PATH-12. CLINICAL AND PATHOLOGICAL ANALYSIS OF 43 PATIENTS WITH EPITHELIOID GLIOBLASTOMA

2021 ◽  
Vol 23 (Supplement_6) ◽  
pp. vi117-vi117
Author(s):  
Junjie Zhen ◽  
Mingyao Lai ◽  
Jiangfen Zhou ◽  
Shaoqun Li ◽  
Linbo Cai
Keyword(s):  
Spinal Cord ◽  
Average Diameter ◽  
Common Symptom ◽  
Karnofsky Performance Score ◽  
Wild Type ◽  
Limb Weakness ◽  
Intracranial Metastasis ◽  
Imaging Characteristics ◽  
Disturbance Of Consciousness ◽  
Epithelioid Glioblastoma

Abstract BACKGROUND AND PURPOSE Epithelioid glioblastoma (eGBM) is a rare and aggressive subtype of glioblastoma. The clinical characteristics, pathological features and radiological findingsare still not well characterized. METHODS The clinical data of 43 patients with eGBM confirmed pathologically after surgery in Guangdong Sanjiu Brain Hospital from June 2015 to January 2021 were reviewed, and to investigate the clinical, pathological and imaging characteristics of the eGBM. RESULTS The range of patients' age was from 5 to 70 years (median 39 years). There were 24 males and 19 females. The median karnofsky performance score (KPS）at diagnosis was 70 (40-90). The most common symptom were headache (81.4%), and other common symptoms included nausea or vomiting (44.1%), limb weakness (30.2%), disturbance of consciousness (20.9%) and seizures (9.3%). Most of the tumors (97.6%) were located in the cerebral hemisphere ,except for one case located in the cerebellum,and with an average diameter of 5.3 cm (2.5-9.4 cm). There were 2 cases of intracranial metastasis and 7 cases of spinal cord metastasis in MRI. In pathologic examination, immunohistochemistry showed all the patients were IDH1 wild-type (43/43) and H3K27M wild-type(34/34). 25 (58.1%) of cases harbored BRAF mutation and 19 (44.2%) cases of MGMT positive. Most of patients were positive for GFAP, P53, Olig-2 and ATRX. CONCLUSION Epithelioid glioblastoma is more common in adults, most of which are located on the supratentorial, intracranial and spinal cord dissemination may occur. Half of patients showed BRFA mutation and maybe benefit from targeted therapy.These findings may help clinicians understand and treat epithelioid glioblastoma better.

scholarly journals Glial Fibrillary Acidic Protein Astrocytopathy in Pediatric Patients: A Retrospective Study

2021 ◽  
Vol 8 ◽  
Author(s):  
Haixia Huang ◽  
Ke Bai ◽  
Yueqiang Fu ◽  
Siwei Lu ◽  
Yunni Ran ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Glial Fibrillary Acidic Protein ◽  
Pediatric Patients ◽  
Clinical Symptoms ◽  
Acidic Protein ◽  
Gadolinium Enhancement ◽  
Urine Retention ◽  
Limb Weakness ◽  
Disturbance Of Consciousness ◽  
Intravenous Steroids

Autoimmune glial fibrillary acidic protein astrocytopathy is a novel form of autoimmune meningoencephalitis related to GFAP autoantibodies. This condition is still being characterized, and few pediatric patients have been identified. Here, we report three patients presenting with fever, nausea, and headache, following progressive disturbance of consciousness, limb weakness, dyspnea, or urine retention. MRI analysis revealed that T2-hyperintense lesions, or enhancement of the meninges and spinal cord. CSF and serum analyses revealed they were positive for GFAP antibody, confirming GFAP astrocytopathy diagnosis. Treating the patients with IVIG, with or without intravenous steroids, gradually improved their clinical symptoms. Our findings indicate that GFAP astrocytopathy should be considered in children who are clinically diagnosed with meningoencephalitis, whether or not myelitis is present, and if the MRI reveals enhancement of meninges or spinal cord, T2-hyperintense lesions, or a pattern of linear perivascular gadolinium enhancement. Suspected cases should be tested for GFAP antibody as soon as possible because these patients may benefit from immunotherapy.

Abnormal Spinal Cord Myelination due to Oligodendrocyte Dysfunction in a Model of Huntington’s Disease

2021 ◽  
pp. 1-8
Author(s):  
Costanza Ferrari Bardile ◽  
Harwin Sidik ◽  
Reynard Quek ◽  
Nur Amirah Binte Mohammad Yusof ◽  
Marta Garcia-Miralles ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Oligodendrocyte Progenitor Cells ◽  
Wild Type ◽  
Specific Expression ◽  
Relative Contribution ◽  
Related Proteins ◽  
The Impact ◽  
Cervical Area

Background: The relative contribution of grey matter (GM) and white matter (WM) degeneration to the progressive brain atrophy in Huntington’s disease (HD) has been well studied. The pathology of the spinal cord in HD is comparatively less well documented. Objective: We aim to characterize spinal cord WM abnormalities in a mouse model of HD and evaluate whether selective removal of mutant huntingtin (mHTT) from oligodendroglia rescues these deficits. Methods: Histological assessments were used to determine the area of GM and WM in the spinal cord of 12-month-old BACHD mice, while electron microscopy was used to analyze myelin fibers in the cervical area of the spinal cord. To investigate the impact of inactivation of mHTT in oligodendroglia on these measures, we used the previously described BACHDxNG2Cre mouse line where mHTT is specifically reduced in oligodendrocyte progenitor cells. Results: We show that spinal GM and WM areas are significantly atrophied in HD mice compared to wild-type controls. We further demonstrate that specific reduction of mHTT in oligodendroglial cells rescues the atrophy of spinal cord WM, but not GM, observed in HD mice. Inactivation of mHTT in oligodendroglia had no effect on the density of oligodendroglial cells but enhanced the expression of myelin-related proteins in the spinal cord. Conclusion: Our findings demonstrate that the myelination abnormalities observed in brain WM structures in HD extend to the spinal cord and suggest that specific expression of mHTT in oligodendrocytes contributes to such abnormalities.

Improving sit-to-stand transition by the saddle-assistive device in the spinal cord injury: A case study

2021 ◽  
pp. 095441192110033
Author(s):  
Akbar Hojjati Najafabadi ◽  
Saeid Amini ◽  
Farzam Farahmand
Keyword(s):  
Spinal Cord ◽  
Spinal Cord Injury ◽  
Lower Limb ◽  
Reaction Force ◽  
Assistive Device ◽  
Limb Weakness ◽  
Sit To Stand ◽  
Lower Limb Weakness ◽  
Wide Range ◽  
Cord Injury

Physical problems caused by fractures, aging, stroke, and accidents can reduce foot power; these, in the long term, can dwindle the muscles of the waist, thighs, and legs. These conditions provide the basis for the invalidism of the harmed people. In this study, a saddle-walker was designed and evaluated to help people suffering from spinal cord injury and patients with lower limb weakness. This S-AD works based on body weight support against the previously report designs. This saddle-walker consisted of a non-powered four-wheel walker helping to walk and a powered mechanism for the sit-to-stand (STS) transfer. A set of experiments were done on the STS in the use of the standard walker and the saddle-assistive device(S-AD). A comparison of the results showed that this device could reduce the vertical ground reaction force (GRF) of the legs up to 70%. Using this device could help a wide range of patients with lower limb weakness and SCI patients in changing from sitting to standing.

Truncated wild-type SOD1 and FALS-linked mutant SOD1 cause neural cell death in the chick embryo spinal cord

2006 ◽  
Vol 21 (1) ◽  
pp. 194-205 ◽  
Author(s):  
Ghanashyam D. Ghadge ◽  
Lijun Wang ◽  
Kamal Sharma ◽  
Anna Liza Monti ◽  
Vytas Bindokas ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Cell Death ◽  
Chick Embryo ◽  
Neural Cell ◽  
Wild Type ◽  
Mutant Sod1

Insulin-like growth factor I treatment reduces clinical deficits and lesion severity in acute demyelinating experimental autoimmune encephalomyelitis

1995 ◽  
Vol 1 (1) ◽  
pp. 2-9 ◽  
Author(s):  
X Liu ◽  
D-L Yao ◽  
Hde F. Webster
Keyword(s):  
Spinal Cord ◽  
Autoimmune Encephalomyelitis ◽  
Limb Weakness ◽  
Inflammatory Lesions ◽  
Factor I ◽  
Immune Mediated ◽  
Igf I ◽  
Blood Spinal Cord Barrier ◽  
Clinical Deficits ◽  
Experimental Autoimmune

Our goal was to test the effects of insulin-like growth factor I (IGF-I) treatment on clinical deficits, lesion number and lesion size in acute demyelinating experimental autoimmune encephalomyelitis (EAE) induced in Lewis rats with an emulsion containing guinea pig spinal cord. In this EAE model, there is severe immune-mediated demyelination, which resembles that seen in actively demyelinating MS lesions. On day 12–13 after EAE induction, a total of 23 pairs of rats with the same mild degree of tail and hind limb weakness were given either intravenous IGF-I or placebo twice daily for 8 days. The daily IGF-I dose used in the first trial was 200 μg (about 0.6 mgkg-1) and in the second and third trials was 1 mg (about 3.0 mgkg-1). IGF-I treatment reduced permeability of the blood-spinal cord barrier to Evans blue-albumin. Maximum clinical deficit scores of IGF-I-treated rats were significantly lower and treated rats recovered faster than controls. IGF-I treatment produced significant reductions in weight loss and hind limb weakness. Treatment also improved treadmill walking, stride length and climbing performance. Morphometric analysis showed that spinal cord inflammatory lesions were significantly smaller and fewer in IGF-I-treated rots. The higher IGF-I dose produced a greater reduction in clinical and pathological deficits. We conclude that IGF-I treatment promotes clinical recovery by reducing EAE-induced blood-spinal cord barrier changes and the associated immune-mediated inflammatory lesions. Our results suggest that IGF-I may be useful in treating patients with multiple sclerosis and other demyelinating diseases.

Neurological manifestations, imaging characteristics, and surgical outcome of intraspinal osteochondroma

2010 ◽  
Vol 12 (5) ◽  
pp. 474-489 ◽  
Author(s):  
Iraj Lotfinia ◽  
Payman Vahedi ◽  
R. Shane Tubbs ◽  
Mostafa Ghavame ◽  
Ali Meshkini
Keyword(s):  
Spinal Cord ◽  
Spinal Cord Compression ◽  
Surgical Outcome ◽  
Posterior Approach ◽  
English Literature ◽  
Cord Compression ◽  
Surgical Removal ◽  
Hereditary Multiple Exostoses ◽  
Imaging Characteristics ◽  
Google Search

ObjectSpinal osteochondromas (OCs) are rare and can originate as solitary lesions or in the context of hereditary multiple exostoses. Concurrent spinal cord compression is a very rare entity. The purpose of this study was to evaluate the authors' 10-year experience with the imaging characteristics and surgical outcome in patients with symptomatic spinal OC.MethodsBetween 1997 and 2007, 8 consecutive cases of symptomatic intraspinal OC with documented spinal cord compression were treated surgically. These patients were analyzed with regard to presentation, imaging, and outcome. The relevant English literature was reviewed using MEDLINE and Google search engines.ResultsThree patients had cervical, 2 had thoracic, and 3 had lumbar lesions. Classic MR imaging characteristics were rarely found. Multiple hereditary exostoses were equally responsible for cervical, thoracic, and lumbar lesions (33%). The origin of the lesion was from the pedicle (25%), lamina (25%), vertebral body (25%), and superior or inferior facets (25%). A posterior approach to the spine was used in 6 patients, and a combined anterior and posterior approach with fusion was performed for 2 thoracic lesions. Surgical outcome was satisfactory in 75% of patients. The prognosis was poor in the patients with thoracic lesions.ConclusionsIn the authors' experience, early detection and surgical removal in cases of symptomatic spinal OC is a key element for the best outcome. Posterior approaches are generally sufficient. The chronicity of symptoms may limit functional recovery postoperatively, especially with cervical and thoracic lesions.

scholarly journals Herpes Simplex Virus Latency-Associated Transcript Sequence Downstream of the Promoter Influences Type-Specific Reactivation and Viral Neurotropism

2007 ◽  
Vol 81 (12) ◽  
pp. 6605-6613 ◽  
Author(s):  
Andrea S. Bertke ◽  
Amita Patel ◽  
Philip R. Krause
Keyword(s):  
Spinal Cord ◽  
Herpes Simplex Virus ◽  
Herpes Simplex ◽  
Acute Infection ◽  
Central Nervous System Infection ◽  
Sensory Nerve ◽  
Lumbar Spinal Cord ◽  
Wild Type ◽  
Simplex Virus ◽  
Hsv 1

ABSTRACT Herpes simplex virus (HSV) establishes latency in sensory nerve ganglia during acute infection and may later periodically reactivate to cause recurrent disease. HSV type 1 (HSV-1) reactivates more efficiently than HSV-2 from trigeminal ganglia while HSV-2 reactivates more efficiently than HSV-1 from lumbosacral dorsal root ganglia (DRG) to cause recurrent orofacial and genital herpes, respectively. In a previous study, a chimeric HSV-2 that expressed the latency-associated transcript (LAT) from HSV-1 reactivated similarly to wild-type HSV-1, suggesting that the LAT influences the type-specific reactivation phenotype of HSV-2. To further define the LAT region essential for type-specific reactivation, we constructed additional chimeric HSV-2 viruses by replacing the HSV-2 LAT promoter (HSV2-LAT-P1) or 2.5 kb of the HSV-2 LAT sequence (HSV2-LAT-S1) with the corresponding regions from HSV-1. HSV2-LAT-S1 was impaired for reactivation in the guinea pig genital model, while its rescuant and HSV2-LAT-P1 reactivated with a wild-type HSV-2 phenotype. Moreover, recurrences of HSV-2-LAT-S1 were frequently fatal, in contrast to the relatively mild recurrences of the other viruses. During recurrences, HSV2-LAT-S1 DNA increased more in the sacral cord compared to its rescuant or HSV-2. Thus, the LAT sequence region, not the LAT promoter region, provides essential elements for type-specific reactivation of HSV-2 and also plays a role in viral neurotropism. HSV-1 DNA, as quantified by real-time PCR, was more abundant in the lumbar spinal cord, while HSV-2 DNA was more abundant in the sacral spinal cord, which may provide insights into the mechanism for type-specific reactivation and different patterns of central nervous system infection of HSV-1 and HSV-2.

scholarly journals Perineural invasion in intramedullary spinal cord metastasis

2020 ◽  
Vol 102 (5) ◽  
pp. e94-e96 ◽  
Author(s):  
N Jayakumar ◽  
H Ismail ◽  
S Athar ◽  
N Ashwood
Keyword(s):  
Spinal Cord ◽  
Brachial Plexus ◽  
Perineural Invasion ◽  
Surgical Excision ◽  
Cervical Cord ◽  
Limb Weakness ◽  
Intramedullary Spinal Cord ◽  
Spinal Cord Metastasis ◽  
Plexus Lesion ◽  
Intramedullary Spinal Cord Metastasis

A woman in her late sixties was referred to the orthopaedic clinic with progressive lower limb weakness and gait disturbance. She was known to have breast cancer with pre-existing infiltrative disease in the left brachial plexus. Magnetic resonance imaging of the spine revealed an intramedullary spinal cord metastasis in the lower cervical cord at C6–C7. She underwent surgical excision but died within six weeks of surgery. This rare case of an intramedullary spinal cord metastasis highlights the extremely poor prognosis in this condition as well as the possibility of perineural invasion into the spinal cord from the brachial plexus lesion. A detailed discussion of the literature on intramedullary spinal cord metastases is also presented.

scholarly journals CCL3 contributes to secondary damage after spinal cord injury

2020 ◽  
Vol 17 (1) ◽  
Author(s):  
Nicolas Pelisch ◽  
Jose Rosas Almanza ◽  
Kyle E. Stehlik ◽  
Brandy V. Aperi ◽  
Antje Kroner
Keyword(s):  
Spinal Cord ◽  
Spinal Cord Injury ◽  
Inflammatory Response ◽  
Lesion Size ◽  
Secondary Injury ◽  
Wild Type ◽  
Locomotor Recovery ◽  
Secondary Damage ◽  
Cord Injury

Abstract Background Secondary damage after spinal cord injury (SCI) is characterized by a cascade of events including hemorrhage, apoptosis, oxidative stress, and inflammation which increase the lesion size which can influence the functional impairment. Thus, identifying specific mechanisms attributed to secondary injury is critical in minimizing tissue damage and improving neurological outcome. In this work, we are investigating the role of CCL3 (macrophage inflammatory protein 1-α, MIP-1α), a chemokine involved in the recruitment of inflammatory cells, which plays an important role in inflammatory conditions of the central and peripheral nervous system. Methods A mouse model of lower thoracic (T11) spinal cord contusion injury was used. We assessed expression levels of CCL3 and its receptors on the mRNA and protein level and analyzed changes in locomotor recovery and the inflammatory response in the injured spinal cord of wild-type and CCL3−/− mice. Results The expression of CCL3 and its receptors was increased after thoracic contusion SCI in mice. We then examined the role of CCL3 after SCI and its direct influence on the inflammatory response, locomotor recovery and lesion size using CCL3−/− mice. CCL3−/− mice showed mild but significant improvement of locomotor recovery, a smaller lesion size and reduced neuronal damage compared to wild-type controls. In addition, neutrophil numbers as well as the pro-inflammatory cytokines and chemokines, known to play a deleterious role after SCI, were markedly reduced in the absence of CCL3. Conclusion We have identified CCL3 as a potential target to modulate the inflammatory response and secondary damage after SCI. Collectively, this study shows that CCL3 contributes to progressive tissue damage and functional impairment during secondary injury after SCI.

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