Rapid Neurological Deterioration Research Articles

Outcomes of patients with intermediate-risk neuroblastoma presenting with motor deficits relating to intraspinal tumor extension: A report from the Children's Oncology Group study ANBL0531.

Tumor invasion of the spinal canal is detected radiographically in approximately 15% of patients with newly diagnosed neuroblastoma (NB). The optimal clinical approach to maintain excellent survival outcomes while minimizing long-term sequelae is yet to be defined. Patients with intermediate-risk neuroblastoma (IR-NB) and radiographically identified intraspinal tumors who were treated on the Children's Oncology Group study ANBL0531 were studied prospectively to evaluate neurologic outcomes related to cord compression. Patients were defined as being symptomatic versus asymptomatic based on reporting of neurologic motor deficits at diagnosis. Patient characteristics, tumor biology, chemotherapy treatment, surgical interventions, and neurologic and disease outcomes are reported. Of the 92 patients with intraspinal tumors, 42 (46%) were symptomatic and most (73%) had complete resolution of symptoms. Age, degree of motor deficit, and duration of symptoms at diagnosis were not associated with complete resolution. While symptomatic patients were more likely to undergo upfront laminectomy, laminectomy was not associated with improvement of motor symptoms. Administration of additional chemotherapy beyond initial treatment assigned per protocol to achieve the treatment end point was not associated with achieving symptom resolution. Patients presenting with motor deficits due to intraspinal tumor had excellent survival and favorable neurologic outcomes, with the majority reporting complete resolution of motor symptoms regardless of severity and duration of symptoms at diagnosis or neurosurgical intervention. Prompt diagnosis and initiation of first-line chemotherapy treatment remain priority, while neurosurgical intervention should be reserved for patients with rapid neurologic deterioration. Biology-based therapy and tumor response should continue to be used to maintain favorable outcomes.

Spirituality in Professional Patient-Centered Care for Adults with Primary Brain Tumors: An Exploratory Scoping Review.

People with primary brain tumors face spiritual challenges due to neuro-cognitive disturbances such as aphasia and rapid neurological deterioration. This scoping review aimed to map the evidence regarding professional spiritual care in this neuro-oncological population. The literature search was conducted utilizing two databases (PubMed, CINAHL) and two peer-reviewed journals ('Spiritual Care', 'Journal of Religion and Health'). In total, 27 studies were analyzed, and five thematic categories were identified: (1) spiritual needs, (2) integrating the spiritual dimension in care, (3) spiritual care generalist interventions, (4) spiritual care specialist interventions, (5) assessments. Early integration of spiritual care and specific training are important.

The HALP (hemoglobin, albumin, lymphocyte, and platelet) score is associated with hemorrhagic transformation after intravenous thrombolysis in patients with acute ischemic stroke.

Hemorrhagic transformation (HT) after intravenous thrombolysis (IVT) with rt-PA can precipitate rapid neurological deterioration, poor prognosis, and even death. The HALP score (hemoglobin, albumin, lymphocyte, and platelet) is a novel indicator developed to reflect both systemic inflammation and the nutritional status of patients. The goal of this study was to reveal the relationship between the HALP score and the risk of HT after IVT in people with acute ischemic stroke (AIS). A total of 753 patients with AIS were included in this study. Patients were divided into quartiles according to baseline HALP score. The HALP score was calculated as follows: hemoglobin (g/L) × albumin (g/L) × lymphocytes (/L)/platelets (/L). Binary logistic regression was used to reveal the connection between HALP score and HT. The baseline HALP score were significantly lower in the HT than non-HT patients (p < 0.001). The HALP score were divided into four quartiles: Q1 (<27.4), Q2 (27.4-37.6), Q3 (37.7-49.6), Q4 (>49.6), respectively. Moreover, the severity of HT increased with decreasing HALP level (p < 0.001). In multivariable logistic regression, taking the Q4 as the reference, the association between Q1 and HT remained, after adjusting for confounding variables [odds ratio (OR) = 3.197, 95% confidence interval (CI) = 1.634-6.635, p = 0.003]. The HALP value can predict the HT risk after IVT in patients with AIS. A lower HALP level was associated with an increased severity of HT post-IVT.

Comparative Efficacy and Safety of External Ventricular Drains and Intraparenchymal Pressure Monitors for Intracranial Pressure Monitoring in Traumatic Brain Injury: A Systematic Review and Meta-analysis.

In the management of traumatic brain injury (TBI), intracranial pressure monitoring (ICPm) is crucial for the timely management of severe cases that show rapid neurological deterioration. External ventricular drains (EVDs) and intraparenchymal pressure monitors (IPMs) are the primary methods used in this setting; however, the debate over their comparative efficacy persists, primarily because of reliance on observational study data. This underscores the need for a meta-analysis to guide clinical decision-making. This study-level meta-analysis aims to assess and compare the efficacy and safety of EVDs versus IPMs in the management of TBI. A database search was conducted until February 13, 2024, to identify studies reporting clinical outcomes of patients with TBI who underwent ICPm with either EVD or IPM. Primary outcomes included mortality, ICPm duration, length of stay, and complications. From an initial pool of 537 articles, eight studies (six retrospective cohort studies and two prospective cohort studies), encompassing 7080 patients, met our inclusion criteria. Mortality rates showed no significant difference between groups (risk ratio 1.11 [95% confidence interval (CI) 0.86to 1.42], p = 0.42). Patients monitored with IPM had shorter intensive care unit length of stay (mean difference 0.90 [95% CI 0.21to1.59], p = 0.01) and ICPm duration (mean difference 0.79 [95% CI 0.33to1.24], p = 0.0007), with a higher risk of requiring surgical decompression. Monitoring-related complications were similar across the two groups. Our findings suggest that EVD and IPM provide similar outcomes in terms of mortality. However, IPM may offer significant advantages in reducing the duration of ICPm and intensive care unit length of stay. EVD may be preferable for certain mid-term to long-term monitoring. The predominance of observational studies in the current literature highlights the need for further clinical trials to compare these interventions.

Question: rapid neurological deterioration.

Question: rapid neurological deterioration.

Rehabilitation in People Living with Glioblastoma: A Narrative Review of the Literature.

Glioblastoma is the most common primary malignant brain tumor. While preliminary data point to the positive effects of rehabilitation for patients with glioblastoma, there are unique challenges for clinicians working with this population, including limited life expectancy and/or rapid neurological deterioration. The aim of this article is to review the literature on rehabilitation of adults with glioblastoma, including the feasibility of interventions, their effectiveness, as well as the current clinical practice. The reviewed literature suggests that rehabilitation has been found beneficial for improving the functional prognosis and quality of life of adults with glioblastoma and is desired by patients. We summarize the qualitative evidence regarding healthcare professionals' and patients' perspectives on the use of supportive care services. We conclude there is a need for the design of effective rehabilitation programs for patients with glioblastoma, as well as for the development of glioblastoma-specific clinical guidelines for rehabilitation practitioners.

External validation and comparison of MBE, EDEMA, and modified EDEMA scores for predicting malignant cerebral EDEMA in Chinese patients with large hemispheric infarction patients without revascularization

BackgroundMalignant cerebral edema (MCE) is a severe condition characterized by rapid neurological deterioration and a potentially poor prognosis. Scoring systems including the malignant brain edema (MBE) score, Enhanced Detection of Edema in Malignant Anterior Circulation Stroke score (EDEMA), and modified EDEMA score, have been developed to predict MCE in patients with large hemispheric infarction (LHI). We aimed to externally validate and comparethe predictive efficacy of these scores in LHI patients within 48 h of onset and not undergoing reperfusion therapy. MethodsDemographic, clinical and radiological data were retrospectively collected from LHI patients within 48 h of onset and not receiving reperfusion therapy. Patients were divided into MCE and non-MCE group. The calibration, discrimination, and clinical practicability of the three scores were verified using Hosmer-Lemeshow goodness-of-fit test, receiver operating characteristic (ROC) curve analysis and decision curve analysis (DCA), respectively. Finally, continuous net reclassification improvement (NRI) and integrated discrimination improvement (IDI) were applied to determine the discrimination performance of the three scores. ResultsA total of 314 patients were included in the study, with 122 cases being MCE patients. The Hosmer–Lemeshow goodness-of-fit test showed excellent fitting ability across the MBE (p = 0.36), EDEMA (p = 0.61), and modified EDEMA scores (p = 0.62) in our patients. The MBE, EDEMA, and modified EDEMA scores had the AUCs of 0.855 (95 % CI 0.818–0.898), 0.782 (95 % CI 0.727–0.837) and 0.878 (95 % CI 0.844–0.919) respectively. The MBE (NRI, 0.33; 95 % CI, 0.11–0.56, p = 0.003 and IDI, 0.11; 95 % CI, 0.03–0.18; p = 0.004) and modified EDEMA scores (NRI, 1.10; 95 % CI, 0.94–1.26; p < 0.001 and IDI, 0.17; 95 % CI, 0.13–0.20, p < 0.001) showed better performance than the EDEMA score. DCA demonstrated that the modified EDEMA score outperformed the other two scores, possessing heightened clinical usefulness. ConclusionsThe MBE, EDEMA, and modified EDEMA scores for predicting MCE are also applicable in non-revascularization LHI patients within 48 h of onset. Both the MBE and modified EDEMA scores demonstrated higher predictive validity as predictive tools for MCE in LHI patients than the EDEMA score. Furthermore, the modified EDEMA score could be a suitable prediction tool in Chinese patients for its excellent clinical utility.

Evaluation of safety and early efficacy of AAV gene therapy in mouse models of vanishing white matter disease

Leukoencephalopathy with vanishing white matter (VWM) is a progressive incurable white matter disease that most commonly occurs in childhood and presents with ataxia, spasticity, neurological degeneration, seizures, and premature death. A distinctive feature is episodes of rapid neurological deterioration provoked by stressors such as infection, seizures, or trauma. VWM is caused by autosomal recessive mutations in one of five genes that encode the eukaryotic initiation factor 2B complex, which is necessary for protein translation and regulation of the integrated stress response. The majority of mutations are in EIF2B5. Astrocytic dysfunction is central to pathophysiology, thereby constituting a potential therapeutic target. Herein we characterize two VWM murine models and investigate astrocyte-targeted adeno-associated virus serotype 9 (AAV9)-mediated EIF2B5 gene supplementation therapy asa therapeutic option for VWM. Our results demonstrate significant rescue in body weight, motor function, gait normalization, life extension, and finally, evidence that gene supplementation attenuates demyelination. Last, the greatest rescue results from a vector using a modified glial fibrillary acidic protein (GFAP) promoter-AAV9-gfaABC(1)D-EIF2B5-thereby supporting that astrocytic targeting is critical for disease correction. In conclusion, we demonstrate safety and early efficacy through treatment with a translatable astrocyte-targeted gene supplementation therapy for a disease that has no cure.

Late Presentation of a Primary Spinal Epidural Non-Hodgkin Lymphoma

Primary spinal epidural lymphomas (PSELs) constitute a unique subset of epidural tumors characterized by the exclusive presence of lymphoma within the epidural space at initial presentation, with no concurrent lymphoma in other sites. We aim to present a unique of PSEL and compare it with the present literature This case report presents a 65-year-old female with delayed-onset primary spinal epidural non-Hodgkin lymphoma (NHL). The patient exhibited paraplegia and walking difficulties, following a year of lower-extremity weakness and tingling sensations. Magnetic resonance imaging revealed a mass in the L1 through L3. Surgical decompression, stabilization, and tumor resection were performed. Microscopic examination confirmed NHL. Chemotherapy with the CHOP regimen was administered postsurgery. Subsequent follow-up revealed motoric and sensory improvements. PSEL primarily affects the thoracic spine and is more common in males. Patient age and gender impact prognosis. PSEL typically exhibits two phases – prodromal and rapid neurological deterioration. Surgical intervention coupled with chemotherapy is recommended, but the optimal surgical approach varies. Combined therapy could lead to improved disease-free survival. Cases of PSEL have shown potential for local and systemic relapse. Late presentation can still yield improvements with surgical and prompt chemotherapeutic intervention. Even without a prior history of malignancy or significant disease, PSEL should still be considered in patients presenting with spinal compression symptoms. Cases of delayed presentation, like the one presented here, can still benefit from surgical and prompt chemotherapeutic intervention.

Spinal epidural abscess mimicking chloroma

Background: Spinal epidural abscess (SEA) is a rare and generally challenging condition. It represents a challenge in terms of diagnosis and treatment due to the low incidence, vague presentation, and rapid neurologic deterioration. Case Presentation: A 40-year-old gentleman who was previously healthy was presented with a 2-week history of decreased vision associated with fever and cough one week before the eye symptoms. The patient was admitted as a case of acute promyelocytic leukemia. And was started on chemotherapy, which was complicated by septicemia and was shifted to the intensive care unit. Three weeks after admission he started to have severe non-radicular neck pain. He also had bilateral lower limb pain spasm-like in nature. Patient denies any history of trauma, weakness, numbness, saddle anesthesia or loss of sphincter control. Labs showed leukopenia, anemia and thrombocytopenia. Bone marrow result showed hypercellularity with active trilineage hematopoiesis and no immunomorphological evidence of abnormal promyelocytes or maturation arrest. He underwent C3-C4 laminectomy and evacuation of epidural pus collection. Intra-operative course was free of complication. Upon post-operative assessment he was hemodynamically stable, his neck pain was better with morphine doses and he was able to mobilize it. Conclusion: Spinal epidural abscess can mimic chloroma clinically and radiologically, however, diffusion with the images and enhancement is essential for proper assessment since a proper medical and surgical intervention is needed.

Acute necrotizing encephalopathy of childhood secondary to parainfluenza infection: A review of two cases

IntroductionAcute necrotizing encephalopathy (ANE) of childhood is a rare inflammatory syndrome, classically associated with preceding febrile illness and followed by fulminant neurological deterioration including encephalopathy, seizures, and focal neurological deficits with multifocal lesions of the thalami, rostral brainstem, and cerebral and cerebellar gray and white matter. ANE is distinctly associated with significant rates of neurological sequelae and death. Multiple infectious and parainfectious etiologies have been associated with ANE, notably influenza A. Case ReportWe report two cases of ANE associated with parainfluenza infection. Both children were treated with high-dose methylprednisolone and intravenous immunoglobulin, and one treated with tocilizumab. However, both patients suffered rapid neurological deterioration and death despite aggressive intervention. ConclusionWe present two cases of ANE with rapid neurological deterioration and death despite aggressive medical therapy. Rapid treatment with immunomodulatory treatments can lead to symptomatic recovery of variable degree in some patients. While high-dose steroids, intravenous immunoglobulin, and plasma exchange are common therapies for ANE, some case reports have suggested interleukin-6 (IL-6) blockade with tocilizumab can be beneficial. We report the nineteenth documented child in pediatric literature treated for ANE with tocilizumab, who did not respond to treatment, suggesting the response is heterogeneous and warrants additional investigation.

Neurological Outcome and Respiratory Insufficiency in Intramedullary Tumors of the Upper Cervical Spine.

Background and Objectives: Intramedullary spinal cord tumors (IMSCT) are rare entities. A location in the upper cervical spine as a highly eloquent region carries the risk of postoperative neurological deficits, such as tetraparesis or respiratory dysfunction. Evidence for respiratory dysfunction is scarce. This study aimed to describe these highly eloquent tumors' early and late postoperative clinical course. Materials and Methods: This is a single-center retrospective cohort study. We included 35 patients with IMSCT at levels of the craniocervical junction to C4 who underwent surgical treatment between 2008 and 2022. The authors analyzed the patients' preoperative status, tumor- and surgery-specific characteristics, and follow-up functional status. Results: The study cohort included twenty-two patients with grade II ependymoma (62.9%), two low-grade astrocytomas (5.7%), two glioblastomas (5.7%), six hemangioblastomas (17.1%), two metastases (5.7%), and one patient with partially intramedullary schwannoma (2.9%). Gross total resection was achieved in 76% of patients. Early dorsal column-related symptoms (gait ataxia and sensory loss) and motor deterioration occurred in 64% and 44% of patients. At a follow-up of 3.27 ± 3.83 years, 43% and 33% of patients still exhibited postoperative sensory and motor deterioration, respectively. The median McCormick Scale grade was 2 in the preoperative and late postoperative periods, respectively. Only three patients (8.6%) developed respiratory dysfunction, of whom, two patients, both with malignant IMSCT, required prolonged invasive ventilation. Conclusions: More than 60% of the patients with IMSCT in the upper cervical cord developed new neurological deficits in the immediate postoperative period, and more than 40% are permanent. However, these deficits are not disabling in most cases since most patients maintain functional independence as observed by unchanged low McCormick scores. The rate of respiratory insufficiency is relatively low and seems to be influenced by the rapid neurological deterioration in high-grade tumors.

Multifocal cerebral haemorrhage in two dogs with erythrocytosis

AbstractAn 8‐month‐old, female toy poodle and a 5‐year‐3‐month‐old, male, neutered Bernese Mountain dog presented with acute onset, progressive neurological deficits. Neurological examination findings were consistent with multifocal forebrain, brainstem and potential C1–C5 spinal cord segment involvement in both dogs. The toy poodle had a packed cell volume of 85% on presentation, and rapidly deteriorated to cardiopulmonary arrest. Postmortem examination revealed multifocal leptomeningeal, white and grey matter haemorrhage. Femoral bone marrow analysis revealed 95%:5% haematopoietic:fat ratio (reference value: 80%:20%). The Bernese Mountain dog had an initial packed cell volume of 81%. Magnetic resonance imaging of the head identified multifocal cerebral haemorrhage. The erythrocytosis was believed to be secondary to marked bilateral nephropathy. Following two phlebotomies, the packed cell volume decreased to 78%. However, the dog deteriorated and had a cardiopulmonary arrest. We report two clinical cases in which erythrocytosis was the cause of rapid and severe neurological deterioration.

Cervical transpedicular irrigation and drainage in a patient with cervical spondylodiscitis after hypopharyngeal cancer treatment A case report

Cervical spondylodiscitis is a rare condition, but it is usually associated with rapid neurological deterioration so urgent surgical intervention mostly is indicated. We are presenting a case of cervical spondylodiscitis in a patient with a history of cancer hypopharynx. The patient had lateral pharyngotomy-wide excision followed by radiotherapy, but the presence of an epidural abscess made surgical intervention inevitable. Anterior cervical debridement might lead to esophageal perforation due to the extensive tissue scaring, so transpedicular irrigation and drainage technique was decided. Our emerging MPPP technique allows us to do safe and effective debridement, however, there were some concerns regarding cervical pedicle screw insertion. So, we are reporting the first case of transpedicular curettage and drainage using the freehand cervical pedicle screw insertion technique for the treatment of cervical SD. After surgery, the infection subsided with no need for further intervention. Ultimately, the transpedicular irrigation and drainage technique is effective and less aggressive treatment for cervical SD after hypopharyngeal cancer treatment.

Outcome of Brain Lymphoma in a High Epstein-Barr Virus–Prevalence Country After Kidney Transplantation

The incidence of post-transplant lymphoproliferative disorder (PTLD) in adult kidney transplant (KTx) recipients is less common in Taiwan. In our institute, we observed that brain lymphoma was the most notorious type. The study describes the clinical, histologic, and radiological features of primary central nervous lymphoma (PCNSL) and the outcomes and associations with Epstein-Barr virus (EBV) infection in our center. Among 1470 KTx recipients, 5 patients had tissue-proven brain lymphoma (0.34%). The brain pathology disclosed diffuse large B-cell lymphoma in all patients. EBV was detected through in situ hybridization for Epstein-Barr encoding region (EBER) to disclose the EBV inclusion in the nuclei of lymphoma cells. The first treatment step was the reduction of immunosuppressants; 4 patients received whole-brain radiotherapy after complete resection of PCNSL, and 1 received concurrent chemoradiotherapy. Only one patient had poor performance status at the time of diagnosis and had a poor response to treatment with steroids. Four patients survived (mean 36.5 months, range 8.6 to 57.6 months), but one died after rapid neurologic deterioration. Epstein-Barr virus inclusion was found in PCNSL in our patients; however, the role of EBV in PCNSL remains to be clarified. Post-transplant lymphoproliferative disorder is a rare malignancy after KTx with a predilection of brain involvement in Taiwan. We report a successful care experience in a patient with primary CNS lymphoma with better survival.

Cervical transpedicular irrigation and drainage in a patient with cervical spondylodiscitis after hypopharyngeal cancer treatment: A case report

Cervical spondylodiscitis is a rare condition, but it is usually associated with rapid neurological deterioration so urgent surgical intervention mostly is indicated

Hepatic Coma Secondary to Fontan Conduit Thrombosis.

The Fontan procedure is used to treat congenital heart defects and has improved long-term survival. Long-term complications include liver disease due to congestive hepatopathy. Fontan-related cirrhosis can manifest with ascites, gastrointestinal bleeding and encephalopathy. A 43-year-old man with history of Fontan surgery was admitted with COVID-19 pneumonia. There was rapid clinical and neurological deterioration, with coma and shock. CT imaging showed thrombosis of the Fontan conduit. The patient was successfully subjected to recanalization of the Fontan circulation, with progressive improvement of coma. Fontan-associated liver disease is a major complication following the Fontan procedure. Clinicians must be aware of this pathology during patient follow-up. The Fontan procedure has improved the long-term survival of patients with single ventricle pathology.Fontan-associated liver disease is a result of haemodynamic changes associated with Fontan circulation; complications include hepatic encephalopathy and coma.Patients with Fontan circulation should be evaluated over time in order prevent and treat systemic complications.

The ferroptosis activity is associated with neurological recovery following chronic compressive spinal cord injury.

Chronic compressive spinal cord injury in compressive cervical myelopathy conditions can lead to rapid neurological deterioration in the early phase, followed by partial self-recovery, and ultimately an equilibrium state of neurological dysfunction. Ferroptosis is a crucial pathological process in many neurodegenerative diseases; however, its role in chronic compressive spinal cord injury remains unclear. In this study, we established a chronic compressive spinal cord injury rat model, which displayed its most severe behavioral and electrophysiological dysfunction at 4 weeks and partial recovery at 8 weeks after compression. Bulk RNA sequencing data identified enriched functional pathways, including ferroptosis, presynapse, and postsynaptic membrane activity at both 4 and 8 weeks following chronic compressive spinal cord injury. Transmission electron microscopy and malondialdehyde quantification assay confirmed that ferroptosis activity peaked at 4 weeks and was attenuated at 8 weeks after chronic compression. Ferroptosis activity was negatively correlated with behavioral score. Immunofluorescence, quantitative polymerase chain reaction, and western blotting showed that expression of the anti-ferroptosis molecules, glutathione peroxidase 4 (GPX4) and MAF BZIP transcription factor G (MafG), in neurons was suppressed at 4 weeks and upregulated at 8 weeks following spinal cord compression. There was a positive correlation between the expression of these two molecules, suggesting that they may work together to contribute to functional recovery following chronic compressive spinal cord injury. In conclusion, our study determined the genome-wide expression profile and ferroptosis activity of a consistently compressed spinal cord at different time points. The results showed that anti-ferroptosis genes, specifically GPX4 and MafG, may be involved in spontaneous neurological recovery at 8 weeks of chronic compressive spinal cord injury. These findings contribute to a better understanding of the mechanisms underlying chronic compressive spinal cord injury and may help identify new therapeutic targets for compressive cervical myelopathy.

Encephalomyelitis in a patient with monkeypox: an unusual complication

A new outbreak of monkeypox has been reported worldwide with CNS complications like encephalitis or myelitis being extremely rare. We present a case of a 30-year-old man with PCR-confirmed diagnosis of monkeypox who developed rapid neurological deterioration with extensive inflammatory involvement of the brain and spinal cord on MRI. Because of the clinical and radiological resemblance to acute disseminated encephalomyelitis (ADEM), it was decided to indicate treatment with high-dose corticosteroids for 5 days (without concomitant antiviral management due to lack of availability in our country). Given the poor clinical and radiological response, 5 days of immunoglobulin G were administered. During follow-up the patient’s clinical condition improved, physiotherapy was started and all associated medical complications were controlled. To our knowledge, this is the first reported monkeypox case with severe CNS complications treated with steroids and immunoglobulin in the absence of specific antiviral treatment.

A rare case of H3K27-altered diffuse midline glioma with multiple osseous and spinal metastases at the time of diagnosis

BackgroundH3K27-altered diffuse midline gliomas are uncommon central nervous system tumors with extremely poor prognoses.Case presentationWe report the case of a 24-year-old man patient with multiple, inter alia osseous metastases who presented with back pain, hemi-hypoesthesia, and hemi-hyperhidrosis. The patient underwent combined radio-chemotherapy and demonstrated temporary improvement before deteriorating.ConclusionsH3K27-altered diffuse midline glioma presents an infrequent but crucial differential diagnosis and should be considered in cases with rapid neurological deterioration and multiple intracranial and intramedullary tumor lesions in children and young adults. Combined radio-chemotherapy delayed the neurological deterioration, but unfortunately, progression occurred three months after the diagnosis.