T2 FLAIR Research Articles

Two Cases of Anti-PD1 Antibody Associated Neurological Immune-Related Adverse Effects (P5-4.001)

<h3>Objective:</h3> We present two cases of neurological immune-related adverse events (irAEs) indefinitely triggered by pembrolizumab. <h3>Background:</h3> Recently, immune checkpoint inhibitors (ICIs) have revolutionized cancer therapy. Programmed cell death protein 1 (PD-1) is a key immune checkpoint receptor expressed by activated T cells. PD-1/PD-L1 antibody therapy is used to treat multiple malignancies; however, it has been associated with an increasing number of irAEs. Neurological irAEs are highly significant complications of ICI therapy that can cause long-term disability and death. Most commonly, neurological irAEs are neuromuscular complications and occur within 4 months after immunotherapy initiation. The anti-PD-1 agent pembrolizumab is currently approved for treatment of several tumors and has been used increasingly due to its more favorable toxicity profile. <h3>Design/Methods:</h3> NA <h3>Results:</h3> NA <h3>Conclusions:</h3> <h3>Case 1:</h3> Our first case is an 84-year-old man with a history of metastatic colon cancer that was treated with pembrolizumab. This is thought to have elicited myositis with elevated CK as well as acetylcholine receptor antibody-positive myasthenia gravis, causing him diffuse and debilitating weakness and functional limitation. He had multiple exacerbations of these symptoms temporally associated with pembrolizumab administration. With the initiation of maintenance treatment and the cessation of pembrolizumab, he required no further admissions for neurologic irAEs, but ultimately he was transitioned to hospice care for cancer progression. <h3>Case 2:</h3> A 65-year-old man with a history of non-small cell carcinoma of the lung on pembrolizumab and zoledronic acid presented with altered mental status and agitation. He had mild pleocytosis on CSF, MRI brain with left mesial temporal T2 FLAIR hyperintensity and nonspecific encephalopathy markers on continuous EEG. He subsequently had complete recovery with IV high-dose steroids followed by plasmapheresis. His symptoms were therefore suspected to be secondary to autoimmune encephalitis elicited by pembrolizumab administration. His oncologist elected to transition to another treatment regimen, and he has had no relapses to date. <b>Disclosure:</b> Ms. Vitek has nothing to disclose. Dr. Hansra has nothing to disclose.

CHANTER Syndrome: Have We Been Overlooking This Catastrophic Opioid Neurotoxicity? (P9-7.002)

<h3>Objective:</h3> To describe the clinical and radiological findings of Cerebellar, hippocampal, and basal nuclei transient edema with restricted diffusion (CHANTER) syndrome in a female presenting with pulseless electrical activity after a potential opioid overdose. <h3>Background:</h3> CHANTER syndrome is a recently described complication of substance abuse, especially opioids, characterized by cytotoxic edema to bilateral hippocampi, basal ganglia and cerebellar cortices. Neuroradiology is distinct from anoxic brain injury as cerebral cortex, thalamus, and subcortical white matter are not involved. <h3>Design/Methods:</h3> Case report with electronic medical chart review. <h3>Results:</h3> 64-year-old African American female with a past medical history of hypertension and depression (on prescribed diazepam), presented after a witnessed cardiac arrest at home with return of spontaneous circulation (ROSC) in the hospital. Pt was intubated on arrival and her initial CT head was unremarkable. Her pocket had multiple opioid pills and urine drug screen was also positive for opiates and benzodiazepines. MRI brain revealed areas of diffusion signal abnormality with corresponding T2 FLAIR hyperintensity in the bilateral cerebellar white matter, the bilateral basal ganglia, and bilateral hippocampi with some patchy involvement of the bilateral (left more than right) occipital lobes suggestive of CHANTER syndrome, as described in limited literature. The electroencephalogram revealed slow background activity but no seizures. A repeat MRI brain on day 8 showed diminished, but unresolved, diffusion restriction involving the supratentorial and infratentorial brain in a symmetric pattern relating to evolution of cytotoxic edema with corresponding improvement of her neurological status. <h3>Conclusions:</h3> CHANTER syndrome’s prognosis depends on early identification and supportive treatment as patients can quickly progress to hydrocephalus. It may be difficult but imperative to differentiate CHANTER syndrome from anoxic brain injury. Further research is needed to identify optimal management strategies for this threatening neurotoxic phenomenon. <b>Disclosure:</b> Dr. Ghani has nothing to disclose. Dr. Yousaf has nothing to disclose. Mr. Kemper has nothing to disclose. Dr. Shi has nothing to disclose. Dr. Sagi has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for SK Life Sciences.

Posterior Reversible Encephalopathy Syndrome (PRES), Hypoxic Injury and Stroke: A Sequelae of Bath Salt Inhalation? (P2-5.012)

<h3>Objective:</h3> To describe a case of bath salt inhalation causing PRES, hypoxic injury, and acute stroke. <h3>Background:</h3> Bath salts contain 3,4-methylenedioxypyrovalerone, a substance similar to cocaine and amphetamines, and can lead to vasospasm by inhibiting dopamine and norepinephrine reuptake. PRES and stroke can be caused by hypertension, but its coexistence in a patient with bath salt inhalation has been not described. <h3>Design/Methods:</h3> This is a case report of a 48-year-old female patient with a history of heroin abuse was found unresponsive. On arrival, was noted to have a systolic blood pressure over 200s and was seizing. On exam, she had expressive and receptive aphasia. Initial labs including a urine toxicology screen were unremarkable. CTA was concerning for vasospasm vs vasculitis. MRI Brain revealed multiple infarcts in bilateral hemisphere with extensive involvement of left posterior cerebral artery and T2 hyperintensities in the bilateral cerebellum and pons. Moreover, T2 FLAIR hyperintensities were noted in bilateral frontal and parietal white matter indicative of PRES. There was also injury to bilateral thalami and watershed vascular distributions suggestive of hypoxic insult. Extensive workup including TEE and LP failed to show an embolic source or vasculitis. <h3>Results:</h3> On further history, mother reported that patient routinely inhales bath salts. PRES findings were improved on repeat MRI 5 days later, but ischemia persisted. Her BP was hard to manage as stroke requires permissive hypertension and PRES requires BP control. She remained aphasic. <h3>Conclusions:</h3> Bath salt inhalation is shown to be associated with ischemic colitis and myocardial infarction, but no instances of stroke or PRES have been reported. The pathophysiological mechanisms between PRES and stroke differ, but may be secondary to vasospasm in the setting of bath salt inhalation. <b>Disclosure:</b> Dr. Mufti has nothing to disclose. Dr. Shi has nothing to disclose. Dr. Mufti has nothing to disclose. Dr. Hasan has nothing to disclose.

Sudden Death in Autoimmune GFAP Astrocytopathy (P5-5.020)

<h3>Objective:</h3> To describe a rare fatal outcome in autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy. <h3>Background:</h3> Our patient was a 42 year old healthy woman who presented with 3 months of low back pain, bilateral upper extremity postural tremor, neck pain which progressed to 1 week of confusion and memory impairment. <h3>Design/Methods:</h3> N/A <h3>Results:</h3> Neurologic exam revealed impaired attention, 3+ deep tendon reflexes throughout, bilateral upper extremity postural tremor and narrow based gait. MRI Brain showed abnormal, diffuse and relatively symmetric periventricular linear enhancement with associated subependymal enhancement. MRI C-spine demonstrated longitudinally extensive T2 FLAIR hyperintensity extending from C2–T2. Lumbar puncture showed opening pressure of 19 cm H2O with lymphocytic pleocytosis. She was treated with 5 days of pulse-dose corticosteroids with significant neurologic improvement and was discharged on prednisone taper. She was at her neurological baseline at Neuroimmunology clinic a week after the discharge. In the interim, CSF studies returned positive for GFAP antibody. Two weeks after the visit, she had new-onset word-finding difficulties, forgetfulness and intermittent staring spells. She was started on prednisone 60 mg daily and had repeat lumbar puncture at the office. Four to five hours after the lumbar puncture, she developed worsening confusion and bilateral upper extremity shaking. She was brought to our ED where her exam revealed dilated pupils with minimal reaction to light. CT head and CTA of the head and neck showed diffuse cerebral edema with absent cortical blood flow. Death by neurologic criteria was met the next day. <h3>Conclusions:</h3> Autoimmune GFAP astrocytopathy has an overall favorable prognosis. Our patient had a fatal outcome secondary to cerebral herniation following lumbar puncture, similar to the first reported fatal case. These two cases suggest that autoimmune GFAP astrocytopathy may present with diffuse cerebral edema and caution should be taken when performing lumbar punctures when this disease is suspected or diagnosed. <b>Disclosure:</b> Dr. Mahat has nothing to disclose. Dr. Dermigny has nothing to disclose. Dr. Alzyoud has nothing to disclose. Dr. Pilato has nothing to disclose.

Same but different. Incidental and symptomatic lower grade gliomas show differences in molecular features and survival

PurposeData on differences in overall survival and molecular characteristics between incidental (iLGG) and symptomatic lower grade Glioma (sLGG) are limited. The aim of this study was to investigate differences between patients with iLGG and sLGG.MethodsAll adult patients with a histologically proven diffuse (WHO°II) or anaplastic (WHO°III) glioma who underwent their first surgery at the authors’ institution between 2010 and 2019 were retrospectively included. Tumor volume on pre- and postoperative MRI scans was determined. Clinical and routine neuropathological data were gained from patients’ charts. If IDH1, ATRX and EGFR were not routinely assessed, they were re-determined.ResultsOut of 161 patients included, 23 (14%) were diagnosed as incidental findings. Main reasons for obtaining MRI were: headache(n = 12), trauma(n = 2), MRI indicated by other departments(n = 7), staging examination for cancer(n = 1), volunteering for MRI sequence testing(n = 1). The asymptomatic patients were significantly younger with a median age of 38 years (IqR28-48) vs. 50 years (IqR38-61), p = 0.011. Incidental LGG showed significantly lower preoperative tumor volumes in T1 CE (p = 0.008), FLAIR (p = 0.038) and DWI (p = 0.028). Incidental LGG demonstrated significantly lower incidence of anaplasia (p = 0.004) and lower expression of MIB-1 (p = 0.008) compared to sLGG. IDH1-mutation was significantly more common in iLGG (p = 0.024). Incidental LGG showed a significantly longer OS (mean 212 vs. 70 months, p = 0.005) and PFS (mean 201 vs. 61 months, p = 0.001) compared to sLGG.ConclusionOur study is the first to depict a significant difference in molecular characteristics between iLGG and sLGG. The findings of this study confirmed and extended the results of previous studies showing a better outcome and more favorable radiological, volumetric and neuropathological features of iLGG.

Abstract Number ‐ 115: A Case of Post‐Pipeline Headache : New Post‐Flow Diversion Headaches for Intracranial Aneurysm

Introduction Flow diversion using devices such as the “pipeline” stent is now a common treatment for unruptured intracranial aneurysms. Though much is known about the efficacy of the device, less is reported regarding potential side effects. We aimed to describe a case of giant aneurysm with new onset and severe headache Post‐ Pipeline flow diversion stent application. Methods A 70‐year‐old female patient was admitted to the neurology outpatient clinic with complaints of diplopia and drooping of the left eyelid that started three months ago. Neurological examination revealed decreased visual acuity and total ophthalmoplegia in the left eye. In neuroimaging,semi‐thrombosed aneurysm, 20*25*20 mm in size, compressing the optic nerve and cavernous structures was observed in the left internal carotid artery cavernous segment. In digital subtraction angiography, a narrow neck, 20*25*22 mm giant aneurysm was observed in the left ICA C4 segment in three‐dimensional imaging. In premedication, were started seven days ago acetylsalicylic acid 100 mg 1*1 and 48 hours ago ticagrelol 2*90 mg and Pipeline 4.25*30 mm flow diverter was applied to cover the aneurysm neck under general anesthesia. One day after the procedure, the patient developed a new headache behind the left eye, throbbing, sensitive to sound and light, unresponsive to paracetamol and non‐steroidal anti‐inflammatory agents.In contrast‐enhanced cranial MRI, an aneurysm causing a central thrombosed hypointense mass effect was observed in the left cavernous structure neighbor with a peripheral enhanced, hyperintense T2 Flair sequence compatible with peripheral vasogenic edema. Pulse 1 gram/day steroid treatment was applied to the patient for three days.The patient’s headache completely regressed at the end of the third month, and perianeurysmal inflammation disappeared in the same contrast‐enhanced cranial MRI.In the 12th month post‐procedure imaging of the patient, it was observed that the aneurysm was completely occluded. Results In the case, we reporta patient with neurologic worsening after flow‐diverter treatment for unruptured cerebral aneurysms. We found MR imaging evidence of perianeurysmal brain inflammation after the therapeutic thrombosis of the sac to be the main cause explaining clinical aggravation. Consequently, we may consider a perianeurysmal brain inflammation when encountering the association of a postprocedure headache with an increase in previous compressive signs, possibly associated with MR imaging signs of inflammation1,2. We tried pulse steroid therapy, which is accepted as the first‐line treatment for postimplantation syndrome after endovascular repair of aortic aneurysms in the literature for a patient who did not respond dramatically to nonsteroidal anti‐inflammatory therapy3.We experienced a significant improvement in the patient’s clinical findings and contrast‐enhanced MRI findings after steroid treatment. Conclusions An inflammatory reaction may aggravate, transiently, clinical symptoms after aneurysm treatment with a flow‐diverter device. Further research is needed to better understand the underlying mechanisms as well as to achieve better prevention strategies.

Atypical Neurological Presentation in a Patient with Uncontrolled Diabetes

Chorea is a disorder characterized by irregular and involuntary hyperkinetic movements associated with malfunctioning the basal ganglia. One of the rarest acquired causes of unilateral or bilateral chorea is hyperglycemia. This is a case of an elderly female diabetic who presented With 3 days history of unilateral involuntary movements of the upper limb and lower limb, irrelevant talk, and altered sensorium. She had a random blood glucose level of 418 mg/dl with hemoglobin A1C (HbA1c) of 15% and normal ketones. Magnetic resonance imaging brain demonstrated T1 hyperintense and T2 flair hypointense right basal ganglia. Diagnosis of nonketotic hyperglycemic chorea was considered. The patient was treated with insulin, antipsychotics, and benzodiazepines and the chorea resolved after proper control of glucose levels. This condition can be reversible with early identification and treatment. This disorder can be prevented with good glycemic control.

Abstract TMP30: High Prevalence Of Unrecognized Cerebral Amyloid Angiopathy Related Inflammation Among Patients With Cerebral Amyloid Angiopathy And White Matter Disease

Introduction: Cerebral amyloid angiopathy related inflammation (CAARI) is an autoimmune inflammatory condition that occurs in patients with cerebral amyloid angiopathy (CAA) and can lead to rapidly progressive cognitive decline. Little is known about the prevalence of subclinical and clinical CAARI. We sought to determine the prevalence of subclinical and clinical CAARI among patients with CAA and white matter hyperintensities (WMH). Methods: In this multicenter, single healthcare system, retrospective cohort study, we reviewed electronic medical records and MRI brain scans of patients presenting with a diagnosis code for amyloidosis or CAA that were evaluated between 1/2010 - 6/2020. We included patients meeting modified Boston criteria for CAA who had WMH on T2 FLAIR sequences. Patients without available hemosiderin sensitive sequences (SWI or GRE) and T2 FLAIR sequences and those without WMH on T2 FLAIR imaging were excluded. Two independent vascular neurologists blinded to background clinical information reviewed each MRI brain scan for the presence of CAARI. The clinical course and outcomes were reviewed and reported. Results: Out of 1100 patients reviewed, 511 met modified Boston criteria for CAA and 193 met the final study inclusion criteria. 55 (28.5% of those with CAA and WMH, and 10.8% of all CAA) patients had MRI brain imaging suggestive of CAA-RI. 21 (38.2%) were male, 38 (69.1%) were Caucasian, and the mean (SD) age was 72.9 (8.9) years. CAA-RI was recognized in only 10 (18.2%) patients initially while 20 (36.4%) were diagnosed up to 9 months later (median 0, IQR 0-9 months). At time of earliest detection of CAARI on imaging, common concurrent findings were cognitive impairment (74.5%), macro-hemorrhages (52.7%), headache (30.9%), seizures (14.5%), and ischemic infarcts (14.5%). Only 18 (32.7%) patients were started on immunosuppression. Nineteen (34.5%) patients expired during the observation period of which only 8 (42.1%) were ever diagnosed with CAA-RI by their treating clinician. Conclusion: The prevalence of subclinical CAA-RI in our study was high. Most cases of radiographic CAARI went unrecognized and untreated. Further studies are needed to assess if treatment of subclinical CAARI may prevent cognitive decline in these patients.

Abstract TP22: Temporal Lobe Involvement In Cerebral Amyloid Angiopathy Related Inflammation Is Associated With Relapse Of Inflammation And Rapidly Progressive Cognitive Decline

Introduction: Cerebral Amyloid Angiopathy related inflammation (CAARI) is an autoimmune cause of rapidly progressive cognitive decline. Little is known about the magnetic resonance imaging (MRI) brain features associated with relapse of CAARI and the effect of treatment on relapse rates therefore we sought to study this. Methods: In this single center prospective cohort study, we analyzed MRI brain scans of 22 patients with CAARI followed at the Cleveland Clinic Cerebrovascular Center between September 2019 and July 2022. We divided patterns of edema on T2 FLAIR imaging into focal, multifocal or diffuse and assessed for temporal lobe involvement (TLI). We analyzed hemosiderin sensitive sequences for the pattern of CAA including cerebral microbleeds (CMB), macrohemorrhage (MH) and/or cortical superficial siderosis (cSS). We collected data on clinical features, treatment duration and cognition after treatment. Relapse was defined as recurrence of edema on T2 FLAIR with cognitive decline or seizure. We report percentages of each clinical feature, imaging pattern and treatment effect on relapse rates. Results: Median age was 74 (35% male, 75% White). Progressive cognitive decline (68%) and seizures (68%) were common but headaches (27.2%) occurred less frequently. Most common CAA features were asymmetric CMB (100%), followed by MH (45%) and cSS (22.7%). The most common pattern of cerebral edema was multifocal (50%), followed by focal (31.8%) and diffuse (27.2%). TLI was seen in 17 (77%) patients. 21 (95.4%) patients were treated with prednisone for median (Q1, Q3) duration of 20 (12, 24) weeks whereas 5 (22.7%) were treated with long term mycophenolate. A total of 6 (27.2%) relapses were seen during a median (Q1, Q3) follow up period of 14 (7, 21) months and occurred at a median (Q1, Q3) of 5.5 (5.12, 5.5) months after stopping steroids. Amongst those who relapsed, none were on mycophenolate, 4 (66.6%) had diffuse edema, 2 (33.4%) had focal edema and all had asymmetric CMB and TLI. No relapses were seen after initiating mycophenolate. Conclusion: Asymmetric CMBs, diffuse or focal cerebral edema with temporal lobe involvement and not being on steroid sparing therapy were associated with relapse of CAARI and rapidly progressive cognitive decline.

Abstract WMP76: Carotid Revascularization Slows Hemispheric White Matter Disease Progression

Introduction: Carotid revascularization is known to prevent stroke in patients with high-grade carotid stenosis, but whether carotid revascularization may slow the progression of hemispheric WMD in patients with carotid stenosis to prevent cognitive decline have not been well described. Methods: We conducted a longitudinal study involving patients age ≥40 years with carotid stenosis on ultrasound from 2011-2015 with serial brain MRI scans at least 1 year apart followed for radiographic and clinical outcomes to 2020. Severity of carotid stenosis was defined as moderate 50-69%, high-grade 70-99%, or occluded. Hemispheric WMD volume was quantified from axial T2 FLAIR images. Patients were stratified by revascularization status. Primary outcome was change in WMD volume per year ipsilateral or contralateral to the index carotid. Hemispheric differences in the annual rate of WMD progression (cc/yr) were compared between revascularized vs not revascularized using multivariable linear regression analysis adjusted for variables that differed between revascularization status. Results: Of the 150 patients, moderate stenosis was present in 37.3%, high-grade stenosis in 42.0%, and carotid occlusion in 20.7%. Mean age was 71±10 years, 36.7% were female. Seventy-three (48.7%) had carotid revascularization with a median follow-up of 5.1 years. Baseline WMD volume was 4.86cc ipsilateral and 4.39cc contralateral to the index carotid. Patients who underwent revascularization trended to have slower WMD progression in the hemisphere ipsilateral to the index carotid compared to those not revascularized (0.32cc/yr vs 0.63 cc/yr, p=0.340). The same trend was not observed in the contralateral hemisphere. In adjusted models, carotid revascularization was associated with a 0.33 cc per year decrease in WMD progression between hemispheres compared to those not revascularized (β= -0.33, p=0.070). Conclusions: In this study with a median follow-up of 5 years, we found that carotid revascularization resulted in a 50% reduction in the annual rate of WMD progression. Intervention trials should test whether carotid revascularization slows WMD progression in patients with cognitive impairment to prevent further cerebrovascular events and cognitive decline.

Structural covariance in subcortical regions in multiple sclerosis and neuromyelitis optica spectrum disorders: An MRI-based study with automated brain volumetry.

This study aimed to investigate the alterations of brain volumetry and associated structural covariance in subcortical regions in multiple sclerosis (MS) and neuromyelitis optica spectrum disorders (NMOSD). Fourty MS patients, 35 NMOSD patients and 34 healthy controls (HC) underwent 3D T1-weighted image and 3D T2 FLAIR of MRI. The volume differences in subcortical regions were compared between the MS, NMOSD, and HC groups by automated brain volumetry. Structural covariance analysis was performed with each pair of these regions to investigate the alterations of anatomical connections in MS and NMOSD compared to HC. Compared with HC, MS patients presented significantly smaller volume in some subcortical and infratentorial regions (P<0.05), while NMOSD patients showed no significant difference of volumetry in any of the brain regions (P>0.05), although they had no significant difference in disease duration (MS 3.95±3.73 ys; NMOSD 3.11±4.61 ys; P>0.05). In addition, the structural covariance analyses revealed synergic volume alteration in subcortical regions both in the MS and NMOSD groups. More extensive additional connections compared with HC were found in MS patients and more extensive missing connections compared with HC were found in NMOSD patients. This study revealed distinct patterns of brain structural damage and reorganization in MS and NMOSD, which could facilitate a better distinction between these two entities.

Acute disseminated encephalomyelitis following the COVID-19 vaccine Ad26.COV2.S, a case report

BackgroundThe severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) pandemic has been leading to dramatic health, social and economic problems around the world. It was necessary to introduce worldwide vaccination program against SARS-CoV-2 virus. Vaccination of billions of people around the world leads to many questions about risk of vaccines and possible side effects. It is well known that acute disseminated encephalomyelitis (ADEM) is a rare, but possible complication of vaccines. Previously, cases of ADEM following various COVID-19 vaccines, including the vaccines from AstraZenica, Pfizer, Sputnik V, SinoVac, Moderna, Sinopharm, have been described. In this case report, we present the first documented case of ADEM following the COVID-19 vaccine Ad26.COV2.S from Johnson & Johnson.Case presentationWe present the case of a 31-year-old female with gradually progression of right-sided weakness and numbness during a three-week period. Four weeks prior to symptom onset, the patient received the single-dose SARS-CoV-2 vaccine Ad26.COV2.S. Neuroimaging revealed five large juxtacortical T2 FLAIR hyperintense lesions with incomplete contrast enhancement on post-contrast T1 images located supratentorial: one in the right cerebral hemisphere and four in left cerebral hemisphere. The patient was followed up for four months. Symptom debut, clinical picture and MRI were typical for ADEM and the patient completely recovered after high dose intravenous methylprednisolone treatment.ConclusionsThis is, to the best of our knowledge, the first case report of ADEM following the COVID-19 vaccine Ad26.COV2.S. This case illustrates, although ADEM is a rare complication following SARS-CoV-2 vaccines, the necessity of maintaining a vaccine safety monitoring system to identify patients at high risk from developing severe complications from the vaccines.

Interrater reliability of modified visual mrı rating scale assessing atrophy and white matter changes.

<p>Cortical atrophy and white matter changes are common findings on magnetic resonance imaging among elderly. Several visual scales have been proposed to evaluate these changes using neuroimaging. We have recently proposed a scale (Modified Visual Magnetic Resonance Rating Scale) recently which allows us to evaluate atrophy, white matter hyperintensities, basal ganglia and infratentorial infarcts together. Our aim in this study was to evaluate the interrater reliability of magnetic resonance visual assessment using this scale between two neurologists and a radiologist.&nbsp;</p>. <p>Randomly selected 30 patients in different ages who underwent brain magnetic resonance imaging between January 2014 and March 2015 were included. Axial T1, coronal T2, and axial FLAIR sequences were visually scored by two neurologists and one radiologist separately. Sulcal, ventricular and medial temporal lobe atrophy, periventricular and subcortical white matter hyperintensities, basal ganglia and infratentorial infarcts were graded according to our scale. The interrater reliability and internal consistency analysis were evaluated by using intraclass correlation coefficient and Cronbach&rsquo;s alpha tests.&nbsp;</p>. <p>The interrater agreements vary between good to excellent. The interrater correlations are moderate to excellent. Interrater correlations were excellent between two neurologists, especially on ventricular atrophy, medial temporal atrophy, basal ganglia infarcts, infratentorial infarcts. When assessing ventricular atrophy, interrater correlations between individual raters were higher than sulcal atrophy. We found good correlations between neurologists and radiologist, and excellent correlations between the two neurologists for medial temporal atrophy. We found excellent interrater correlations between neurologists and radiologist for white matter hyperintensities.</p>. <p>Our scale is a reliable tool assessing both atrophy and white matter hyperintensities with a good interrater reliability. Ventricular atrophy seems to be a more reliable marker than sulcal atrophy when assessing the atrophy on neuroimaging of a patient with memory decline. We think that the total score of the scale will also guide us in clinical practice.</p>.

Chordoid gliomas of the third ventricle

Chordoid glioma is a rare slow-growing tumor of the central nervous system. Available world experience includes no more than 200 cases (lesion of the third ventricle in absolute majority of cases). Recognition and treatment of chordoid glioma are currently difficult problems due to small incidence of this disease. To describe clinical manifestations and surgical treatment of chordoid glioma of the third ventricle considering literature data and own experience. There were 12 patients (6 men and 6 women) with chordoid glioma between 2004 and 2023 (10 patients with lesion of the third ventricle, 1 - lateral ventricle, 1 - pineal region). Only patients with tumors of the third ventricle were analyzed. Total and subtotal resection was performed in 1 and 3 cases, respectively. Five patients underwent partial resection, 1 patient underwent biopsy. The follow-up data were available in 7 out of 10 patients (mean 25 months). Radiotherapy was performed in 4 patients (continued tumor growth in 2 cases). One patient died. Chordoid glioma is a benign tumor predominantly localized in the third ventricle. Preoperative MRI and CT in some cases make it possible to suspect chordoid glioma and differentiate this tumor from craniopharyngioma, meningioma and pituitary adenoma by such signs as isointense signal in T1WI, hyper- or isointense signal in T2WI, homogeneous contrast enhancement and edema of basal ganglia in T2 FLAIR images. The only effective treatment for chordoid glioma is surgery. Total resection is often impossible or extremely dangerous due to location of tumor, large size and invasion of the third ventricle. Postoperative mental disorders and diabetes insipidus, including severe hypernatremia, are common that requires mandatory monitoring of water and electrolyte balance.

Leukoaraiosis and Dizziness: Are They Related? – A Case Series

Dizziness and imbalance are one of the most common complaints seen in elderly people. Age-related ischemic diseases have varying degrees of manifestations and imbalance problems. Cerebral small vessel white matter disease has recently been a topic of interest in view of its association with geriatric syndromes–development of cognitive decline, dementia, falls, etc., but the association between small vessel disease and dizziness has not established clearly yet. Magnetic resonance imaging (MRI) brain is the most commonly used imaging modality for neurological disorders. White matter hyperintensities (WMHs) on T2-weighted sequences on MRI are known as leukoaraiosis (LA) and are suggestive of small vessel white matter disease and represent a high risk of cerebral, cerebellar, and brainstem stroke. The purpose of the present study is to investigate the correlation between unexplained vertigo and radiological changes of LA (small vessel disease) and videonystagmography (VNG) findings of such patients. First patient, a 55-year-old male patient had complaints of short-lasting episodes of spinning sensation and imbalance for the past 2 years. MRI brain showed small vessel ischemic changes (leukoaraiosis) with right vascular loop type 3 (anterior inferior cerebellar artery) without compressive symptoms. VNG showed broken smooth pursuit in horizontal and vertical planes; a hypometric saccades tracing in both planes and microsaccadic oscillations in spontaneous gaze. Second patient, a 60-year-old male had recurrent episodes of vertigo for the past 15 years. His VNG showed micro-oscillations in the saccades and impaired smooth pursuit. MRI revealed bilateral symmetrical T2 FLAIR hyperintensities in white matter and lateral ventricles. Third patient, a 69-year-old female presented with giddiness, nausea, and vomiting for 5 days. Pure-tone audiogram showed mild sensorineural hearing loss. MRI brain showed diffuse cerebral atrophy with small vessel ischemic changes. VNG showed impaired smooth pursuit and microsaccadic oscillations when the gaze was fixed. All three patients were managed successfully by vestibular rehabilitation and antioxidants and Coenzyme Q10. Through this study, we would like to highlight the possibility of a relation between leukoaraiosis and unexplained dizziness among elderly patients.

Sex disparity of cerebral white matter hyperintensity in the hypertensive elderly: The Shanghai Changfeng study.

White matter hyperintensity (WMH) is associated with vascular hemodynamic alterations and reflects white matter injury. To date, the sex difference of tract-specific WMH and the relationship between high blood pressure (BP) and tract-specific WMH remain unclear. We recruited 515 subjects from the Shanghai Changfeng study (range 53-89 years, mean age 67.33 years). Systolic and diastolic blood pressure (SBP and DBP) were collected and used to calculate pulse pressure (PP). Magnetic resonance T1 and T2 FLAIR images were acquired to measure WMH and calculate WMH index. The ANCOVA test was performed to test the difference between sexes, and the linear regression model was used to examine the associations between BP and WMH index. Men showed higher WMH index than women in all white matter tracts (p < .001, respectively) except for the bilateral superior longitudinal fasciculus (SLF) and its left temporal part (tSLF). High SBP and PP was associated with a lower WMH index on the left corticospinal tract (CST), SLF, tSLF and right cingulum in hippocampus (p ≤ .001, respectively) in women, while high DBP was associated with a higher WMH index on the bilateral CST (left p < .001; right p=.001), left inferior longitudinal fasciculus (p < .001) and inferior fronto-occipital fasciculus (p=.002) in men. Men tend to have more WMH compared to women. A high SBP/PP relates to a lower WMH burden in women. This suggests that women could benefit from higher blood pressure in older age.

Unsupervised machine learning using K-means identifies radiomic subgroups of pediatric low-grade gliomas that correlate with key molecular markers

IntroductionDespite advancements in molecular and histopathologic characterization of pediatric low-grade gliomas (pLGGs), there remains significant phenotypic heterogeneity among tumors with similar categorizations. We hypothesized that an unsupervised machine learning approach based on radiomic features may reveal distinct pLGG imaging subtypes. MethodsMulti-parametric MR images (T1 pre- and post-contrast, T2, and T2 FLAIR) from 157 patients with pLGGs were collected and 881 quantitative radiomic features were extracted from tumorous region. Clustering was performed using K-means after applying principal component analysis (PCA) for feature dimensionality reduction. Molecular and demographic data was obtained from the PedCBioportal and compared between imaging subtypes. ResultsK-means identified three distinct imaging-based subtypes. Subtypes differed in mutational frequencies of BRAF (p < 0.05) as well as the gene expression of BRAF (p<0.05). It was also found that age (p < 0.05), tumor location (p < 0.01), and tumor histology (p < 0.0001) differed significantly between the imaging subtypes. ConclusionIn this exploratory work, it was found that clustering of pLGGs based on radiomic features identifies distinct, imaging-based subtypes that correlate with important molecular markers and demographic details. This finding supports the notion that incorporation of radiomic data could augment our ability to better characterize pLGGs.

1029. Clinical Characteristics and Outcomes of Neuroinvasive West Nile Virus Infection in Immunocompromised and Immunocompetent Individuals

Abstract Background Despite the increasing incidence of neuroinvasive West Nile virus (NiWNV) infection in the US, the spectrum of disease characteristics and neuroimaging findings in immunosuppressed (IS) individuals are not adequately described. We aimed to compare the clinical characteristics and outcomes of NiWNV infection in IS and immunocompetent (IC) patients. Methods We extracted relevant data from all NiWNV patients hospitalized 7/2003-10/2021 at Mayo Clinic hospitals. Cohort was inclusive of patients from the recent historic WNV outbreak in Arizona in 2021. Chi-Square or Kruskal-Wallis and logistic regression were used to compare relevant variables and determine predictors of mortality respectively. Results We included 115 patients (72 IC and 43 IS), mean age 63.5 years; neurologic syndromes included meningoencephalitis (85.2%), encephalomyelitis (8.7%) and myeloradiculitis (6.1%). Presenting symptoms were malaise (72%), fever (66%), altered mentation (64%), gastrointestinal (47%) and myalgia (35.7%). MRI brain was abnormal in 62.8% (49/78), demonstrating T2/FLAIR hyperintensities in 47.4% (brainstem, thalamus, temporal lobes), leptomeningeal enhancement (16.7%) and diffusion restriction (20.5%). Altered mental status (76.7% vs 56.9%), myalgia (44.4% vs. 20.9%), myoclonus (18.6% vs. 4.2%) and thalamic MRI T2 FLAIR abnormalities (11.4% vs 0%) were more common in IS patients. Higher CSF WBC counts were observed in IC vs IS patients (P&amp;lt; 0.05). Immunosuppressed patients were more likely to be treated with intravenous immunoglobulin (44.2% vs 8.3% p=&amp;lt; 0.001) and/or interferon therapy (32.6% vs 6.9%, p=0.0003) and had increased odds of 90-day mortality on multivariable analysis (Adjusted Odds Ratio, AOR 2.22; 95% CI 1.065-4.627, p=0.0334). In the IS subgroup, ICU admission, mechanical ventilation, and Glasgow coma scale of &amp;lt; 8 were associated with reduced overall survival/increased 90-day mortality (p&amp;lt; 0.005). Conclusion Individuals presenting in summer/fall months with the aforementioned symptoms and/or MRI abnormalities should be evaluated for NiWNV infection. Compared to the immunocompetent, immunosuppressed patients with NiWNV are at a significantly greater mortality risk. Novel and effective antiviral therapies aimed at improving outcomes are warranted. Disclosures All Authors: No reported disclosures.

500: DIAGNOSIS OF WERNICKE ENCEPHALOPATHY IN A PATIENT WITH UNDERLYING PSYCHIATRIC ILLNESS

Introduction: Wernicke’s encephalopathy (WE) is an acute neuropsychiatric syndrome caused by thiamine deficiency. Common causes include alcohol use, bariatric surgery, and hemodialysis. Description: Our case involves a 65-year-old female who initially presented with seizure-like activity. Subsequent presentations over months included dysphagia, behavioral changes, and confusion. Initial workup with EEG, CT head, MRI brain, CT soft tissue neck, and endoscopy was unremarkable. Further history revealed that after her mother’s death four months prior, she began to self-isolate and reduce oral intake. Patient was evaluated by psychiatry and was diagnosed with conversion disorder. The clinical course was complicated by worsening neurological symptoms and respiratory distress requiring mechanical ventilation. Repeat MRI brain showed increased T2 FLAIR signal in the periaqueductal gray matter and along the dorsomedial thalamus consistent with WE. Patient was started on thiamine and showed symptomatic improvement. Discussion: WE is a triad of oculomotor dysfunction, ataxia, and confusion. In an observational study by Chamorro et al, 92.7% of cases were due to excessive alcohol intake, 47.1% due to malnutrition, and 5.9% due to psychiatric illness. The diagnosis of WE is primarily clinical, yet the classic triad is reported in only 10% of cases. The most consistent characteristic is a change in mental status. Caine et al developed the clinical diagnostic criteria, which include dietary deficiencies, oculomotor abnormalities, cerebellar dysfunction, and altered mentation or mild memory impairment. A case series based on this criteria found that clinical features of WE vary between alcohol and non–alcohol-related etiologies. The classic triad was more common in alcoholics than in non-alcoholics. One of the main challenges is the rate of underdiagnosis, which can be due to a lack of clinical suspicion or atypical presentation. Hence, MRI is a useful test to support the diagnosis of WE with a sensitivity of 53% and specificity of 93%. MRI may demonstrate lesions with increased symmetrical signal intensity in the periaqueductal region, medullary bodies, medial thalami, and the tectum of the midbrain. Diagnosis of WE should be considered in patients with psychiatric illnesses and atypical neurologic presentation.

Acute Autoimmune Encephalitis With Features of Bickerstaff Brainstem Encephalitis (BBE) and Two Abnormal Autoantibodies Presenting With Prominent Cerebellar Abnormality on MRI–A Case Report

Objective To present an unusual cerebellar imaging finding of a patient with clinical features of BBE Background BBE is characterized by progressive ataxia, ophthalmoplegia and impaired consciousness. Magnetic resonance imaging (MRI) of the brain is usually normal. However, rare T2 Flair changes have been reported. Scarcity of cerebellar findings on imaging led to the controversy of peripheral vs central etiology for the ataxia. Despite other modalities including positron emission tomography, magnetic resonance spectroscopy and molecular level evidence pointing towards involvement of the cerebellum, MRI is usually unrevealing. Design/Methods A 62-year-old woman presented with acute onset ataxia with multiple falls, dysarthria, diplopia, and blurred vision that started 3 days prior to presentation. She had left face angioedema couple days after her flu inoculation 6 weeks prior to presentation. Her exam revealed normal mental status, scanning speech, bilateral dysmetria and ataxia as well as left-sided facial palsy, square wave jerks and hyperreflexia. CSF showed 30 RBC, 17 WBC, normal glucose, and elevated protein of 68 mg/dl. No infectious etiologies were identified. MRI brain showed infratentorial leptomeningeal enhancement with T2 hyperintensities in the both cerebellar hemispheres. Anti-GQ1b antibodies were 51 IV (negative &lt; 30 IV) and anti-GAD65 antibodies were also weakly positive only in serum, 0.12 nmol/L (negative &lt; 0.02 nmol/L). Results She was diagnosed with autoimmune encephalitis and treated with IV methylprednisolone and IVIG. She rapidly improved clinically, and her imaging findings resolved. Conclusions We demonstrated prominent cerebellar imaging findings and good recovery in a patient with anti-GQ1b and mild anti-GAD65 seropositive autoimmune encephalitis. Our case is the first reported double positive autoimmune encephalitis with features of BBE and direct cerebellar involvement