scholarly journals Spinal Cord Diffuse Midline Glioma in a 4-Year-Old Boy

2019 ◽  
Vol 6 ◽  
pp. 2329048X1984245 ◽  
Author(s):  
Ashutosh Kumar ◽  
Salman Rashid ◽  
Sumit Singh ◽  
Rong Li ◽  
Leon S. Dure

Objective: We report a child presenting with spinal myelopathy secondary to H3K27M mutant diffuse midline glioma. Case Report: A 4-year-old boy presented with a 3-week history of progressive gait difficulty. Examination revealed bilateral hand and lower extremity weakness, left leg hypertonia with ankle clonus, and a right hemisensory deficit. Magnetic resonance imaging of neuroaxis showed cervical and thoracic spinal cord with expansion and irregular areas of enhancement. Serum and cerebrospinal fluid studies were unremarkable for infectious, autoimmune, inflammatory, and neoplastic causes but showed mild cerebrospinal fluid pleocytosis, hypoglycorrhachia, and high protein level. A thoracic cord biopsy revealed a diffuse midline glioma (World Health Organization grade IV). Consequently, the tumor involved intracranial structures and patient died within 4 months after diagnosis. Conclusion: High-grade spinal cord gliomas are very rare but should be considered in the differential diagnosis of pediatric myelopathy. Tissue biopsy is recommended in indeterminate cases to facilitate diagnosis and to guide management.

2000 ◽  
Vol 92 (2) ◽  
pp. 229-232 ◽  
Author(s):  
Federico Roncaroli ◽  
Bernd W. Scheithauer ◽  
H. Gordon Deen

✓ A case of multiple hemangiomas of the cauda equina nerve roots, conus medullaris, and lower spinal cord is described. The 74-year-old male patient presented with a 9-month history of progressive bilateral leg weakness. He had a history of lymphoma at the age of 39 years and renal cell carcinoma in his early 40s. Neither disease was evident at the time of this presentation. A magnetic resonance image revealed multiple enhancing nodules in the cauda equina region as well as on the pial surface of the lower thoracic spinal cord and conus medullaris. The patient underwent an L2–3 laminectomy. Cauda equina nerve roots were found to be studded with numerous purple nodules, the largest measuring 6 to 8 mm. The nodules were adherent to nerve roots from which they could not be resected. Two lesions were histologically examined and found to be capillary hemangiomas. Twelve months into an uneventful postoperative course, the patient is neurologically unchanged. This unique case might represent a distinct form of hemangiomatosis confined to the cauda equina nerve roots and spinal cord.


2008 ◽  
Vol 45 (6) ◽  
pp. 934-938 ◽  
Author(s):  
C. J. Gibson ◽  
N. M. A. Parry ◽  
R. M. Jakowski ◽  
D. Eshar

A 2–year-old, female hedgehog presented with an 8–month history of progressive, ascending paresis/paralysis and was tentatively diagnosed with wobbly hedgehog syndrome. She died awaiting further diagnostic tests, and the owners consented to postmortem examination. Grossly, the bladder was large and flaccid and the cervical and lumbar spinal cord were regionally enlarged, light grey, and friable with multifocal hemorrhages. The thoracic spinal cord was grossly normal. Microscopically all regions of the spinal cord had similar changes, although the cervical and lumbar sections were most severely affected. These regions were completely effaced by a moderately cellular infiltration of highly pleomorphic polygonal to spindle shaped cells, mineralization, and necrosis, which were most consistent with anaplastic astrocytoma. The thoracic spinal cord white matter was similarly infiltrated by the neoplastic cells, with perivascular extension into the otherwise normal grey matter. A diagnosis of anaplastic astrocytoma was confirmed using immunohistochemical stains that were positive for glial fibrillary acidic protein and S100.


2021 ◽  
Vol 8 ◽  
Author(s):  
Ran Cheng ◽  
Da-Peng Li ◽  
Nan Zhang ◽  
Ji-Yin Zhang ◽  
Di Zhang ◽  
...  

Background: Diffuse midline glioma (DMG) with histone H3 K27M mutation is a recently identified entity documented in the 2016 World Health Organization (WHO) Classification of Tumors of the Central Nervous System. Spinal cord DMGs with H3 K27M-mutant are commonly reported in adults. Herein, we reported a pediatric patient with spinal cord H3 K27M-mutant DMG.Case Report: A 7-year-old girl with 1-month history of neck pain and 3-week history of progressive weakness in the right hand was presented. Spinal magnetic resonance imaging showed an intramedullary lesion with slight enhancement at the C2-7 levels. With intraoperative neuroelectrophysiological monitoring, the lesion was subtotally resected. Histopathological examination revealed a DMG with histone H3 K27M mutation corresponding to WHO grade IV. Postoperatively, the neck pain was relieved, and the upper-extremity weakness remained unchanged. Oral temozolomide was administrated for 7 months, and radiotherapy was performed for 22 courses. After an 18-month follow-up, no tumor recurrence was noted.Conclusion: Spinal cord H3 K27M-mutant DMGs are extremely rare in pediatric patients. Preoperative differential diagnosis is challenging, and surgical resection with postoperative chemoradiotherapy may be an effective treatment.


2000 ◽  
Vol 6 (3) ◽  
pp. 223-234 ◽  
Author(s):  
F. Sala ◽  
Y. Niimi ◽  
A. Berenstein ◽  
V. Deletis

The decision whether or not to embolise during endovascular procedures for arteriovenous malformations (AVMs) of the spinal cord under general anesthesia, relies primarily on neurophysiological results of provocative tests with Lidocaine and short-acting barbiturates. Because of the complex haemodynamics of spinal AVMs, when either sensory (CSEPs) or muscle motor evoked potentials (mMEPs) are used independently, they can mislead the interpretation of provocative tests. This report illustrates the specific but complementary role played by provocative tests using CSEPs and mMEPs during embolisation of a low thoracic spinal cord AVM. We present the case of a 46 year old male with six year history of right lower extremity weakness. At that time, Magnetic Resonance (MR) imaging of the spine disclosed an intramedullary AVM at T11. He remained neurologically stable up to seven months before admission, when he developed sudden onset of low back pain, followed by progressive paraparesis, numbness in lower extremities, urinary retention and fecal incontinence. A new MR imaging study indicated venous thrombosis of the AVM. A two-stage embolisation was performed. During the first procedure, after provocative tests did not affect either CSEPs or mMEPs, an embolisation was performed through a sulco-commisure feeder from the anterior spinal artery (ASA) at T9. Conversely, provocative tests with Lidocaine performed from a right posterior spinal artery (PSA) feeder to the AVM nidus resulted in a significant (>50%) decrease of CSEPs, while mMEPs remained unchanged. The repeatedly positive tests warranted further investigation of the vascular anatomy which disclosed a normal right PSA distal to the nidus; the distal normal PSA was protected with coils. A repeated Lidocaine test was negative and the posterior feeder was embolised with no subsequent changes in CSEPs or mMEPs. After the procedure, the patient experienced only a mild transitory increase in right leg numbness, but no additional motor deficits. Five days later, the embolisation through the ASA feeder at T9 was completed on the basis of negative provocative tests. No additional neurological deficits were observed. Favoring either CSEPs or MEPs during endovascular procedures in the spinal cord is not justified by a solid scientific background. This case report illustrates that monitoring both CSEPs and mMEPs combined with provocative tests allows the safest and most effective embolisation of spinal cord AVMs under general anesthesia.


2019 ◽  
Author(s):  
Toshiki Endo ◽  
Tomoo Inoue ◽  
Shinichiro Sugiyama ◽  
Ryuta Saito ◽  
Teiji Tominaga

Abstract BACKGROUND Spinal cord high-grade glioma has poor prognosis. Especially, no treatment protocols have been established for recurrent cases. OBJECTIVE To apply a novel treatment method, convection-enhanced delivery (CED), for recurrent high-grade glioma. CED can deliver chemotherapeutic agents directly into the intramedullary lesion and possibly lead to remarkable regression of enlarging tumors that are, otherwise, difficult to control. METHODS Two patients developed high-grade glioma in the thoracic spinal cord. Partial resection and chemotherapy and radiotherapy induced remission of the disease. However, following the initial treatment, recurrence was noted in the spinal cord at 6 and 12 mo, respectively. No effective treatment was available for these recurrent lesions. Therefore, the authors decided to use CED to infuse nimustine hydrochloride (ACNU) directly into the spinal cord. During the procedure, the infusion cannula was inserted into the spinal cord lesion under intraoperative computed tomography scan. RESULTS After ACNU CED, successive magnetic resonance imaging confirmed remarkable shrinkages of the tumors in both cases. However, the patient's preinfusion symptoms, including bilateral lower extremity weakness, did not change after the treatment. Importantly, overall survivals of the 2 patients were as long as 67 and 33 mo. CONCLUSION The authors report the first 2 cases of recurrent spinal cord high-grade glioma. ACNU CED dramatically regressed enhanced mass lesions and provided local tumor controls in the spinal cord.


Neurosurgery ◽  
2019 ◽  
Vol 66 (Supplement_1) ◽  
Author(s):  
Toshiki Endo ◽  
Ryuta Saito ◽  
Teiji Tominaga

Abstract INTRODUCTION Spinal cord high-grade glioma has poor prognosis. Especially, no treatment protocols have been established for recurrent cases.This paper describes a novel treatment method, convection enhanced delivery (CED), for recurrent high-grade glioma. CED can deliver chemotherapeutic agents directly into the intramedullary lesion and possibly lead remarkable regression of enlarging tumors that are otherwise difficult to control. METHODS Two patients developed high-grade glioma in the thoracic spinal cord. Partial resection and chemo- and radiotherapy-induced remission of the disease. However, following the initial treatment, recurrence was noted in the spinal cord 6 and 12 mo, respectively. No effective treatment was available for these recurrent lesions. Therefore, the authors decided to use CED to infuse nimustine hydrochloride (ACNU) directly into the spinal cord. During the procedure, the infusion cannula was inserted into the spinal cord lesion under intraoperative computed tomography scan. RESULTS After ACNU CED, successive magnetic resonance imaging confirmed remarkable shrinkages of the tumors in both cases. However, the patient's preinfusion symptoms including bilateral lower extremity weakness, did not change after the treatment. Importantly, overall survivals of the two patients were as long as 67 and 33 mo. CONCLUSION The authors report the first 2 cases of recurrent spinal cord high-grade glioma. ACNU CED dramatically regressed enhanced mass lesions and provided local tumor controls in the spinal cord.


Spine ◽  
1989 ◽  
Vol 14 (6) ◽  
pp. 643-645 ◽  
Author(s):  
SHLOMI CONSTANTINI ◽  
DOV SOFFER ◽  
TALI SIEGEL ◽  
MORDECHAI N. SHALIT

2011 ◽  
Vol 2011 ◽  
pp. 1-4 ◽  
Author(s):  
Gregory Helsen ◽  
Stefaan J. Vandecasteele ◽  
Ludo J. Vanopdenbosch

We describe a farmer who presented with a clinical picture of a transverse thoracic myelitis. MRI showed inflammatory lesions in brain and thoracic spinal cord. Toxocariasis was suspected because of eosinophilia in blood and cerebrospinal fluid, and this diagnosis was confirmed immunologically. He was successfully treated with antihelminthics in combination with corticosteroids. Neurotoxocariasis is rare and diagnosis can be difficult because of the different and atypical clinical manifestations. It should be considered in every case of central neurological syndrome associated with eosinophilia.


Neurosurgery ◽  
2015 ◽  
Vol 77 (3) ◽  
pp. E492-E499 ◽  
Author(s):  
Eduardo Martinez-del-Campo ◽  
Karam Moon ◽  
Samuel Kalb ◽  
Hector Soriano-Baron ◽  
Nicholas Theodore

Abstract BACKGROUND AND IMPORTANCE: Thoracic spinal cord herniation (TSCH) is rare and likely underdiagnosed. It is characterized by ventral herniation of the spinal cord through a dural defect, effacement of the anterior subarachnoid space, and increased posterior subarachnoid space. We present here a case of TSCH diagnosed and surgically treated at Barrow Neurological Institute, along with supplemental intraoperative video. CLINICAL PRESENTATION: A 61-year-old man with a history of progressive myelopathy causing left lower-extremity weakness with associated numbness, impaired gait, foot drop, incontinence, and sexual impotence was referred without any previous treatment. Computed tomographic myelography and magnetic resonance imaging of the thoracic spine showed ventral spinal cord herniation at T3-T4. Neurological monitoring was recorded preoperatively and intraoperatively. The patient underwent left-sided posterolateral exploration via T3-T4 laminectomies and costotransversectomy for intradural cord release/detethering of the spinal cord with additional superior and inferior extension and repair of the dural defect. Arthrodesis was not considered necessary. After cord release, motor evoked potentials showed immediate improvement from baseline. Dural duplication was considered the cause of TSCH in this case. Total reduction of herniation was evident in postoperative images. The postoperative course was uneventful, and at the last follow-up, the patient had regained ambulation and sphincter control. CONCLUSION: Anterior displacement of the thoracic spinal cord should elicit consideration of herniation to prevent misdiagnosis and inadequate surgery. Surgical cord release and enlargement of the dural defect are safe and associated with good clinical outcomes.


Sign in / Sign up

Export Citation Format

Share Document