Parietal Lobe Lesions Research Articles

Visuospatial function impairment in patients with subacute-chronic isolated frontal lobe ischemic stroke: A cross-sectional study using MoCA-Ina in a private hospital

Background. Stroke is one of the leading causes of disability, resulting in both limb disability and also cognitive impairment. Cognitive impairment can occur post-stroke due to lesions in specific cortex areas. Visuospatial function, a domain of cognitive function related to visual imaging, is commonly affected by parietal lobe lesions. However, visuospatial processing also requires sustained attention, planning, and error correction, functions of the frontal lobe. There is a paucity of studies evaluating the relationship between isolated frontal lobe stroke and patients’ visuospatial function. Objective. To analyze the relationship between subacute-chronic frontal lobe ischemic stroke and visuospatial function in post-ischemic stroke patients using the MoCA-Ina. Material and methods. Patients from Siloam Karawaci General Hospital’s neurology outpatient department with ages under 65 years, Glasgow Coma Scale of 15, diagnosis of first-time stroke based on CT scan result, and ischemic lesion confined to one brain lobe were recruited using non-probability consecutive sampling for this cross-sectional study. Patients were divided into frontal lobe and non-frontal lobe stroke subgroups. Visuospatial function was assessed using MoCA-Ina and analyzed using Mann Whitney U test. Outcomes. Fifty patients were included; 25 had frontal lobe stroke, and 25 had non-frontal lobe stroke. The median visuospatial score was lower in the frontal lobe group (2, min/max = 0/4) compared to the non-frontal lobe group (3, min/ max = 0/5), with a p-value of 0.044. There was no significant difference in visuospatial scores between different frontal hemisphere locations. Conclusion. Subacute-chronic frontal lobe ischemic stroke affects visuospatial function in post-ischemic stroke patients.

Abstract CT204: Efficacy of Bria-IMT regimen in inducing CNS metastasis regression

Abstract Objectives: Breast cancer metastasis to the central nervous system (CNS) often leads to severe morbidity and mortality, and while systemic treatments manage primary tumors, their impact on CNS metastases is limited. This retrospective analysis evaluates the efficacy of Bria-IMT as a monotherapy or in combination with an immune checkpoint inhibitor (ICI) on CNS tumor regression. Methods: Post-hoc analysis of a subset of advanced MBC patients with CNS metastases from previous phase 1 and 2, and ongoing randomized phase 2 trials involving SV-BR-1-GM. SV-BR-1-GM is a GM-CSF secreting, antigen-presenting immortalized breast cancer cell line. Bria-IMT (SV-BR-1-GM ~20x106 cells, intradermally 48-72 hours after cyclophosphamide 300 mg/m2, followed by interferon-alpha at the inoculation sites 2 days later) was administered q3wks. In the combination setting, an ICI was administered with each cycle. Patients A002 and 02-003 received Bria-IMT as monotherapy. Patients 06-005 and 06-007 received Bria-IMT in combination with an ICI. Results: Median pt age of 65 (range 58-70). All pts had ≥1 HLA match with SV-BR-1-GM. Significant CNS tumor regression was observed in all pts. Pt A002 (ER+/PR+, HER2-) received 6 cycles, achieving an 83% reduction in breast lesions. ~3 months after the last cycle, imaging revealed the onset of multiple brain metastases and recurrence of right breast lesions. Following 3 additional cycles, marked measurable regression in both brain metastases and breast lesions was noted. Pt 02-003 (ER-/PR-, HER2 1+ (IHC)) received 5 cycles and observed a 60% reduction (5mm ->2mm) in a left parietal periventricular lesion, and complete resolution (CR) of a left parietal lobe lesion. Pt 06-005 (ER+/PR-, HER2 1+(IHC)) demonstrated a 56% (9mm -> 4mm) reduction in the thickness of a dural lesion overlying the left anterior temporal lobe after 6 cycles of SV-BR-1-GM + ICI (pembrolizumab). After 2 additional cycles of SV-BR-1-GM + ICI (retifanlimab) and 1 cycle of SV-BR-1-GM (ICI was skipped on final cycle due to grade 2 AE), patient achieved CR of a left orbital mass observed at baseline (24mm -> 0m). Circulating cancer-associated macrophage-like cells (CAMLs) varied throughout the treatment, with levels ranged from a baseline of 90 to 24 by Cycle 13. Pt 06-007 (ER-/PR-, HER2 1+ (IHC)) demonstrated significant reductions in CNS lesions after 3 cycles of SV-BR-1-GM + ICI (pembrolizumab): a 67% reduction in a right frontal lobe lesion, 29% in the right parietal, 30% in the right thalamus, and 67% in the right precentral gyrus lesions. CAML levels remained low throughout treatment. Conclusions: This review consolidates evidence for the efficacy of the Bria-IMT regimen in CNS metastasis regression, both alone and with ICIs. The consistent regression seen across all breast cancer subtypes among heavily pretreated patients highlights the potential of SV-BR-1-GM in managing CNS metastasis. Ongoing trials will determine the full extent. Citation Format: Sailaja Kamaraju, Blaise Bayer, Mingjin Chang, William Williams, Charles Wiseman, Giuseppe Del Priore. Efficacy of Bria-IMT regimen in inducing CNS metastasis regression [abstract]. In: Proceedings of the American Association for Cancer Research Annual Meeting 2024; Part 2 (Late-Breaking, Clinical Trial, and Invited Abstracts); 2024 Apr 5-10; San Diego, CA. Philadelphia (PA): AACR; Cancer Res 2024;84(7_Suppl):Abstract nr CT204.

Evaluating the Rate of Primacy and Recency in Learning Word Series in Neglected Patients with Right Posterior Parietal Lobe Lesion

Evaluating the Rate of Primacy and Recency in Learning Word Series in Neglected Patients with Right Posterior Parietal Lobe Lesion

Neuropsychological syndromes of disorders of mnestic activity in patients with strokes in the right (subdominant) hemisphere

The work carried out a neuropsychological assessment of different types of memory and determined the profiles of mnestic disorders depending on the type of hemispheric asymmetry and localization of the lesion. The analysis of the features of the course of stroke in the right (subdominant) hemisphere was carried out. The main symptoms of impaired types of memory, for which the subdominant hemisphere is responsible, were studied. The presented data correlate with the data found in the world literature: stroke in the subdominant hemisphere has a less pronounced effect on memory impairment, is associated with a better prognosis, while visual memory, spatial, logical, and mechanical suffer more often. A detailed topographic analysis of the syndromatics is presented, comparing instrumental data on visualization of the lesion and psychological tests that reveal a deficiency of a particular type of memory. The data obtained make it possible to identify targets and opportunities for early neuropsychological correction of disorders by attracting compensatory brain capabilities, which is important when planning an individual rehabilitation program. The analysis of mnestic activity in patients with strokes in the right (subdominant) hemisphere was carried out. The applied set of patho- and neuropsychological methods: The Wexler memory scale, Indirect memorization according to A.N. Leontiev, the "Pictograms" technique, Benton's Visual retention, the "Memorizing 10 words" Technique, the "Memorizing faces" technique, the "Memorizing shapes" method., the MOSS test. For the first time, a comprehensive clinical, psychological and experimental psychological study was conducted, which made it possible to characterize the quality and hierarchy of impaired and preserved types of mental activity in the defeat of the right (subdominant) hemisphere. For the first time, a comparative analysis of disorders of the mnestic activity of the dominant and subdominant hemispheres of the brain was carried out, which allowed us to identify the specifics of memory disorders depending on the lateralization of lesions. The novelty is also represented by the systematization of mnestic disorders carried out by the author, accompanied by the isolation of neuropsychological syndromes of disorders of mnestic activity in the defeat of various parts of the right (subdominant) hemisphere in the acute period of syndromes of occipital, temporal, parietal lobe lesions and their combinations. Based on the data obtained, the necessity of studying the features of mnestic activity in order to restore and further rehabilitate higher mental functions in patients with cerebral infarction in the right hemisphere is justified.

Abstract TMP36: Anatomical Predictors of Gastrostomy Tube Placement After Large Vessel Occlusion Ischemic Stroke

Dysphagia is a common neurologic deficit after ischemic stroke; as a result, patients often require percutaneous endoscopic gastrostomy (PEG) tube placement for safe maintenance of sufficient caloric intake. Right hemispheric strokes have previously been associated with post-stroke dysphagia. We examined whether topographic locations of post-stroke diffusion-weighted MRI (DWI) lesions associated with the need for placement of PEG tube. A registry of 898 patients evaluated for acute treatment of suspected large vessel occlusion (LVO) stroke was used. 65 patients underwent post-stroke PEG placement, and 65 additional patients were selected as propensity matches based on age, baseline NIH Stroke Scale score, and recanalization status. Binary masks of 24-to-72-hour post-stroke DWI lesions were co-registered to standard template space. Voxel-based lesion symptom mapping (V2.6), rewritten to perform logistic regression at each voxel, was used to generate statistical maps of lesion contribution to PEG placement. Uncorrected t-statistic maps demonstrated voxels in the right frontal, parietal, and temporal regions were associated with post-stroke PEG placement. Upon controlling for age and/or recanalization status, lesions in the right parietal lobe were associated with need for PEG. After controlling for lesion volume, this association weakened; once all variables were controlled for, there were no topographical regions associated with PEG placement. In this limited series, there is not a topographic region on post-stroke diffusion MRI that is significantly associated with need for PEG placement in patients with LVO stroke after controlling for appropriate covariates. PEG tube requirement may be a composite outcome, dependent on various inputs notably including lesion volume.

10126-ACT-10 FACTORS AFFECTING THE PATTERN OF RECURRENCE OF IDH-WILDTYPE GLIOBLASTOMA

Abstract INTRODUCTION IDH-wildtype glioblastoma is a quite poor prognosis disease with which almost all cases recur in spite of postoperative radiation and chemotherapy. Survival rates are particularly low after dissemination. METHODS We included 120 patients with IDH-wild type glioblastoma treated at our hospital from January 2015 to December 2019. Patient backgrounds, MGMT promotor methylation status, imaging findings, and recurrence patterns were analyzed. Subependymal, subarachnoid, and spinal dissemination, plus distant metastasis, were defined as non-local recurrence. RESULTS Of 105 patients with a single lesion at diagnosis, 65 had local recurrence and 15 had non-local recurrence. 36 of 65 patients had local recurrence and 12 had non-local recurrence in the second recurrence. 17 of 36 patients had local recurrence and 8 had non-local recurrence in the third recurrence. The cumulative number of non-local recurrences was 54 (45.0%). Surgical ventricular opening and tumor proximity to the ventricles were associated with non-local recurrence (p=0.006, p=0.019). The patients with tumor proximity to the ventricle had non-local recurrence at the first recurrence and those with non-proximity to the ventricle had non-local recurrence at the second or later recurrence (p=0.038). MGMT promotor methylation status and postoperative ischemia were not significantly different from non-local recurrence (p=0.122, p=0.088), but MGMT promotor unmethylation was a risk factor for early non-local recurrence, especially within 1 year (p=0.033). Frontal lobe lesions were less frequently disseminated and more frequently MGMT promotor methylated (p=0.032, p=0.024). Parietal lobe lesions had more MGMT promotor methylation (p=0.006), and temporal lobe lesions had more dissemination (p=0.023). Distant metastasis commonly occurred from the frontal lobe to the ipsilateral or contralateral, and the frontal lobe was frequently associated. CONCLUSION IDH-wildtype glioblastoma is characterized by repeated local recurrence and cumulative non-local recurrence. The pattern of recurrence differs depending on the tumor localization, especially adjacent to ventricle, and MGMT promotor methylation status.

PATH-56. SUPRATENTORIAL NEUROEPITHELIAL TUMORS WITH NOVEL PLAGL1 FUSIONS: 2 CASE REPORTS

Abstract Neuroepithelial tumors with pleomorphic adenoma gene-like (PLAGL1) fusions represent a distinct subgroup of neoplasms that have been recently identified. These fusions are quite rare and most commonly associated with fusions in the EWSR1 or FOXO1 genes. Limited reports in literature have indicated a median age of diagnosis of 6.2 years and poor progression-free survival at 35 months. Here we present two cases of patients with neuroepithelial tumors with PLAGL1 fusions. Case 1 was a 49 year old male who underwent resection of a left frontal lobe mass at 2 year old that was reported as a low grade ependymoma. He did not receive radiation or chemotherapy and subsequently developed seizures in childhood. At age 47, he presented with worsening seizures and MRI brain revealed a 4.5 cm mass in the left frontal lobe with serpiginous enhancement. He underwent surgical resection and molecular profiling demonstrated PLAGL1-FOXO1 fusion. Case 2 was an 18 year old female presenting with generalized tonic-clonic seizures. MRI brain demonstrated a 1.4 x 1.2 cm cortical and subcortical lesion in the right parietal lobe. Following surgical resection, pathology reported a low grade neuroepithelial neoplasm with ependymal-like features. Molecular profiling showed PLAGL1-EWSR1 fusion. As PLAGL1 fusions are not yet characterized in the WHO classification system, these cases aid in tumor characterization and highlight the importance of molecular and methylation analysis for accurate tumor classification. Case 2 presents a notable variation from reported age of presentation of 6.2 years with diagnosis at 18 years of age, indicating these tumors may also present in young adulthood. Additionally, Case 1 demonstrated 45 years progression-free survival compared to previously reported median of 35 months, suggesting outcomes may be heterogeneous with potential for prolonged survival. Further research is needed to better elucidate the imaging, clinical course, and prognosis for tumors with this novel fusion.

QOL-16. GLIOBLASTOMAS LOCATED IN THE RIGHT INFERIOR PARIETAL LOBE CAUSE A POOR POSTOPERATIVE PERFORMANCE LEVEL

Abstract OBJECTIVE Managing quality of life has become more important for patients with glioblastoma (GBM). Maintaining independence levels should be given attention while treating GBM. Postoperatively, non-dominant hemispheric GBMs often decrease the Karnofsky Performance Status (KPS) score. This study aimed to investigate the factors that lead to poor KPS scores after tumor resection in non-dominant hemispheric GBM. METHODS Forty-one patients newly diagnosed GBM in the right cerebral hemisphere who underwent resection were included. Brain functions were assessed preoperatively and postoperatively. First, the KPS scores before and after surgery were compared. Second, univariate and multiple regression analyses and voxel-based lesion-symptom mapping (VLSM) were performed to analyze the functions and brain regions related to KPS, respectively. Third, factors associated with prognosis were analyzed using the log-rank test. RESULTS Compared with the preoperative status, KPS significantly decreased after the surgery (81.0% vs. 73.6%, p = 0.020). Brain functions affecting KPS include general cognitive function, processing speed, attention, memory, visuospatial cognition, emotion recognition, and executive function. Among these, multiple regression analysis revealed visuospatial cognition and emotion recognition to be significantly influenced KPS in the chronic postoperative phase (p = 0.030 and 0.048, respectively). VLSM demonstrated that resection of the inferior parietal lobe caused a significant decline in KPS. Visuospatial cognition and emotion recognition were impaired with a higher probability (p = 0.035 and 0.048, respectively) in patients with tumors located in the inferior parietal lobe. In addition, lesions in the inferior parietal lobe were associated with shorter overall survival (p = 0.046). CONCLUSIONS GBMs in the right inferior parietal lobe are more likely to result in poor postoperative KPS due to impairment of visuospatial cognition and emotion recognition, which relate to a poor prognosis.

IMMU-32. CAN PEMBROLIZUMAB TREAT RECURRENT GLIOBLASTOMA AND REVERSE HIV LATENCY?

Abstract Immunotherapy revolutionized treatment for many solid tumors. Early studies show that immunotherapy may be effective for recurrent glioblastoma. The ongoing Phase 2 LEEP-005 clinical trial (pembrolizumab+lenvima) is currently enrolling glioblastoma patients, but excludes patients with HIV. However, there is a strong need to treat this special patient population. One of the barriers to achieving cure in HIV patients is HIV latency within infect CD4+ T cells. Recently published data suggests that pembrolizumab can reverse HIV latency by forcing the HIV out of intracellular hiding into the plasma where anti-retroviral therapy can be effective. We present a case of a 39 year-old male with HIV and a Left Parietal Glioblastoma who had progressive disease following a subtotal resection and radiation alone due persistent neutropenia. Post-radiation MR brain wwo showed progressive disease in the left parietal lobe and new dural-based lesions in the left frontal and temporal lobes. HIV viral load was < 20 and CD4 count was 406 on HAART. The patient declined lenvima, but was amenable to bevacizumab. At 1 month follow-up, after 1 infusion of pembrolizumab 200mg and 2 infusions of bevacizumab, he achieved CR in the original site of disease and stable disease in the left frontal and temporal lobes. HIV viral load was 52. CD4 count was 660. Though it is too early to draw any firm conclusions, in our patient, pembrolizumab appears to induce HIV reversal, which is supported by an increase in the increase in his HIV viral load. The combination of pembrolizumab and bevacizumab appears to be effective at short-term follow-up. Our study is significant because there is limited evidence on the use of PD-L1 inhibitors in active HIV patients with cancer. To our knowledge, our case is the first reported case of the use of pembrolizumab in a patient with HIV and glioblastoma.

Newly Diagnosed Anaplastic Astrocytoma: > 23 Year Survival in a 31-Year and 11-Month-Old Female Treated with Antineoplastons

Rationale: Anaplastic astrocytoma (AA), a malignant brain tumor that arises from astrocytic cells, has a poor prognosis. It accounts for 6–7% of gliomas and 1-2% of brain tumors. Objectives: A 31-year and 11-month-old female with an AA is presented to discuss the efficacy of Antineoplastons A10 (Atengenal) and AS2-1 (Astugenal) in the treatment of AA. She presented to the Burzynski Clinic (BC) for treatment and was enrolled in Protocol BT-08, “Phase II Study of Antineoplastons A10 and AS2-1 in Adult Patients with Anaplastic Astrocytoma”, receiving both intravenous (IV) and oral Antineoplastons A10 and AS2-1 (ANP therapy). IV ANP therapy was delivered continuously via subclavian catheter and infusion pump. Tumor response was determined by comparison of baseline brain magnetic resonance imaging (MRI) to sequential brain MRIs during therapy. Findings: The patient presented to the BC with no prior treatment and a one-month history of right arm clumsiness and right leg weakness. Brain MRI and stereotactic biopsy performed elsewhere had demonstrated an AA. Baseline MRI at the BC showed a 2.0 cm2 nonenhancing lesion and two enhancing lesions (0.02 cm2 and 0.15 cm2 ) in the left parietal lobe. IV ANP therapy was given over 56 days, and a complete response (CR) was demonstrated after one month of therapy. Oral ANP therapy was subsequently provided for 17 months. At last follow-up, in June 2023, the patient was healthy and showed no evidence of recurrent disease. She had an overall survival (OS) of > 23 years and one month since diagnosis and an OS of > 23 years since the start of IV ANP therapy. Conclusions: The utilization of ANP therapy in an adult female patient with AA is presented. The patient achieved a CR and prolonged OS, suggesting that ANP therapy may be an effective therapeutic option for adults with AA.

BALANCE, GAIT AND LATEROPULSION DISORDERS IN THE ACUTE ISCHEMIC STROKE

Introduction: Stroke mortality rates has decreased to a considerable value over the past few decades owing to the improved medical facilities and technology. This has lead to more number of stroke survivors in the aging population. A strong predictor of independent living after stroke is maintaining body balance and it was showing strong correlation with the disability perceived at the time of patient's discharge from the stroke rehabilitation unit Methodology: All patients with acute ischemic stroke were taken for study. These initial assessments collected information about handedness, all motor decits, postural instability and gait disorders, and disability affecting motor function. The second assessment was performed after one week of stroke at the discharge after daily physiotherapy and only focused on postural and gait disorders. Motor weakness was assessed by a standardized examination of muscle strength adapted for participants with central neurological disorders. Lateropulsion, gait and balance disorders were identied by specic protocols. Results: It was observed that the lesions of frontal lobe, thalamus, basal ganglia and internal capsule had signicant assosciation with balance and gait abnormality post stroke while lesions of parietal lobe, temporal lobe and occipital lobe did not lead to any signicant gait or balance decits. Individuals with lateropulsion improves well on daily physiotherapy in both Lw/oP (r = -0.65) and for pushers (r = -0.61) to a signicantly improved state( r= -0.5 for upright and r= -0.24 for pushers).Conclusion: Lateropulsion is a main factor in determining recovery of balance and gait in the subacute stage of stroke recovery. Special emphasis should be given to the impaired body orientation with respect to gravity in stroke rehabilitation stage and techniques to improve lateropulsion has to be stressed during physiotherapy for better recovery of balance and gait post stroke

Post-infectious Epilepsy from Probable Neurocysticercosis in a 32 Year Old Pregnant Female (P14-10.003)

<h3>Objective:</h3> To report a case of neurocysticercosis management in a first trimester pregnant female. <h3>Background:</h3> Neurocysticercosis is a parasitic infection known to be endemic to some countries and is a leading cause of acquired epilepsy worldwide. Available data on the diagnosis and management of neurocysticercosis infection in pregnancy remains limited. <h3>Design/Methods:</h3> Case report <h3>Results:</h3> A 32-year-old female presented with headaches after a camping trip in the Ozarks. MRI head revealed leptomeningeal hyperintensity in the left parieto-occipital junction. Three weeks later, she presented again with seizures and was 11 weeks pregnant. The semiology was right hand somatosensory to focal clonic, then generalized. She had recently travelled to South America. Neurological exam was significant for concentration difficulty, right pronator drift, finger extension weakness, and bradykinesia with dysdidokinesia in the right hand. MRI with and without contrast demonstrated one multi-cystic lesion in the left parietal lobe without a discernable scolex, with extensive surrounding vasogenic edema. Work up demonstrated peripheral eosinophilia, and unremarkable lumbar puncture (WBC 3). Lesion biopsy demonstrated eosinophils, demyelination, and inflammation of the meninges. Given the clinical and radiographic findings, suspicion for a parasitic infection was high on the differential and a diagnosis of probable Neurocysticercosis was made. Patient was managed with levetiracetam (with high dose folate), high dose IV steroids, and a prolonged oral steroid taper for vasogenic edema. Surveillance MRIs revealed slow improvement over weeks and complete resolution to a gliotic scar without calcification by six months. <h3>Conclusions:</h3> Neurocysticercosis represents a treatment challenge in pregnancy, given the teratogenicity of antiparasitic agents and the potential for acute worsening of peri-lesional edema. Symptomatic treatment aimed at controlling seizures and the inflammatory response can help support the immunocompetent pregnant patient in the right clinical setting, while the cysts undergo natural involution. <b>Disclosure:</b> Dr. Shamim has nothing to disclose. Dr. Nwabueze has nothing to disclose. Dr. Leever has nothing to disclose. Dr. Gronseth has received personal compensation in the range of $0-$499 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Brain &amp; Life. Dr. Gronseth has received personal compensation in the range of $10,000-$49,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Neurology. Dr. Gronseth has received personal compensation in the range of $0-$499 for serving as a Member/EBM consultant Guideline Development Subcommittee with AAN.

Thanatotic Infestation: Ekbom’s Syndrome as an Exordium to Cotard’s Delusion

AbstractIntroductionEkbom’s delusion as a prelude to Cotard’s syndrome, has not heretofore been described.MethodsCase study: A 45-year-old woman with a past diagnosis of bipolar disorder with psychotic features was admitted, having been up all night conversing with spirits, proclaiming that she had made a deal with Satan. Convinced that her grandmother was possessed by the devil, she smashed her grandmother’s head with a two-by-four. Results: Mental Status Examination: self conversing with her eyes darting around the room. Poor hygiene. Behavior: guarded and withdrawn. Oriented x2. Speech: hyperverbal. Insight and judgment: poor. Mood: hostile, aggressive, and angry. Thought process disorganized, incongruent, and tangentiality. She was convinced she was infested with little black bugs crawling around her insides which had been placed there by the devil. After two days of olanzapine she reported the bugs were no longer present, but rather that she herself was dead and that her organs were decomposing, which persisted through the remainder of the hospitalization.DiscussionNeuroimaging abnormalities in Ekbom’s syndrome involve the striatum, basal ganglia (putamen and caudate nucleus), insular and cingulate cortices, cortex (prefrontal, right parietal, and temporal lobes), right lingual and orbitofrontal gyri, and thalamus. In Cotard’s syndrome, abnormalities have been identified in the striatum, frontal and temporal lobes, and right-sided and bilateral hemispheres. An overlap between the delusions exists in the striatum, inferior parietal, and temporal lobes. A single lesion in the nondominant inferior parietal lobe may cause both syndromes, due to its substantial interconnection with the temporo-limbic areas. Since the parietal lobe is also involved in somatosensory processing, peradventure distorted sensory perception with associated sensation of formication may have been the nidus for the delusional infestation as well as a nidus for the perception of thanatos habitus. Such misperception may have then been amplified into a delusion because of a hyperconnection between the parietal lobe and the limbic system. This may represent a variant of the two-factor hypothesis of delusions whereby a distorted sensory perception is then misrepresented in a delusion. Dysfunction of the right hemisphere, which normally acts to censor the left, allows the delusion to manifest. A single lesion of the inferior parietal lobule may be sufficient for both sensory distortions and loss of inhibition of delusional interpretation of distorted sensation by the frontal lobe, yclept the sensorialist hypothesis.ConclusionIn those with monothematic delusions, the search for transient fluctuation in delusional states may be revealing.FundingNo Funding

Clinical factors predictive of new-onset seizure in patients with AIDS-related brain parenchymal lesion

PurposeThis study aimed to determine the clinical characteristics and risk factors of new-onset seizure in patients with AIDS-related brain parenchymal lesion. MethodsA retrospective case-control study from January 2015 to December 2021 was conducted to determine the clinical characteristics and etiology of seizures in patients with AIDS-related brain parenchymal lesion. Univariate and multivariate logistic regression analyses were used to identify risk factors associated with seizures. Receiver operating characteristic (ROC) curve was used to analyze seizure prediction efficiency. ResultsAmong a total of 343 patients with AIDS-related brain lesions, 222 had brain parenchymal lesions. Of the 222 patients in the series (range: 16–81 y), 69 reported an episode of at least one seizure. A logistic regression analysis showed that tuberculoma, cortex involvement, and lesions in parietal lobe were found to have a strong association with higher incidence of seizures, whereas lesions in the periventricular area was less prone to seizure. The area under the ROC curve of these factors was 0.733, indicating these factors could predict seizure effectively. Amongst the 69 patients with seizures in multivariate analysis using logistic regression, multiple lesions significantly associated with focal to bilateral tonic-clonic seizures, and lesions in temporal lobe independently associated with focal impaired awareness seizure. ConclusionsOur study identified the underlying predictors between seizures and the clinical characteristics in a large population of patients with AIDS-related brain parenchymal lesions. These findings would provide further insights into developing effective prevention and treatment strategies aimed at improving the quality of life in the HIV population.

Non-cerebellar ataxias: posterior column-like ataxia and cerebellar-like ataxia

Ataxia is not only due to cerebellar lesions, but also due to non-cerebellar lesions such as those in the brain, spinal cord, dorsal root (DR), peripheral nerve. In this article, optic ataxia is excluded and 'vestibular ataxia' is briefly referred. Non-cerebellar ataxias are generically called sensory ataxia or posterior column ataxia. However, since non-cerebellar lesions, e.g. frontal lobe lesions, may develop "cerebellar-like ataxia" (Hirayama, 2010). At the same time, non-posterior column lesions, e.g. parietal lobe lesion, can show "posterior column-like ataxia". From these viewpoints, I here describe various non-cerebellar ataxia in some disorders such as tabes dorsalis and sensory neuropathies and emphasize a role of a peripheral sensory input to the cerebellum via the DR ganglia and spinocerebellar tract for sensory ataxia because there is the International Consensus (2016) that the ataxia in Miller Fisher syndrome is suggested cerebellar-like clinicophysiologically.

Gerstmann Syndrome Case-Control Study: Correlation between Brain Lesions & Functional Disability.

Deep functional and structural neuroimaging of a series of Gerstmann's syndrome patients required high accuracy, and our results avoided false overlaps of heterogeneous brain lesions by handling each case of our study subjects separately as an individual case regarding functional and neuroimaging tests. Six patients with Gerstmann tetrad (one with dominant acalculia, one with dominant left and right disorientation, two with writing disabilities and two with finger agnosia) and 6 control subjects with close ages were recruited in the current study. In the main phase, we assessed brain activation in response to experimental and interventional settings using neuroimaging techniques (FMRI-Functional Magnetic Resonance Imagingwhere twelve pictures were taken on a Dell inspiration 3T all-body scanner with sequences of echo pictures, 80o angled, TE 35 ms) of the subject's brain to declare lesions existence and locations that might result in one of the four cognitive impairment domains of Gerstman's syndrome tetrad. We assessed statistically significant differences of patient images vs. control images as well as the images of patients presenting specific symptomatic cognitive dysfunction domain vs. the images of patients presenting the three other domains. Neuroimages were analyzed using multiple databases such as T1 weighted and free sequence types. Gerstmann's syndrome is mainly connected to injury in the dominant parietal lobe, so images comparisons and analysis were only restricted to the left parietal lobe region. P values <0.05were only considered as statistically significant difference in comparisons of functional tests time and accuracy of patients vs. in addition to comparisons of brain images parameters of patient group vs. control group and specific symptomatic domain patients vs. other symptomatic domains patients. Regarding functional testing, Patients group took significantly higher time compared to control group. Regarding brain images parameters, patients in each domain showed significantly different lesions compared to other domains. Moreover, control subjects showed no lesions in the left parietal lobe compared to significant lesions in the patient groups. These results oppose the theory of Gerstmann that a common brain structural injury may result in the combination of all of the four symptomatic dysfunction domains. This may be due to the fact that Gerstmann examined incomplete cases which represent a considerable criticism to his scientific basis. Moreover, he excluded patients with speech difficulties and apraxia.

A case report of epileptic seizures caused by Rosai Dorfman disease followed by a literature review

Rosai Dorfman disease is a rare benign histiocytoproliferative disorder that occurs in the intracranial area, which occurrs typically in lymph nodes. Extrapnodal Rosai Dorfman disease rarely develops in the central nervous system and is often a focal lesion based on the dura. Based on imaging and clinical symptoms, RDD may be misdiagnosed as meningioma, and some lesions can also occur in the brain parenchyma. In the case of benign disease, the final diagnosis is made by pathological tissue diagnosis. For chronic diseases, progression may be chronic or remitting and relapsing. A 54-years-old man was hospitalized after experiencing paroxic convulsions and being unconsciousness. A head magnetic resonance imaging demonstrates a strip of lesions in the right parietal lobe. No obvious abnormality is found in the laboratory data. We diagnosed meningioma of right parietal lobe and secondary epilepsy, and prescribed oral sodium valproate to treat him. The lesion is located in the right parietal lobe on neuroimaging prior to surgery, which was taken for immunohistochemical examination. If it is found that immunohistochemistry reveals histiocytes are positive for CD68, S-100, but negative for CD1a, it is identified as RDD. For patients who are seizure-free following surgery, symptomatic management is used. Following parietal lesion resection, patients are seizure-free during the follow-up period (44 months). Basing on studying and summarizing relevant literatures, RDD is described in the report in terms of its diagnosis, pathology, treatment, and clinical outcome, in order to improve the diagnosis and identification of intracranial RDD by physicians.

MPC-20 CLINICAL AND MOLECULAR FEATURES OF GLIOBLASTOMAS ARISING IN THE SETTING OF LI FRAUMENI SYNDROME

Abstract Introduction Li Fraumeni Syndrome (LFS) is a rare autosomal dominant tumor predisposition syndrome caused by germline alterations of the TP53. Brain tumors are included in LFS core cancers. Choroid plexus carcinomas and medulloblastomas tend to present in pediatric LFS patients, while gliomas tend to present in adult LFS patients. There are few previous reports about gliomas arising in the setting of LFS. Here, we report clinical and molecular features of three cases of glioblastoma arising in the setting of LFS. Results Mean age at diagnosis was 40 years (range: 32-45). Two patients were male. Two patients had medical history of multiple tumors and family history of brain tumors. Magnetic resonance imaging of each patients revealed a left cerebellar peduncle lesion, a right parietal lobe lesion and two lesions in the pons and right parietal lobe, respectively. Pathological diagnoses of all cases were glioblastomas, IDH-wildtype. All patients underwent chemoradiotherapy after surgery. Mean progression free survival was 10.4 months (range: 5.1-17.0). All patients were admitted to palliative care mean 17.3 months (range: 14.4-21.4) after diagnosis. All patients had heterozygous germline mutations of TP53. Genetic analysis of tumor tissues revealed TP53 mutation and PDGFRA amplification in all patients. In all cases, mutation spots of TP53 in tumor-derived DNA were consistent with those of germ line mutations. In all cases, copy number alteration of TP53 gene in tumor-derived DNA were not detected. EGFR amplification, the combination of gain of chromosome 7 and loss of chromosome 10 (7+/10-) and mutations of IDH, H3F3A, BRAF genes and TERT gene promoter were not detected in all tumor-derived DNA. Conclusion We experienced three cases of glioblastoma, IDH-wildtype arising in the setting of LFS. Clinical courses of all cases were poor. Notably, all tumor tissues harbored TP53 mutation and PDGFRA amplification, while no tumor tissues harbored TERTp mutation, EGFR amplification and 7+/10-.

PATH-19. CLINICAL AND MOLECULAR FEATURES OF GLIOBLASTOMA, IDH-WILDTYPE ARISING IN THE SETTING OF LI FRAUMENI SYNDROME

Abstract INTRODUCTION Li Fraumeni Syndrome (LFS) is a rare autosomal dominant tumor predisposition syndrome caused by germline alterations of the TP53. Brain tumors are included in LFS core cancers. Choroid plexus carcinomas and medulloblastomas tend to occur in pediatric LFS patients, while gliomas tend to occur in adult LFS patients. There are few previous reports about gliomas arising in the setting of LFS. Here, we report clinical and molecular features of three cases of glioblastoma, IDH-wildtype arising in the setting of LFS. RESULTS Mean age at diagnosis was 40 years (range: 32-45). Two patients were male. Two patients had medical history of multiple tumors and family history of brain tumors. Magnetic resonance imaging of each patients revealed a left cerebellar peduncle lesion with faint contrast-enhancement, a right parietal lobe lesion with nodular contrast-enhancement and two lesions in the pons (no enhancement) and right parietal lobe (ring enhancement), respectively. All patients underwent chemotherapy including temozolomide and/or bevacizumab combined with radiation therapy (60Gy) after surgery. Mean progression free survival was 10.4 months (range: 5.1-17.0). All patients were admitted to palliative care mean 17.3 months (range: 14.4-21.4) after diagnosis. All patients harbored heterozygous germline mutations of TP53. Whole exome sequencing and copy number analysis of tumor tissues revealed TP53 mutation and PDGFRA amplification in all patients. However, EGFR amplification, the combination of gain of chromosome 7 and loss of chromosome 10 (7+/10-) and mutations of IDH, H3F3A, BRAF and TERT promoter were not detected in all tissues. CONCLUSION We experienced three cases of glioblastoma, IDH-wildtype arising in the setting of LFS. Although tumor locations and radiographic features were varied, all clinical courses were poor. Notably, all tumor tissues harbored TP53 mutation and PDGFRA amplification, while no tumor tissues harbored TERT promoter mutation, EGFR amplification and 7+/10-.

INNV-05. CO-OCCURRENCES OF A HIGH-GRADE GLIOMA WITH CAVERNOUS MALFORMATIONS AND PATHOGENIC VARIANTS IN PDCD10 AND SMARCA4

Abstract INTRODUCTION The co-occurrence of multiple disease processes can make for more challenging diagnoses. Here we report an unusual case of a patient found to have an IDH1-mutant high-grade glioma along with multiple cerebral cavernous malformations and pathogenic germline variants in PDCD10 and SMARCA4. Case Description: A 17-year-old female presented with left arm paresthesia and weakness along with persistent headaches within the frontal and occipital regions that progressed in intensity to include nausea and emesis. A fast sequence magnetic resonance imaging (MRI) of her head was obtained that revealed the presence of multiple bilateral cystic lesions suspicious for cavernomas with the most notable lesion in the right parietal lobe. Ophthalmology consultation revealed grade III papilledema bilaterally. A full brain MRI with and without contrast was obtained and demonstrated a right anterior parietal lobe lesion with associated mass effect, as well as multiple bilateral supratentorial and left cerebellar cavernous malformations. The patient underwent tumor debulking of her dominant lesion. Pathology revealed an IDH1 mutant diffuse astrocytoma, WHO grade 3. Germline genetic testing was pursued which revealed a PDCD10 pathogenic variant consistent with familial cerebral cavernous malformation syndrome and a likely pathogenic variant in SMARCA4. Treatment of her high-grade-glioma included radiation therapy followed by maintenance oral temozolomide. DISCUSSION This case illustrates the unusual co-occurrences of a high-grade glioma with familial cavernous malformation syndrome and germline pathogenic variants in PDCD10 and SMARCA4. Our patient continues to do well clinically, but because of her now significant risk of developing ovarian cancer she has elected to undergo a prophylactic bilateral salpingo-oophorectomy. Recognition of abnormal genetic results is critical in the setting of multiple disease processes and can play a crucial role in the on-going care for a patient.