although some lesions can be managed successfully with embolization and surgery, AVM size, location, and angioarchitecture precludes treatment in many circumstances. Given the poor outlook for such patients, and building on the successful experience with radiosurgical ablation of cerebral AVMs, our group at Stanford University has used CyberKnife (Accuray, Inc., Sunnyvale, CA) stereotactic radiosurgery (SRS) to treat selected spinal cord AVMs since 1997. In this article, we retrospectively analyze our preliminary experience with this technique. METHODS: Fifteen patients with intramedullary spinal cord AVMs (nine cervical, three thoracic, and three conus medullaris) were treated by image-guided SRS between 1997 and 2005. SRS was delivered in two to five sessions with an average marginal dose of 20.5 Gy. The biologically effective dose used in individual patients was escalated gradually over the course of this study. Clinical and magnetic resonance imaging follow-up were carried out annually, and spinal angiography was repeated at 3 years. RESULTS: After a mean follow-up period of 27.9 months (range, 3–59 mo), six of the seven patients who were more than 3 years from SRS had significant reductions in AVM volumes on interim magnetic resonance imaging examinations. In four of the five patients who underwent postoperative spinal angiography, persistent AVM was confirmed, albeit reduced in size. One patient demonstrated complete angiographic obliteration of a conus medullaris AVM 26 months after radiosurgery. There was no evidence of further hemorrhage after CyberKnife treatment or neurological deterioration attributable to SRS. CONCLUSION: This description of CyberKnife radiosurgical ablation demonstrates its feasibility and apparent safety for selected intramedullary spinal cord AVMs. Additional experience is necessary to ascertain the optimal radiosurgical dose and ultimate efficacy of this technique.


Neurosurgery ◽  
1991 ◽  
Vol 29 (5) ◽  
pp. 651-657 ◽  
Author(s):  
Jacques Brotchi ◽  
Olivier Dewitte ◽  
Danielle Balériaux ◽  
Arlette Vandesteene ◽  
Christian Raftopoulos ◽  
...  

Abstract Between January 1984 and December 1990. 65 intramedullary spinal cord tumors were diagnosed and operated on. In this series, all patients underwent magnetic resonance imaging investigations and were operated on with the Cavitron ultrasonic surgical aspirator whenever necessary. Major surgical difficulties have been found in patients previously treated by radiotherapy with or without biopsy. We found magnetic resonance imaging to be a highly sensitive imaging procedure and the method of choice for visualizing tumors within the spinal cord. Nevertheless, accurate diagnosis may only be suggested by magnetic resonance imaging, rather than made definitively. Surgery is necessary in every case in order to obtain a definite diagnosis. Radical surgery can be performed when a plane exists between the tumor and the normal spinal cord: biopsy or debulking with the Cavitron ultrasonic surgical aspirator should be performed when the tumor is infiltrative. We have performed 33 so-called total resections, 22 partial resections, and 10 biopsies, among which 5 were performed on lipomas. Surgical results were assessed at 3 months after surgery, showing 35 improvements (53%), 24 stabilizations (37%), and 6 deteriorations (10%).


Spinal Cord ◽  
2014 ◽  
Vol 52 (10) ◽  
pp. 769-773 ◽  
Author(s):  
H Arima ◽  
T Hasegawa ◽  
D Togawa ◽  
Y Yamato ◽  
S Kobayashi ◽  
...  

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