Residual Neurological Deficits Research Articles

Optimization of laboratory diagnostics of neuromyelitis optica spectrum disorders: indications and algorithms

Neuromyelitis optica spectrum disorders are a group of autoimmune demyelinating diseases of the central nervous system characterized by severe exacerbations with development of residual neurological deficit. Anti-aquaporin-4 antibody is one of key factor in diagnosing, differentiating, and prescribing pathogenetic therapy. The paper discusses tests and methods of detecting anti-aquaporin-4 antibodies.

МУЛЬТИСИСТЕМНЫЙ ВОСПАЛИТЕЛЬНЫЙ СИНДРОМ, АССОЦИИРОВАННЫЙ С COVID-19 У ДЕТЕЙ, ОСЛОЖНЕННЫЙ ВЕНОЗНЫМИ И АРТЕРИАЛЬНЫМИ ТРОМБОЗАМИ: СЕРИЯ КЛИНИЧЕСКИХ НАБЛЮДЕНИЙ

SARS-CoV-2 causative agent may result in adulthood in hyperinflammatory syndrome and cytokine storm in some patients leading to the microvascular bed thromboses as well as those of large venous and arterial vessels. In the COVID-19 critical form, multisystem inflammatory syndrome in children (MIS-C), thromboses are less common. Authors represent a series of 8 patients’ cases with both thromboses and MIS-C associated with COVID-19 of different localization hospitalized in Nov. 2020-Nov. 2022 aged 4 months to 17 years old (Me 7.5 y/o): 4 aged 4 m/o to 5 y/o and 4 aged 8 to 17 y/o; 6 boys/2 girls. The incidence of thromboses was 13.3% (8/60 pediatric patients with MIS-C); venous thrombosis occurred in 6 (10% of 60), arterial thrombosis in 4 (6.6%), large cerebral vessels thrombosis coupled with the development of bilateral stroke occurred in 2 (3.3%), secondary thrombotic microangiopathy (TMA) in 3 (5%), distal gangrene in a single case (1.7%) and the cerebral venous sinus thrombosis in a single case (1.7%) as well. Treatment was carried out with anticoagulants, aspirin, immunomodulators (intravenous immunoglobulin, glucocorticosteroids, genetically engineered biological drugs). One of 8 patients with thromboses had died from pulmonary embolism (1.7% among all 60 patients with MIS-C), 6 of the 7 survivors continue to be followed up without complications and a single patient continues to have residual severe neurological deficits. Conclusion: venous thrombosis, secondary TMA and large cerebral arteries thrombosis predominated. Treatment of thrombosis with unfractionated heparin intravenously by continuous infusion with a transition to low molecular weight heparins against the background of pathogenetic therapy for MIS-C with immunomodulators was effective in most patients. Acetylsalicylic acid was used in 3 (with coronary dilatation and/or absence of severe thrombocytopenia). Risk factors for thromboses were male gender, severe congenital underlying diseases.

Pulsed field ablation versus very high power short duration radiofrequency ablation in atrial fibrillation: what is better?

Abstract Background The newly introduced non-thermal pulsed field ablation (PFA) is a promising technology for catheter ablation (CA) of atrial fibrillation (AF) with high acute success rates and good safety features. However studies have shown that very high power short duration (VHPSD) ablation is also highly effective and fast with potentially less arrhythmia recurrence compared to conventional radiofrequency ablation. Data comparing this two methods are lacking Objective The aim of this study is to compare two source of energy used for CA of AF in terms of procedural, clinical and outcome data Methods We conduced a retrospective observational study enrolling all patients (pts) from september 2021 to may 2023 who underwent CA of AF with PFA system and VHPSD. In the PFA group pulmonary veins isolation (PVI) was obtained using 2KV with eight application each vein, posterior wall isolation (PWI) of left atrium (LA) was obtained with application in the flower configuration of the catheter and then additional lesion were deployed at the operator’s discretion. In the VHPSD group the encircling of PV was obtained with 90W for 4 seconds radiofrequency pulses in posterior portions of the LA including LA PW, whereas 50W with ablation index target of 500 was used in the anterior portions of the PV and additional lesions. General anesthesia or deep sedation was carried out in all pts Results A total of 205 pts were included, n = 86 (42%) in the PFA group and N = 119 (58%) in the VHPSD group: paroxysmal (n = 62[72%], N = 64[54%]), persistent (n = 19[22%], N = 43[36%]), long standing persistent (n = 5[5%], N = 10[8%]). PVI was successful in all pts and additional lesions were delivered in n = 26 (30%) in PFA group and N = 76 (64%) in HVPSD group, mostly at the PW (n = 24[92%]; N = 50[66%]). The PFA group revealed a shorter procedura duration (80 ± 29 min vs 108 ± 39 min; p = 0,00001) but longer fluoroscopic time (21 ± 8 min vs 14 ± 10 min; p = 0,00001). The VHPSD group revealed more complications (N = 7[6%] vs n = 3[3%]) but without statistically significant difference (p = 0,43), the most frequent in the PFA group was vascular access complications instead in the VHPSD group was post-procedural pericarditis. Only one patient in VHPSD group had major complication with a post-procedural stroke but without residual neurological deficits. At follow-up after median of 14 (26-6) months, n = 61 pts in the PFA group (71%) and N = 91 in the VHPSD group (76%) were free from atrial arrhythmia (p = 0,79). In both group 7 pts recurrenced as atypical atrial flutter (n = 7[28%] PFA; N = 7[25%] VHPSD; p = 0,061) Conclusion PFA and VHPSD are effective and safe to CA of AF with comparable arrhythmia recurrenses. However procedure duration with PFA is significantly shorter and therefore may be of potential benefit in particular for elderly and frail pts. Further research, including randomized controlled trials, is needed to validate and compare these techniques more comprehensively

Phase I Trial of GD2.CART Cells Augmented With Constitutive Interleukin-7 Receptor for Treatment of High-Grade Pediatric CNS Tumors.

T cells modified with chimeric antigen receptors (CARTs) have demonstrated efficacy for hematologic malignancies; however, benefit for patients with CNS tumors has been limited. To enhance T cell activity against GD2+ CNS malignancies, we modified GD2-directed CART cells (GD2.CARTs) with a constitutively active interleukin (IL)-7 receptor (C7R-GD2.CARTs). Patients age 1-21 years with H3K27-altered diffuse midline glioma (DMG) or other recurrent GD2-expressing CNS tumors were eligible for this phase I trial (ClinicalTrials.gov identifier: NCT04099797). All subjects received standard-of-care adjuvant radiation therapy or chemotherapy before study enrollment. The first treatment cohort received GD2.CARTs alone (1 × 107 cells/m2), and subsequent cohorts received C7R-GD2.CARTs at two dose levels (1 × 107 cells/m2; 3 × 107 cells/m2). Standard lymphodepletion with cyclophosphamide and fludarabine was included at all dose levels. Eleven patients (age 4-18 years) received therapy without dose-limiting toxicity. The GD2.CART cohort did not experience toxicity, but had disease progression after brief improvement of residual neurologic deficits (≤3 weeks). The C7R-GD2.CART cohort developed grade 1 tumor inflammation-associated neurotoxicity in seven of eight (88%) cases, controllable with anakinra. Cytokine release syndrome was observed in six of eight (75%, grade 1 in all but one patient) and associated with increased circulating IL-6 and IP-10 (P < .05). Patients receiving C7R-GD2.CARTs experienced temporary improvement from baseline neurologic deficits (range, 2 to >12 months), and seven of eight (88%) remained eligible for additional treatment cycles (range 2-4 cycles). Partial responses by iRANO criteria were observed in two of seven (29%) patients with DMG treated by C7R-GD2.CARTs. Intravenous GD2.CARTs with and without C7R were well tolerated. Patients treated with C7R-GD2.CARTs exhibited transient improvement of neurologic deficits and increased circulating cytokines/chemokines. Treatment with C7R-GD2.CARTs represents a novel approach warranting further investigation for children with these incurable CNS cancers.

Use of thrombolytic therapy for treatment of stroke with ischemic type within the standard therapeutic window

The study focuses on stroke, the second leading cause of death worldwide. It specifically addresses the ischemic type of stroke, which constitutes the majority of cases globally and in Ukraine, driving the search for innovative and effective diagnostic and treatment strategies. Among these, reperfusion of brain tissue within the first hours of the disease can prevent irreversible brain damage or minimize its extent, thus reducing the degree of residual neurological deficit. Objective — to compare the outcomes between standard therapy and thrombolytic therapy (TT) in treating ischemic stroke, evaluating the efficacy of TT within the standard therapeutic window to optimize patient care. Materials and methods. The study was conducted in the Neurological Department No. 2 of Kyiv City Clinical Hospital No. 4 from July to December 2023. A total of 247 cases of ischemic type stroke were analyzed. 69 patients who were admitted within the standard therapeutic window were selected. They were divided into two groups. The experimental group included 42 patients aged between 42 and 89 years (average age — (71.0 ± 17.8) years) without contraindications, who agreed (themselves or through relative’s consent) to undergo TT. Alteplase was administered intravenously at a dose of 0.9 mg/kg body weight, 10 % of the dose as a bolus (over 1 minute), and 90 % within one hour. The control group consisted of 27 patients aged between 47 and 88 years (average age — (76.11 ± 8.9) years), who had contraindications for TT. They received standard therapy. Evaluations were conducted using the modified Rankin Scale, which allows for assessing the level of disability or dependence on external assistance in patients’ daily lives after a stroke. Results. The study demonstrated that TLT significantly improves the functional status of patients compared to standard therapy, as evidenced by a decrease in the level of disability to 0—1 points on the modified Rankin Scale at discharge from the hospital: in the control group — in 3 (11.1 %) patients, in the experimental group — in 16 (35.7 %, p = 0.047), i. e. the use of TLT significantly improved the functional status of more patients. In the experimental group, 4 patients died (9.5 %), in the control group — 5 (18.5 %). Conclusions. TLT effectively promotes the restoration of neurological functions and reduces the level of disability in patients with ischaemic cerebral stroke, which opens up prospects for further research. The results obtained indicate the need for wider use of TLT in clinical practice, taking into account individual patient risks and contraindications.

Large Single-Center Experience with Short-Term Follow-up of Neqstent-Assisted Coiling.

Endovascular treatment of wide-neck bifurcation aneurysms has historically proved difficult with variable outcomes. Different endovascular techniques such as balloon-assisted coiling, stent-assisted coiling, or intrasaccular devices provide a varied range of efficacy and safety. Neqstent-assisted coiling is a new device and technique that aim to provide a maximum of both. We analyzed the early clinical and radiologic outcomes after the use of this new technique and device in our practice. This study was a retrospective analysis of ruptured and unruptured intracranial aneurysms treated with the Neqstent. The primary radiologic outcomes were quantified on DSA, CTA, or MRA using the modified Raymond-Roy criteria. The outcomes were defined as immediate complete occlusion (modified Raymond-Roy criteria 1) and complete (modified Raymond-Roy criteria 1) and adequate occlusion (modified Raymond-Roy criteria 1 and modified Raymond-Roy criteria 2) at 6 months posttreatment. The primary safety outcome was the rate of device-related adverse events. Secondary safety outcomes included time to discharge and change in the mRS score at 6-month follow-up. Twenty patients were treated with the Neqstent from November 2020 to January 2023. Nine had unruptured aneurysms, and 11 were patients with subarachnoid haemorrhage due to ruptured aneurysms. Eighteen of 20 aneurysms (90%) treated demonstrated complete occlusion (modified Raymond-Roy criteria 1) on immediate postembolization angiograms. Sixteen of 17 aneurysms treated (94.1%) remained adequately occluded on 6-month follow-up (modified Raymond-Roy criteria 1 and modified Raymond-Roy criteria 2). Immediate postoperative complications occurred in 2 patients; only 1 patient had residual neurologic deficits at 6 months (mRS = 2). Management of large, wide-neck aneurysms remains difficult, with high rates of recurrence and complications. The use of the Neqstent shows promising short-term results for the treatment of complex wide-neck aneurysms. Initial complication rates for our cohort were relatively high. However, this result is likely related to the initial learning experience of device deployment and the use of antiplatelets.

Patterns and Outcomes of Acute Central Nervous System Complications During Treatment of Childhood Acute Lymphoblastic Leukemia: A Single-center Experience.

Central nervous system (CNS) complications are considered adverse events during the treatment of pediatric acute lymphoblastic leukemia (ALL). This study aimed to assess the incidence, types, clinical and radiologic patterns, risk factors, and the fate of different CNS complications during the treatment of pediatric ALL. A retrospective study included 390 patients with pediatric ALL, treated according to St. Jude total XV protocol at the National Cancer Institute, Cairo University, from January 2012 to December 2017. Thirty-nine (10%) patients developed different types of CNS complications. Nineteen (4.9%) patients had cerebrovascular complications, 12 (3.1%) patients had posterior reversible encephalopathy syndrome (PRES), and 6 (1.5%) patients had leukoencephalopathy; both CNS infections and leukemic infiltrates were diagnosed in one patient each. CNS complications were significantly higher in patients older than 10 years old, patients with high-risk disease, and patients who were classified as CNS III status with a statistically significant P value of 0.040, 0.020, and 0.002, respectively. There were 31 (79.5%) cases that achieved complete recovery, 6 (15.4%) patients who died, and 2 (5.1%) patients who developed residual neurological deficits. In conclusion, pediatric patients with ALL, who presented with older age, high-risk disease initially, and had initial CNS III status, were at higher risk of developing acute CNS complications during their treatment period. Patients who developed visual disturbances were associated with unfavorable outcomes. Despite that, around 80% of patients showed complete recovery, but still, 15% of them died from these complications.

Autoimmune Parkinsonism: A Newer Manifestation of Contactin-Associated Protein-Like 2 Autoimmunity: A Case Report

Contactin-associated protein-like 2 (CASPR2) antibodies are part of an expanding spectrum of disorders. Although they were initially associated with Morvan’s syndrome and peripheral nerve hyperexcitability, their clinical manifestations are more varied than previously recognized. In this report, we present a rare case of a middle-aged woman who experienced gait disturbances, sleep disturbances, behavioral changes, and postural abnormalities over a period of five months. A thorough examination revealed a Parkinsonian phenotype. Considering the timeline and symptomatology, an autoimmune work-up was conducted, which detected CASPR2 antibodies in the patient’s serum. Treatment with high-dose intravenous Methylprednisolone followed by rituximab effectively reversed her clinical manifestations without residual neurological deficits.

Painless nondiabetic lumbosacral radiculoplexus neuropathy with complete recovery: a case report

BackgroundLumbosacral radiculoplexus neuropathy, also known as amyotrophy, is an uncommon monophasic disorder characterized by inflammation of the lumbosacral nerve roots and plexuses. Lumbosacral radiculoplexus neuropathy is usually associated with diabetes mellitus, is typically painful at presentation, and often associated with long-term residual neurologic deficits. We report a case of painless, nondiabetic lumbosacral radiculoplexus neuropathy in a young Chinese woman, who made a full recovery after treatment with intravenous immunoglobulin, adding an atypical case to the scarce literature on lumbosacral radiculoplexus neuropathy.Case presentationA 35-year-old Chinese woman presented to our emergency department with 1-week history of painless left lower limb weakness and numbness. Examination revealed weakness confined to the left lower limb but spanning various nerves and myotomes, with abnormal sensation. Clinical localization to the lumbosacral plexus was supported by neurodiagnostic tests, and magnetic resonance imaging of the lumbosacral plexus showed that the nerve roots were also involved. After treatment with intravenous immunoglobulin for nondiabetic lumbosacral radiculoplexus neuropathy, the patient had a full recovery.ConclusionOur patient’s case highlights that lumbosacral radiculoplexus neuropathy, an already rare disorder, can occur in the absence of diabetes mellitus and pain, making it even harder to recognize. A systematic and meticulous clinical approach, supported by intelligent selection of adjunctive tests, is required for localization and diagnosis. With an accurate diagnosis, our case also demonstrates that appropriate and prompt treatment can lead to complete recovery, despite previous reports suggesting a high prevalence of long-term residual deficits after lumbosacral radiculoplexus neuropathy.

GLOBRYTE: A Phase III, Open-Label, Multicenter, Randomized Trial Evaluating Glofitamab Monotherapy in Patients with Relapsed or Refractory Mantle Cell Lymphoma

Background and Significance: There is currently no standard of care for patients (pts) with relapsed or refractory (R/R) mantle cell lymphoma (MCL). Although Bruton tyrosine kinase inhibitors (BTKi) have improved clinical outcomes in R/R MCL, there is a high unmet need for new treatment options for pts who do not respond to, or progress through, BTKi therapy. Chimeric antigen receptor (CAR) T-cell therapies, such as brexucabtagene autoleucel, have shown promising outcomes, but alternative systemic therapies are still needed in this pt population. Glofitamab is a CD20xCD3 T-cell-engaging bispecific antibody that redirects T cells to eliminate B cells. A Phase I/II trial of glofitamab monotherapy with step-up dosing (SUD) and obinutuzumab pretreatment (Gpt; 1000 or 2000mg) to mitigate the risk of cytokine release syndrome (CRS) showed high and durable complete response (CR) rates (73.0%) and manageable, mostly low-grade CRS in heavily pretreated pts with R/R MCL (n=37), most of whom had failed prior BTKi therapy (Phillips et al. ASH 2021, Phillips et al. ASH 2022). Study Design and Methods : The GLOBRYTE study (GO43878; EU CT: 2023-503206-37-00) is a Phase III, open-label, multicenter, randomized, controlled trial that will evaluate the efficacy, safety, pharmacokinetics (PK), and pharmacodynamics (biomarkers) of glofitamab monotherapy in pts with R/R MCL, in comparison with an investigator's choice of rituximab + bendamustine (BR) or rituximab + lenalidomide (R-Len). Pts with histologically confirmed R/R MCL who have received ≥1 prior line of systemic therapy (including a BTKi) and have an Eastern Cooperative Oncology Group performance status of 0-2 are eligible for enrollment. Exclusion criteria include leukemic, non-nodal MCL; prior CAR T-cell therapy or CD20xCD3 bispecific antibodies; primary or secondary central nervous system (CNS) lymphoma or history of CNS lymphoma; and current or prior CNS disease (including stroke or transient ischemic attack in the past 2 years and residual neurologic deficits, epilepsy, or CNS vasculitis). Eligible pts will be randomly assigned (1:1) to receive glofitamab or investigator's choice of BR or R-Len stratified by line of therapy (1 vs ≥2) and response to last therapy (relapsed vs refractory). Pts will receive intravenous (IV) Gpt (2000mg) on Day (D) 1 of Cycle (C) 1, 7 days prior to the first glofitamab dose (Gpt may be split over 2 days as 1000mg doses if needed: the second 1000mg dose must be administered ≥1 day before initial glofitamab treatment). Pts will receive glofitamab for a fixed duration of 12 cycles, unless progressive disease (PD) or unacceptable toxicity occurs earlier. Glofitamab IV SUD will be given on C1D8 (2.5mg), C1D15 (10mg), and then the target dose (30mg) on D1 of C2-12 (21-day cycles). Pts randomized to the investigator's choice will receive 375mg/m 2 IV rituximab on D1 in combination with either 90mg/m 2 IV bendamustine on D1-2 of each cycle (BR for six cycles) or 20mg/day oral lenalidomide on D1-21 of each cycle (R-Len until PD); the cycle length for BR or R-Len is 28 days. Crossover to glofitamab from the investigator's choice is permitted in pts with radiologic confirmation of PD ( Figure). The primary endpoint is progression-free survival (PFS) determined by independent review committee (IRC). Treatment comparisons will be made with a 2-sided, level 0.05 stratified log-rank test. Secondary endpoints include overall survival (OS); IRC- and investigator-assessed CR rate, objective response rate, duration of response, and duration of CR; PFS (investigator-assessed); safety; health-related quality of life including time to deterioration in physical functioning or fatigue evaluated by the EORTC QLQ-C30; glofitamab PK; and immune response to glofitamab (including presence of anti-drug antibodies). Response will be determined according to Lugano criteria (Cheson et al. J Clin Oncol 2014). Stratified hazard ratios with 95% confidence intervals (95% CI) will be calculated for PFS and OS, and odds ratios (95% CI) will be calculated for the difference in CR rate and overall response rate between treatment arms. Predictive and prognostic biomarkers (to characterize high-risk subgroups and minimal residual disease kinetics) will also be explored. An estimated 80 sites globally will enroll approximately 182 pts with R/R MCL (glofitamab n=91; BR or R-Len n=91 total) starting in Oct-Nov 2023 (APAC, US, and EU regions).

OTHER-11 DEMENTIA AND EPILEPSY REVERSED SUCCESSFULLY THROUGH STAGED EMBOLISATION OF A SYMPTOMATIC HYPERVASCULAR PARAGANGLIOMA : A CASE REPORT

Abstract INTRODUCTION Paragangliomas (PGs) are rare neuroendocrine tumours that can arise from parasympathetic glossopharyngeal and vagus nerves. Atypical presentations involving seizures and dementia can occur due to excessive catecholamine release. Physiological intracranial arteriovenous fistulae (AVF) can occur concurrently due to increased vascularity, especially in the cavernous and sigmoid sinuses. METHODS We present a case of a patient who developed a highly vascular AVF from an ipsilateral glomus jugulare PG, presenting with rapidly progressive but reversible dementia and epilepsy. CASE: A 64-year-old lady presented on several occasions with new-onset stereotyped episodes of confusional dysphasia suggestive of seizures, confirmed on electroencephalography. Further history revealed two years of progressive deafness and imbalance, and one year of increasing forgetfulness and executive dysfunction, which had deteriorated rapidly in the period of the patient's current presentations. Montreal cognitive assessment (MoCA) score was 21/30 at baseline (normal score &amp;gt;25/30), and she had no lateralising signs. Serial brain magnetic resonance imaging (MRI) revealed progressively worsening vasculopathic white matter hyper-intensities (WMHs). Eventual MR angiography showed a 14x22×23mm right glomus jugulotympanicum PG with numerous feeding arteries, and associated multiple AVFs (Cognard grade III). She was initially managed with anti-seizure medications and then discussed in our multidisciplinary neurosciences meeting. The patient underwent staged right glomus jugulare embolization over several weeks. Her cognitive function gradually improved, and at three months she had fully returned to normal functioning with a MoCA of 30/30 and no residual neurological deficit. The WMHs disappeared on follow-up MRI scans. CONCLUSION Our case highlights a rare clinical presentation of dementia and seizures from a right glomus jugulare PG with associated AVF. Multidisciplinary involvement was key in excluding other causes, and definitive treatment of the PG led to complete reversal of the patient’s dementia.

Clinical characteristics and long-term follow-up outcomes of myelin oligodendrocyte glycoprotein antibody-associated disease in Han Chinese participants.

Myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease (MOGAD) is an immune-mediated inflammatory demyelinating disease of the central nervous system. This study aimed to delineate the clinical manifestations, imaging features, and long-term outcomes in Chinese patients with MOGAD and analyze the recurrence-associated factors. The phenotypic and neuroimaging characteristics of 15 Han Chinese patients with MOGAD were retrospectively analyzed. Demyelinating attacks, MOG antibodies in the cerebrospinal fluid/serum, response to immunotherapy, follow-up outcomes, and recurrence-associated factors were recorded. The median age at disease onset was 34 years (range, 4-65 years). The most common initial presentations included vision loss (10/15, 66.7%) and seizures (5/15, 33.3%). Serum MOG-Ab titers in 14/15 cases were higher than those in the cerebrospinal fluid and were detected in 3/6 relapsed patients. Brain magnetic resonance imaging during acute attacks showed lesions in 10/15 patients (66.7%), mostly in the cortex/subcortical white matter (5/15, 33.3%). Recurrence occurred in 6/15 patients (40.0%); in 4 patients, recurrence occurred shortly after immunotherapy discontinuation. Residual neurological deficits were present in 5/15 patients (33.3%), including visual impairment, incapacitation, cognitive impairment, and speech reduction. Optic neuritis was the most common clinical manifestation of MOGAD. magnetic resonance imaging findings were heterogeneous and the cerebral cortex/subcortical white matter was the most susceptible brain region. Although patients in the acute phase responded well to methylprednisolone pulse therapy, the long-term recurrence rate was high. Consistently detected serum MOG antibodies and inappropriate maintenance immunotherapy may be associated with recurrence, and residual neurological deficits should not be ignored.

Powassan Virus Encephalitis: A Tertiary Center Experience.

Powassan virus (POWV) is an emerging arthropod-borne flavivirus, transmitted by Ixodes spp. ticks, which has been associated with neuroinvasive disease and poor outcomes. A retrospective study was conducted at Mayo Clinic from 2013 to 2022. We included clinical and epidemiologic data of probable and confirmed neuroinvasive POWV cases. Sixteen patients with neuroinvasive POWV were identified; their median age was 63.2 years, and 62.5% were male. Six patients presented with rhombencephalitis, 4 with isolated meningitis, 3 with meningoencephalitis, 2 with meningoencephalomyelitis, and 1 with opsoclonus myoclonus syndrome. A median time of 18 days was observed between symptom onset and diagnosis. Cerebrospinal fluid analysis showed lymphocytic pleocytosis with elevated protein and normal glucose in the majority of patients. Death occurred within 90 days in 3 patients (18.8%), and residual neurologic deficits were seen in 8 survivors (72.7%). To our knowledge, this is the largest case series of patients with neuroinvasive POWV infection. We highlight the importance of a high clinical suspicion among patients who live in or travel to high-risk areas during the spring to fall months. Our data show high morbidity and mortality rates among patients with neuroinvasive disease.

Iatrogenic Skull Base Defect Accompanied by Brain Injury After Endoscopic Sinus Surgery: A Report of Two Cases

Although iatrogenic skull base injuries after endoscopic sinus surgery (ESS) are rare (overall complication rate, 0.5%), they can be fatal or cause significant morbidity. Conventionally, skull base injuries were repaired using an external approach. However, in recent years, most skull base injuries after ESS have been repaired using an endoscopic transnasal approach due to its lower morbidity, lower risk of postoperative complications, and shorter hospital stay. We report two cases of iatrogenic skull base injury accompanied by brain injury following ESS and describe the skull base repair techniques employed for each case. In both cases, the skull base defects were successfully repaired using an endoscopic transnasal approach, although craniotomy was also performed in the first case to remove bone fragments from the right frontal base and lateral ventricle. Both patients recovered without residual neurologic deficits.

Posterior reversible encephalopathy syndrome: A rare complication of rituximab therapy in rheumatoid arthritis.

Rituximab, a murine-human chimeric monoclonal antibody targeting CD20-positive B lymphocytes, has established itself as an effective and relatively safe biologic therapy for patients with refractory rheumatoid arthritis. Most common side effects associated with its use include infusion related reactions and cytopenia. Rare adverse effects such as progressive multifocal leukoencephalopathy and posterior reversible encephalopathy syndrome (PRES) have also been reported. Diagnosis of PRES following rituximab treatment requires a high index of suspicion correlated with clinical and radiological features in individuals at risk. Early diagnosis and prompt treatment is associated with a favorable prognosis. We present a case of a young man who developed PRES following rituximab administration on account of active rheumatoid arthritis. Timely diagnosis and prompt treatment ensured his uneventful recovery without residual neurological deficit.

Intracranial invasive group A streptococcus: a neurosurgical emergency in children.

Invasive group A streptococcus (iGAS) infections are associated with a high rate of morbidity and mortality. CNS involvement is rare, with iGAS accounting for only 0.2%-1% of all childhood bacterial meningitis. In 2022, a significant increase in scarlet fever and iGAS was reported globally with a displacement of serotype, causing a predominance of the emm1.0 subtype. Here, the authors report on iGAS-related suppurative intracranial complications requiring neurosurgical intervention and prolonged antibiotic therapy. The authors performed a retrospective chart review of consecutive cases of confirmed GAS in pediatric neurosurgical patients. Five children with a median age of 9 years were treated for intracranial complications of GAS infection over a 2-month period between November 2022 and December 2022. All patients had preceding illnesses, including chicken pox and upper respiratory tract infections. Infections included subdural empyema with associated encephalitis (n = 2), extradural empyema (n = 1), intracranial abscess (n = 1), and diffuse global meningoencephalitis (n = 1). Streptococcus pyogenes was cultured from 4 children, and 2 were of the emm1.0 subtype. Antimicrobial therapy in all patients included a third-generation cephalosporin but varied in adjunctive therapy, often including a toxin synthesis inhibitor antibiotic such as clindamycin. Neurological outcomes varied; 3 patients returned to near neurological baseline, 1 had significant residual neurological deficits, and 1 patient died. Despite the worldwide increased incidence, intracranial complications remain rarely reported resulting in a lack of awareness of iGAS-related intracranial disease. Awareness of intracranial complications of iGAS and prompt referral to a pediatric neurology/neurosurgical center is crucial to optimize neurological outcomes.

Two rare cases of myelin oligodendrocyte glycoprotein antibody-associated disorder in children with leukodystrophy-like imaging findings

BackgroundChildren with acquired demyelinating syndromes (ADS) whose sera are positive for myelin oligodendrocyte glycoprotein (MOG) immunoglobulin (IgG) can be diagnosed with MOG-IgG associated disorder (MOGAD). Cases with leukodystrophy-like imaging findings with recurrent MOGAD have rarely been reported.Case presentationTwo children with MOGAD, whose onset age was 6 months and 3 years, respectively, were admitted to the hospital due to fever and altered consciousness. In both children, MOG-IgG was detected in the serum using live cell-based assay. Brain magnetic resonance imaging (MRI) revealed leukodystrophy-like lesions with diffuse bilateral white matter. Cerebrospinal fluid (CSF) analysis showed mild pleocytosis with normal or slightly increased protein levels and no oligoclonal bands. Metabolic and inflammatory blood/CSF markers were all negative. Full exon gene testing revealed normal results, and nuclear and mitochondrial DNA were normal. Despite regular immunotherapy and reduction of lesions based on brain MRI results, the patients repeatedly relapsed and had residual neurological dysfunction at 3–4 years of follow-up.ConclusionsAlthough MOGAD is a monophasic and benign condition, certain MOGAD patients can experience multiple relapses and residual neurologic deficits. The spectrum of clinical manifestations in MOGAD is wider in children than in previously reported cases, including cases with leukodystrophy-like imaging findings. Such imaging findings along with MOG-IgG may occur recurrently and result in severe neurological prognosis. Patients with extensive and confluent white matter lesions should undergo early testing of MOG-IgG to ensure early therapy. In refractory cases, MOGAD treatment may need to be escalated beyond the current therapy, which means second-line immunotherapy should be performed as early as possible and hormone levels should not be rapidly reduced. Early diagnosis and appropriate treatment may improve the prognosis of children with MOGAD.

Late Postpartum Posterior Reversible Encephalopathy Syndrome: Case Series and Literature Review

Abstract Posterior reversible encephalopathy syndrome (PRES) is a reversible neurological entity characterized by seizures, headache, visual symptoms and other focal neurological findings. It is caused by wide variety of causes ultimately leading to vasogenic cerebral edema of occipital and parietal lobes of the brain. Prompt and early diagnosis of PRES is key in reducing the risk of permanent neurological sequelae. Occurrence of PRES during pregnancy or early postpartum period is well known. However, occurrence of PRES between 48 hours and one month postpartum in women who had normal pregnancy and delivery and no signs of preeclampsia is rare. In this case series we present three cases of PRES that occurred in young women during late postpartum period. All these three patients were normotensive during antenatal and early puerperal period but developed hypertension and vision loss in late puerperium. The prompt diagnosis and management of symptoms resulted in complete reversal of the symptoms with no permanent neurological deficit. Posterior reversible encephalopathy syndrome (PRES) is a reversible neurological entity characterized by the presence of white matter edema affecting the occipital and parietal lobes. The predominantly affected area of the cerebral cortex determines presentation in the patient. The reversibility of these symptoms is one of the hallmarks of the disease. Diffusion-weighted MRI is the gold standard in diagnosis of PRES as it helps to distinguish between vasogenic edema from cytotoxic edema. Prompt diagnosis and initiation of early treatment may lead to complete recovery with no residual neurological deficit.

Clinical presentation, diagnosis, treatment, and outcome in 8 dogs and 2 cats with global hypoxic-ischemic brain injury (2010-2022).

Global hypoxic-ischemic brain injury (GHIBI) results in variable degrees of neurological dysfunction. Limited data exists to guide prognostication on likelihood of functional recovery. Prolonged duration of hypoxic-ischemic insult and absence of neurological improvement in the first 72 hours are negative prognostic indicators. Ten clinical cases with GHIBI. Retrospective case series describing 8 dogs and 2 cats with GHIBI, including clinical signs, treatment, and outcome. Six dogs and 2 cats experienced cardiopulmonary arrest or anesthetic complication in a veterinary hospital and were promptly resuscitated. Seven showed progressive neurological improvement within 72 hours of the hypoxic-ischemic insult. Four fully recovered and 3 had residual neurological deficits. One dog presented comatose after resuscitation at the primary care practice. Magnetic resonance imaging confirmed diffuse cerebral cortical swelling and severe brainstem compression and the dog was euthanized. Two dogs suffered out-of-hospital cardiopulmonary arrest, secondary to a road traffic accident in 1 and laryngeal obstruction in the other. The first dog was euthanized after MRI that identified diffuse cerebral cortical swelling with severe brainstem compression. In the other dog, spontaneous circulation was recovered after 22 minutes of cardiopulmonary resuscitation. However, the dog remained blind, disorientated, and ambulatory tetraparetic with vestibular ataxia and was euthanized 58 days after presentation. Histopathological examination of the brain confirmed severe diffuse cerebral and cerebellar cortical necrosis. Duration of hypoxic-ischemic insult, diffuse brainstem involvement, MRI features, and rate of neurological recovery could provide indications of the likelihood of functional recovery after GHIBI.

The Outcome of Traumatic Intra-diploic Meningoencephalocele with Recurrent seizures - A case report and literature review of 20 cases (P12-1.004)

<h3>Objective:</h3> We highlight an under-recognized epileptic pathology in a 56-year-old left-handed female with progressive right facial numbness and weekly focal seizures characterized by episodic aphasia. Additionally, we provide a thorough literature review of reported IDME patients along with their clinical presentation and outcomes. <h3>Background:</h3> Delayed neurological symptoms may arise years later in traumatic intradiploic meningoencephalocele (IDME) which creates potential diagnostic and therapeutic challenges. <h3>Design/Methods:</h3> Retrospective chart review and literature search. <h3>Results:</h3> 56-year-old left-handed female presented with progressive right facial numbness and focal seizures characterized by episodes of aphasia for a year. Episodes are characterized by worsening right facial numbness followed by garbled speech, word-finding difficulty, and grammatical errors when she attempts to talk or text over the phone, while she remains conscious and is aware of her surroundings. Her only epilepsy risk factor was minor head trauma ten years prior to presentation. Episodes were evaluated in the Epilepsy Monitoring Unit at Cleveland Clinic Foundation. An ictal event characterized by rhythmic sharply contoured delta and theta activity in the left hemisphere, most prominently in the left frontocentral region, This was clinically associated with numbness in the right side of the face progressing into an aphasic seizure. She was found to have left frontoparietal intradiploic meningoencephalocele (IDME) on imaging studies. Positron emission tomography (PET) showing mild metabolism corresponding to Intradiploic meningoencephalocele in left parietal bone, and subtle hypometabolism in the adjacent posterior left frontal lobe operculum. She had a craniotomy for encephalocele resection and mesh cranioplasty. At 3-year follow-up visit, patient was seizure free without residual neurological deficits. She remains on lacosamide 100 mg BID as seizures recurred after attempting to discontinue it. <h3>Conclusions:</h3> Traumatic IDME is a rare disorder and a presentation with seizures specifically is rarely encountered. A cranioplasty and dural repair, with or without resecting the projecting parenchyma, is likely the definitive treatment. <b>Disclosure:</b> Dr. Alsallom has nothing to disclose. Dr. Alzahrany has nothing to disclose. Dr. Gonzalez has nothing to disclose. Dr. Jehi has received personal compensation in the range of $500-$4,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for wolters kluwer. Dr. Jehi has a non-compensated relationship as a PI with Cleveland Clinic that is relevant to AAN interests or activities.