Areas Of Restricted Diffusion Research Articles

200 DWI and ADC Changes in Patients With Glioblastoma IDH-Wild Type Following Surgical Tumor Resection

INTRODUCTION: Current standard of care for glioblastoma (GBM) entails maximal tumor resection to achieve better outcomes while preserving neurological function. Early postoperative DWI has elucidated that peritumoral ischemia is common following glioma resection, but radiographic characteristics, risk factors, and outcomes have not been well described. METHODS: This retrospective cohort included 324 patients (18-86 years) who underwent surgical resection for glioblastoma IDH-wildtype between 2005 and 2022. All patients had structural MRI and DWI acquired preoperatively and within 72 hrs after resection. Areas of restricted diffusion on postoperative DWI were classified as rim, sector, mixed, or remote. RESULTS: Areas of restricted diffusion on postoperative DWI were found in 173 (53%) patients. 55 (32%) were rim-shaped, 103 (59%) sector-shaped, 10 (6%) mixed, and 5 (3%) remote. Preoperative tumor volume was positively correlated with areas of ischemia (p = 0.048), but peritumoral DWI was not correlated with decreased overall survival (OS) or Karnofsky Performance Score (KPS). In the full cohort analysis, gross total resection (GTR) correlated with improved OS compared to subtotal total resection (STR) (p = 0.016); however, subgroup analysis revealed that this survival benefit was exclusively found in patients without peritumoral infarction on DWI (21 months vs. 13 months, p = 0.043). CONCLUSIONS: Larger tumor volume was a risk factor for larger peritumoral infarction. The survival benefit of GTR was only seen in patients who did not experience postoperative peritumoral infarction, underscoring the importance of maximal safe tumor resection. Future study on specific causes of postoperative peritumoral infarctions may improve surgical management and outcomes in glioblastoma patients.

Acute leukoencephalopathy with restricted diffusion – Case Series

Acute leukoencephalopathy with restricted diffusion (ALERD) is a clinicopathological diagnosis that is characterized by severe encephalopathy and seizures with extensive areas of restricted diffusion in cerebral parenchyma. Case 1: Apparently healthy 13-month-old male child presented with acute febrile encephalopathy with a flurry of seizures with raised intracranial pressure. Investigations were consistent with multiorgan dysfunction along with severe metabolic acidosis, ketosis, and hyperammonemia. Initial magnetic resonance imaging (MRI) Brain was normal. However subsequent MRI brain suggested features of diffuse ALERD. With immunotherapy and supportive management, he improved and was discharged without sequelae. Case 2: A 9-month-old male child who was premorbidly normal presented with fever, status epilepticus, and encephalopathy. MRI Brain was suggestive of central sparing ALERD. He was discharged with sequelae with the advice of rehabilitation. Unfortunately, he got succumbed due to aspiration. Case 3: A 10-day-old neonate presented with the refusal of feeds, multiple seizures, and encephalopathy. MRI Brain revealed central sparing ALERD. Improved with immunotherapy and was developmentally normal at five months of age. ALERD is a clinic-radiological syndrome characterized by acute encephalopathy with restricted areas of diffusion in subcortical white matter on MRI. Reported two categories are Diffuse and Central sparing ALERD. Diffuse ALERD has severe manifestations. However, Case 1 had a good outcome, suggesting a variable prognosis. Central sparing ALERD is a milder form, but when basal ganglia are involved, it may have a worsened outcome, as seen in Case 2. Case 3 had central sparing ALERD, and had a good prognosis as described in literature. This study highlights the varied signs and symptoms of ALERD, including neonatal age of onset. Diagnosis is based on the restricted diffusion in white matter on diffusion-weighted imaging and the apparent diffusion coefficient in MRI of the brain. MRI may be normal in the initial course; hence, it’s important to repeat if strongly suspected. Prompt immunotherapy and supportive care are emphasised.

P-27 Sweet moves - Hemichorea associated with non-ketotic hyperglycemia

Abstract Introduction Hemichorea is a movement disorder characterized by involuntary, irregular, and often violent movements of one hemibody, typically caused by dysfunction in the basal ganglia. There are several potential causes of hemichorea, namely vascular lesions, autoimmune conditions, structural lesions, drugs, or metabolic disturbances, among others. Hemichorea associated with non-ketotic hyperglycemia is a rare condition with a reported prevalence of 1 in 100.000. Although its exact mechanisms are not fully understood, it is believed to be linked to metabolic changes and vascular abnormalities associated with diabetes and its management involves optimizing blood sugar levels. Clinical Case This is the case of a female patient, 76 years old, caucasian with known medical history of hypertension, type 2 diabetes, and dyslipidemia. The woman, admitted to the Neurology department, presented with involuntary movements of the left hemibody and face, with sudden onset, one day before admission. There was no history of visual changes, headache, dizziness, syncope, or other symptoms and there were no recent changes in her usual medication. The neurological examination didn't show any defects other than the choreic movements of the left hemiface and hemibody. Blood tests revealed an HbA1c level of 9.3% and a glucose level of 254 mg/dL. A brain CT scan revealed a possible hypodensity of the right insula, besides signs of microangiopathic leukoencephalopathy. A brain MRI was ordered and showed, on the right putamen, hyperintensity on T1, mild hyperintensity on Flair (Fig.1), and mild hypointensity on T2, without areas of diffusion restriction suggesting ischaemic vascular damage - radiologic findings consistent with hemichorea associated with non-ketotic hyperglycemia. Strict glycemic control was achieved in cooperation with the Endocrinology department, with complete resolution of symptoms. Conclusion Although rare, hyperglycemia should be considered as a potential cause of hemichorea, as correction of the underlying condition will lead to rapid improvement of the movements and is the most important feature for diagnosis. This case shows the myriad of clinical presentations that a person living with Diabetes can have.Figure 1.Brain MRI scan.Brain MRI scan showing, on the right putamen, mild hyperintensity on Flair.

Differentiation of multiple brain metastases and glioblastoma with multiple foci using MRI criteria

ObjectiveGlioblastoma with multiple foci (mGBM) and multiple brain metastases share several common features on magnetic resonance imaging (MRI). A reliable preoperative diagnosis would be of clinical relevance. The aim of this study was to explore the differences and similarities between mGBM and multiple brain metastases on MRI.MethodsWe performed a retrospective analysis of 50 patients with mGBM and compared them with a cohort of 50 patients with multiple brain metastases (2–10 lesions) histologically confirmed and treated at our department between 2015 and 2020. The following imaging characteristics were analyzed: lesion location, distribution, morphology, (T2-/FLAIR-weighted) connections between the lesions, patterns of contrast agent uptake, apparent diffusion coefficient (ADC)-values within the lesion, the surrounding T2-hyperintensity, and edema distribution.ResultsA total of 210 brain metastases and 181 mGBM lesions were analyzed. An infratentorial localization was found significantly more often in patients with multiple brain metastases compared to mGBM patients (28 vs. 1.5%, p < 0.001). A T2-connection between the lesions was detected in 63% of mGBM lesions compared to 1% of brain metastases. Cortical edema was only present in mGBM. Perifocal edema with larger areas of diffusion restriction was detected in 31% of mGBM patients, but not in patients with metastases.ConclusionWe identified a set of imaging features which improve preoperative diagnosis. The presence of T2-weighted imaging hyperintensity connection between the lesions and cortical edema with varying ADC-values was typical for mGBM.

Diffusion Restriction in the Splenium: A Comparative Study of Cytotoxic Lesions of the Corpus Callosum (CLOCCs) versus Lesions of Vascular Etiology.

Cytotoxic lesions of the corpus callosum (CLOCCs) have broad differential diagnoses. Differentiating these lesions from lesions of vascular etiology is of high clinical significance. We compared the clinical and radiological characteristics and outcomes between vascular splenial lesions and CLOCCs in a retrospective cohort study. We examined the clinical and radiologic characteristics and outcomes in 155 patients with diffusion restriction in the splenium of the corpus callosum. Patients with lesions attributed to a vascular etiology (N = 124) were older (64.1 vs. 34.6 years old, p < 0.001) and had >1 vascular risk factor (91.1% vs. 45.2%, p < 0.001), higher LDL and A1c levels, and echocardiographic abnormalities (all p ≤ 0.05). CLOCCs (N = 31) more commonly had midline splenial involvement (p < 0.001) with only splenial diffusion restriction (p < 0.001), whereas vascular etiology lesions were more likely to have multifocal areas of diffusion restriction (p = 0.002). The rate of in-hospital mortality was significantly higher in patients with vascular etiology lesions (p = 0.04). Across vascular etiology lesions, cardio-embolism was the most frequent stroke mechanism (29.8%). Our study shows that corpus callosum diffusion restricted lesions of vascular etiology and CLOCCs are associated with different baseline, clinical, and radiological characteristics and outcomes. Accurately differentiating these lesions is important for appropriate treatment and secondary prevention.

Abstract 099: Reversible Cerebral Vasoconstriction Syndrome Secondary to Weight Loss Patch: A Case Report​

Introduction Reversible Cerebral Vasoconstriction Syndrome (RCVS) is a clinico‐radiographical syndrome characterized by severe thunderclap headache and reversible multifocal arterial vasoconstriction1. RCVS can be spontaneous or related to a trigger such as vasoactive medication/supplements. There is no previous data on the association of weight loss patch formulation in the genesis of unexplained RCVS. Methods A woman in her 40s presented with five days of recurrent acute onset severe right frontal headaches after starting Thrive weight loss patch supplementation. Head CT showed right frontal lobe hemorrhage with bilateral SAHs (Figure1). Brain MRI redemonstrated hemorrhages, numerous scattered foci of restricted diffusion, and a large area of restricted diffusion in the left insular cortex (Figure 2). Cerebral angiogram showed diffuse multifocal narrowing with a ‘beads‐on‐a‐string’ appearance of the cerebral vasculature and no aneurysms (Figure 3). Thrive patch contains undisclosed amounts of sympathomimetics and vasoactive compounds. Given the history, clinical symptoms of recurrent thunderclap headaches, and angiographic findings, the diagnosis of RCVS was made. Brain MRA 3 months after discharge showed no areas of cerebrovascular focal stenosis (Figure 4). At 4 month follow up, she reported 90% improvement of her symptoms. Results We report a temporal relationship between RCVS in a woman in her 40s and usage of a Thrive weight loss patch, which contains green tea extract, L‐arginine, 5‐hydroxytryptophan (5‐HTP), and Yerba Mate. We believe this report represents the first case of RCVS triggered by a weight loss patch. Multiple compounds within the Thrive patch have vasoactive properties that may contribute to RCVS genesis. Epigallocatechin (component of green tea extract) and 5‐HTP are metabolic compounds with potent vasoactive properties. They are components of weight loss and SSRI/SNRI medications respectively. Numerous studies have documented a temporal relationship between their use and subsequent development of RCVS2,3. Resolution of RCVS is reported after their discontinuation. Only one other case in our literature review investigated a patch modality in a temporal relationship with RCVS. It reported development and resolution of RCVS six days after nicotine patch use and discontinuation4. While Thrive patch does not contain nicotine, this case provides a further avenue for exploration as to whether pharmacokinetics of a patch modality influences a patient’s presentation with RCVS, as all prior reports document oral ingestion of substances. Conclusion Clinicians should be aware of the role of weight loss patches in RCVS, and their use should be assessed in large RCVS cohorts to clarify the association described in this report.

Xanthinuria: A Rare Case Report

INTRODUCTION: Classical xanthinuria is an autosomal recessive hereditary disease which manifests as a result of deficiency of xanthine dehydrogenase which converts hypoxanthine, and xanthine into uric acid. Molybdenum cofactor deficiency, an inherited form of xanthinuria, causes hyperreflexia, microcephaly, and other central nervous system symptoms in newborns. Molybdenum cofactor deficiency, an inherited form of xanthinuria, causes hyperreflexia, microcephaly, and other central nervous system symptoms in newborns. The function of three different enzymes (xanthine dehydrogenase, aldehyde oxidase, and sulfite oxidase) depends on a molybdenum-containing cofactor that is congenitally defective in this disorder. CASE REPORT: A full term female baby born to a G3P1L1A1 mother via normal vaginal delivery was referred from outside hospital on day of life 10 in view of one episode of convulsion on day 3 of life. Baby was screened for inborn errors of metabolism which revealed increased excretion of Xanthine in urine. MRI of brain was done which showed extensive areas of diffusion restriction involving bilateral cerebral hemispheres, bilateral corticospinal tracts and corpus callosum suggestive of Acute hypoxic ischaemic encephalopathy. DISCUSSION: Because of enzymatic deficiency, xanthine dehydrogenase cannot be converted into uric acid which leads to increase in blood levels, and urinary excretion of hypoxanthine, and xanthine. Since these substances have a lower solubility in urine, they accumulate in the urinary system leading to formation of stones. Reduced or undetectable serum uric acid levels associated with MoCD are a result of the deficiency of xanthine dehydrogenase. Most of the patients with classical xanthinuria are asymptomatic, and in 30% of the cases urolithiasis develops. CONCLUSION: Refractory seizures, encephalopathy, and the absence of radiologic signs of HIE a positive family history of perinatal asphyxia, and refractory seizures are contributors in newborn fatalities that demonstrate a significant tendency to develop xanthinuria. A thorough research regarding its prevalence and prognosis should be done. Keywords: xanthinuria, autosomal recessive hereditary disease, newborn

Neonatal Cerebral Sinovenous Thrombosis: A Case Report

Neonatal cerebral sino-venous thrombosis (CSVT) is a rare disorder, associated with long-term neurological sequelae. Cerebral sino-venous thrombosis (CSVT) is a focal or diffuse disruption of cerebral blood flow secondary to occlusion of cerebral veins and/or sinuses. The most commonly presented clinical manifestations were feeding difficulties, lethargy, respiratory distress, loss of consciousness, and seizures. Here we report one such rare case of Neonatal Cerebral Sinovenous Thrombosis in a full-term male admitted to the NICU in view of respiratory distress due to meconium-stained liquor and cried immediately after birth. The baby had convulsions on day 5 of life, MRI Brain was done and reported as multifocal areas of diffusion on restriction and large areas of diffusion restriction involving various parts of brain. Subcutaneous low molecular heparin was started. There is limited literature describing outcomes: 60?80% of all infants have some neurological deficits; cognitive impairment is reported in 10?60%, and epilepsy in 30?40%.

Posterior Reversible Encephalopathy Syndrome Causing Takotsubo Cardiomyopathy or Vise a Versa: How to Differentiate (P4-4.002)

<h3>Objective:</h3> N/A <h3>Background:</h3> Posterior reversible encephalopathy syndrome (PRES) and Takotsubo Cardiomyopathy (TC) are constructs with reversible clinical symptoms and radiographic features with proposed shared molecular signaling pathways are a rare entity to occur concomitantly. PRES-Takotsubo dual diagnosis is attributed to a single catecholamine surge, leading to endothelial dysfunction in cerebral arteries and coronary arteries and myocardial stunning. This theory, however, is seemingly oversimplified and does not address the intricacies of cytotoxic/inflammatory communication between systems. There are no case that describes if PRES precedes TC or vice a versa and how it would improve patient outcome or avoid further life-threatening conditions such as cardiogenic shock which occurred in our patient. <h3>Design/Methods:</h3> N/A <h3>Results:</h3> 72-year-old female with extensive cardiac history with mechanical mitral valve presented with left gaze preference, facial twitching followed by unresponsiveness raising suspicion of seizure versus stroke. CT of head showed hypodensities in bilateral occipital lobes, followed with MRI that showed bilateral white matter hyperintensities in the occipital and frontal lobes, area of restricted diffusion in the right occipital lobe favoring atypical PRES. EKG sowed no ST segment changes and troponin 0.086. Treatment with levetiracetam improved her encephalopathy, but hospitalization was complicated by cardiogenic shock requiring ionotropic support. Echocardiogram findings were consistent with TC. No clear etiology of PRES identified. Repeat TTE in few days showed EF back at 60%. <h3>Conclusions:</h3> Considering patient initial presentation of seizure with no ST segment changes on EKG and mild troponin elevation supports hypothesis of PRES further causing TC with no clear inciting factor for PRES. The case concludes that patient could have idiopathic PRES with extensive cardiac history. Cardiac biomarkers and EKG should be obtained. If any cardiac markers abnormal thorough cardiologic work up should be performed however conversely patient with TC if patient has any neurologic clinical symptoms further neurological work up is warranted <b>Disclosure:</b> Dr. Srinivasan has nothing to disclose. Dr. Kauffman has nothing to disclose. Dr. Shah has nothing to disclose. Dr. Uqdah has nothing to disclose. Dr. Kaleta has nothing to disclose.

AARS2-related Leukodystrophy Without Ovarian Failure Mimicking Multiple Sclerosis in a Female: A Case Report With a Novel Pathogenic Variant (P4-12.010)

<h3>Objective:</h3> N/A <h3>Background:</h3> <i>AARS2</i> mutations affect mitochondrial alanyl-transfer RNA (tRNA) synthetase. This enzyme is responsible of charging alanine to its cognate tRNA for protein translation within mitochondria. Individuals with <i>AARS2</i> mutations were reported to present either severe infantile cardiomyopathy or progressive leukoencephalopathy as the main clinical presentation. <i>AARS2</i>-related leukoencephalopathy is an ultra-rare white matter disorder, characterized by spasticity, ataxia, and impaired cognition. Further disease progression manifests as psychiatric illness and executive dysfunction. Ovarian failure is reported in almost all females with <i>AARS2</i>-related leukoencephalopathy. <h3>Design/Methods:</h3> N/A <h3>Results:</h3> A 32-year-old female was referred to the clinic due to the development of progressive neurological symptoms since 2011. Ascending numbness of the lower limbs was the first clinical symptom that further extended to her abdomen. Patient, then, developed visual impairment in both eyes. Other symptoms included cognitive decline and language impairment that began abruptly with fast progression. She has one child and her menstrual cycles have been always regular. Multiple sclerosis was initially diagnosed and she was treated with corticosteroid, natalizumab, and rituximab without achieving improvements. The brain magnetic resonance imaging (MRI) of a patient performed in our institute showed multiple focal and confluent areas of diffusion restriction in bilateral periventricular and deep white matter, involving bilateral centrum semiovale/ corona radiate. Diffuse brain volume loss and bilateral thinning of optic nerves were observed. The MRI of thoracic spinal cord showed non-enhancing short segment T2 hyperintense lesions within the right ventral cord at T8-T9 and T12-L1. Molecular study reported a novel homozygous variant of c.929G&gt;T (p.Gly310Val) in <i>AARS2</i>. <h3>Conclusions:</h3> <i>AARS2</i>-related leukoencephalopathy in females can present without ovarian failure. The overlapping clinical and imaging features of white matter disorders may lead to misdiagnosis of the disease. Detailed review of clinical characteristics, MRI patterns, and molecular study can be helpful to reach to an accurate diagnosis. <b>Disclosure:</b> Mr. Amanat has nothing to disclose. Julie Cohen, 377 has received personal compensation in the range of $500-$4,999 for serving as a Consultant for PTC Therapeutics. Julie Cohen, 377 has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Illumina. The institution of Julie Cohen, 377 has received research support from A Cure for Ellie. Dr. Fatemi has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Bluebirdbio. Dr. Fatemi has received personal compensation in the range of $10,000-$49,999 for serving as an Expert Witness for RCO law. The institution of Dr. Fatemi has received research support from Minoryx. The institution of Dr. Fatemi has received research support from Viking Therapeutics. The institution of Dr. Fatemi has received research support from Swanbio Therapeutics . The institution of Dr. Fatemi has received research support from Autobahn Therapeutics. The institution of Dr. Fatemi has received research support from Nuvelution. The institution of Dr. Fatemi has received research support from NIH. The institution of Dr. Fatemi has received research support from A Cure for Ellie Foundation. The institution of Dr. Fatemi has received research support from Brian’s Hope Foundation . Dr. Fatemi has received intellectual property interests from a discovery or technology relating to health care. Dr. Fatemi has a non-compensated relationship as a Director (Board member) with ALDConnect that is relevant to AAN interests or activities. The institution of Dr. Fine has received research support from NIH.

Cryptococcal Meningoencephalitis and Immune Reconstitution Inflammatory Syndrome in a patient on Tocilizumab Therapy: A Case Report (P1-10.005)

<h3>Objective:</h3> To describe a case of cryptococcal meningoencephalitis complicated by immune reconstitution inflammatory syndrome (IRIS), vasculitis, ischemic strokes, and hydrocephalus. <h3>Background:</h3> Cryptococcus neoformans is the most common cause of fungal meningitis worldwide and typically affects individuals with defects in cell-mediated immunity. A 71-year-old female with giant cell arteritis on daily prednisone and monthly Tocilizumab infusions presented with worsening headaches for 2 weeks and new-onset confusion for 3 days. Initial workup revealed hyponatremia (sodium 125 mmol/L) and community acquired pneumonia. She was treated with antibiotics, but her mental status rapidly declined necessitating endotracheal intubation. MRI Brain demonstrated multifocal areas of diffusion restriction in the bilateral cerebral and cerebellar hemispheres, basal ganglia, and brainstem with diffuse leptomeningeal enhancement. Cerebrospinal fluid (CSF) studies revealed an elevated opening pressure of 28 cm H₂O, positive CSF cryptococcal antigen, and CSF fungal cultures grew cryptococcus neoformans. She was started on empiric liposomal amphotericin B and flucytosine before the cryptococcal antigen and cultures resulted. Despite treatment, her neurological status continued to decline with repeat MRI showing new ischemic infarctions, subarachnoid hemorrhages, and leptomeningeal and parenchymal enhancement. Digital subtraction angiography revealed cerebral vasospasm and possible vasculitis. Repeat lumbar puncture demonstrated persistently elevated opening pressure, continued pleocytosis, and increased protein. She was continued on antifungal therapy and received IV methylprednisolone for IRIS. She additionally underwent ventriculoperitoneal (VP) shunt placement. Despite these interventions, her clinical status continued to deteriorate and she was palliatively extubated. <h3>Design/Methods:</h3> N/A <h3>Results:</h3> N/A <h3>Conclusions:</h3> This patient presented with cryptococcal meningoencephalitis in the setting of immune suppression from prednisone and tocilizumab, a monoclonal antibody that targets the IL-6 receptor. Despite early and appropriate treatment, she continued to worsen with multiple complications including IRIS, a dysfunctional inflammatory response to infection precipitated by immune recovery. In patients treated appropriately for cryptococcal meningoencephalitis, a failure to respond should raise concern for complicating conditions. <b>Disclosure:</b> Dr. Navalkar has nothing to disclose. Dr. Sui has nothing to disclose. The institution of Dr. Bashir has received research support from NIH. The institution of Dr. Bashir has received research support from PICORI. The institution of Dr. Bashir has received research support from Sanofi. Dr. Bashir has received research support from GWSP. The institution of Dr. Bashir has received research support from Roche. The institution of Dr. Bashir has received research support from Novartis. The institution of Dr. Bashir has received research support from Hoffmann-La Roche.

Ischemic Cerebellar Stroke in a Young Patient with Neurofibromatosis Type 2, a Case Report (P12-5.026)

<h3>Objective:</h3> We present a case of brainstem ischemia in a young patient with neurofibromatosis 2 (NF2) and review the existing literature on similar cases, which together suggest a possible NF2-associated syndrome of which stroke neurologists and pediatric neurologists should be aware. <h3>Background:</h3> NF2 is a multifaceted disease characterized primarily by benign CNS tumors, especially meningiomas and vestibular schwannomas. There have been several cases of brainstem ischemic stroke in young NF2 patients reported in the literature. To date, there is no unified theory about the connection between NF2 and pediatric stroke. <h3>Design/Methods:</h3> We present a case of ischemia in the left cerebellar peduncle of a young patient with NF2, as well as a narrative review of the literature on previous cases. <h3>Results:</h3> The patient presented to the Emergency Department with left facial paresthesia and dizziness. Serial Magnetic Resonance Images (MRI’s) displayed an initial area of restricted diffusion in the left cerebellar peduncle with peripheral enhancement which did improve over several months, consistent with maturing infarct. <h3>Conclusions:</h3> Our case joins several others with similar presentations and findings, including pediatric patients for whom brainstem ischemia was the presenting symptom of NF2. The primary theory for the cause of brainstem ischemia in juvenile NF2 is a microvascular cause due to genetic deficiency of <i>neurofibromin 2</i>, a regulator of endothelial development, whose absence is likely to disrupt vascular homeostatic mechanisms. Multicenter studies with large NF2 cohorts are needed to better characterize this syndrome. Understanding brainstem ischemia in young NF2 patients will inform the development of treatment guidelines for such patients. <b>Disclosure:</b> An immediate family member of Mrs. Trautman has received personal compensation for serving as an employee of Hydro Extrusions. Mrs. Trautman has received personal compensation for serving as an employee of University of South Dakota. Dr. Sandhu has nothing to disclose.

Abstract Number ‐ 225: COVID‐19 As A Potential Inducer Of Antiphospholipid Syndrome In A Patient With Acute Ischemic Stroke

Introduction COVID‐19 has been linked to hypercoagulability and acute ischemic stroke, though the precise mechanism is not well understood [1]. Various mechanisms have been suggested, including the potential release of autoantibodies‐ either via self‐production or exacerbation of the release of already produced autoantibodies [2‐3]. Antiphospholipid antibodies have been identified in severe disease [3‐4] and implicated in thrombosis [1‐2], though the significance is unclear. In this report, we describe a young woman with asymptomatic COVID‐19 presenting with acute ischemic stroke and postulate it was a manifestation of activated autoimmunity from COVID‐19. Methods Case Report Results A 22 year‐old previously healthy woman presented with acute onset dysarthria and right upper extremity weakness and numbness. On presentation, blood pressure was 158/101. NIHSS was 2 for right upper extremity drift and decreased sensation. Neurologic exam was otherwise unremarkable. Thrombolytic therapy was not administered given presentation outside the time window. CT head showed scattered left centrum semiovale hypodensities without hemorrhage. MRI brain demonstrated multifocal areas of diffusion restriction in the left centrum semiovale, anterior frontal lobe, and parietal lobe. CT angiogram demonstrated a possible thrombus at the left carotid bifurcation which was redemonstrated on MR angiography of the neck. No atherosclerotic plaque, hemodynamically significant stenosis, nor other vascular abnormality were appreciated. Heparin drip was started. Hemoglobin was 6.9 for which 1 unit of packed red blood cells was transfused. White blood cell count was 9.02. Platelet count was 761. PT/INR and PTT were 13.4/1.2 and 30.1. HIV was negative, RPR was positive at 1:128 titer, and COVID PCR was positive. She did not experience respiratory symptoms. Urine drug screen was positive for cannabinoids. LDL was 66. Transthoracic echo demonstrated minimal right to left shunting, suggesting patent foramen ovale. Ejection fraction was 60–65% and chamber sizes were normal. Bilateral lower extremity ultrasound was negative for deep vein thrombosis as a potential embolic source given cardiac shunt. Given history of miscarriage and extensive family history of sickle cell trait, possible lupus anticoagulant, and thrombosis at young ages, hypercoagulable workup was sent. Sickle cell preparation and ANA were negative. ESR was 12. High sensitivity CRP was 2.71. Anticardiolipin IgM antibody was elevated significantly at 125.9 MPL and IgG antibody was indeterminately elevated at 19.2 GPL. Beta‐2 glycoprotein IgM antibodies were also elevated at 42 SMUnits. Atypical neurosyphilis was considered given positive titer, middle cerebral artery distribution, and young age, but deemed unlikely given embolic rather than inflammatory stroke pattern [5]. Ultimately, with a possible diagnosis of antiphospholipid syndrome, anticoagulation was transitioned to warfarin via enoxaparin bridge with plan for repeat hypercoagulable workup as outpatient. Conclusions COVID‐19 has been observed in association with hypercoagulable states such as deep venous thrombosis and stroke. Here we present a case of COVID‐19 associated ischemic stroke with concomitant antiphospholipid antibodies. This case highlights the potential for even asymptomatic/mild COVID‐19 to induce autoimmunity through modulation of host antibody activity. This may be more profound in those with a predisposition to thrombosis. Autoimmunity should be considered in cases of concurrent COVID‐19 and acute ischemic stroke without clear etiology.

The relationship between radiation dose and bevacizumab-related imaging abnormality in patients with brain tumors: A voxel-wise normal tissue complication probability (NTCP) analysis.

Bevacizumab-related imaging abnormality (BRIA), appearing as areas of restricted diffusion on magnetic resonance imaging (MRI) and representing atypical coagulative necrosis pathologically, has been observed in patients with brain tumors receiving radiotherapy and bevacizumab. We investigated the role of cumulative radiation dose in BRIA development in a voxel-wise analysis. Patients (n = 18) with BRIA were identified. All had high-grade gliomas or brain metastases treated with radiotherapy and bevacizumab. Areas of BRIA were segmented semi-automatically on diffusion-weighted MRI with apparent diffusion coefficient (ADC) images. To avoid confounding by possible tumor, hypoperfusion was confirmed with perfusion imaging. ADC images and radiation dose maps were co-registered to a high-resolution T1-weighted MRI and registration accuracy was verified. Voxel-wise normal tissue complication probability analyses were performed using a logistic model analyzing the relationship between cumulative voxel equivalent total dose in 2 Gy fractions (EQD2) and BRIA development at each voxel. Confidence intervals for regression model predictions were estimated with bootstrapping. Among 18 patients, 39 brain tumors were treated. Patients received a median of 4.5 cycles of bevacizumab and 1-4 radiation courses prior to BRIA appearance. Most (64%) treated tumors overlapped with areas of BRIA. The median proportion of each BRIA region of interest volume overlapping with tumor was 98%. We found a dose-dependent association between cumulative voxel EQD2 and the relative probability of BRIA (β0 = -5.1, β1 = 0.03 Gy-1, γ = 1.3). BRIA is likely a radiation dose-dependent phenomenon in patients with brain tumors receiving bevacizumab and radiotherapy. The combination of radiation effects and tumor microenvironmental factors in potentiating BRIA in this population should be further investigated.

Incidental Tc-99m MDP Uptake in Cortical-subcortical Parietotemporal Cerebral Area in a Patient with a History of Recent Ischemic Cerebrovascular Event who Underwent Whole-body Bone Scan.

The authors present Tc-99m methylene diphosphonate (MDP) uptake in the right parietotemporal area at whole-body bone scan (WBBS) in 75 years male patient with prostate adenocarcinoma Gleason score 3+4 (pT2N0Mx). No residual or metastatic disease was detected in the patient's Gallium-68 prostate-specific membrane antigen positron emission tomography/computed tomography four months before WBBS. The patient had undetectable prostate-specific antigen levels and underwent WBBS to restage prostate cancer due to equivocal findings in previous WBBS. Current WBBS planar views revealed heterogeneous Tc-99m MDP uptake in the right parietotemporal area and the sphenoid bone in addition to equivocal uptake on the lower lumbar vertebrae. Single-photon emission computed tomography study to identify the MDP-avid lesion on the right cranial area revealed heterogeneous Tc-99m MDP uptake in the right parietotemporal area and sphenoid bone. The patient had a history of transsphenoidal surgery for a hypophyseal tumor two years ago and a recent cerebrovascular event (CVE). Diffusion-weighted magnetic resonance imaging revealed a cortical-subcortical patchy area of restricted diffusion in the parietotemporal region compatible with acute ischemia. Heterogeneous Tc-99m MDP uptake in the right parietotemporal area was attributed to recent CVE and secondary vascular-tissue change-related dystrophic calcification.

Clinical Characteristics, Imaging Findings, and Outcomes of Cerebral Air Embolism.

Arterial cerebral air embolism (CAE) is an uncommon but potentially catastrophic event. Patients can present with focal neurologic deficits, seizures, or coma. They may be treated with hyperbaric oxygen therapy. We review the causes, radiographic and clinical characteristics, and outcomes of patients with CAE. We performed a retrospective chart review via an existing institutional database at Mayo Clinic to identify patients with arterial CAE. Demographic data, clinical characteristics, and diagnostic studies were extracted and classified on predefined criteria of diagnostic confidence, and descriptive and univariate analysis was completed. Fifteen patients met criteria for inclusion in our study. Most presented with focal deficits (80%) and/or coma (53%). Seven patients (47%) had seizures, including status epilepticus in one (7%). Five presented with increased muscle tone at the time of the event (33%). Computed tomography (CT) imaging was insensitive for the detection of CAE, only identifying free air in 4 of 13 who underwent this study. When obtained, magnetic resonance imaging typically showed multifocal areas of restricted diffusion. Six patients (40%) were treated with hyperbaric oxygen therapy. Age, Glasgow Coma Scale score at nadir, and use of hyperbaric oxygen therapy were not associated with functional outcome at 1year in our cohort. Twenty-six percent of patients had a modified Rankin scale score of 0 one year after the event, and functional improvement over time was common after discharge. A high index of clinical suspicion is needed to identify patients with CAE because of low sensitivity of free air on CT imaging and nonspecific clinical presentation. Acute alteration of consciousness, seizures, and focal signs occur frequently. Because improvement over time is possible even among patients with severe presentation, early prognostication should be approached with caution.

Preclinical model of anterior circulation intracranial stenting

BackgroundPreclinical testing of intracranial stents is currently performed in the peripheral circulation, and rarely in the basilar artery of the dog.ObjectiveTo test the feasibility of intracranial stenting in the middle...

PSUN248 Mitochondrial diabetes - a rare but important cause of diabetes

Abstract Background Mitochondrial Encephalopathy, Lactic Acidosis, and Stroke-like episodes (MELAS) syndrome is a rare mitochondrial disorder which presents with mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes. Mitochondrial diabetes is the cause in 1% of all diabetic patients. Here we present a case of a patient with insulin dependent diabetes mellitus (DM) who experienced sensorineural deafness and was diagnosed with MELAS. Case 33-year-old female presented with stroke like symptoms in December 2018. Medical history was significant for Diabetes Mellitus (DM), bilateral deafness and end stage renal disease requiring peritoneal dialysis with subsequent kidney transplant. On presentation, she had left sided weakness, lab work revealed lactic acidosis. MRI brain showed large areas of restricted diffusion in right hemisphere and cerebral edema. CSF analysis was positive for lactate and pyruvate. Left lower extremity muscle biopsy and mitochondrial genome analysis were positive for m.3243A&amp;gt;G in the MT-TL1 with 80% heteroplasmy, confirming the diagnosis of MELAS. Patient was started on Arginine, CoQ10, Levetiracetam, phenytoin, IVIG and her symptoms improved. DM was diagnosed in 2013 when patient was on peritoneal dialysis. Family history significant for type 2 DM in maternal uncle. Discussion Mitochondrial diseases are rare, maternally inherited, heterogeneous conditions caused by mutations in mitochondrial DNA and present with a variety of clinical symptoms. Mitochondrial diabetes results from the 3243 A&amp;gt;G gene mutation, responsible for both MELAS and Mitochondrial inherited diabetes and deafness (MIDD). MELAS presents with encephalopathy, stroke like symptoms and lactic acidosis (1). MIDD presents with nerve deafness, which usually occurs at an early age (2). In our patient, she presented with neurological symptoms suggesting MELAS and bilateral deafness suggesting MIDD. Her presentation is unusual and constitutes an overlap between both disorders. Conclusion Early recognition and diagnosis of mitochondrial diabetes is important. The diagnosis has important genetic implications for other family members. This case report highlights the presentation of MELAS and MIDD and importance of genetic studies in the confirmation of diagnosis 1) D. M. Sproule and P. Kaufmann, "Mitochondrial encephalopathy, lactic acidosis, and strokelike episodes: basic concepts, clinical phenotype, and therapeutic management of MELAS syndrome," Annals of the New York Academy of Sciences, vol. 1142, pp. 133–158, 20082) Murphy R, Turnbull DM, Walker M, Hattersley AT. Clinical features, diagnosis and management of maternally inherited diabetes and deafness (MIDD) associated with the 3243A&amp;gt;G mitochondrial point mutation. Diabetic Medicine 2008. Apr;25(4): 383-99. doi: 10.1111/j.1464-5491.2008.02359.x.Epub 2008 Feb 18. PMID: 18294221. Presentation: Sunday, June 12, 2022 12:30 p.m. - 2:30 p.m.

Persistent headache attributed to previous ischemic stroke: a prospective cohort study

Introduction Headache is a frequent sign of stroke, occurring both in the acute phase and persisting for 3 months after the stroke. The most recent International Classification of Headache Disorders has included persistent headache attributed to past stroke. Diagnosis depends on the presence of headache in the acute phase of stroke, although the headache persists for more than 3 months after the cerebrovascular event. Objectives To assess the incidence, course and impact of persistent headache attributed to a past ischemic stroke. To identify risk factors for the development of persistent headache attributed to a past stroke. Methods This was a prospective, observational cohort study. Inpatients with stroke, admitted within 72 hours of the onset of symptoms, were assessed at the Real Hospital Português de Beneficência de Pernambuco. Diagnosis of ischemic stroke occurred through the presence of an area of restricted diffusion in the magnetic resonance examination within a compatible clinical context. A semi-structured questionnaire was applied to characterize the sociodemographic information, the cerebrovascular disease and the headaches according to the diagnostic criteria of the International Classification of Headache Disorders. The NIHSS Stroke Scale and the HIT-6 scale were also used. Patients were assessed in person by the researchers in the acute phase of stroke and by telephone after 1 year to assess persistent headache attributed to past stroke. Results Initially, 221 patients were included, most of them male (59.3%) and whose mean age was 68.2 years ± 13.8. One hundred and nineteen patients (53.9%) answered the questionnaire by telephone 1 year after the stroke. Persistent headache attributed to a past stroke presented a frequency of 10.1% in the sample (95%CI: 5.3 to 17.0%) 1 year after the stroke. Headache presented a median frequency of 2.5 (1 – 4) days... (To see the complet abstract, please, check out the PDF.)

WITHDRAWN

SESSION TITLE: Critical Care of Immunocompromised Patients Case Posters SESSION TYPE: Case Report Posters PRESENTED ON: 10/19/2022 12:45 pm - 01:45 pm INTRODUCTION: Hyperpyrexia is defined as a core body temperature greater than 41.5 °C or 106.7°F. In modern times, hyperpyrexia is most prevalent in central nervous system hemorrhages. It can be seen in severe bacterial infections, though uncommon in modern times. Other more common causes of severe hyperthermia can include heat stroke and drug toxicity. This case presents a much less obvious cause of hyperpyrexia. CASE PRESENTATION: 27-year-old previously healthy female presents dysarthria and aphasia. Vitals revealed a temperature of 106.8 °F. Patient was admitted to ICU. Initial laboratory evaluation revealed renal failure, liver failure, rhabdomyolysis, troponin leak and hemolytic anemia. Nephrology started dialysis. MRI brain showed focal area of restricted diffusion involving the splenium of the corpus callosum. A CT chest/abdomen/pelvis showed no acute abnormalities. The patient's GCS deteriorated and was intubated. Rheumatology and hematology were consulted. High dose steroids were started. Due to high suspicion for an autoimmune process, the patient underwent plasmapheresis. Work-up revealed multiple positive autoimmune serologies. Extensive infectious workup was negative. the patient was extubated and kidneys began to recover. Renal biopsy revealed active focal and membranous lupus nephritis, ISN/RPS III + V. A diagnosis of severe lupus flare was made. Mycophenolate was initiated at this time. Steroids were tapered. The fever decreased but never resolved. She was then transferred to a tertiary center for biologics. DISCUSSION: Systemic Lupus Erythematosus is a relatively common autoimmune disease that involves multiple organ systems. Fever is a common manifestation of SLE and can occur in 36–86% of patients (1). According to the 2019 EULAR/ACR criteria, Fever is a low weighted diagnostic criterion for SLE. However, lupus flare fever is usually low-grade, rarely exceeding 102 F (2). The patient was found to be negative for infection, drug toxicity, heat stroke, intracranial bleed, hypothalamic infarct, or large vessel occlusion. It was therefore postulated that the biopsy proven acute SLE flare caused the hyperpyrexia. However, it is unclear if the entirety of the poly organ failure was caused by autoimmune reaction with organs themselves or by the hyperpyrexia. Only the Lupus Nephritis was confirmed by biopsy. Temperature of 38.5°C in non-infected patients result in increased mortality and adverse outcomes. (3). In this case, we presume that most of the patient's poly-organ dysfunction was either caused or perpetuated by hyperpyrexia. The full extent that the fever contributed to any specific organ dysfunction cannot be concretely demonstrated. CONCLUSIONS: This case demonstrates that in patients with hyperpyrexia or severe hyperthermia, autoimmune etiology should not be overlooked. This case presents a previously healthy, young female with hyperpyrexia caused solely by severe lupus flare. Reference #1: 1. Falagas ME, Manta KG, Betsi GI, Pappas G. (2007). Infection-related morbidity and mortality in patients with connective tissue diseases: a systematic review. Clin Rheumatol, 26: 663– 670. Reference #2: 2. Bernatsky S, Boivin JF, Joseph L, et al. (2006). Mortality in systemic lupus erythematosus. Arthritis Rheum, 54: 2550– 2557. Reference #3: 3. Edward Walter, Oliver R. Gibson. (2020). The efficacy of steroids in reducing morbidity and mortality from extreme hyperthermia and heatstroke—A systematic review. Pharmacol Res Perspect. 2020; 8:e00626 DISCLOSURES: No relevant relationships by Syed Akbarullah No relevant relationships by Sara Alleyasin No relevant relationships by Kory Cummings No relevant relationships by LLOYD Del Mundo No relevant relationships by Gerard DiChiara No relevant relationships by Jerome Hruska No relevant relationships by Amber McDonald No relevant relationships by Xenia Schneider No relevant relationships by Paul Stewart