Neurological Examination Research Articles

Long-term prognosis and related factors of neonatal and small infants meningitis caused by streptococcus agalactiae

Objective: To explore the long-term prognosis and related factors of neonates and infants meningitis caused by Streptococcus agalactiae. Methods: The Clinical data of neonates and small infants with Streptococcus agalactiae meningitis admitted to the Children's Hospital of Zhengzhou University from January 2015 to May 2022 were retrospectively collected, and long-term follow-up of neurological development was conducted for the patients, with a follow-up period of ≥18 months. According to long-term neurological prognosis indicators, the patients were divided into good prognosis group and poor prognosis group, and the multivariate logistic regression model was used to analyze the factors affecting the long-term prognosis of the patients. Results: A total of 86 children were included, with 50 males and 36 females, and their age ranged from 3 to 85 (29.7±18.5) days. The follow-up duration was 20 to 78(47.2±17.0) months, and the age at the end of follow-up was 1.8 to 6.6(4.2±1.3) years. Among them, there were 54 cases in good long-term prognosis group and 32 cases in poor prognosis guoup. Infectious shock [56.3%(18/32) vs 13.0%(7/54), P<0.001], hypoproteinemia [46.9%(15/32) vs 22.2%(12/54), P=0.017], surgical intervention for neurological complications [31.0%(13/32) vs 7.4%(4/54), P=0.003], abnormal neurological examination at discharge [37.5%(12/32) vs 7.4%(4/54), P<0.001], and abnormal cranial imaging at discharge [53.1%(13/32) vs 7.4%(4/54), P<0.001] were higher in the poor prognosis group than those in the good prognosis group. The peripheral blood WBC count [M(Q1, Q3)] [4.7(3.4, 7.6)]×109/L at the onset of the disease was lower in the poor prognosis group than that in the good prognosis group [10.1(3.8, 14.1)]×109/L, and the cerebrospinal fluid protein concentration [3.0(2.1, 3.8)] g/L was higher in the poor prognosis group than that in the good prognosis group [2.5(1.9, 2.8)] g/L (both P<0.05). The multivariate logistic regression model found that peripheral blood WBC<4.0×109/L (OR=5.501,95%CI:1.430-21.170,P=0.013), infectious shock (OR=4.600,95%CI:1.008-21.004,P=0.047), neurological complications undergoing surgical intervention (OR=8.961,95%CI:1.792-44.812,P=0.008), and abnormal head imaging at discharge (OR=12.636,95%CI:2.502-63.829,P=0.002) were related factors for poor long-term prognosis in neonatal and infants meningitis caused by Streptococcus agalactiae. Conclusions: Neonates and infants meningitis caused by Streptococcus agalactiae have a high incidence of long-term adverse prognosis. Peripheral blood WBC<4.0×109/L, infectious shock, neurological complications undergoing surgical intervention, and abnormal cranial imaging at discharge are related factors for poor long-term prognosis.

Smartphone tests quantify lower extremities dysfunction in multiple sclerosis

IntroductionIncreasing shortage of neurologists compounded by the global aging of the population have translated into suboptimal care of patients with chronic neurological diseases. While some patients might benefit from expanding telemedicine, monitoring neurological disability via telemedicine is challenging. Smartphone technologies represent an attractive tool for remote, self-administered neurological assessment. To address this need, we have developed a suite of smartphone tests, called neurological functional test suite (NeuFun-TS), designed to replicate traditional neurological examination. The aim of this study was to assess the ability of two NeuFun-TS tests—short walk and foot tapping—to quantify motor functions of lower extremities as assessed by a neurologist.MethodsA cohort of 108 multiple sclerosis (MS) patients received a full neurological examination, imaging of the brain, and completed the NeuFun-TS smartphone tests. The neurological exam was digitalized using the NeurEx™ platform, providing calculation of traditional disability scales, as well as quantification of lower extremities-specific disability. We assessed unilateral correlations of 28 digital biomarkers generated from the NeuFun-TS tests with disability and MRI outcomes and developed machine learning models that predict physical disability. Model performance was tested in an independent validation cohort.ResultsNeuFun-TS-derived digital biomarkers correlated strongly with traditional outcomes related to gait and lower extremities functions (e.g., Spearman ρ &amp;gt; 0.8). As expected, the correlation with global disability outcomes was weaker, but still highly significant (e.g., ρ 0.46–0.65; p &amp;lt; 0.001 for EDSS). Digital biomarkers also correlated with semi-quantitative imaging outcomes capturing locations that can affect lower extremity functions (e.g., ρ ~ 0.4 for atrophy of medulla). Reliable digital outcomes with high test-retest values showed stronger correlation with disability outcomes. Combining strong, reliable digital features using machine learning resulted in models that outperformed predictive power of best individual digital biomarkers in an independent validation cohort.DiscussionNeuFun-TS tests provide reliable digital biomarkers of lower extremity motor functions.

Diagnostic and Therapeutic Challenges of Focal Cortical Dysplasia in Mali: A Case Report

Focal Cortical Dysplasias (FCDs) are a common cause of pharmaco-resistant epilepsy. FCDs are rare conditions, and their pathogenesis remains poorly understood. We report a 12-year-old patient seen in our clinic for focal onset epileptic seizures. Neurological examination and brain CT-scan were nearly normal. However, an electroencephalogram (EEG) revealed a slightly slow background rhythm with slow spikes in the medial and right parietal regions and a brain magnetic resonance imaging (MRI) showed a focal cortical thickening in the right parietal lobe with FLAIR hypersignal, indicating a focal cortical dysplasia. Ongoing seizures marked the disease course under antiepileptic polytherapy after 15 months of treatment. Keywords: Focal cortical dysplasia, pharmaco-resistant epilepsy, Mali, Africa.

NOT THE USUAL SUSPECTS: GLIOBLASTOMA OF THE CEREBELLOPONTINE ANGLE MASQUERADING AS A VESTIBULAR SCHWANNOMA

Introduction Most tumoral processes involving the cerebellopontine angle (CPA) are extraneuraxial and usually comprise of vestibular schwannomas and meningiomas, followed by less common lesions such as epidermoid cysts, metastases and arachnoid cysts. Glioblastomas located in the CPA are rarely diagnosed. Uncommonly, intraneuraxial tumors in the CPA may grow in an exophytic manner compressing the surrounding cranial nerves, resembling the clinical picture of a more common extraneuraxial tumour. Clinical Case A 47 year old male presented to our department accusing gait imbalance, vertigo, vomiting and nausea, symptoms that started 2 months prior to admission. Upon neurological examination, the patient additionally presented right facial paresis and hypoesthesia, right sided hypoacusis, hypogeusia and diplopia. A contrast enhanced cerebral MRI revealed a right cerebellopontine angle mass with inhomogenous contrast enhancement, relatively well delineated, exerting mass effect on the cerebellum and pons with reduced perilesional edema but with involvement of the internal acoustic meatus. The indication for surgical removal of the tumour was made. The patient was placed in sitting position and a right retrosigmoid approach was performed using the operating microscope. Upon retraction of the cerebellar hemisphere, a greyish, highly vascularized and soft lesion was seen, exhibiting a spread growth, engulfing the V, VII and VIII nerves, without infiltrating them, resembling anything but a schwannoma. A frozen section of the tumour revealed a surprising result: high grade glioma. The surgical excision continued in a piece-meal fashion, using the ultrasonic aspirator with preservation of the cranial nerves until an almost complete removal was achieved. The immediate postop evolution of the patient was favourable. Nevertheless, the final histopathological diagnosis confirmed a grade IV glioblastoma. Oncological treatmend followed, but the tumour relapsed in 2 months after surgery, the control MRI revealing a multilocular tumoral dissemination in the cerebellum and brainstem which was surgically unsalvageable. Conclusion Glioblastoma of the CPA, manifesting both imagistically and clinically as a vestibular schwannoma is a rare entity but nevertheless extant, that dramatically changes the prognosis and postop evolution of a patient. Physicians involved in the diagnosis and treatment of such tumours must always be suspicious of the usual suspects in the CPA.

DEALING WITH TWO SYNCHRONOUS LESIONS

Introduction Multiple synchronous intracranial lesions present a significant diagnostic and therapeutic challenge, in most cases appearing as metastatic tumors or same-cell type tumors. Case Report We report the case of a 73-year-old patient admitted in our department with light left hemiparesis (MRC MMT 4/5), light left hypoesthesia and transitory and remitted state of temporo-spatial confusion that debuted around six weeks prior to presentation. The neurological exam confirmed the sensorimotor deficit and displayed the existence of left neglect syndrome. The patient’s MRI revealed a giant temporo-parieto-occipital mass almost 7 cm in diameter that appeared to have an intraventricular starting point and showed a relatively homogeneous contrast enhancement with significant mass effect. A concomitant superficial parieto-temporal lesion was also noted. Both lesions were excised in a successive manner from superficial to deep, using a right parieto-temporal craniotomy. While the giant mass had a yellowish color and an extremely hard consistency, the superficial one was easily suctionable and had a grey-reddish color and was extremely well vascularized, raising the suspicion of concomitant brain lesions of different histology. Postoperatively the patient underwent a routine thoracic CT scan in the context of a position related soft tissue injury that revealed the existence of two pulmonary lesions, further deepening our suspicion of concomitant brain lesions of different histology. Conclusions Although extremely rare, the appearance of two histologically distinct brain lesions in patients with no evidence of a genetic condition should be taken into consideration especially in the context of intraoperative differences in order to successfully guide the patients’ follow-up treatment.

MULTUM IN PARVO-THE GLASGOW COMA SCALE

Introduction Coma assessment scales have been developed to facilitate ease of communication between emergency team members and to facilitate ease of clinical assessment for patients with severe impairment of consciousness. In 1974 Graham Teasdale and Bryan Jennett published in the Lancet a scale which theoretically helped physicians get a quick and accurate status of comatose patients. The scale they described assessed patient behaviour regarding three key aspects – motor reactivity, verbal communication and eye opening. As the two authors were working in Glasgow, the scale was dubbed the Glasgow Coma Scale (GCS) a name which all neurologists and neurosurgeons are well-accustomed with. Material The use of the GCS is based on the patient’s capacity to react using language and motion to external stimuli. Eye movement (1-4 points), Speech (1-5 points) and Motion (1-6 points) for a maximum total of 15 points or a minimum total of 3 points. A patient with a Glasgow Coma Score of 3 is completely non-reactive, while a patient with a Glasgow Coma Score of 15 is perfectly aware. This scale introduced in neurotrauma has simplified enormously the communication in neurosurgery, accompanying neurotrauma to the whole spectrum of neurosurgical pathology. Despite its wide use today, the GCS has been seriously criticised due to its incapacity to determine the functional status of brainstem structures. Therefore, various improvements and updates were performed for the Glasgow Coma Scale. Conclusions Over the years, the use of the GCS extended in the entire medical meme despite its criticism. The simplicity and ease of use which characterize the GCS made it a very useful instrument for neurological examination since the first moment a patient is seen by a medical professional. The Glasgow Coma Scale which recently reached its 50th birthday became a universal language for physicians. Since it has been in use neurological status can be expressed with great ease and without loss of meaning.

SURGICAL RESECTION OF A COMPLEX TUBERCULUM SELLAE MENINGIOMA ENCASING MAJOR ARTERIES

Introduction Though constituting only a minor proportion (1%) of sellar masses, suprasellar meningiomas pose a significant diagnostic challenge within the region of sellar and suprasellar tumors. Pituitary adenomas, at nearly 90% prevalence, dominate this anatomical region. Distinguishing these two entities is paramount, as both clinical presentation and, to some extent, biochemical profiles exhibit notable overlap. Accurate preoperative differentiation is critical due to the contrasting behaviors of these tumors. Meningiomas, with their robust vascularity supplied by dural and osseous arteries, present a heightened risk of intraoperative hemorrhage. Clinical Case A 53-year-old woman presented with a two-month history of worsening headaches and gradual vision loss. Neurological examination revealed only visual acuity decline. Her hormone profile showed elevated prolactin but lacked clinical features of prolactin excess, suggesting a possible prolactin-secreting pituitary macroadenoma. A contrast-enhanced MRI confirmed a significant sellar and suprasellar mass with homogeneous enhancement, extending to the right paramedian region and encasing the right internal carotid artery (ICA) at its critical segment involving the cavernous and supraclinoid portions. Furthermore, the right middle meningeal artery (M1) and anterior cerebral artery (A1) originated from this same location, highlighting the high surgical risk. Surgical resection was deemed necessary. The patient underwent a right pterional craniotomy, revealing a grayish, extradural soft mass lesion encasing the ICA bifurcation. Careful microsurgical dissection with basal devascularization was performed, followed by near-complete tumor removal using an ultrasonic aspirator to minimize damage to the encased arteries. Postoperative follow-up CT scan confirmed successful near-total tumor ablation. Conclusion This case underscores the critical role of meticulous preoperative diagnosis in managing complex sellar tumors. While pituitary adenomas dominate this region, suprasellar meningiomas, despite their rarity, demand a high index of suspicion. Overlapping clinical and biochemical presentations highlight the reliance on advanced imaging for accurate differentiation. The intricate vascularity of meningiomas, as exemplified by the encasement of critical arteries in this case, necessitates meticulous surgical technique with devascularization strategies. This successful near-complete tumor removal emphasizes the importance of such surgical expertise in navigating these diagnostically challenging sellar lesions.

Clinical and neurophysiological aspects of peripheral neuropathy in patients with myelodysplastic syndromes.

Myelodysplastic syndromes (MDS) are a heterogeneous group of clonal disorders, manifesting multiple clinical autoimmune inflammatory phenomena, including rarely peripheral neuropathy. Twenty-four patients diagnosed with MDS and 29 healthy subjects were enrolled in this prospective study in a 5-year period. Every subject was assessed by symptoms questionnaire and clinical neurological examination followed by nerve conduction studies, quantitative sensory testing and skin biopsy. Peripheral neuropathy was diagnosed in 12 subjects (50%). Our study indicated that peripheral neuropathy involving large and small nerve fibres, with a symmetrical length-dependent pattern, is not uncommon between patients with MDS.

Barriers and Facilitators Associated With Remote Concussion Physical Assessments From the Perspectives of Clinicians and People Living With Workplace Concussions: Focus Group Study.

Evaluating the clinical status of concussions using virtual platforms has become increasingly common. While virtual approaches to care are useful, there is limited information regarding the barriers and facilitators associated with a virtual concussion assessment. This study aims to identify the barriers and facilitators associated with engaging in virtual concussion assessments from the perspective of people living with workplace concussions; identify the barriers and facilitators to completing virtual concussion assessments from the perspectives of clinicians; and identify the clinical measures related to 4 clinical domains that would be most appropriate in virtual practice: general neurological examination and vestibular, oculomotor, and cervical spine assessment. We also evaluated effort. Separate online focus groups were conducted with expert concussion clinicians and people living with workplace concussions. A moderator led the focus groups using a semistructured interview guide that targeted a discussion of participants' experiences with virtual assessments. The discussions were recorded, transcribed, and analyzed by 2 reviewers using content analysis. Barriers and facilitators associated with completing the physical concussion examination were categorized based on the domain of the concussion examination and more general barriers and facilitators. Clinician-selected measures believed to work best in a virtual practice were described using frequency counts. A total of 4 focus groups with 15 people living with workplace concussions and 3 focus groups with 14 clinicians were completed using Microsoft Teams. Barriers were identified, such as triggering of symptoms associated with completing an assessment over video (mentioned 13/162 (8%) and 9/201 (4%) of the time for patient and clinician participants, respectively); challenges with location and setup (mentioned 16/162 (10%) of the time for patient participants); communication (mentioned 34/162 (21%) and 9/201 (4%) of the time for patient and clinician participants, respectively); and safety concerns (mentioned 11/162 (7%) of the time for patient and 15/201 (7%) for clinician participants). Facilitators were identified, such as having access to support (mentioned 42/154 (27%) and 21/151 (14%) of the time for patient and clinician participants, respectively); implementing symptom management strategies throughout the assessment (mentioned 11/154 (7%) of the time for patient participants); and having access to resources (mentioned 25/151 (17%) of the time for clinician participants). From the perspective of the clinician participants included in this study, the clinical measures recommended most for a virtual practice were finger to nose testing; balance testing; the Vestibular/Ocular Motor Screening tool; saccades; and cervical spine range of motion within their respective domains (ie, neurological examination, vestibular, oculomotor, and cervical spine assessment). Virtual assessments appear to be useful for both people living with workplace concussions and clinicians. While barriers were identified, such as challenges associated with exposure to screens, virtual assessments have benefits such as improved access to care. The clinician-selected measures that were considered best in a virtual practice will be investigated in an upcoming evaluative study. RR2-10.2196/40446.

Predictive Modeling and Early Detection of Parkinson's Disease Using Machine Learning

Parkinson's disease is a progressive neurodegenerative disorder that impacts millions of citizens in the USA, mainly targeting the motor system and causing debilitating symptoms such as rigidity, tremors, and bradykinesia. The diagnosis of Parkinson's disease is presently heavily dependent on clinical evaluations and neurological examinations, targeting the detection of motor dysfunction. The principal aim of this study was to use machine learning as a means for early detection and prediction of Parkinson's disease. The dataset utilized for this study was the Parkinson’s Disease Dataset, retrieved from the UCI Machine Learning Repository, which included comprehensive biomedical voice measurements from a cohort of individuals, 23 of whom are diagnosed with Parkinson’s disease and 8 who are healthy controls. This dataset included a set of features extracted from voice recordings. These include parameters like fundamental frequency (pitch), amplitude variation, jitter, shimmer, and several phonation-related measures known to reflect early vocal impairments associated with the disease in question. This research project deployed three credible and proven algorithms, namely, logistic regression, random forest, and the Support Vector Machines. Besides, this study employed a combination of metrics, including accuracy, precision, recall, F1-score, and ROC-AUC, which are more holistic toward the model performance. According to the metric performance results of the three models, several key insights were drawn into the early detection of Parkinson's Disease. Particularly, it was clear that the Random Forest model had superior accuracy and was the most reliable in classifying positive cases and healthy patients, which could be that this model turned out to be most reliable in an early detection setting. In that respect, predictive modeling in Parkinson's Disease is a capability frontier that has the potential to make much difference in clinical decision-making. Supported by Machine Learning algorithms, clinicians can trace the minute patterns in patient data, which are not easily visible through any other diagnostic means. In such cases, early detection of Parkinson's Disorder with vocal biomarkers or motor assessment may afford healthcare professionals opportunities for early interventions that might retard the disease process.

Abstract 4117646: The Ultimate Test in Hemocompatibility: HeartMate3 Restart After Prolonged Pump Shut Down

Introduction: The HeartMate 3 (HM3) LVAD, was shown to have a higher survival free of hemocompatibility related adverse events (HRAE) compared to its predecessors (HM2, HVAD). Superior HM3 outcomes are attributed to wide blood flow pathways coupled with frictionless movement and intrinsic pulsatility, reducing shear stress and blood stasis. It is unknown if improved hemocompatibility can withstand pump restart after prolonged shutdown. We herein report a case of HM3 pump stoppage without subsequent HRAE. Case Presentation: A 41-year-old male underwent HVAD implant in 2019 for advanced non-ischemic cardiomyopathy. This was exchanged to HM3 for recurrent neurological events despite therapeutic anticoagulation. Ten months after exchange, he awakened one morning to find his LVAD had been off for an unknown period but had not heard any device alarms (85dB if on battery power, 165dB if on wall power). Since he felt well he changed to battery power resulting in immediate pump restart. Log file analysis showed pump stoppage 2 am – 10 am, without preceding low flow alarms or power elevations. Management and Outcomes: In the ER INR was 1.5 (2.8 one week prior) and systemic heparin was started. Evaluation included: 1) CT brain without acute infarcts 2) echocardiogram without intracardiac thrombus 3) CT Angiography with patency of the inflow cannula and outflow graft 4) stable serial LDH measurements. The controller was exchanged, and analysis noted normal function. 1-year later the patient is maintained on warfarin and aspirin 325mg without further HRAE. Given the patient’s neurologic history and pump stoppage event, we did not invoke ARIES trial guidance and thus continued aspirin. Conclusion: Pump stoppage occurs when there is complete battery depletion, disconnection of both power leads, or the percutaneous lead from system controller. Our case is unique in that the duration of pump shutdown was 8 hours and INR subtherapeutic, without HRAE in the background of neurologic events on HVAD support. It has been previously reported that complete outflow graft thrombosis can occur shortly after LVAD decommissioning. The HM3 User Manual recommends restarting the pump immediately if off for a few minutes and using clinical judgment for longer durations due to increased risk for thromboembolic events. Our case adds to the paucity of existing data on improved hemocompatibility of the HM3 during rare circumstances of the ultimate test in hemocompatibility: complete pump shut down.

Assessing corneal dendritic cells in glucose dysregulation small-fibre neuropathy.

Small-fibre neuropathy (SFN) is associated with glucose dysregulation, including impaired glucose tolerance (IGT) and type 2 diabetes (T2D). Corneal confocal microscopy (CCM) offers a non-invasive tool to assess corneal nerve damage and dendritic cell density (DCD). In this study, we investigated corneal DCD in patients with SFN and glucose dysregulation, defined as IGT or T2D. We enrolled 38 patients with SFN + glucose dysregulation, 51 with SFN + non-glucose dysregulation and 20 healthy controls. All participants underwent neurological examination, neurophysiology and CCM. Individuals with SFN and glucose dysregulation had higher DCD compared with healthy controls (p = .01), and mature DCD was higher in IGT SFN patients than in T2D patients. Higher DCD in IGT compared with controls and patients with established T2D may suggest that DCD is a biomarker of early neuropathy.

NCOG-43. THE APPLICATION OF INDUCTION CHEMOTHERAPY COMBINED WITH RADIOTHERAPY IN YOUNG ADULTS WITH CNS GERM CELL TUMORS

Abstract BACKGROUND Central nervous system (CNS) germ cell tumors are rare brain neoplasms, accounting for approximately 0.3-0.5% of all CNS tumors. A combination of radiotherapy and chemotherapy is the standard of care (SOC) in pediatric patients. However, there is no SOC for young adult or adult patients. This study evaluates a regimen of induction chemotherapy followed by radiation in treating young adults with CNS germ cell tumors. METHOD Nine young adult patients diagnosed with pure CNS germinomas were identified in our institution. They underwent a treatment regimen of induction chemotherapies that were planned for four cycles and consisted of bleomycin (three doses per cycle), cisplatin (five doses per cycle) and etoposide (five doses per cycle) followed with whole brain and/or ventricular radiotherapy. The patients’ overall survival (OS), neurological function (including cognition), and adverse events (AE) were analyzed. RESULT Among the nine patients (seven males and two females), the median age is 20 years (range: 17-33 years). They received bleomycin, cisplatin and etoposide (median: two cycles, range: 1-4), and subsequent radiotherapy with plans based on tumor location by radiation oncologists, with a median radiotherapy dose of 45 Gy (range: 36-50.4). Seven patients had a complete response after chemotherapy. The median OS is 53 months (range: 39-121). There is no identifiable cognitive impairment by neurological exam. All patients experienced at least one, reversible, grade 3 AE of myelosuppression. All patients return to their normal baseline including employment. Results of neuropsychological evaluation and all AEs will be presented at the conference. CONCLUSION Induction chemotherapy followed by radiotherapy is potentially safe and effective with manageable and reversible side effects from chemotherapy without cognitive and functional impairment in the nine young adult patients with CNS germ cell tumors. A prospective study of this approach in a clinical trial setting is warranted.

NCMP-05. CLINICAL AND RADIOGRAPHIC IMPROVEMENT WITH REPLACEMENT OF FOLATE METABOLITES IN A PATIENT WITH INTRATHECAL METHOTREXATE-INDUCED MYELOPATHY

Abstract Methotrexate (MTX)-induced myelopathy is a rare but serious adverse effect of intrathecal (IT) MTX, which may mimic subacute combined degeneration (SCD). MTX disrupts the normal synthesis of methionine, which plays an important role in maintaining myelin sheath integrity, resulting in degeneration of the dorsal columns. This degeneration does not respond to classic treatment with vitamin B12 and folate supplementation, unlike SCD. Here, we describe a case of a 45-year-old woman with Philadelphia-positive B-cell acute lymphoblastic leukemia treated with hyperCVAD regimen with ponatinib, who after 14 doses of prophylactic IT MTX, developed progressive lower extremity weakness, paresthesias, gait instability, saddle anesthesia, and bowel/bladder dysfunction. Her neurologic exam was also notable for diffuse hyporeflexia. The onset of symptoms followed a diarrheal infection, and an initial MRI spine showed no evidence of abnormal enhancement. She was initially treated with intravenous immunoglobulins for presumed acute inflammatory demyelinating polyneuropathy. She then re-presented one week later with progressively worsening symptoms. A repeat MRI spine revealed a new longitudinally extensive T2 hyperintense signal within the dorsal columns from T7 to T12. CSF results were notable for mild pleocytosis and increased myelin basic protein; the latter of which is often seen in MTX-induced myelopathy. The patient received high-dose administration of key metabolites of the methyl-transfer pathway including S-adenosylmethionine (PO 400mg three times daily), folinate (IV 20mg four times daily), cyanocobalamin (PO 1000mcg once daily) and methionine (PO 5mg once daily) for 7 days. This treatment regimen resulted in both clinical and radiographic improvement. Subsequent MRI spine demonstrated complete resolution of the abnormal signal in the thoracic cord. This case highlights (1) the diagnostic complexity of IT MTX-induced myelopathy (2) the role response to treatment plays in uncovering this diagnosis, and (3) how the replacement of folate metabolites facilitates both radiographic and clinical improvement in IT MTX-induced myelopathy.

SURG-45. OUTCOMES OF PATIENTS RECEIVING SURGICAL RESECTION AND STEREOTACTIC RADIOSURGERY VS. STEREOTACTIC RADIOSURGERY ALONE FOR MOTOR CORTEX METASTASES

Abstract BACKGROUND Local therapy for brain metastases typically involves stereotactic radiosurgery (SRS), with surgical resection considered for larger and/or symptomatic tumors. For lesions in eloquent areas, surgical management is controversial given the risks of neurologic deficits. This study compared clinical outcomes in patients with motor cortex metastases who received surgery + SRS or SRS alone. METHODS Patients treated for motor cortex metastases from 1/2018 – 12/2022 were identified. Demographic/preoperative variables included age, gender, race, tumor histology, adjuvant therapies, pre-treatment neurological exam, and Karnofsky Performance Scale. Treatment variables included the number of brain metastases, maximum tumor diameter, and radiation dose/number of fractions. Outcome measures included post-treatment neurological exam, seizures, and overall survival (OS). RESULTS 351 charts were identified – 80 surgery + SRS and 271 SRS alone. 50 consecutive patients in each group were included in this analysis. Surgery + SRS patients were younger (59.0 vs. 63.9; P=0.020) with larger tumors (2.8 vs. 0.83cm; P&amp;lt;0.001), while the SRS only group had more brain metastases per patient (2 vs. 6; P&amp;lt;0.001). No differences were seen in the remaining demographic/preoperative variables. Pre-treatment, surgery + SRS patients had significantly increased frequency (45/50, 90% vs. 25/50, 50%; P&amp;lt;0.001) and severity (4 vs. 5; P=0.001) of motor deficits, and a higher likelihood of seizures (25/50, 50% vs. 8/50, 16%; P&amp;lt;0.001). Despite the surgery + SRS group being more symptomatic pre-treatment, the frequency and severity of motor deficits and seizures was similar between cohort’s post-therapy. Surgery + SRS patients also demonstrated higher rates of pre- to post-treatment improvement in motor function (29/45, 64% vs. 3/25, 12%; P&amp;lt;0.001) and longer OS from the date of treatment (7.5 vs 4 months; P=0.021). CONCLUSION Given the equivalent neurological outcomes, higher rates of functional improvement, and improved oncologic outcomes, surgery should be a consideration for patients with brain metastases in eloquent areas, particularly those with symptomatic lesions.

NCOG-11. FACTORS PREDICTING SPEECH AND GAIT RECOVERY IN CHILDREN WITH POST-OPERATIVE POSTERIOR FOSSA SYNDROME (PFS)

Abstract Posterior fossa syndrome/cerebellar mutism syndrome is a surgical complication of posterior fossa tumor resection, especially midline tumors. Initially thought to involve complete mutism, PFS is now recognized as a complex syndrome involving loss of language (complete [PFS1] and incomplete [PFS2]). Additional symptoms include apraxia, movement disorder, motor and coordination deficits, and emotional lability. The aim of this study was to identify factors that influence delayed speech and independent gait recovery in children with PFS. We prospectively studied 73 children ≥3 years of age with medulloblastoma who developed PFS (SJMB12, NCT01878617). Enrolled children received standardized neurologic examinations and serial imaging before, during and up to five years after completion of therapy. Neurological examinations included scale for assessment and rating of ataxia (SARA), CTCAE grading of neurologic deficits, and severity of injury to the superior cerebellar peduncles (SCP) on post-operative MRI. Median time to speech recovery was 1.4 months (range 0.3-14.3); 81% had return of speech at 3 months and 97% at 12 months. On multivariable Cox regression analysis, complete mutism (HR 0.17, p&amp;lt;0.0001) and inability to sit (HR 0.40, p=0.001) were associated with delayed speech return. Independent ambulation was regained by 80.8% at a median of 2.3 (range 0.3-71.3) months. 70% had a return of ambulation at 12 months and 77% at 24 months. On multivariable Cox regression analysis, complete mutism (HR 0.32, p=0.016), inability to sit (HR 0.30, p=0.002), SARA score (HR 0.94, p=0.002), and right SCP injury (HR 0.47, p=0.014) were significantly associated with delayed return of ambulation. Presence of mutism, inability to sit unassisted, higher SARA score, and right SCP injury at the time of PFS diagnosis are associated with time to recovery of speech/language and ambulation. Our findings have prognostic implications for families and to help identify children in need of more intensive interventions.

SURG-31. HYBRID THERAPY FOR NEWLY DIAGNOSED AND RECURRENT GLIOBLASTOMA: STAGED PROCEDURE INTEGRATING OPEN SURGICAL RESECTION WITH LASER INTERSTITIAL THERMAL THERAPY

Abstract BACKGROUND Glioblastoma (GBM) is the most common and aggressive primary malignant brain tumor in adults with a median overall survival (OS) of 15.6 months. Despite advancements, 5-year survival rates have not significantly improved. Laser interstitial thermal therapy (LITT) has been employed to treat both newly diagnosed (nGBM) and recurrent GBM (rGBM) with demonstrable safety and efficacy. Traditionally, LITT has been limited to smaller lesions given physical and thermal constraints. OBJECTIVE We present two case examples of a novel, hybrid treatment approach to GBM, utilizing both surgery and laser ablation as part of a single-staged procedure. METHODS Two geriatric (&amp;gt;65 years) patients with nGBM or rGBM were treated with surgical resection followed by LITT the subsequent day. Both patients underwent preoperative magnetic resonance imaging (MRI) showing multifocal disease with a dominant component, causing mass effect in the second case, and an unresectable component. RESULTS The first case underwent surgical resection of a large, multicentric rGBM in the parieto-occipital region, followed by LITT of an unresectable lesion in the inferior temporal gyrus. The second patient underwent surgical resection of the dominant temporal lobe component, and LITT of the insular component. Neither patient had intraoperative or ablation complications. Both patients had stable postoperative neurological exams and were discharged on the day after ablation, with no new deficits at follow-up. CONCLUSION The prognosis for GBM remains dismal despite significant research and attempts at advancements in patient management. Surgical resection is a critical component of the GBM treatment paradigm. However, surgery alone is not sufficient in many patients. LITT has shown promise for improving GBM outcomes, mainly due to its minimal invasiveness, enhanced immune responses, BBB disruption, and cytoreductive potential. We demonstrate a novel, hybrid approach to GBM using hybrid surgical resection and LITT in a single-staged procedure to overcome traditional constraints of LITT.

Small fiber neuropathy associated with COVID-19 infection and vaccination: A prospective case-control study.

Small fiber neuropathy (SFN) after both COVID-19 infection or vaccination has been reported in sporadic cases, but a detailed description and comparison are missing. We aimed to screen a large cohort of patients complaining of pain and autonomic symptoms after COVID-19 natural infection or vaccination to ascertain the presence of SFN and its correlation with autoimmune diseases. We prospectively recruited for this case-control study 66 patients: 33 developing sensory and autonomic symptoms after a natural COVID-19 infection (P-COVID) and 33 after a mRNA vaccination against COVID-19 (P-VAC). We also used 33 matched healthy controls (HC) collected before 2019 when the COVID-19 virus appeared. Patients underwent neurological examination and clinical scales, an extensive serum screening, and skin biopsy to detect small nerve fiber involvement. Clinical scales showed higher scores for autonomic symptoms in P-COVID patients than in P-VAC patients, but the other scales did not differ. P-COVID and P-VAC patients showed a significant decrease in somatic small nerve fibers compared with HC, whereas autonomic innervation did not differ. SFN was more frequent in P-COVID patients (94%) than in P-VAC patients (79%). Epidermal innervation was correlated with clinical scales for pain and autonomic dysfunctions. Autoimmune abnormalities were frequent in both groups but importantly they were not correlated with SFN. Somatic SFN was frequently found in both P-COVID and P-VAC patients, with a higher incidence in the former group. Spared skin autonomic innervation was spared in both groups although a subtle autonomic involvement in P-COVID patients was suggested by a high COMPASS-31 scale score. SFN was not correlated with autoimmune dysfunctions, although autoimmune diseases were frequent in both groups.

SURG-59. A RARE CASE OF COMBINED OCCURRENCE OF GLIOBLASTOMA MULTIFORME AND PHEOCHROMOCYTOMA: A CASE REPORT AND LITERATURE REVIEW

Abstract Glioblastoma is most common and malignant primary brain tumors. However, combined other solid organ malignancies sometimes occurred. To our knowledge, the combination of glioblastoma multiforme and pheochromocytoma is an exceedingly rare occurrence. As a result, we present the case of a 61-year-old male patient who suffered from slurred speech and unsteady gait due to left frontal glioblastoma with concurrent pheochromocytoma. The clinical course was retrospectively collected from medical records, including the lab data, imaging, surgical procedure and neurological examination results. We also provide a comprehensive review and discussion of the association between pheochromocytoma and glioblastoma, including genetic disorder the management strategy. The fluctuated blood pressure during brain surgery implied suspicious concurrent endocrine tumor in this patient. The patient was ultimately diagnosed with adrenal pheochromocytoma after left adrenalectomy and radical nephrectomy by urologist. No other malignancies or genetic disorders were found in this patient. Subsequently, this patient recovered well without any neurologic deficit and with effectively controlled blood pressure. The presence of a concomitant pheochromocytoma may precipitate paroxysmal catecholamine surges, fluctuations in cerebral perfusion, and an elevated risk of significant intra-cranial blood loss during neurosurgery. Once patient of brain tumors presented with uncontrolled or fluctuated blood pressure occurred, concurrent endocrine tumor should be considered.

Split hand and minipolymyoclonus in spinocerebellar ataxia type 3: a case report

BackgroundSpinocerebellar ataxia type 3 (SCA3), also known as Machado–Joseph disease, is an autosomal dominant neurodegenerative disorder caused by CAG repeat expansion in exon 10 of ATXN3. Extra-cerebellar manifestations, including external ophthalmoplegia, dystonia, Parkinsonism, and peripheral neuropathy, are predominantly present in SCA3 cases. Here, we report a case of SCA3 presenting with a split hand and minipolymyoclonus.Case presentationA 73-year-old female patient presented with a 5-year history of ataxic gait. Neurological examination revealed cerebellar ataxia and minipolymyoclonus in the digits on both sides and muscle atrophy in the right hand, consistent with the split hand pattern. Electrodiagnostic studies demonstrated decreased amplitude of compound muscle action potentials and neurogenic motor unit potentials, indicating lower motor neuron involvement.ConclusionsOur patient’s case indicated a split hand and minipolymyoclonus in SCA3. Clinicians should consider these extra-cerebellar manifestations in patients with SCA3. Although neither split hand nor minipolymyoclonus are likely to directly result in a specific etiological diagnosis, a common pathophysiological mechanism for both may be lower motor neuron involvement. This extracerebellar manifestation contributes to narrowing down the diagnostic possibilities for cases presenting with progressive cerebellar ataxia.