Progressive Neurological Deficits Research Articles

P.068 Malignant transformation of a spinal dermoid cyst into carcinosarcoma

Background: Spinal dermoid cysts are uncommon, benign tumours of ectodermal origin, often associated with spinal dysraphism. Malignant transformation of spinal dermoid cysts is an exceptionally rare entity, with transformation into carcinosarcoma not previously reported. Methods: Case report and literature review Results: A 41-year-old male presented with a recurrent lumbar intradural mass, 28 years after resection of a dermal sinus tract and associated dermoid cyst. Intraoperative appearance and subsequent pathology were again consistent with a dermoid cyst. The patient re-presented 2 weeks after surgery with diplopia and headache due to hydrocephalus, thought to be due to chemical meningitis. Following ventriculoperitoneal shunt implantation, the patient rapidly deteriorated with progressive neurologic deficits and widespread leptomeningeal enhancement. A repeat spinal leptomeningeal biopsy was pursued, which revealed malignant transformation of the dermoid cyst into invasive carcinosarcoma. Without curative treatment options, the patient was palliated and died 85 days after admission. Conclusions: Malignant transformation of spinal dermoid cysts should be considered in the differential diagnosis of patients with dermoid cysts and progressive leptomeningeal enhancement. False negatives can occur with initial tumour pathology and repeat sampling may be warranted for diagnostic clarity. To the authors knowledge, this is the first report of a spinal dermoid cyst with malignant transformation into carcinosarcoma.

Medical management and surgery versus medical management alone for symptomatic cerebral cavernous malformation (CARE): a feasibility study and randomised, open, pragmatic, pilot phase trial

The highest priority uncertainty for people with symptomatic cerebral cavernous malformation is whether to have medical management and surgery or medical management alone. We conducted a pilot phase randomised controlled trial to assess the feasibility of addressing this uncertainty in a definitive trial. The CARE pilot trial was a prospective, randomised, open-label, assessor-blinded, parallel-group trial at neuroscience centres in the UK and Ireland. We aimed to recruit 60 people of any age, sex, and ethnicity who had mental capacity, were resident in the UK or Ireland, and had a symptomatic cerebral cavernous malformation. Computerised, web-based randomisation assigned participants (1:1) to medical management and surgery (neurosurgical resection or stereotactic radiosurgery) or medical management alone, stratified by the neurosurgeon's and participant's consensus about the intended type of surgery before randomisation. Assignment was open to investigators, participants, and carers, but not clinical outcome event adjudicators. Feasibility outcomes included site engagement, recruitment, choice of surgical management, retention, adherence, data quality, clinical outcome event rate, and protocol implementation. The primary clinical outcome was symptomatic intracranial haemorrhage or new persistent or progressive non-haemorrhagic focal neurological deficit due to cerebral cavernous malformation or surgery during at least 6 months of follow-up. We analysed data from all randomly assigned participants according to assigned management. This trial is registered with ISRCTN (ISRCTN41647111) and has been completed. Between Sept 27, 2021, and April 28, 2023, 28 (70%) of 40 sites took part, at which investigators screened 511 patients, of whom 322 (63%) were eligible, 202 were approached for recruitment, and 96 had collective uncertainty with their neurosurgeon about whether to have surgery for a symptomatic cerebral cavernous malformation. 72 (22%) of 322 eligible patients were randomly assigned (mean recruitment rate 0·2 [SD 0·25] participants per site per month) at a median of 287 (IQR 67-591) days since the most recent symptomatic presentation. Participants' median age was 50·6 (IQR 38·6-59·2) years, 68 (94%) of 72 participants were adults, 41 (57%) were female, 66 (92%) were White, 56 (78%) had a previous intracranial haemorrhage, and 28 (39%) had a previous epileptic seizure. The intended type of surgery before randomisation was neurosurgical resection for 19 (26%) of 72, stereotactic radiosurgery for 44 (61%), and no preference for nine (13%). Baseline clinical and imaging data were complete for all participants. 36 participants were randomly assigned to medical management and surgery (12 to neurosurgical resection and 24 to stereotactic radiosurgery) and 36 to medical management alone. Three (4%) of 72 participants withdrew, one was lost to follow-up, and one declined face-to-face follow-up, leaving 67 (93%) retained at 6-months' clinical follow-up. 61 (91%) of 67 participants with follow-up adhered to the assigned management strategy. The primary clinical outcome occurred in two (6%) of 33 participants randomly assigned to medical management and surgery (8·0%, 95% CI 2·0-32·1 per year) and in two (6%) of 34 participants randomly assigned to medical management alone (7·5%, 1·9-30·1 per year). Investigators reported no deaths, no serious adverse events, one protocol violation, and 61 protocol deviations. This pilot phase trial exceeded its recruitment target, but a definitive trial will require extensive international engagement. National Institute for Health and Care Research.

Navigating Diagnostic Dilemmas in Subacute Subdural Hemorrhage: A Case Report

In this case report a 64-year-old male patient with recent past medical history of head injury complicated by zygomatic arc fracture and mild subarachnoid hemorrhage is studied. He had been presented to the Emergency Department because of progressive neurological symptoms and neurological deficits in the physical examination that could have been indicating subcacute subdural hemorrhage. However, the patient was reluctant to undergo diagnostic imaging due to concerns about radiation exposure. After several explanations, a CT scan was done, which revealed a bilateral subacute subdural hematoma. Neurosurgical management was initiated and intravenous corticosteroid therapy was administered to reduce local edema. The challenge of this case is based on the subtlety of symptoms that might cause patients to delay seeking medical attention. Additionally, patient reluctance to undergo diagnostic tests can complicate management, emphasizing the importance of patient education and therapeutic alliance. Multidisciplinary management involving Neurology and Neurosurgery is crucial for optimal patient care in such cases. This report underscores the significance of effective communication and collaborative decision-making between healthcare providers and patients to ensure timely and appropriate management of complex medical conditions.

Abstract 1449: Targeting squalene epoxidase for breast cancer brain metastasis: The evaluation of a new therapeutic target for brain extravasation and colonization using animal models

Abstract Breast cancer brain metastasis is one of the most common forms of breast cancer metastasis and a major cause of morbidity and mortality. Patients who develop brain metastases tend to be associated with progressive neurologic deficits and short overall survival, which represents an unmet medical need. Extensive research has been conducted to elucidate the mechanism of cancer metastasis with limited information gained as to how cancer cells extravasate and colonize to the brain. Recently, we identified a novel mechanism by which squalene epoxidase (SQLE), the second rate-limiting enzyme in cholesterol biosynthesis, plays a critical role in the process of breast cancer metastasis to the brain. Thus, toward a better understanding of the underlying molecular mechanisms and applications of molecular target(s) for brain metastasis therapy, murine models were employed for investigation of brain extravasation and colonization with intracardiac or direct intracranial injections of GFP/FLuc-labeled human MDA-MB-231 metastatic breast carcinoma cells. The MDA-MB-231 cells were found to metastasize to the brain and other organs following intracardiac implantation into nude mice and were further isolated from each tissue. Interestingly, the isolated brain-metastasized tumor cells (MDA-MB-231-BrM) exhibited a significant upregulation of SQLE expression when compared with those that metastasized to other distal sites. The essential role of SQLE in the specific steps of the breast cancer-to-brain metastatic process was evaluated by ex vivo immunofluorescence analysis and immunohistochemistry staining of brain slices from the study subjects. Our data demonstrate that high SQLE expression is essential for MDA-MB-231-BrM to extravasate into the parenchyma, as well as the formation of micro- and macro-metastases in the brain. Interestingly, we found that blood vessel co-option and the surrounding neuronal cells play key roles in supporting MDA-MB-231-BrM to develop brain macro-metastases. In vitro blood-brain barrier (BBB) models further demonstrated the critical role of SQLE in promoting tumor cell invasion and penetration through the BBB. To verify SQLE as an oncogenic factor that can be selected as a potential therapeutic target in suppressing breast cancer brain metastasis, we evaluated the inhibitory effects of terbinafine (a SQLE inhibitor) in both the MDA-MB-231-BrM orthotopic and intracardiac mouse models. Pharmacologic inhibition of SQLE by terbinafine suppressed MDA-MB-231-BrM tumor growth at the mammary fat pad and distal metastases to the brain, suggesting that targeting SQLE represents a therapeutic opportunity for breast cancer brain metastasis. Citation Format: Pony Yu-Ling Lee, Kun-Yuan Lin, Chao-Di Chang, Shan-Yun Cheng, Ya-Wen Hung, Chih-Chieh Yang, Yu-Hsien Chang, Chien-Chang Shen, Jia-Yun Yeh, Yung-Lung Yu, Shu-Ping Wang. Targeting squalene epoxidase for breast cancer brain metastasis: The evaluation of a new therapeutic target for brain extravasation and colonization using animal models [abstract]. In: Proceedings of the American Association for Cancer Research Annual Meeting 2024; Part 1 (Regular Abstracts); 2024 Apr 5-10; San Diego, CA. Philadelphia (PA): AACR; Cancer Res 2024;84(6_Suppl):Abstract nr 1449.

Abstract 3339: TTFields for the management of spinal metastases in in vitro and in vivo models

Abstract Spine metastases often affect cancer patients, leading to significant morbidity and mortality. Their prevalence has increased over time due to the progressive improvements in cancer management which led to a longer survival of these individuals. Radiation therapy is the mainstay treatment for local control of these lesions, with surgery reserved for selected cases (e.g., spinal instability, neurological deficits secondary to neural compression requiring urgent decompression, separation surgery). Chemotherapy addresses the diffusely metastatic spread. However, when spinal metastases progress despite using all the appropriate available treatment, there follows a progressive decline in function and further treatment options are limited, highlighting the need for development of novel therapies. Tumor treating fields (TTFields) has demonstrated to impair tumor cell replication and are influenced by surrounding tissue, such as bone (presents non-conductive properties). Our goal was to investigate the effects of TTFields in in vitro and in vivo spinal metastasis models. We hypothesize that the deposition of TTFields is increased in bone, and this would ultimately result in vertebral metastasis growth suppression. A computational model using a simulated spine resection cavity with metallic hardware was used to investigate the TTFields intensity in various tissues. For in vitro studies, luciferase tagged KRIB osteosarcoma and A549 lung adenocarcinoma cell lines were cultured in demineralized human bone graft. These were treated with TTFields and cell viability was quantified. In vivo assays were performed using a previous validated murine orthotopic tumor model. The effects of TTFields in these animals were monitored with imaging (bioluminescence and MRI) and clinical findings (progressive neurological deficits milestones that corresponds to the degree of spinal cord compression). At the end of the study, tissue was collected for histologic analysis. Sham stimulation was used for control groups. Computational simulation demonstrated an elevated TTFields intensity in the tumor-bone interface of the resected vertebral body. In all experimental models, TTFields significantly suppressed tumor cell growth. In vivo models, this effect on cell proliferation prevented tumor growth into the spinal canal, delaying, or even preventing, neurological deficits. TTFields inhibited tumor growth in three-dimensional cultures and orthotopic murine models of spinal metastases, preventing functional loss. The bony environment surrounding the tumor demonstrated to enhance the intensity of electric fields on a computational simulation, which stands as an advantage with the use of TTFields for vertebral tumors. These results provide solid evidence supporting future clinical trials investigating the effects of this novel and promising technology for the treatment of spine metastases. Citation Format: Romulo Augusto Andrade de Almeida, Daniel Ledbetter, Xizi Wu, Ariel Naveh, Chirag Patel, Queena Gonzalez, Thomas H. Beckham, Robert Y. North, Laurence Rhines, Christopher A. Alvarez-Breckenridge, Claudio E. Tatsui. TTFields for the management of spinal metastases in in vitro and in vivo models [abstract]. In: Proceedings of the American Association for Cancer Research Annual Meeting 2024; Part 1 (Regular Abstracts); 2024 Apr 5-10; San Diego, CA. Philadelphia (PA): AACR; Cancer Res 2024;84(6_Suppl):Abstract nr 3339.

Surgical strategies in acute subdural hematoma: a meta-analysis of decompressive craniectomy vs. craniotomy.

Acute subdural hematoma (ASDH) stands as a significant contributor to morbidity after severe traumatic brain injuries (TBI). The primary treatment approach for patients experiencing progressive neurological deficits or notable mass effects is the surgical removal of the hematoma, which can be achieved through craniotomy (CO) or decompressive craniectomy (DC). Nevertheless, the choice between these two procedures remains a subject of ongoing debate and controversy. We conducted a comprehensive literature review, utilizing prominent online databases and manually searching references related to craniotomy and craniectomy for subdural hematoma evacuation up to November 2023. Our analysis focused on outcome variables such as the presence of residual subdural hematoma, the need for revision procedures, and overall clinical outcomes. We included a total of 11 comparative studies in our analysis, encompassing 4269 patients, with 2979 undergoing craniotomy and 1290 undergoing craniectomy, meeting the inclusion criteria. Patients who underwent craniectomy displayed significantly lower scores on the Glasgow Coma Scale (GCS) during their initial presentation. Following surgery, the DC group exhibited a significantly reduced rate of residual subdural (P = 0.009). Additionally, the likelihood of a poor outcome during follow-up was lower in the CO group. Likewise, the mortality rate was lower in the CO group compared to the craniectomy group (OR 0.63, 95% CI 0.41-0.98, I2 = 84%, P = 0.04). Our study found that CO was associated with more favorable outcomes in terms of mortality, reoperation rate, and functional outcome while DC was associated with less likelihood of residual subdural hematoma. Upon further investigation of patient characteristics who underwent into either of these interventions, it was very clear that patients in DC cohort have more serious and low pre-op characteristics than the CO group. Nonetheless, brain herniation and advanced age act as independent factor for predicting the outcome irrespective of the intervention.

Videoocular assessment of eye movement activity in an ataxia-telangiectasia: a case study.

Ataxia-telangiectasia (A-T) is an autosomal recessive disorder characterized by progressive neurological deficits, including prominent oculomotor dysfunction. We report 5 cases of eye movement assessment in children 9-15years old with A-T. Three different oculomotor tasks (gaze holding, visually guided saccades and visual search) were used, and video-oculography was performed. Additionally, the scale for the assessment and rating of ataxia(SARA) score was used to assess severity of the cerebellar ataxia. Unstable gaze holding, nystagmus and saccadic intrusions were found. In addition to psychophysiological assessment results, we provide quantitative analysis of oculomotor activity, revealing a specific abnormal oculomotor pattern, consisting of (i) marked saccade hypermetria, (ii) unstable gaze holding, and (iii) gaze-evoked nystagmus. Our study opens the prospect to evaluate efficacy and safety of alternative methods for supporting the patient and improving his/her life quality.

Headache and Other Pain Syndromes in Multiple Sclerosis: A Narrative Review.

Multiple sclerosis is a chronic and progressive neurological disease, with an important socio-economic burden. Over time, an increased incidence of headaches like migraines and tension headaches has been observed among these patients. Headaches have not been considered as multiple sclerosis-related symptoms, even representing a red flag for multiple sclerosis diagnosis. It is uncertain whether the headache-multiple sclerosis association could be explained by the presence of common triggers or a common physiopathological mechanism (involvement of tertiary B-cell follicles). An important differential diagnosis is between multiple sclerosis attacks and migraines with aura, which can also be associated with neurological deficits. Another important aspect is the occurrence or exacerbation of the cephalalgic syndrome after the initiation of therapy for multiple sclerosis (DMTs), or the improvement of headache after the initiation of certain DMT drugs. In addition to headaches, individuals diagnosed with multiple sclerosis often report experiencing diverse pain syndromes, contributing to an additional decline in their overall quality of life. These syndromes are frequently neglected, the focus being on slowing down the progression of neurological deficits. This review aims to evaluate the characteristics of multiple-sclerosis-related headaches (frequency, possible correlation with attacks, and disease-modifying therapies) and the key distinctions in imaging characteristics between demyelinating lesions in multiple sclerosis and those observed in cases of primary headaches.

GM1 and GM2-Gangliosidosis: Clinical Features, Neuroimaging Findings and Electroencephalography.

Gangliosidosis is one of the hereditary metabolic diseases caused by the accumulation of Gangliosid in the central nervous system, leading to severe and progressive neurological deficits. Regarding phenotype, GM1 and GM2-Gangliosidosis are divided into Infantile, Juvenile, and Adult. In this study, thirty-seven patients with GM1 and GM2-Gangliosidosis were referred to the neurology department of Mofid Children's Hospital in Tehran, Iran, whose disease was confirmed from September 2019 to December 2021. This study assessed age, sex, and developmental status before the onset of the disease, clinical manifestations, brain imaging, and electroencephalography. 97.20% of patients were the result of family marriage. Approximately 80% of juvenile patients were developmentally normal before the onset of the disease. Developmental delay was more common among infantile GM1-Gangliosidosis than infantile GM2-Gangliosidosis, but in total, more than 50% of GM1&GM2-Gangliosidosis patients had reached their developmental milestone before the onset of the disease. With the onset of disease symptoms, 100% of patients regressed in terms of movement, 97.20% of them mentally, and 75% of them had seizures during the disease. The most common clinical findings were cherry-red spot, Mongolian spot, macrocephaly, organomegaly, hyperacusis, and scoliosis. The most common brain imaging findings included bilateral thalamus involvement, brain atrophy, PVL, and delayed myelination. The most common finding in electroencephalography was background low voltage with abnormal sharp waves. This study concluded that most of the patients are the result of family marriage, and most of the juvenile patients are developmentally normal before the onset of the disease. In addition, more than 50% of infantile patients reach their developmental milestones before the onset of the disease. The most common clinical findings of these patients are seizures, cherry-red spot, macrocephaly, hyperacusis, Mongolian spot, and bilateral involvement of the thalamus.

Outcomes of Operated Cases of Central Nervous System Metastases at a Tertiary Cancer Hospital – An Analysis

Abstract Background: Recent advances in the treatment have increased the survival of patients with common cancers. As a result, the incidence of symptomatic central nervous system (CNS) metastases has increased over time. Although systemic therapies remain the mainstay of metastatic cancers, surgical treatment is often required for progressive neurological deficits and to buy time for other modalities to be effective. The present retrospective study aimed to analyze various demographic, neurological, and short-term survival outcomes among operated cases of symptomatic CNS metastases. Materials and Methods: All surgically treated symptomatic brain and spine metastasis patients between August 2018 and March 2022 were included in the study. Patients who underwent procedures for primary tumors of the brain and spine were excluded. The details of demographic and clinical profile, neurological data, surgical treatment, and short-term survival data were retrieved from hospital medical records and analyzed. Patients with motor deficits (power grade 3/5 or less) were analyzed to look for improvement in motor deficits after surgery. Results: The first 100 operated cases of symptomatic CNS metastases were reviewed. The mean age of presentation was 46.42 years (females – 44.28, males – 49.13). Breast malignancy was the most common source of primary overall (29%) and lung malignancy (15%) was second most common. A total of 53 brain surgeries and 47 spine surgeries were performed. Preoperative motor deficits were seen more commonly among spine metastases (30/47) than brain metastases (17/53) patients. Around 64% of patients who presented with neurological deficits (motor deficits) had improvement postsurgery. Such improvement from deficits was observed more in patients with spine metastases (23/30; 76.7%) than brain metastases (7/17; 41.2%). Conclusion: Neurosurgery has an important role in the management of CNS metastases, and timely surgical intervention in a selected group of symptomatic CNS metastases can improve the quality of life of the patients.

Multifocal Acquired Demyelinating Sensory and Motor Neuropathy (MADSAM) with Cranial Nerve Involvement. Case Report

BACKGROUND: The multifocal acquired demyelinating sensory and motor neuropathy (MADSAM) is a rare adult onset subvariant of the chronic inflammatory demyelinating polyneuropathy (CIDP). The latter affects 1-9 cases per million adults and may pose a diagnostic challenge (antibody and electrophysiological overlap). The hallmark of the disease spectrum is an immune mediated structural myelin breakdown. Urgent differentiation of MADSAM from the other variants of CIDP is indicated due to its distinct pathogenesis, management, and long-term therapy response to immunomodulators. The disease presents with progressive asymmetrical motor and sensory deficits initially located typically in one limb. Rarely the disease manifests with cranial nerve involvement. The prognosis of patients suffering from MADSAM is reliant on rapid diagnosis and therapeutic response, but neurological deficits reside. Due to its rarity and diagnostic challenge, misdiagnosis is common. THE CASE: A 84 years old Caucasian male diagnosed with multifocal acquired demyelinating sensory and motor neuropathy (MADSAM) presented to our department with progressive neurological deficits. His neurological examination and history revealed paresthesia, hypesthesia, diminished vibration sense (pallhypesthesia), distally pronounced paresis in the upper extremities with a wrist drop on the left hand. Gait examination exposed coordination deficits. The history of the patient and the initial presentation of the disease in 1998 exemplifies the diagnostic challenge of MADSAM, due its mimicry of other diseases, like multifocal motor neuropathy (MMN). The initial symptoms were asymmetrical motor and sensory deficit starting at the upper extremities. Before the patient presented to our facility, he was diagnosed with neuroborreliosis and treated accordingly. The correct diagnosis was set at our department according to clinical presentation, nerve conduction velocity parameters and conduction block during electroneurography, typical changes in nerve ultrasound suggesting an inflammatory neuropathy and fasciculations of the gastrocnemius muscle. After an ineffective steroid therapy and long standing intravenous immunoglobulins the patient is now on a therapeutic scheme of 1000mg Rituximab (CD20 Ab) every 6 months and 1000mg Mycophenolatmofetil. The patient subjectively reports improvement of his status and slower progression of the disease since the Rituximab paradigm. Furthermore, an evident atrophy of the orbicularis oculi muscle was now noted during follow up examination, which highlights facial nerve involvement. CONCLUSION: This case exemplifies the challenge of diagnosing the multifocal acquired sensory and motor neuropathy, but prompt diagnosis and therapeutic intervention is associated with a better prognosis and slower progression of the disease in patients suffering from MADSAM.

Characterizing mJOA-defined post-surgical recovery patterns in patients with degenerative cervical myelopathy

BackgroundDegenerative cervical myelopathy is a spinal disorder resulting in progressive cord compression and neurological deficits that are assessed using the modified Japanese Orthopedic Association (mJOA) questionnaire. It is difficult to predict which patients will recover neurological function after surgery, making it challenging for clinicians to set postoperative patient expectations. In this study, we used mJOA subscores to identify patterns of recovery and recovery timelines in patients with moderate and severe myelopathy. MethodsFifty-three myelopathy patients were enrolled and completed the mJOA questionnaire both pre-surgery, and six weeks and six months post-surgery. Pearson chi-square tests were performed to assess relationships of both recovery patterns and recovery timelines with severity of disease. ResultsModerate myelopathy patients were significantly more likely than severe myelopathy patients to experience full recovery of upper extremity, lower extremity, and sensory domains. Disease severity did not significantly impact the timeline during which recovery occurs. Overall, >90% of patients experienced at least partial recovery by six months post surgery, 80% of which demonstrated it within the first six weeks. ConclusionsThis study shows the more severe the disease experienced by myelopathy patients, the more likely they will be left with permanent disabilities despite surgery. Early identification and treatment are therefore necessary to prevent worsening quality of life and increased costs of functional dependence. The recovery timelines for each subscore are similar and provide new values to guide patient expectations in their potential post-operative recovery. The overall recovery timeline is more generalizable though potentially lacking the specificity patients seek.

Comparison of lumbar microdiscectomy and unilateral biportal endoscopic discectomy outcomes: a single-center experience.

Lumbar microdiscectomy (LMD) is still the gold-standard treatment for lumbar disc herniations with progressive neurological deficits that are refractory to conservative treatment. With improvement of endoscopic systems in recent years, endoscopic discectomy techniques have been developed as an alternative to LMD. The unilateral biportal endoscopic discectomy (UBE) technique is one of these endoscopic techniques, and its popularity has increased in recent years because it does not require high-cost specialized endoscopes, many microsurgical instruments are compatible with this system, and it is similar to LMD in terms of anatomical orientation. This study compared results between LMD and UBE techniques in patients with lumbar disc herniations performed by the same spine surgeons at a single center. The data of patients with lumbar disc herniation who were operated on with LMD and UBE techniques were retrospectively reviewed. The data obtained were statistically evaluated. The operative video of one of the patients who underwent UBE was edited for demonstration. Between January 2021 and June 2022, 93 patients were operated on for lumbar disc herniation. LMD was performed in 39 patients, and UBE was performed in 54 patients. There were no significant differences in the complications, recurrence, postoperative back and leg pain, patient satisfaction rates, and quality of life index results of the patients in the two groups. The operation time was shorter in the LMD group. In the UBE group, estimated blood loss was lower and postoperative hospitalization was shorter. Although LMD is still the gold-standard treatment for lumbar disc herniation, the results of UBE are comparable to those of LMD, and it may be a good alternative for spine surgeons who prefer minimally invasive surgery.

Rasmussen’s Encephalitis in Children: A Case Report

Rasmussen’s Encephalitis (RE) is one form of chronic and progressive focal encephalitis of children presented with intractable epilepsy and progressive neurologic deficits. The cause of the disease is still unknown but thought to be an immunological disorder following a viral infection. Brain imaging is one of the best tools for diagnosis. We presented a case of Rasmussen’s encephalitis. BANGLADESH J CHILD HEALTH 2022; VOL 46 (1) : 35-38

Combined endoscopic and microsurgical approach for the drainage of a multisegmental thoracolumbar epidural abscess: illustrative case.

Spinal epidural abscess is a rare but serious infectious disease that can rapidly develop into a life-threatening condition. Therefore, the appropriate treatment is indispensable. Although conservative treatment is justifiable in certain cases, surgical treatment needs to be considered as an alternative early on because of complications such as (progressive) neurological deficits or sepsis. However, traditional surgical techniques usually include destructive approaches up to (multilevel) laminectomies. Such excessive approaches do have biomechanical effects potentially affecting the long-term outcomes. Therefore, minimally invasive approaches have been described as alternative strategies, including endoscopic approaches. The authors describe a surgical technique involving a combination of two minimally invasive approaches (endoscopic and microsurgical) to drain a multisegmental (thoracolumbar) abscess using the physical phenomenon of continuous pressure difference to minimize collateral tissue damage. The combination of minimally invasive approaches, including the endoscopic technique, may be an alternative in draining selected epidural abscesses while achieving a similar amount of abscess removal and causing less collateral approach damage in comparison with more traditional techniques.

The Important Role of Hemispherotomy for Rasmussen Encephalitis: Clinical and Functional Outcomes

BackgroundRasmussen encephalitis (RE) is characterized by pharmacoresistant epilepsy and progressive neurological deficits concurrent with unilateral hemispheric atrophy. Evidence of an inflammatory autoimmune process has been extensively described in the literature; however, the precise etiology of RE is still unknown. Despite data supporting a beneficial effect of early immunosuppressive and immunomodulatory interventions, surgical disconnection of the affected hemisphere is considered the treatment of choice for these patients. The aim of this study was to report a series of children and adolescents who underwent hemispheric surgery (HS) for the treatment of RE, analyzing their clinical, electrographic, and neuroimaging features pre-operatively, as well as their postoperative status, including seizure and functional outcomes. MethodsAll patients with RE who underwent HS in the Epilepsy Surgery Center (CIREP) of the University Hospital of Ribeirão Preto Medical School, between 1995 and 2020 were retrospectively reviewed. Preoperative and postoperative analyses included gender; age at epilepsy onset; seizure semiology; seizure frequency; interictal and ictal electroencephalographic (EEG) findings; age at surgery; duration of epilepsy; surgical complications; duration of follow-up; histopathological findings; and postoperative seizure, cognitive, and functional outcomes. ResultsForty-four patients were evaluated. Mean age at seizure onset and epilepsy duration was 6 years and 2.5 years, respectively. Mean age at surgery was 9 years, with an average follow-up ranging from 3 months to 23 years. All patients presented with severe epilepsy and distinct neurological abnormalities on MRI. Before HS, different degrees of abnormal intellectual performance as well as hemiparesis were seen in 86% and 90%, respectively. Histopathology examination confirmed this diagnosis in 95% patients. At the last follow-up, 68% of patients were seizure free, and 70% were classified as Engel Class I or II. Postoperatively, the cognitive status remained unchanged in 64% of patients. Likewise, the gross motor function remained unchanged in 54% of patients and 74% had functional hand ability after HS. ConclusionsConsidering the progressive damage course of RE, hemispheric surgery should be offered to pediatric patients. It has manageable risks and results in good seizure outcome, and the preoperative functional status of these children is often preserved (even when the left hemisphere is involved), thus improving their quality of life.

Neuroinflammatory Part of the Systemic Inflammatory Process in the Development of Neurodegenerative Conditions

Background: neurodegenerative processes are prognostically dramatic conditions. They are actively studied by both neurologists and psychiatrists. Nosological forms are diverse and include regressive autism, dementia, multiple sclerosis, Parkinson’s disease, Alzheimer’s disease, progressive epilepsy of infancy and childhood, generalized hyperkinesis, Tourette’s syndrome and many other conditions that are common to progressive neurological deficit, loss of cognitive functions, the appearance of nonspecific symptoms of brain irritation (epileptic seizures, hyperkinesis, dystonic states, stereotypes, psychotic episodes and other symptoms). Despite many ongoing studies the treatment of the described conditions is limited to temporary, incomplete and pharmacologically dependent relief of symptoms, since the problem of the etiopathogenesis of these conditions remains open. The aim of review is to present current immunological concepts of pathogenesis of neurodegenerative diseases. Material and method: using keywords “neuroinflammation, neurodegeneration, multiple sclerosis, Parkinson disease, epilepsy, parasitosis, microbiota” selected relevant scientific publications in domestic and international databases. Conclusion: recent advances in neuroimmunology show a significant role of neuroinflammation in the triggering and development of neurodegenerative processes, some of which are autoimmune in nature, which is confirmed by the indicators of immunity intensity introduced into clinical practice and by the detected antibody titers to brain tissues. In recent decades in a series of scientific works attention has been paid to the study of infectious agents detected in the immunological screening of patients with neurodegeneration, however, these data are fragmented and contradictory, and therefore are ignored by the medical community. This review presents a modern view of the etiopathogenesis of neurodegenerative diseases from the point of view of the summative antigenic burden, assessment of the immunological potential, homeostatic resource, and compensatory capabilities of the human body, which as a result can become a starting point for proposing new therapeutic strategies for the treatment of these intractable diseases.

Cerebral Vasculopathy in Children with Neurofibromatosis Type 1.

Cerebrovascular abnormalities are a severe and often underrecognized complication of childhood neurofibromatosis type 1 (NF1). There are no prospective studies of cerebral vasculopathy in NF1; thus, the estimated frequency of vasculopathy varies between studies. The data is difficult to interpret due to the retrospective data collection and variability in whether imaging is done based on screening/surveillance or due to acute neurologic symptoms. The prevalent NF1-associated cerebral vasculopathy is moyamoya syndrome (MMS). Vascular changes can present without symptoms or with acute TIA or stroke-like symptoms or a range of progressive neurologic deficits. Advanced imaging may enhance sensitivity of neuroimaging in children. Medical and/or surgical interventions may prevent short- and long-term complications. Challenges for establishment of a screening protocol for cerebral vasculopathy in children with NF1 include the relatively large number of patients with NF1, the potential need for sedation to achieve quality imaging and the broad age range at time of detection for cerebral vascular changes. The goal of this review is to present the epidemiology, clinical presentation, imaging features and medical/surgical management of cerebral arteriopathies in children with NF1.

Multidisciplinary management of invasive vertebral hemangioma: the role of different neurointervention modalities—a retrospective study

BackgroundCatastrophic intraoperative bleeding is a major complication of surgical removal of invasive vertebral hemangioma. Interventional neuroradiology techniques could be more useful tools to manage such hemorrhagic lesions.ResultsRetrospective analysis of cases of invasive vertebral hemangioma revealed 17 cases treated at the Alexandria University School of Medicine from 2006 to 2020. The study included 52.9% of males with a mean age of 38.4 ± 18.6 years old. All patients reported local and sometimes radicular pain; 64.7% exhibited progressive neurological deficits. Imaging revealed thoracic spine affection in 11 cases, lumbar vertebral in four cases, and cervical vertebra in two cases. Vertebral involvement occurred in 10 cases and paravertebral extension in 13 cases. Neurointervention modalities included transarterial embolization followed by corpectomy and fixation (one case with C4 lesion), direct surgery with corpectomy and anterior fixation (one case with C7 lesion), vertebroplasty alone (four cases), vertebroplasty with fixation (seven cases), and direct transpedicular alcohol injection with immediate devascularization and necrosis of the vascular channels inside the lesions (six cases). The alcohol injection use ranged from 4 to 10 ml in each pedicle. All patients did well during the follow-period post-intervention. The neurological deficits improved over six months. All patients showed improved Nurick grade regardless of the intervention (preoperative mean 2.7 ± 1.9 vs. postoperative mean 1.1 ± 1.3, p value 0.0001). Two patients were completely paraplegic, but with intact deep sensation, they improved dramatically and can walk unsupported post-intervention.ConclusionsVertebral hemangioma can present in an invasive manner that necessitates intervention. Preoperative embolization, alcohol injection, or vertebroplasty are helpful methods to decrease intraoperative catastrophic hemorrhage. Alcohol injection is cost-effective with immediate devascularization of the lesion. The extensive 360 surgery utilization can be decreased with the use of alcohol and vertebroplasty. More cases are needed to validate these conclusions.

P08.05.A A LONGITUDINAL CASE STUDY OF PATIENTS' EXPERIENCES IN THE INITIAL PHASE OF A BRAIN CANCER PATHWAY - AIMING TO IMPROVE PATIENT CARE

Abstract BACKGROUND A brain cancer diagnosis is life-transforming and involves accelerated diagnostics and treatment and care involving multiple settings and healthcare professionals. Patients are confronted with an uncurable cancer disease but also face progressive neurological and cognitive deficits over time, adding to the uncertain and stressful situation. Knowledge of patients' experiences during their hospital stay in the initial diagnostic phase is sparse. This study aimed to explore patients' experiences at the hospital during the diagnostic workup, the brain surgery, and the post-operative period until discharge and to describe what matters to patients when diagnosed with a malignant brain tumour. MATERIAL AND METHODS We used a descriptive longitudinal single-case study design to produce in-depth, context-dependent knowledge and capture real-life situations in patients' day-to-day care experiences from admission to discharge across three different departments throughout the initial diagnostic phase. To explore patients' views directly in the actual situations unfolding in practice, we followed one patient at a time and did 180 hours of field observation and 16 semi-structured interviews. We analyzed field notes and interviews by applying systematic text condensation. RESULTS An overarching theme, "A fast transition", was identified, describing the patients' experiences in the two central themes; "From feeling fine to an unknown journey" and "Patient involvement determined by system factors". Within the two themes, six subthemes emerged from the data: Overwhelming uncertainty and fear, Putting life on hold, An unfamiliar dependency and Being unprepared and side-lined, The significance of close relatives, and Managing care needs and demands. CONCLUSION The results add to understanding patients' experiences during the initial diagnostic phase. While part of the findings are a fact of life for patients diagnosed with a brain tumour, others point to specific areas for improvement. Small changes in care delivery can enhance patient involvement by proactively informing patients about their care plan, ensuring the presence of relatives during information giving and identifying patients' care needs and preferences. Establishing a patient and public involvement group in 2023 will provide more knowledge gaps for future research.