West Nile Meningoencephalitis in a Patient with Leiomyosarcoma Presenting with Neutropenic Fever

Case Report: West Nile Virus (WNV) was first isolated from the West Nile district of Northern Uganda in 1937, but was first detected in the United States well over half a century later in 1999. The arthropod-borne virus has since persisted, with 2,401 cases reported to the CDC on average annually. The infection typically causes a nonspecific acute systemic febrile illness with occasional gastrointestinal and skin manifestations;however, in less than 1% of infected patients, it can cause severe and potentially fatal neuroinvasive disease, presenting as meningitis, encephalitis or acute flaccid paralysis. Immunosuppression is one of the risk factors associated with the development of neuroinvasive disease, and chemotherapy thus places patients at risk. Uterine leiomyosarcoma is a rare gynecological malignancy. Palliative chemotherapy is common in late stage disease, but may predispose patients to conditions that present as neutropenic fever, leading to a diagnostic conundrum. This is the first case report where patient with neutropenic fever was found to have West Nile neuroinvasive disease, so it is important to include West Nile disease in the differential diagnosis. Case Description: This is a case of a 45-year-old female with history of diabetes, hypothyroidism and recently diagnosed uterine leiomyosarcoma status post tumor debulking with metastasis on palliative chemotherapy with gemcitabine that presented to the Emergency Room for a fever of 103.8 degrees Fahrenheit. Given the history of advanced leiomyosarcoma, the patient was admitted for neutropenic fever with an absolute neutrophil count of 1000. During the hospitalization, the patient became acutely altered and confused. CT head without contrast and lumbar puncture were performed. Due to clinical suspicion of meningitis, she was started on broad spectrum antibiotics. Lumbar puncture revealed leukocytosis of 168 with lymphocytic predominance and elevated protein level in the cerebrospinal fluid, therefore acyclovir was started due to high suspicion of viral meningoencephalitis. An EEG showed severe diffuse encephalopathy as the patient was persistently altered. A broad workup of infectious etiology was considered including HIV, syphilis, hepatitis A, B, C, COVID-19, adenovirus, pertussis, influenza, WNV, HHV6, coccidiomycosis, aspergillus, and tuberculosis. Patient was ultimately found to have elevated IgM and IgG titers for West Nile Virus. Discussion(s): It is important to consider a broad spectrum of diagnosis in patients with metastatic carcinoma presenting with new-onset fever and acute encephalopathy. This includes working up for other causes of altered mental status including cardiac, neurologic, psychiatric, endocrine, metabolic, electrolyte, drug, and infectious etiology. While uncommon in the healthy population, WNV encephalitis should be on the radar for any patient who is immunocompromised or on immunosuppressive therapy, especially those who present with a neutropenic fever.

POSTINFECTIOUS COVID-19 ENCEPHALITIS RESPONSIVE TO PULSE-DOSE STEROIDS AND IV IMMUNOGLOBULINS

SESSION TITLE: Post-COVID-19 Infection Complications SESSION TYPE: Case Report Posters PRESENTED ON: 10/17/2022 12:15 pm - 01:15 pm INTRODUCTION: Severe Acute Respiratory Syndrome-Coronavirus-2 (SARS-CoV-2) is the causative agent of coronavirus disease-2019 (COVID-19). Post-infectious encephalitis secondary to SARS-CoV-2 may present with delirium, seizures, or transient comatose state. The mechanism of encephalitis in patients with COVID-19 is multifactorial. Cytokine release syndrome, a systemic hyperinflammatory condition, might have an integral part in the pathophysiology of this manifestation. Beneficial effects of pulse dose glucocorticoid therapy, with and without plasma exchange or IVIG, have been described. (1, 2) In this case report, we disclose a case of a young healthy male that presented with acute encephalopathy after 10 days of contracting SARS-CoV-2 and aim to discuss the potential role of IVIG and pulse dose steroid. CASE PRESENTATION: A 37-year-old previously healthy Caucasian man initially presented to urgent care with fatigue and generalized weakness and was diagnosed with acute COVID-19 infection through positive PCR. Four days later, he developed shortness of breath, syncope and vomiting. He was taken to the ER, where he had a witnessed seizure complicated by status epilepticus requiring endotracheal intubation for airway protection. He was then airlifted to our University Hospital. Upon arrival, labs were notable for elevated troponin, leukocytosis, and mildly elevated liver enzymes. An echocardiogram revealed stress induced (Takotsubo) cardiomyopathy. CT head was normal and continuous EEG showed focal electrographic seizures of left temporal onset. MRI of brain with/without contrast showed subtle areas of cortical diffusion hyperintensity involving left cerebral hemisphere including left posterior temporal lobe, lateral occipital lobe, posterior lateral frontal lobe and posterior lateral parietal lobe with subtle patchy areas of cortical enhancement on postcontrast T1-weighted images. CSF analysis was benign and CSF PCR for SARS-CoV-2 was negative. One gram daily IV methylprednisolone and IVIG therapy was given for total 5 days. On Day 2 of therapy, seizures subsided, and patient was successfully extubated after. Repeat MRI brain with/without contrast done after day of therapy showed improvement in previously demonstrated findings. He improved clinically and was discharged home on hospitalization day. DISCUSSION: Post-infectious COVID-19 encephalitis falls under the spectrum of disease described under neurological syndromes related to SARS-CoV-2 infection.(3) Diagnosis is based on Clinical presentation, positive COVID PCR on nasopharyngeal swab and Imaging demonstrating cortical enhancement on post contrast T1-weighted imaging. Out of various treatment options described in literature (1,2), our patient responded well to pulse dose steroids and IVIG therapy for 5 days. CONCLUSIONS: Careful selection of patients and therapies should be considered when post-infectious COVID-19 encephalitis is suspected. Reference #1: Cao A, Rohaut B, Le Guennec L, et al. Severe COVID-19-related encephalitis can respond to immunotherapy. Brain. 2020;143(12):e102. doi:10.1093/brain/awaa337 Reference #2: Pugin D, Vargas MI, Thieffry C, et al. COVID-19-related encephalopathy responsive to high-dose glucocorticoids. Neurology. 2020;95(12):543-546. doi:10.1212/WNL.0000000000010354 Reference #3: Al-Ramadan A, Rabab'h O, Shah J, Gharaibeh A. Acute and Post-Acute Neurological Complications of COVID-19. Neurol Int. 2021;13(1):102-119. Published 2021 Mar 9. doi:10.3390/neurolint13010010 DISCLOSURES: No relevant relationships by Ali Ahmad No relevant relationships by Varun Halani No relevant relationships by Michael Lasky No relevant relationships by Posan Limbu

A Peculiar Case of an Acute Increase in Sodium From 152 mmol/L to 192 mmol/L

We present a case in which a long-term hospital patient had a serum sodium level that increased from 152mmol/L to 192 mmol/L in a time frame less than 12 hours. Inspite of aggressive treatment, he succumbed to his illness. Guidlines for hypernatremia treatment need to be updated 75 yo male with PMH of malnutrition, CKD Stage 4, chronic hypoxia and hypertension was brought to the ED for hypertensive emergency. He had a complicated hospital course which included COVID contraction and diarrhea. Nephrology was consulted for chronic hypernatremia with a sodium of 152, BUN of 100 and creatinine of 4.50 along with acute encephalopathy. Hemodialysis was recommended. Repeat labs later that evening showed a sodium of 192. He was started on HD with the highest sodium bath available to the institution at 148 mmol/L. Pt’s sodium 18 hours after HD & IV fluids was 172 mmol/L . He later developed hypotension requiring vasopressors. Due to family wishes;patient was transitioned to comfort care with the last noted sodium of 168mmol/L. The brain adapts to hypernatremia. There were several factors in this patient that would have prevented adequate adaptation, namely hypoxia, malnutrition and hypotension. Hence a decision was made to rapidly lower serum sodium. There are many reports where chronic hypernatremia has been rapidly corrected by dialysis without development of cerebral edema. In our case, there was a dilemma whether to treat this as acute or chronic hypernatremia. The guideline for correction of chronic hyponatremia is based on pediatric patients. Many however, do not agree with this recommendation as there are reports where the sodium has been rapidly lowered without complications. Further studies are needed in adults to ascertain the correct correction rate. (Table Presented) (Figure Presented)

Severe Tracheal Stenosis in a Patient with History of Prolonged Intubation

Post-intubation tracheal stenosis (PiTS) is a complication that occurs in 10-22% of patients after prolonged intubation or tracheostomy. Predisposing factors include localized infection, soft tissue trauma, granulation at the site of stoma, hypotension, tracheal cartilage damage, and metabolic disorders. Despite advances in the design of endotracheal tubes and cuffs, PiTS continues to remain an undesired consequence that warrants increased attention, especially in the COVID pandemic, which has led to increased number of tracheostomies and intubations. Consequently, prompt diagnosis and management is imperative to improve patient survival and quality of life. Herein, we present a 52-year-old male with history of tracheostomy in the setting of prolonged intubation from intraparenchymal hemorrhage with subsequent decannulation who was admitted for the management of pulmonary embolism. His course was complicated by encephalopathy and acute hypercapnic respiratory failure requiring intubation. In the intensive care unit, the patient was extubated after improvement in his respiratory and mental status, however developed shortness of breath and the feeling of impending doom a few hours after extubation. Chest radiograph was obtained and suggested tracheal stenosis (Image-1A). Given this finding, follow-up urgent chest computed tomography demonstrated severe lateral collapse of the subglottic space with critical tracheal stenosis (Image-1B). There was >90% cross sectional area reduction at the site of greatest stenosis (Image-1C). The measured area at the site of greatest stenosis was noted at 0.32 cm2 (average tracheal area is 2.8-3.4 cm2). His tracheal stenosis was suspected to be secondary to his history of tracheostomy. Given these findings, patient was reintubated for airway protection and urgently underwent rigid bronchoscopy. Initially, argon plasma coagulation was used in between stenotic areas, however there was minimal improvement in the airway, requiring a silicone 18 x 14 x 14 cm Y-stent to be placed. Since stent placement, patient has required frequent bronchoscopies to assist with mucus and secretion clearing. The remainder of his hospital course was benign. PiTS is an infrequent yet important complication that clinicians must consider in individuals with a history of prolonged intubation who have sudden difficulty breathing postextubation. Prompt recognition is required to manage these patients effectively. Factors considered in the management include location, length, and the severity of the stenosis. Therefore, if 1) critical stenosis is suspected, 2) the facility is capable of resection and reconstruction, and 3) the patient has minimal comorbidities, surgery should be considered. Otherwise, bronchoscopy with stent placement can be performed.

Severe Multifocal Cortical Encephalopathy Following COVID-19 Vaccination: A Case Report

Objective: This clinical case history details a woman who received the COVID-19 vaccine BNT162b2 and developed an acute, severe encephalopathy associated with new onset seizures within days of her first vaccination. Background: BNT162b2, an mRNA-LNP-vaccine, has been strategically purified and modified to suppress immunogenicity. It still possesses powerful intrinsic immune-stimulatory features that induce type-I INF production, which has been associated with both inflammation and potentially autoimmunity in several mRNA-vaccine phase-I/II clinical trials (HIVNCT02413645, influenza-NCT03076385, rabies-NCT02241135 and Zika virus-NCT03014089). Design/Methods: A 69-year-old woman who received her first dose of BNT162b2 five days prior, was found unresponsive. Within twenty-four hours of arrival, she developed focal seizures with secondary generalized, and remained in non-convulsive status epilepticus on longterm EEG monitoring until day 5 of admission despite three anti-seizure medications and a propofol drip. Brain MRI on day 3 of admission showed gyriform-pattern diffusion restriction in the right hemisphere and left frontoparietal region without features suggestive of an acute vascular event. Vascular imaging was normal. Cerebrospinal fluid examination revealed an elevated protein level (135 mg/dl), with negative findings for infections from bacteria, fungi, mycobacteria, HIV, syphilis, and viruses, including COVID-19 PCR, as well as paraneoplastic and autoimmune encephalitis panels. Serum COVID-19 IgG antibody was negative on post-immunization day 8. Results: There was no significant improvement following empiric methylprednisolone and meningitis antibiotic/antiviral treatment. She was discharged in a deeply comatose status on day 30 of hospital admission. Repeat MRI brain on day 14 showed similar findings to her previous scan with the addition of Wallerian degeneration in the right cerebral peduncle. Conclusions: In a patient who develops an acute encephalopathy with new onset of seizure within days of BNT162b2 vaccine, do consider mRNA-vaccine related encephalopathy in the differential diagnosis.

Clinically mild encephalitis/encephalopathy with a reversible splenial lesion of the corpus callosum in childhood: a single-center experience

Background/aim: Mild encephalitis/encephalopathy with a reversible splenial lesion (MERS) is a rare clinicoradiological syndrome that typically presents with central nervous system symptoms such as loss of consciousness, seizure, headache, and ophthalmoparesis. Materials and methods: Here, we highlight the characteristics of this syndrome together with the clinical and MRI findings of 6 pediatric patients with MERS. Results: Between January 2017 and October 2020, 6 patients with MERS (3 boys and 3 girls) presented to our center. The mean age was 122 ± 54.6 (min-max: 44-180) months. None of the patients had a chronic disease. In our study, infectious agents were detected in 4 patients (66.6%), while noninfectious causes (one seizure and the other hyponatremia) were detected in two patients. All of our cases were discharged without any sequelae after an average of 12.1 ± 7 (min–max: 4–20) days of hospitalization. In 1 patient (case 6), control MRI could not be performed, and the radiological recovery of our other patients was shown to be between 14 days and 2 months. Conclusion: MERS is an acute encephalopathy with good prognosis and should be considered by neurologists in differential diagnosis due to its variable clinical presentation and specific MRI findings.

A case of acute encephalopathy with biphasic seizures and delayed diffusion changes

Acute encephalopathy with biphasic seizures and delayed diffusion changes (AESD) is an extremely rare syndrome outside Asia. AESD is diagnosed based on clinic-radiological correlation. Influenza or human herpesvirus 6 infection have been most commonly isolated in previously healthy children with AESD and there is possible correlation with genetic abnormalities such as mutations in the SCN1A gene. We report a 2 year old previously fit and well white female who presented febrile status epilepticus at day 3 of an upper respiratory tract infection with subsequent prolonged left facial palsy and left hemiparesis. Brain MRI on day 4 showed swelling and diffusion restriction of the right hippocampus, without any other focal abnormality or signs of ischaemia. On day 6 she presented clusters of focal left sided seizures, non-responding to treatment Levetiracetam and sodium valproate add on. Repeated brain MRI on 7 showed prolonged and diffuse diffusion restriction over the whole right hemisphere, which along with the characteristic clinical presentation led to the diagnosis of AESD. She received high dose IV steroids with taper over 6 weeks starting on day 9. The seizures stopped on day 10 but motor function recovery was slow. CSF analysis showed no signs of infection. Nasopharyngeal swab was positive for Bocavirus and she had positive antibodies for SARS-COV-2 with negative PCR. Genetic investigations were requested and are pending. This case illustrates the importance of repeated brain imaging in abrupt onset refractory seizures with abnormal neurological examination which can lead to early diagnosis and treatment. More studies are needed to investigate the correlation between SARs-COV-2 or Bocavirus and AESD.

GCS-neuro-COVID: Neurologic manifestations and outcomes in children with and without comorbidities

INTRODUCTION: Children with comorbidities are at increased risk of severe disease due to SARS-CoV-2 (COVID-19) infection and Multisystem Inflammatory Syndrome-Children (MIS-C). We hypothesized that children with comorbidities hospitalized with COVID-19 or MIS-C will experience more neurologic manifestations and worse outcomes compared to children without comorbidities. METHODS: Secondary study of the Global Consortium Study of Neurological Dysfunction in COVID-19 (GCS-NeuroCOVID) study, a multinational study enrolling children < 18 years of age hospitalized with confirmed/presumed COVID-19 or MIS-C. Neurological manifestations, lengths of hospital and intensive care unit (ICU) stay, hospital disposition and mortality were analyzed by comorbidity status. A multivariable logistic regression was performed to analyze the association of comorbidity with neurologic manifestation. RESULTS: Overall, 824 (55%) children had any comorbidity and 646 (43%) had any neurologic manifestation. Children with comorbidity were older (median [interquartile range] 9.5 [4-15] vs. 6.4 [0.5-12], had more COVID-19 (58%) vs. MIS-C (32%) and ICU admissions (39% vs. 29%), and longer hospital length of stay (9 [2-9] vs. 5 [2-6] days), all p< .001;mortality was similar (1.5% vs. 0.5%, p=.067). The most common comorbidities were neurologic and respiratory (20% each). Children with comorbidity more frequently had any neurologic manifestation (61% vs. 39%), and seizures/status epilepticus (11% vs. 4%), p< .001). There were no differences between comorbidity vs no comorbidity groups for the most common neurologic manifestations including headache (21% vs. 20%) and acute encephalopathy (17% vs. 15%). Older age (odds ratio 1.1 [95% confidence interval 1.1-1.1]), ICU stay (2.6 [1.9-3.4]), MIS-C (2.2 [1.5-3.2]), and neurologic (2.8 [1.9-4.1]) comorbidity were associated with neurologic manifestation while cardiovascular morbidity was protective for neurologic manifestation (.5 [.3-.8]), all p< .05. CONCLUSIONS: Children with comorbidity, especially neurologic, who are hospitalized with COVID-19 related conditions are at increased risk of neurologic manifestations. Assessment of post-hospital neurodevelopmental outcomes to determine the impact of neurologic manifestations in children with comorbidity and COVID-19 related conditions is critically needed.