Glial fibrillary acidic protein astrocytopathy in a patient with recent mRNA SARS-CoV-2 vaccination: COVERSHEET

Background: Literature describing triggers of GFAP astrocytopathy (GFAP-A) is limited. We report a case of GFAP-A in a patient with recent messenger ribonucleic acid (mRNA) severe acute respiratory syndrome corona virus 2 (SARS-CoV-2) vaccination and discuss the possible pathogenesis. Case description: A 45-year-old gentleman presented with features of meningoencephalitis 31 days after the first dose and 4 days after the second dose of mRNA SARS-CoV-2 vaccination. He sequentially developed brainstem/cerebellar, autonomic and cord dysfunction. Cerebrospinal fluid was positive for GFAP autoantibody. Clinical improvement occurred after intravenous methylprednisolone and immunoglobulins. Conclusion(s): Although we are uncertain of a causal link of GFAP-A to mRNA vaccine, indirect activation of an underlying dysregulated immune milieu is plausible.Copyright © 2021 The Author(s)

An unusually fulminant case of encephalomyelitis in an 80 year old

Background: There have been reports of demyelinating syndromes in association with COVID-19 and to a much lesser extent COVID 19 vaccines. The association between demyelination and vaccines, in general, remains controversial. We review a presentation of fulminant demyelination, and discuss antecedent COVID-19 vaccination, the formulation of a broader differential diagnosis and ultimately the pathologic diagnosis. Case presentation: An 80-year-old woman presented with seizure, encephalopathy, quadriparesis and ultimately expired. She received a SARS-CoV-2 vaccine one day prior. Imaging revealed contrast enhancing cerebral lesions, longitudinally extensive transverse myelitis. CSF was markedly inflammatory. Pathologic examination of the CNS lesions revealed demyelination and inflammation beyond white matter, not restricted to a perivenular distribution. Conclusion(s): This case depicts a seemingly fulminant course of a diffuse demyelinating syndrome characterized clinicopathologically as Marburg's variant of multiple sclerosis. There are several unique aspects of this case including the extremely rapid course, the unusual evolution of CSF abnormalities, with hypoglycorrhachia and markedly elevated protein. The proximity to vaccination is a pertinent association to document, though we cannot unequivocally prove causation.Copyright © 2022 The Authors

Maternal COVID-19 Infection: A Cause of Neonatal Stroke?

Perinatal arterial ischemic stroke (PAIS) is a rare cause of neonatal seizures, with an incidence of 1 in 2500 to 4000 live births, globally. This is a case of a neonate with PAIS due to transpla-cental passage of COVID-19 IgG antibodies from the mother. A term, male neonate, born to a primigravida with an unevent-ful antenatal history was presented on the second day of life with multiple episodes of focal clonic seizures involving the right upper and lower limbs. Magnetic resonance imaging revealed an acute infarct in the left frontal lobe, extending into the parietal region, anterior limb, and genu of internal capsule suggestive of arterial ischemic stroke. The known causes of PAIS were evaluated and ruled out. The result of reverse transcription polymerase chain reaction analysis for SARS-CoV-2 antigen was negative for both the mother and the neonate. COVID-19 IgG antibodies in the mother and neonate were elevated. Seizures were controlled with antiepileptics. The neonate had no further seizure episodes and was discharged on oral levetiracetam. The infant was developmentally and neurologically normal at 3 months of age. PAIS is a rare cause of neonatal seizures, and maternal COVID-19 infection may be associated with neonatal stroke.Copyright © 2022, Himalaya Wellness Company. All rights reserved.

Status epilepticus 2020. / Status epilepticus 2020

Status epilepticus is the second most common neurological emergency with 15‒25% mortality rate. The principle of "time is brain" is also true for the treatment of status epilepticus: the earlier we start an adequate treatment, the more likely we are to stop progression. With treatment protocols based on high-level evidence, the progression of status epilepticus can be prevented in 75­90% of cases: we can avoid the induced coma or death. At the beginning of status epilepticus, parenteral benzodiazepine should be given immediately: intramuscular midazolam (0.2 mg/kg, max. 10 mg). In the case of easy veinous access, benzodiazepines can also be given intravenously. If the first benzodiazepine bolus does not stop the status epilepticus, we speak about established (benzodiazepine refractory) status epilepticus. In this case, a fast-acting non-benzodiazepine antiepileptic drug should be given: intravenous valproate (40 mg/kg, max. 3000 mg, within 10 minutes) or levetiracetam (60 mg/kg, max. 4500 mg, within 10 minutes). Refractory status epilepticus that persists for more than 1 hour and does not respond to either benzodiazepines or antiepileptics should be treated with general anesthesia (full narcosis). Induced coma can be achieved with fast-acting anesthetics, a combination of propofol with midazolam is the most frequently used one. Orv Hetil. 2020; 161(42): 1779­1786.

A case report of multiple sclerosis after COVID-19 infection: causality or coincidence?

Introduction: Since the declaration of COVID-19 pandemic, several cases of demyelination of both peripheral and central nervous systems have been reported. The association of viral infection and the development of CNS demyelination has long been studied, and this link has recently been reported following SARS-CoV-2 infection as well. Case report: We report a case of a 36-year-old male who developed CNS demyelinating disease, that fulfilled the diagnostic criteria of multiple sclerosis (MS), 2 months after laboratory-confirmed infection with SARS-CoV-2. Conclusion(s): To our knowledge, this is the second published case report of MS in association with COVID-19 infection, and the first case from Middle East and North Africa (MENA) region, adding to the growing literature of a probable causal relationship between SARS-CoV-2 infection and the development of MS.Copyright © 2021 The Author(s)

Shortages of antiseizure medications in Australia and the association with patient switching, and adherence in a community setting.

PURPOSE: To quantify sponsor-reported shortages of oral antiseizure medications in Australia, estimate the number of patients impacted, and the association between shortages and brand or formulation switching, and changes in adherence. METHODS: A retrospective cohort study of sponsor-reported shortages (defined as where the supply of a medicine will not or will not be likely to meet the demand over a 6-month period) of antiseizure medications reported to the Medicine Shortages Reports Database (Therapeutic Goods Administration, Australia); cross-referencing shortages to the IQVIA-NostraData Dispensing Data (LRx) database, a deidentified, population-level dataset collecting longitudinal dispensation data on individual patients from ∼75% of Australian community pharmacy scripts. RESULTS: Ninety-seven sponsor-reported ASM shortages were identified between 2019 and 2020; of those, 90 (93%) were shortages of generic ASM brands. Of 1,247,787 patients dispensed ≥1 ASMs, 242,947 (19.5%) were impacted by shortages. Sponsor-reported shortages occurred more frequently before the COVID-19 pandemic versus during the pandemic, however, shortages were estimated to affect more patients during the pandemic than before the pandemic. An estimated 330,872 patient-level shortage events were observed, and 98.5% were associated with shortages of generic ASM brands. Shortages occurred at a rate of 41.06 shortages per 100 person-years in patients on generic ASM brands versus 0.83 shortages per 100 person-years in patients on originator ASM brands. In patients taking a formulation of levetiracetam affected by a shortage, 67.6% switched to a different levetiracetam brand or formulation during shortages compared with 46.6% in non-shortage periods. CONCLUSIONS: Approximately 20% of patients on ASMs were estimated to have been impacted by an ASM shortage in Australia. The rate of patient-level shortages was approximately 50 times higher for patients on generic ASM brands versus originator brands. Shortages of levetiracetam were associated with formulation and brand switching. Improved supply chain management amongst sponsors of generic ASMs is needed to maintain the continuity of supply in Australia.

Pharmacological management of seizures in patients with COVID-19: a systematic review.

Background: Some patients with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) have been reported to exhibit neurological symptoms such as seizures and impaired consciousness. Our study reviews reported cases to assess the pharmacological approach to managing seizures in SARS-CoV-2 patients and associated outcomes. Methods: A systematic review of case reports on the incidence of seizures following coronavirus disease 2019 (COVID-19) among patients that reported use of antiepileptic drugs (AEDs) in management was performed by using the PRISMA (preferred reporting items for systematic reviews and meta-analysis) guidelines. Databases used included EMBASE, PubMed, SCOPUS, and Google Scholar. Data was presented as qualitative and descriptive data. Results: In total, 67 articles were selected for full-text assessment, of which 19 were included in the final review. Patients had a median age of 54 years, most of whom were male. Remdisivir, dexamethasone, Laminavir, hydroxychloroquine, azithromycin, and Lopinavir-ritonavir were common agents used in the management of COVID-19. Most patients presented with either generalized tonic-clonic seizures or status epilepticus. Most patients received levetiracetam as drug choice or as part of their regimen. Other AEDs commonly prescribed included midazolam and sodium valproate. Some patients received no antiepileptic drug therapy. Most of the patients who died had more than one comorbidity. Also, most of the patients who died received COVID-19 treatment drugs. None of the patients who received midazolam as drug choice or as part of their regimen developed recurrent seizures in contrast to patients who received levetiracetam and sodium valproate as drug choice or as part of their regimen. Interestingly, none of the patients who received no AEDs suffered recurrent seizures or died. Conclusions: Standard guidelines for managing seizures in COVID-19 patients may be required. A limitation of this review is that it involved the use of case reports with no controls and a small number of patients.

Haematological Malignancy Presenting in an Unusual Manner

Chronic lymphocytic leukaemia is a haematological malignancy that occurs due to an increased proliferation of mature B lymphocytes. It is considered to be the most common leukaemia in adults. Hyponatremia is commonly seen in such patients. This case report is about a 75-year-old male, who presented with giddiness, followed by altered sensorium. However, the patient had no motor weakness or sensory loss. Initially, a diagnosis of posterior circulation stroke was made but Magnetic Resonance Imaging (MRI) brain did not show associated signs. The routine investigations showed highly elevated total leukocyte count and hyponatremia. The patient was worked up for malignancy and diagnosed with Chronic lymphocytic leukaemia. Oncology reference was taken and treated with tablet Ibrutinib. On discharge, the patient's mentation improved, and he is on regular follow-up.

A Case of Autosarcophagy in a Pediatric Patient With Depression

BACKGROUND: Self-mutilating behavior in the pediatric population is associated with psychiatric and psychosocial factors. Autosarcophagy, or self-cannibalism, is an extremely rare form of self-mutilation and is predominantly seen with psychosis or substance use.1 We report a case of oral autosarcophagy in a pediatric patient in the absence of substance use or psychosis. OBJECTIVE: To learn about autosarcophagy and its treatment in the pediatric population and to explore other neuropsychiatric disorders in which it is a predominant manifestation. METHODS: Review of a case using electronic medical records and relevant literature. Key terms: 'autosarcophagy,' 'body focused repetitive behavior,' 'oral self injury,' 'pediatric self-mutilation' using Medscape and Google Scholar. RESULTS: We present a 14-year-old female with history of seizure disorder in full remission, depression, self-cutting behavior, and suicidal ideation with 2 psychiatric hospitalizations, who presented to the pediatric emergency department with oral bleeding after eating one-third of her tongue over the course of a month. Evaluation was notable for poverty of speech and constricted affect. Patient stated she was 'trying to remove an infection' and alleviate discomfort. She denied that this behavior was an attempt to end her life but endorsed past suicidal ideations and cutting behavior. History revealed emergency room evaluation for aggressive behavior and episodes of volitional enuresis. We diagnosed major depressive disorder, recurrent episode in remission without psychosis. Drug screen, complete blood count, complete metabolic panel, COVID-19, urinalysis, thyroid-stimulating hormone, head computed tomography, and beta-human chorionic gonadotropin were negative. Patient continued home oral medications aripiprazole 10 mg daily, fluoxetine 30 mg daily, and levetiracetam 500 mg twice daily and was discharged the next day. CONCLUSIONS: Self-harm is observed in 17.2% of adolescents, 13.4% of young adults, and 5.5% of older adults.2 Cases of self-mutilation in pediatric patients typically present as cutting, burning, or head banging.3 Our differential diagnoses include borderline personality disorder due to repeated impulsivity and self-harm, and body focused repetitive behavior disorder (obsessive-compulsive disorder-related disorder), which presents with repetitive strain injuries and dental malocclusions. Treatment of self-mutilation involves treating the underlying psychiatric condition with psychotropic medications.4,5 In pediatric patients, dialectical behavioral therapy has been shown to reduce parasuicidal behaviors after 1 year of therapy.6 Our patient, under constant 24-hour observation, was cleared by medical, psychiatric, and dental teams. The patient followed up with outpatient psychotherapy and psychiatry. We are presenting this rare case for clinicians to identify and manage pediatric patients presenting with unique forms of self-harm tendencies.

POSTERIOR REVERSIBLE ENCEPHALOPATHY SYNDROME in A PATIENT with SYSTEMIC LUPUS ERYTHEMATOSUS after COVID-19 VACCINATION: A CASE REPORT

Background: The COVID-19 pandemic has infected millions of people around the world and there has been a new surge of virulent strains in many parts of the world[2]. Patients with Systemic Lupus Erythematosus (SLE) were reported to be at higher risk of SARS-CoV-2 infection and worse outcomes from COVID-19, possibly due to their intrinsic immune dysfunction, demographics, disease activity, medications, associated organ damage, comorbidities and as such, have been among the frst to receive the vaccines [3]. The most common reason for vaccine refusal in patients with SLE is fear of SLE disease fare. Additionally, SARS-CoV-2 mRNA vaccines could potentially induce interferon production, associated with increased SLE disease activity[1]. Objectives: we report a case of SLE presented with lupus fare after receiving the 1st dose of phizer vaccine. Methods: A 30-year-old female patient, kown case of SLE since 2011 well controlled on low dose steroids, hydroxychloroquine and azathioprine. Upon receiving her 1st shot of Pfzer-BioNTech COVID-19 Vaccine, she developed high grade fever associated with generalized tender papulovesicular skin eruption mainly on the back of the trunk and the outer surface of both thighs, then she developed generalized tonic-clonic convulsions and transferred for Intensive Care Unit (ICU), intubated, mechanically ventilated and received intravenous anti-epileptic medications. During her admission, Cerebrospinal fuid (CSF) examination and Magnetic Resonance Imaging (MRI) brain were done.she regained her consciousness, extubated after 48 hours. Results: The initial laboratory invwstigations revealed COVID19-PCR: nega-tive,ESR: 35 mm/hr,CRP: 78,C3: 70 mg/dL (90-180) and C4: 8 mg/dL (10-40). CSF examination revealed proteins: 116.9 mg/dL (15-45),glucose: 46.3 mg/dL (50-60% of serum),LDH: 49.1 U/L (10% of serum) and no cells.Emergency MRI brain was performed revealed multiple bilateral symmetrical mainly cortical and subcortical abnormal signal with cortical swelling are seen mainly involving both occipito-temporo-parietal lobes with patchy enhancement of left cerebellar hemisphere, cerebellar vermis, both thalami, medulla and pons,Picture suggestive of Posterior Reversible Encephalopathy Syndrome (PRES).Accordingly the patient received received pulse steroid therapy for 3 days under cover of oral acyclo-vir.She also received levetiracetam and Oxcarbazepine.the condition markedly improved and discharged from the hospital for follow up after one month. Conclusion: 1)The mRNA COVID Vaccine may rarely cause CNS affection, or even SLE fare so, SLE patients must be well controlled before giving the Vaccine. 2) SLE patients must be monitored closely by clinical examination and laboratory investigations after taking mRNA COVID Vaccine.

Dysgeusia and Appetite Loss: Thinking Beyond MDD and COVID-19

Background: Dysgeusia is a distortion of taste sensation. Etiologies can include medications and Covid-19, among others. Dysgeusia may lead to appetite loss which is nonspecific and can have multiple causes, including major depressive disorder (MDD) (Coulter, 1988). Although post-marketing data revealed no association between nifedipine and dysgeusia (Ackerman, 1997), case reports of dysgeusia from nifedipine exist (Ackerman, 1997). We present a case of nifedipine-induced dysgeusia mistaken for depression. Case Report: A 42-year-old man with hypertension and diabetes was admitted to the hospital following right thalamocapsular and intraventricular hemorrhages. Hypertension was managed with metoprolol, lisinopril, nifedipine, and chlorthalidone. Levetiracetam was started for seizure prophylaxis. Medications included pantoprazole, simethicone, transdermal lidocaine, insulin, metformin, docusate, senna, and subcutaneous heparin. Psychiatric consultation was requested out of concern that appetite loss indicated depression. The day before psychiatric evaluation, mirtazapine 15 mg at bedtime for mood and appetite was started. Nifedipine 90 mg daily had been started 9 days prior to his first complaint of decreased appetite. The patient reported feeling disconnected from his family and “sad" for ∼10 years, complaining that family members “talk behind his back.” He was otherwise without paranoia. He denied insomnia, anhedonia, hopelessness, poor concentration, suicidal ideation, homicidal ideation, guilt, mania, or hallucinations. He reported poor appetite due to epigastric discomfort and bad taste to foods. Covid-19 testing was not yet widely available. No other signs or symptoms suggestive of Covid-19 were present. Although alert and fully oriented, concentration was impaired with sometimes tangential thought processes. Affect was full without depression. A diagnosis of adjustment disorder was made. The psychiatry team suspected nifedipine-induced dysgeusia and advised discontinuing nifedipine. Appetite improved two days later. Discussion: This case highlights the importance of considering alternative causes of nonspecific symptoms of depression, including decreased appetite, that may have non-psychiatric causes. Dysgeusia is widely recognized as a symptom of Covid-19. Other causes, including medications may be underrecognized and amenable to intervention. Conclusion: It would be helpful to consider medication side-effects as potential causes for taste distortion alongside psychiatric diagnoses, and COVID-19. References: 1. Coulter DM: Eye pain with nifedipine and disturbance of taste with captopril: a mutually controlled study showing a method of post marketing surveillance BMJ 1988;296: 1086–8. 2. Ackerman BH, Kasbekar N: Disturbances of taste and smell induced by drugs. Pharmacotherapy 1997;17(3):482-96.

Cerebral Infarction Following Correction of Aortic Coarctation Surgery in Children

Background: Coarctation of the aorta (CoA) is a congenital heart defect. Due to the narrowing of the descending aorta, blood flow mainly reduces after the stenosis, and CoA can occur at any region in the thoracic and abdominal aorta. Cardiac surgeons and cardiologists are familiar with postoperative complications of CoA;however, there are also some other complications that have not been reported to date. Case Presentation: The present study investigated three cases of CoA undergoing reconstructive surgery. Nevertheless, a couple of days after the surgery, they manifested symptoms suspected of cerebral infarction. Ischemic infarction was observed after performing brain computed tomography. Additionally, we discuss possible pathophysiology and reasons that can lead to this problem. Conclusions: In this case report, we presented three cases of CoA patients who underwent reconstructive surgery and manifested cerebral infarction as an adverse effect of the reconstructive surgery.

Myoclonus after Vaccination for SARS COVID19

Objective: To report two patients with movement disorders after vaccination for SARS COVID 19 with non-replicating viral vector vaccine. Background: As a result of the SARS COVID 19 pandemic, different types of vaccines have been developed to reduce the worldwide spread of the virus. Non-replicating viral vector vaccines (recombinant viruses) offer a rapid immune response, may require booster doses to acquire long-lasting immunity and may exhibit reactogenicity. Methods: We studied the clinical features and complementary examinations of two patients, who presented myoclonus after vaccination for SARS COVID19. Results: Case 1: Male, 75 year old with Parkinson's disease since 2003 treated with levodopa and amantadine. Hoehn&Yahr: II. He lived alone and was actively working as a psychologist. After the first dose of SARS COVID vaccine he felt severe asthenia. On the fourth day postvaccination, he presented upper and lower limbs myoclonus that made him unable to walk, and myoclonus in phonatory muscles. He was treated with levetiracetam up to a dose of 1500 mg/day with clear improvement of symptoms.Brain MRI showed no pathological findings. Caso 2: Female 80 year old with hemifacial spasm due to herpes zoster. One day after the second dose of SARS COVID vaccine she developed rhythmic involuntary movement in her left hand (3 Hz). Two months later, the involuntary movement spontaneously improved, remaining a mild myoclonic jerk. Brain MRI and Fluorodopa PET were normal. Conclusion: Both patients presented myoclonus one to four days after vaccination. In patient two, the initial interpretation of the involuntary movement as a tremor, led us to indicate a Fluorodopa PET to rule out dopamine deficient related movement disorder.Sputnik and Covishield share the same platform;they use non-replicating viral vectors also called recombinant virus vaccines.Based on the similar mechanism of action, we hypothesize that an immune-mediated mechanism, related to the faster occurrence of myoclonus in patient two (who had already been exposed to the vaccine) as compared to the delayed symptoms in patient one (who received his first dose), may underly the occurrence of symptoms.A detailed description and report on these findings can alert to the health organizations to contribute to a better understanding of the adverse effects profile associated with these different types of immunizations and to the level of evidence.

Meta-analysis of175 Patients with COVID-19 and Seizures, Status Epilepticus, or Cortical Myoclonus: An Individual Patient Data Analysis

Objective: To characterize management and outcomes of seizures, status epilepticus, and cortical myoclonus in COVID-19, with individual patient data analysis of published literature. Background: Seizure disorders in COVID-19 have been sparingly reported. Lack of large-scale studies create challenges in identifying clinically relevant associated factors. Design/Methods: Systematic literature review was conducted in accordance with PRISMA guidelines. Criteria included new-onset seizures, status epilepticus, and/or cortical myoclonus developing prior to or during hospitalization, with concomitant COVID-19. COVID-19 severity was dichotomized into mild and severe cases, based on severity of respiratory symptoms. Good outcome was defined as discharge without severe deficits, and/or return to near baseline. Results: A total of 105 studies reporting 175 patients (male 56.6%;mean age 47.9, SD 25.7) were included. Status epilepticus occurred in 44 patients (25.1%) and myoclonus in 38 (21.7%). Any seizure-like activity on electroencephalography (EEG) was noted in 53/102 patients (52.0%). Abnormal cerebrospinal fluid analysis was reported in 32/83 patients (38.6%). Most common underlying diagnosis was encephalitis (autoimmune or infectious) in 42/175 patients (24.0%), followed by infarct (15/175;8.6%) and intracerebral hemorrhage (ICH) (13/175;7.4%). The most common treatment was levetiracetam (92/130;70.8%). Overall, 106/160 patients (66.3%) had good outcomes while 24/156 died (15.4%). Encephalitis was associated with good outcomes (p=0.005). Severe COVID-19 was associated with more myoclonus, poor outcome, and mortality (all p<0.001), with a trend towards more EEG abnormalities (p=0.066). In multivariate regression, only severe COVID-19 was associated with reduced odds of good outcome (OR=0.095;p=0.006), and higher odds of mortality (OR=4.60, p=0.040). Conclusions: Encephalitis, infarct, and ICH are common underlying etiologies in COVID-19 patients with seizure disorders. Overall, most patients achieved good outcome, thus highlighting the necessity of aggressively treating seizures, and identifying any treatable underlying etiology. Future research should investigate long-term neurocognitive outcomes in COVID-19 patients with seizure disorders.

Hemiconvulsion-Hemiplegia-Epilepsy Syndrome in the Setting of COVID-19 Infection and Multisystem Inflammatory Syndrome in Children

Objective: To present a case of Hemiconvulsion-Hemplegia-Epilepsy (HHE) Syndrome in a child with COVID-19 infection and Multisystem Inflammatory Syndrome in Children (MIS-C). Background: HHE Syndrome is a rare pediatric epilepsy syndrome that presents with prolonged unilateral convulsive status epilepticus in the setting of fever, followed by hemiparesis, unilateral hemispheric swelling and atrophy, and the development of epilepsy. Though it was first described over six decades ago, the pathophysiology is still poorly understood with multiple factors contributing, including hyperthermia, inflammation, and cytotoxic edema from prolonged ictal activity. Prognosis is variable, from the resolution of hemiplegia and seizures to permanent hemiparesis and refractory epilepsy. Design/Methods: This is a case report based on a chart review. Results: The patient is a 2-year-old boy with a history of one prior complex febrile seizure who presented with greater than one hour of convulsive status epilepticus in the setting of fever. The patient had a generalized tonic-clonic seizure with more prominent convulsions on the right side. The patient required intubation and was initially given multiple anti-seizure loads though continued to have persistent electrographic and electroclinical seizures. EEG showed lefthemispheric high amplitude spike/polyspike and wave discharges. The patient required continuous midazolam infusion with eventual control of seizures on levetiracetam, phenobarbital, and clobazam. The examination was notable for persistent right-sided hemiparesis with gradual improvement. MRI brain without contrast revealed T2 signal abnormality and restricted diffusion diffusely throughout the left cerebral hemisphere. Infectious workup was significant for positive COVID-19 PCR and elevated inflammatory markers, consistent with MIS-C. Conclusions: Our patient had prolonged focal convulsive status epilepticus in the setting of acute febrile illness secondary to COVID-19 and MIS-C leading to hemiparesis and diffuse left cerebral hemisphere edema on MRI brain consistent with HHE syndrome. More research is needed to elucidate further HHE syndrome's pathophysiology and assess long-term outcomes in patients with HHE syndrome.

Atypical Presentation of Lance Adams Syndrome in the setting of Clonazepam overdose and SARS-CoV-2 pneumonia

Objective: To report an atypical presentation of Lance-Adams Syndrome presenting from severe respiratory depression rather than cardiac arrest and to highlight the importance of distinguishing it from post hypoxic myoclonic variants. Background: Clinicians often face difficulty distinguishing Lance-Adams Syndrome (LAS) from Myoclonic Status Epilepticus (MSE). Similarities between the two conditions frequently result in confusion when diagnosing, managing and prognosticating for post-hypoxic myoclonus patients. Design/Methods: A 23-year-old male with a history pertinent for Hemophilia B, depression, opiate and alcohol abuse and chronic pain was found down in his home next to an empty bottle of clonazepam. He was hypoxic with oxygen saturation in the 40s and intubated in the field. Upon arrival to the Emergency room, neurological examination revealed intact corneal reflexes but no gag reflex, cough, or purposeful movements of the extremities. The patient exhibited stimulus induced myoclonic jerking which lasted >30 minutes despite being loaded on valproic acid and levetiracetam. Jerking subsequently ceased with propofol drip. Chest X-ray confirmed interstitial opacities and tested positive for SARS-CoV-2. On attempting to wean sedation, patient exhibited full-body myoclonus including face and palate with inability to follow commands and lack of spontaneous movements. As the EEG showed BIPEDS greater than 2.5 HZ, we decided to burst suppress him and treat with targeted temperature management. After 10 days, the patient was successfully weaned from sedation and extubated, but remained on multiple anti-seizure medications. Results: Patient responded well despite his diffuse cerebral anoxic injury. He regained the ability to follow commands upon discharge but had residual moderate expressive aphasia and post-hypoxic action-induced myoclonus, consistent with LAS. Conclusions: The atypical presentation of this case emphasizes the importance of distinguishing LAS from MSE to guide neurologists to aggressively treat LAS to improve outcome, particularly since MSE historically results with a 90-100% mortality rate.

New-Onset Refractory Status Epilepticus After Pfizer-BioNTech COVID-19 Vaccination

Objective: NA Background: Previous case reports have described 3 cases of autoimmune encephalitis and 1 case of new-onset refractory status epilepticus (NORSE) following COVID-19 viral vector vaccinations. However, no cases have been documented in association with COVID-19 mRNA vaccinations. We describe a case of NORSE after vaccination with Pfizer-BioNTech COVID-19 vaccine. Design/Methods: Case report. Results: A 56 year old healthy man presented with three days of fever, fatigue, and aphasia beginning 2 weeks after he received his first dose of the Pfizer-BioNTech COVID-19 vaccine. Video EEG showed temporally predominant seizures occurring independently bilaterally (right greater than left). Clinical seizures were characterized by head turn to the left and right hand movements. He then developed sustained right frontotemporal spike and slow wave activity consistent with non-convulsive status epilepticus. CSF demonstrated mild lymphocytic pleocytosis with WBC 16 cells/mm3, protein 24, glucose 76, and an opening pressure of 47. CSF bacterial and viral encephalitis panels, HSV, lyme, West Nile virus, and VDRL were all negative. Oligoclonal bands, paraneoplastic panel, and encephalopathy panel were negative. Systemic malignancy workup was negative. Initial MRI brain was unremarkable, but 1 week after symptom onset he developed bilateral hippocampal edema. The patient was empirically treated with broad spectrum antibiotics and antivirals which were later discontinued. Due to presumed diagnosis of autoimmune encephalitis, he was treated with high dose steroids, plasmapheresis, IVIG, and rituximab. He was treated with progressively escalating anti-seizure medications including midazolam, propofol, and ketamine continuous infusions and eventually stabilized on levetiracetam, lacosamide, phenobarbital, clobazam, zonisamide, oxcarbazepine, and perampanel. At the time of discharge, mental status had improved and aphasia resolved. Conclusions: To our knowledge, this is the first case of NORSE reported after Pfizer COVID-19 vaccination. While no test exists to definitively establish causality, these findings warrant further investigation of the possible association between COVID-19 vaccination and autoimmune encephalitis.

POSTERIOR REVERSIBLE ENCEPHALOPATHY SYNDROME (PRES) IN MILD AND SEVERE COVID-19

As the COVID-19 pandemic progresses neurological complications are increasingly being reported. Posterior reversible encephalopathic syndrome (PRES) is a clinico-radiological syndrome characterised by headache, visual loss, encephalopathy and seizures, and the development of vasogenic white matter lesions in a classically parieto-occipital distribution. The pathophysiology of PRES is incompletely understood, but both hyperperfusion secondary to hypertension, and endothelial dysfunction leading to vasogenic oedema have been implicated. Here we present a case series of 2 hospitalised COVID-19 patients with markedly different disease severity, both of whom developed PRES. Patient 1 presented with confusion and headache without significant systemic features, on a background of known hypertension. Patient 1 had a single generalised seizure and was managed with levetiracetam and antihypertensives, and showed complete clinical recovery. In contrast, patient 2 presented with respiratory distress, metabolic disturbance and encephalopathy requiring critical care admission. Patient 2 had a protracted admission, developing marked visual disturbance and generalised seizures requiring multiple agents. In both cases initial CT/MRI showed characteristic posterior PRES-like leukoencephalopathy with resolution on follow-up imaging, and CSF biochemistry, cytology and virology were normal. This case series highlights the potential for neurological complications in COVID-19 patients across the spectrum of disease severity.

Hashimoto's encephalopathy in a patient with severe hypothyroidism

Introduction: Hashimoto's encephalopathy (HE) manifests itself with neuro-psychiatric disorders, which can reach coma;HE usually occurs in euthyroid, being less frequent states of thyroid dysfunction. We present the case of a patient with HE and severe hypothyroidism. Case Description: A 71-year-old woman;history of rheumatoid arthritis, hypertension and cerebral infarction without sequelae. The family referred a 3-week history of illness characterized by disorientation and "jerking" movements, she progressed to drowsiness with left hemiparesis, focal epileptic seizures and myoclonus. The brain CT showed atrophy and left frontal malacia;diagnosed status epilepticus and cerebral infarction, indicating phenytoin. During hospitalization she was added valproic acid and levetiracetam, without improvement. Brain MRI showed cerebral atrophy and periventricular leukoaraiosis. The study of the CSF: hyperproteinorraquia;EEG: diffuse slowing and periodic lateralized discharges in the right hemisphere. They request an endocrinology evaluation for TSH: > 75uIU/ml, Free T4: 0.31ng/dl, we expand with Anti-TPO > 1000 and Anti-thyroglobulin > 3000, and we indicate treatment for severe hypothyroidism with levothyroxine for nasogastric tube and intravenous hydrocortisone for 5 days and with minimal recovery from sensory disorder. After the withdrawal of hydrocortisone, she developed drowsiness and intensified myoclonus despite an improvement in the blood concentration of thyroid hormones. Having excluded infectious, metabolic and paraneoplastic etiology of encephalopathy, with the presence of elevated antithyroid antibodies in blood and CSF, the diagnosis of Hashimoto's encephalopathy was raised, receiving methylprednisolone (1 g/day for 5 days), then human immunoglobulin (25g for 5 days) Faced with poor response, she received 5 sessions of plasmapheresis with favorable clinical and paraclinical evolution. After overcoming hospital infection by COVID-19, she was discharged at 3 months. In the follow-up, cognitive deterioration, partial dependence, without epileptic seizures or myoclonus were reported. Discussion: HE is rare, with variable clinical presentation. HE has a good response to corticosteroids, although it sometimes requires other interventions. The alteration of thyroid function itself can be confusing at the time of diagnosis. Severe hypothyroidism can present neurological complications such as seizures, dementia, or psychosis;but it differs from HE in that manifestations improve when thyroxine is replaced.