Covid-19 vaccine (covishield) heralding Moyamoya angiopathy

Indroduction: Thrombotic complications leading to cerebrovascular events occuring in conjunction with Covid-19 vaccination though rare, is well-documented. Moyamoya Angiopathy is a progressive intracranial vasculopathy leading to recurrent strokes. Case presentation: We present two index cases of young patient presenting with stroke and TIA following Covid-19 vaccination (COVISHIELD) leading to unmasking of Moyamoya Angiopathy. Conclusion(s): Arterial stroke following Covid-19 vaccination is documented, but uncommon. However, in the background of a vasculopathy, it may not be so rare. Moyamoya Angiopathy has been closely studied in the model of inflammatory pathophysiology in genetically predisposed patients leading to progressive vaso-occlusive disease. Few reports of Covid-19 infection potentiating Moyamoya Angiopathy symptoms are also documented. Thus, as an extrapolation of the inflammatory etiopathogenesis of Moyamoya Angiopathy, Covid-19 vaccination can similarly affect the Moyamoya symptomatology. These two index cases open new lines of enquiry regarding the interplay of Covid-19 vaccination and neurological destabilization in patients with underlying vasculopathy of inflammatory pathophysiology.Copyright © 2022

Guillain-Barre syndrome following COVID-19 vaccination: a case report and an updated review

Background: Coronavirus disease-2019 (COVID-19) has caused a pandemic that has recently affected every aspect of life. Fortunately, many vaccines with high safety and efficacy profiles were developed timely to face this pandemic. In a very short time, billions of people were vaccinated. In the meantime, a wide range of neurological syndromes are being reported. Guillain-Barre syndrome (GBS) which is a rare immune-mediated post-infectious peripheral neuropathy was reported after both the COVID-19 infection itself and many types of its vaccines. Method(s): We are reporting a case of post-AstraZeneca vaccine GBS and reviewing the literature of all reported post-COVID-19 vaccines GBS till July 2021. Result(s): 29 adult patients were reported. Of them 58.6% were males. Their mean age is 58.2 years. The median time to clinical onset after vaccine administration was 13.2 days. 86.2% of patients had their symptoms following immunization with the 1st dose of AstraZeneca vector-based covid vaccine. Facial palsy was the most predominant single symptom in 75.8% of patients. Conclusion(s): Guillain-Barre syndrome is a well-recognized but still rare adverse event following vaccination against COVID-19. Although preliminary data incriminates viral vector-based vaccines more than the other types, active post-vaccination surveillance and more powerful statistics are mandatory to reach a solid conclusion regarding the presence of a causal relation.Copyright © 2022

An Astra-Nomical Headache

Introduction We present a case of myelin-oligodendrocyte glycoprotein antibody disease (MOGAD) requiring long-term immunosuppression triggered by a dose of the AstraZeneca COVID-19 vaccination. Relapsing MOGAD is thus far an unknown complication of COVID-19 vaccination. Case Description: A 58-year-old lady developed headache, nausea, dizziness, facial numbness, ataxia and slurred speech 8 days after the COVID-19 AstraZeneca vaccination. Her imaging showed acute disseminated encephalomyelitis (ADEM) with a white matter lesion in the left cerebellum and bilateral smaller lesions. Her cerebrospinal fluid showed 38 white cells and elevated protein. She initially responded well to steroids, however relapsed with optic neuritis 7 months later, requiring long-term immunosuppres- sion with mycophenolate mofetil. Discussion Although there have been some case reports of MOGAD following COVID-19 infection, to our knowledge this is only the second reported case of MOGAD following vaccination against COVID-19, and the first such case to require long-term immunosuppression. The other reported case also occurred following the COVID-19 AstraZeneca vaccine, and also presented with ADEM. This is in contrast to reported cases of MOGAD following COVID-19 infection, where adults mostly presented with optic neuritis. We wanted to highlight the possibility of this vaccine-related neurological complication occurring, particularly in the context of potentially frequent ongoing COVID-19 booster vaccinations.

Covid-19 Vaccine safety and adverse event analysis from Pakistan

Covid immunization commenced on 2nd Feb 2021 in Pakistan and as of 7th Sep 2021, over 84 million vaccine doses were administered in Pakistan, of which 72% procured by the government, 22% received through Covax and 6% were donated. The vaccines rolled out nationally included: Sinopharm, Sinovac and CanSinoBIO (China), AstraZeneca (UK), Moderna and Pfizer (USA), Sputnik (Russia), and PakVac (China/Pakistan). About half of the eligible population in Pakistan (63 m) had received at least one dose of Covid vaccine as of Sep 2021. Pakistan National Pharmacovigilance Centre (PNPC) in coordination with WHO, MHRA and Uppsala Monitoring Centre (UMC) established pharmacovigilance centers across Pakistan. The Covid vaccine AEFIs in Pakistan were mainly reported via NIMS (National Immunization Management System), COVIM (Covid-19 Vaccine Inventory Management System), 1166 freephone helpline and MedSafety. There have been 39,291 ADRs reported as of 30th Sept 2021, where most reported after the first dose (n = 27,108) and within 24-72 h of immunization (n = 27,591). Fever or shivering accounted for most AEFI (35%) followed by injection-site pain or redness (28%), headache (26%), nausea/vomiting (4%), and diarrhoea (3%). 24 serious AEFIs were also reported and investigated in detail by the National AEFI review committee. The rate of AEFIs reports ranged from 0.27 to 0.79 per 1000 for various Covid vaccines in Pakistan that was significantly lower than the rates in UK (~4 per 1000), primarily atrributed to underreporting of cases in Pakistan. Finally, Covid vaccines were well tolerated and no significant cause for concern was flagged up in Pakistan's Covid vaccine surveillance system concluding overall benefits outweighed risks.Copyright © 2022

DURAL VENOUS SINUS THROMBOSIS: A RARE PRESENTING CONSEQUENCE OF COVID-19

SESSION TITLE: Post-COVID-19 Infection Complications SESSION TYPE: Case Report Posters PRESENTED ON: 10/17/2022 12:15 pm - 01:15 pm INTRODUCTION: The severe acute respiratory syndrome coronavirus (SARS-COV2) and its resulting coronary virus 2019 syndrome (COVID-19) has resulted in an unprecedented global pandemic affecting more than 250 million people and resulting in at least 5 million deaths worldwide. Clinical manifestations of the Covid-19 disease process include but are not limited to respiratory dysfunction and failure, coagulopathy, malaise and cytokine storm. We report a case of dural sinus thrombosis (DST) as a sequelae to COVID-19. CASE PRESENTATION: A 26-year-old woman with a history of migraines presented with sudden diffuse headache and photosensitivity. She reported no palpitations, oral ulcers, dizziness, diaphoresis, slurred speech, weakness, paresthesias or recent head trauma. Her presenting vital signs were within normal range. Physical exam was negative for focal neurologic deficits, weakness, or sensory loss. A rapid pregnancy test was negative. D-dimer was 7,200 ng/mL (reference <500 ng/mL). A COVID test was positive. A computed tomography (CT) of the head revealed diffuse hypodensity in the torcula and the transverse sinuses bilaterally extending into the cerebellar folia, suspicious for DST, which was confirmed on magnetic resonance venography. A full hypercoagulable panel resulted negative. It was determined that the patient's coronavirus disease infection resulted in a prothrombotic state and her dural sinus vein thromboses. The patient was started on a high intensity heparin drip for seven days, then transitioned to Dabigatran and Topiramate for management of headache upon discharge. DISCUSSION: COVID-19 typically manifests as fever, hypoxia, and dyspnea. If coagulopathy were to occur, the most common of them are deep vein thromboses. Cerebral thrombotic events, specifically, a DST has been underreported in literature. It is suspected that the burden of cerebral thrombosis in COVID-19 patients is 0.08%. In the same study, it was also identified that 31% of those who developed a cerebral thrombosis also had other hypercoagulable risk factors not present in this patient. Advancement in neuroimaging has allowed these thrombotic issues to be identified, however, early recognition, especially with a lack of risk factors, creates a less straightforward management plan. Our patient manifested a DST in the setting of an active COVID-19 infection. Higher levels of evaluation are required in patients who test positive for Covid-19 when clinically indicated. Such indications include headaches that are new in onset, severe in nature, and diffuse. Delayed diagnosis and management can be permanently damaging. CONCLUSIONS: Dural venous sinus thrombosis is a rare, yet deadly complication of COVID-19. All risk factors and other etiologies of hypercoagulable states should be ruled out followed by early detection based on clinical and physical exam, and accompanied by appropriate imaging followed by prompt intervention. Reference #1: Baldini, T., Asioli, G. M., Romoli, M., Carvalho Dias, M., Schulte, E. C., Hauer, L., Aguiar De Sousa, D., Sellner, J., & Zini, A. (2021). Cerebral venous thrombosis and severe acute respiratory syndrome coronavirus-2 infection: A systematic review and meta-analysis. European journal of neurology, 28(10), 3478–3490. https://doi.org/10.1111/ene.14727 Reference #2: Hemasian, H., & Ansari, B. (2020). First case of Covid-19 presented with cerebral venous thrombosis: A rare and dreaded case. Revue neurologique, 176(6), 521–523. https://doi.org/10.1016/j.neurol.2020.04.013 Reference #3: Thompson, A., Morgan, C., Smith, P., Jones, C., Ball, H., Coulthard, E. J., Moran, E., Szewczyk-Krolikowski, K., & Rice, C. M. (2020). Cerebral venous sinus thrombosis associated with COVID-19. Practical neurology, practneurol-2020-002678. Advance online publication. https://doi.org/10.1136/practneurol-2020-002678 DISCLOSURES: No relevant relationships by Steven Douedi No relevant relationships by slam Elkherpitawy No relevant relationships by Justin Ilagan No relevant relationships by David Kountz No relevant relationships by Anton Mararenko No relevant relationships by Mihir Odak

GIANT CELL ARTERITIS-RELATED STROKE in A MAN with ELDERLY-ONSET RHEUMATOID ARTHRITIS

Background: Giant cell arteritis-related stroke is rare, with high early mortality and major morbidity in survivors. Objectives: To increase the awareness of coexistence of giant cell arteritis-re-lated stroke and rheumatoid arthritis. Methods: A case report and discussion. Results: A 73 year-old man with seronegative elderly-onset rheumatoid arthritis (EORA) presented to the emergency department (ED) with a one week history of frontal headache, vomiting and dizziness. He had multiple cardiovascular comor-bidities and took multiple medications, including methotrexate and sulfasalazine. He also had long-standing history of thrombocytopenia without requiring any treatment. Neurological examination performed in the ED was unremarkable. His C-reactive protein (CRP) was 69mg/L and erythrocyte sedimentation rate (ESR) 82mm/hour. Computed tomography (CT) of the brain was normal. The headache settled with analgesia. A diagnosis of probable tension-type headache, with underlying active EORA, was made. One month later, he presented to an ophthalmologist with recurrence of headache associated with visual disturbance and was diagnosed with giant cell arteritis (GCA). Both CRP (77mg/L) and ESR (85mm/hour) remained raised. Neither temporal artery biopsy nor temporal artery ultrasound were possible due to the coronavirus disease 2019 (COVID-19) pandemic. The headache and visual symptoms resolved completely a week after prednisolone 60mg daily was prescribed. In parallel, the CRP dropped to 2mg/L and ESR 16mm/hour. The patient's glucocorticoid dose was then tapered. While on prednisolone 20mg daily, about 3 weeks later, he developed slurred speech and generalized weakness. Examination showed cerebellar signs and MRI brain showed acute cerebellar infarct. He was treated pragmatically as an atherosclerotic stroke with clopidogrel, and the steroid was rapidly tapered in view of absence of headache and normalization of infammatory markers. Four weeks later, he was noted to have persistent confusion and unsteadiness of gait. CRP was elevated at 92mg/L. An urgent positron emission tomography-CT (PET-CT) scan showed infammation in the vertebral arteries [Figure 1] and cerebellar stroke. Prednisolone 40mg daily was restarted which led to a rapid improvement in his symptoms and normalization of infammatory markers. The glucocorticoids were tapered in a slower manner this time. A diagnosis of GCA-related cerebellar stroke with vertebral vasculitis was made and, with glucocorticoids, the patient made a good clinical recovery. His infam-matory joints pain also improved in parallel. Conclusion: Stroke or transient ischemic stroke are rare complications, reported in 2.8-16% of patients with active GCA. Most studies report strokes as occurring between the onset of GCA symptoms and 4 weeks after commencement of glucocorticoids1-3. Vertebrobasilar territory is involved in 60-88% of cases of GCA-related stroke1-3. In contrast, the vertebrobasilar territory is affected only in 15-20% of atherosclerotic strokes1,2. One study reported fatal outcomes in 11 out of 40 patients (28%) with GCA-related stroke, 7 within 2-13 days of stroke2. To conclude, this case demonstrates that high-dose glucocorticoids with slower tapering were able to control GCA-related stroke due to vertebral vasculitis in patient with EORA on background methotrexate and sulfasalazine.

A CLASSIC CASE OF CREUTZFELDT-JAKOB DISEASE (CJD)

CASE: A 64-year-old woman was brought in by husband for inability to care for patient. Previously active, she developed gait instability, slurred speech, and memory lapse to the point of selective mutism and being bed-bound within three months. Her medical history was notable for hypertension and Covid four months prior. She had had mild upper respiratory symptoms and recovered in ten days. Examination revealed general encephalopathy, dysarthria, limited ability to follow commands. She had decreased strength but increased tone and rigidity in all extremities. She had rhythmic jaw movement and bradykinesia with scatter myoclonic movements. Cerebellar exam was notable for ataxia, but she had normal cranial nerve and sensory exams and normal reflexes. MRI of the brain revealed restricted diffusion and T2/Flair signal abnormality involving bilateral basal ganglia, ventral medial thalami, hippocampi, and cerebral cortices. Toxic metabolic workup was unrevealing. CSF was positive for 14-3-3 protein and elevated total tau protein, confirming Creutzfeldt-Jakob disease. IMPACT/DISCUSSION: Creutzfeldt-Jakob Disease (CJD) is a prion disease with one in a million prevalence. Patients present with rapidly progressing dementia, myoclonus, and signs of cerebellar, corticospinal and extrapyramidal involvement including nystagmus, ataxia, hyperreflexia, spasticity, hypokinesia, bradykinesia, dystonia, and rigidity. CJD is fatal within months to two years. Patients with end stage disease may have akinetic mutism. Magnetic resonance imaging (MRI), electroencephalogram (EEG), and cerebrospinal fluid (CSF) analysis are important for evaluation of CJD. Most sensitive in early stages, MRI Brain commonly shows hyperintense signal involving the cerebral cortex, corpus striatum, caudate, and putamen. EEG may capture pattern of periodic bi-or triphasic period sharp wave complexes. CSF might detect 14-3-3 protein with elevation of tau protein but real-time quaking-induced conversion (RT-QuIC) has the highest specificity for diagnosis for CJD. Though brain biopsy is the sole method of definitive diagnosis, results of MRI, EEG, and CSF analysis along with presenting signs and symptoms are sufficient for clinical diagnosis of CJD. Our patient's dementia, myoclonus, ataxia, hypokinesia, bradykinesia, dystonia, and rigidity all progressing to akinetic mutism within three months are classic presentation of CJD. EEG was normal, but MRI with hyperintensity of basal ganglia and cerebral cortices and CSF analysis with positive 14-3-3 and elevated tau proteins are all lead to diagnosis of CJD. CONCLUSION: This case illustrates a classic case of a Creutzfeldt-Jakob Disease, a rare prion disease marked by rapidly progressive dementia with neuropsychiatric features.

Cerebellar Symptoms: An Uncommon Presentation of Legionnaires' Disease

A 27- year-old previously healthy man presented to the emergency department with a 3-day history of confusion, urinary retention, cough, slurred speech, intermittent vertigo, and unsteady gait after being started on outpatient abx for diagnosis of community acquired pneumonia at an outside facility. On presentation, his vital signs were significant for a Temp. of 102.4 F, a HR of 118 bpm, a RR of 22 bpm, a BP of 135/75 mmHg, O2 sats down to the 80s% with a new oxygen requirement of 3 L/min. His neurological exam was remarkable for dysarthria, a wide based and unsteady gait, forward drift on standing, bilateral dysmetria and was otherwise non-focal. Bilateral basilar rales were noted on lung exam. Initial workup was significant for a sodium level of 129, potassium 3.1, AST 178, ALT 31. His urine legionella antigen test was positive and COVID-19 PCR test was negative. CT of the chest demonstrated multifocal pneumonia. The predominant neurological picture on presentation warranted a lumbar puncture as well as an MRI of the brain, both of which came back negative. HIV test was negative as well. The spectrum of neurological manifestations of legionnaire's disease is quite wide ranging from simple headaches to acute disseminated encephalomyelitis (ADEM). While up to 40% of patients may present with headaches and some confusion, the specific cerebellar presentation is quite rare and has been described in case reports. According to Shelburne(1), there has been instances where the neurological manifestations have lasted up to 3 years after resolution of the pulmonary disease. In a review article detailing 29 cases of cerebellar dysfunction in Legionnaire's disease, a lumbar puncture performed in 16 of the cases showed that the majority had no abnormalities in CSF studies. The propensity for Legionella to specifically manifest as cerebellar Sx remains to be studied. Interestingly, another case report of two patients with neurological symptoms, showed hypoperfusion of the cerebellar and frontal lobes on single photon emission while CT and MRI imaging of the brain were normal. We add to the literature another 'cerebellar' presentation of Legionnaire's disease.

Post-acute sequelae of SARS-CoV-2 infection (PASC): peripheral, autonomic, and central nervoussystem featuresin a child

Objective: To describe treatment with intravenous immune globulin (IVIG) of severe central, peripheral and autonomic (CNS, PNS, ANS) post-acute sequelae of SARS-CoV-2 infection (PASC) in a child. Background: PASC is defined as failure to recover from acute COVID-19 in those persistently symptomatic for>30 days from onset of infection with any pattern of tissue injury that remains evolving including the nervous system. Design/Methods: A child underwent extensive evaluation of the CNS, PNS and ANS according to the authors protocol for COVID-19 neurologic illness. Results: A 12-year-old girl was initially well until March 2020 until exposure to other family members testing positive for COVID-19 infection she contracted an upper respiratory infection illness with loss of taste, and excessive fatigue followed in July 2020 by burning, weakness, slurred speech and impaired cognition leading to a bedbound state and a concern she was suffering from conversion disorder. Examination in September 2020 showed mild delirium, tetraparesis, stocking sensory loss and areflexia. Electrodiagnosis showed mixed chronic distal demyelinating and axonal changes. Epidermal nerve fiber studies showed reduced calf and thigh densities. Autonomic studies showed symptomatic hypotension with tilting and reflex tachycardia. Brain FDG PET/MRI showed hypometabolism of bilateral anterior and mesial temporal, superior parietal, and lateral occipital lobes, anterior cingulate cortices, and the cerebellar hemispheres with hippocampus volumes <5% of age-matched controls. Lumbar puncture showed a total protein of 136 mg/dL. EEG and Mayo Clinic ENS2 panel did not show evidence of autoimmune encephalitis. From October 2020 to February 2021, she received monthly 2 g/kg/month of intravenous immune globulin (IVIg) with overall clinical improvement. Conclusions: The underlying basis of PASC, especially in the CNS, has not yet been fully appreciated awaiting controlled clinical and autopsy studies. IVIg is effective initial therapy of PASC to modulate neurologic post-infectious immunity. COVID Long Hauler and Long COVID are inappropriate terms for PASC.

Refractory Rhombencephalitis Secondary to SARS-CoV-2 Infection: A Case Report

Objective: To describe a case of rhombencephalitis secondary to severe acute respiratory syndrome coronavirus type 2 (SARS-CoV-2) Background: Rhombencephalitis is an inflammation of the brainstem and cerebellum. Etiologies include infectious, inflammatory, and autoimmune causes. Rhombencephalitis has varied presentations but frequently includes encephalopathy, cranial neuropathies, long tract signs and cerebellar dysfunction. To date, SARS-CoV-2 has been reported as the cause of rhombencephalitis in 4 cases. Design/Methods: Authors searched PubMed and Google Scholar for articles using the keywords: “COVID-19”, SARS-CoV-2', “Rhomboencephalitis”, “Rhombencephalitis”. Results: 30-year-old African American man with poorly controlled type 1 diabetes mellitus presented with dysgeusia, slurred speech, night sweats, left-sided hypoesthesia, paresthesias, ataxic gait, and light-headedness. Exam was notable for, left-sided hypoesthesia of the face and left upper extremity weakness as well as ataxia. MRI brain revealed diffuse pontine edema and central areas of diffusion restriction. COVID-19 nasal PCR and COVID-19 IgG antibodies were positive. Extensive infectious, autoimmune and paraneoplastic workup was unrevealing. Pulse-dose steroids resulted in improvement of edema and patient was discharged with diagnosis of a monophasic infectious rhombencephalitis due to COVID-19. Patient re-presented 8 days following discharge with acute left-sided headache and vomiting. Exam was notable for mild cranial nerve seven palsy and ataxia in all extremities. MRI brain displayed increased edema, mass effect and enhancement throughout the brainstem extending superiorly to include optic tracts and hypothalamus. CSF studies were remarkable for leukocytosis and increased protein. Repeat infectious, autoimmune and paraneoplastic studies again negative. Re-treatment with pulse-dose steroids followed by prolonged taper resulted in clinical and radiographic improvement at 1 month follow-up. Conclusions: The complete picture of neurological sequelae from COVID-19 is developing as the pandemic continues. Our case adds to the literature of SARS-CoV-2 associated rhombencephalitis and highlights the need for close monitoring and slow titration of immunotherapies such as steroids to minimize the potentially devasting effects of rhombencephalitis.

Acute disseminated encephalomyelitis after COVID-19 vaccination: A case report

Objective: To report a case of a patient who developed acute disseminated encephalomyelitis in the setting of recent COVID-19 vaccination. Background: NA Design/Methods: Data was obtained through the medical records system of Westchester Medical Center in Valhalla, New York. Results: We report on a 53-year-old female patient with a past medical history of obstructive sleep apnea who presented in the emergency room (ER) with left-sided numbness and weakness, ataxia, vertigo, and slurred speech in the context of a three month long history of headaches that started one day after she received her first dose of COVID-19 vaccine. Brain imaging studies showed findings most suggestive of acute disseminated encephalomyelitis (ADEM). Initial pretreatment cerebrospinal fluid (CSF) analysis showed markedly elevated protein with normal glucose level. Extensive workup was negative for infectious, oncologic, autoimmune, and psychiatric etiologies. Over the course of hospitalization, the patient was found to have isolated elevated liver function tests (LFTs) with transaminitis and hepatomegaly on ultrasound. The initial treatment of a five-day methylprednisolone course did not result in significant clinical improvement. The patient was then started on plasma exchange therapy (PLEX) and successfully recovered by the end of the last session. Conclusions: Our case report exemplifies a rare but severe neurologic condition observed in the setting of recent COVID-19 vaccination in a previously healthy woman.

Isolated Hypoglossal Nerve Palsy: Two Case Reports

Objective: NA Background: Although extremely rare, hypoglossal nerve palsy(HNP) can occur in isolation causing dysphagia, dysarthria, and tongue deviation. Documented cases of isolated hypoglossal nerve palsy(IHNP) were found to be caused by various etiologies, which include: tumor, trauma, surgery, (post)infection, sarcoidosis, atlanto-occipital dislocation, aberrant ectatic vessel in the hypoglossal canal, and stroke. We report 2 cases of IHNP, one of which had an etiology yet to be reported before. Design/Methods: NA Results: Case 1, a 78-year-old male with history of arthritis who awoke to find he had slurred speech, difficulty swallowing, and tongue deviation to the right. No tongue tumors, edema, or tenderness on exam. No recent history of infection, cancer, neck surgeries, or COVID-19. MRI head and neck revealed erosive arthritis at the C1-C2 articulation, hypertrophic pannus surrounding the atlantoaxial articulation. Pannus seen extending into the spinal canal and foremen magnum, displacing the medulla and crowding the right hypoglossal nerve at the canal. No reported cases of infiltrative pannus causing IHNP was found in literature review. Surgery was scheduled to resect the infiltrating tissue. Case 2, an 18-year-old female who presented to an urgent care for slurred speech, tongue deviation, and only being able to swallow if she shifts her jaw and food to the left side of her mouth. Stroke work-up was negative. Laboratory and imaging studies were within normal limits. She later reported that she had a sinus infection 2-3 weeks prior which required antibiotics. With a post-infectious etiology, she was treated with prednisone 60 mg daily for 7 days with a short taper after. Conclusions: IHNP is a rare disorder stemming from a variety of causes, here we have reported 2 cases demonstrating the wide ranges of age, etiologies and interventions. With increasing etiologies, it is important clinicians recognize that IHNP requires targeted intervention whether medical or surgical.

Transverse myelitis in a pediatric patient with COVID-19

Case Report Transverse myelitis is the segmental inflammation of the spinal cord with motor and sensory abnormalities at and below the level of the lesion. Often, the etiology is unknown but may be attributed to autoimmune conditions or viruses. Here we describe a rare case of transverse myelitis secondary to severe acute respiratory syndrome coronavirus 2 [SARS-CoV-2]/coronavirus disease (COVID-19). Case A 5-year-old male with a history of asthma presented for vomiting and altered mental status. The patient was noted to be altered, lethargic, and in respiratory distress. Intubation was performed. After family collateral was obtained, it was revealed that patient possibly ingested Sertraline and/or Risperidone at an unknown time prior to arrival. History also revealed that he had slurred speech, ataxia, and a fall with trauma to forehead 1 day prior to arrival. He tested positive for COVID-19 via PCR and chest x-ray revealed RLL consolidation. Dexamethasone was started. When sedation was weaned in hopes of extubation, patient was noted to be alert, but not moving extremities and had minimal gag and cough reflex. MRI of Brain and Spine were conducted and revealed findings suggestive of long segment transverse myelitis involving C2 to C3. LP was performed with unremarkable CSF studies and IV Solumedrol was started. In light of active COVID-19 infection, and worsening respiratory status, patient started on 5 days Remdesivir. Further, patient underwent ten sessions of plasmapheresis. Repeat MRI was consistent with previous. Physical and occupational therapy initiated at the onset of illness in hopes of achieving musculoskeletal improvement. Patient had some minimal musculoskeletal improvement, however, given his condition, decision was made for patient to undergo placement of gastrostomy and tracheostomy tubes. Patient was weaned off of sedatives and withdrawal was treated with a clonidine taper. Once stabilized, patient was transferred to neurological inpatient rehabilitation center. Discussion Neurological manifestations in children affected by SARS-CoV-2 are relatively common but are often non-specific. Worldwide data reports only 1% of children with COVID-19 present with severe symptoms of encephalopathy, seizures, and meningeal signs. Pathophysiology is multifactorial, including direct invasion of the CNS, vascular insufficiency, immune dysregulation and autoimmunity. Imaging is paramount in the diagnosis of transverse myelitis. Treatments are emerging and may include steroids, immunoglobulin, plasmapheresis, and monoclonal antibodies. Conclusion Much is unknown about COVID-19. Information is emerging and evolving daily. Cases of transverse myelitis in COVID-19 have been reported in few adult patients and minimal pediatric patients. Practitioners should keep transverse myelitis on their list of differentials for neurological complications of SARS-CoV-2 infections and initiate aggressive treatment with a multidisciplinary approach.

P.0177 Partial ornithine-transcarbamylase deficiency, autoimmunity and psychosis – a complex case report

Background: Ornithine-transcarbamylase deficiency (OTC) is the most common type of urea cycle disorder, and it is the only one with X-linked inheritance. The clinical presentations can vary from severe symptoms caused by hyperammonemia in childhood or adolescence to milder cases with late-onset in adulthood (similar to delirium or acute psychosis) [1], in the context of precipitating factors such as pregnancy, high protein intake, acute stress, infections, certain medications (valproate, steroids, haloperidol) [2]. Method: We present a case of a 31-year-old female, with no history of mental disorders, with a personal history of Hashimoto thyroiditis and urticaria, and a family history of OTC deficiency (her two-year-old niece). She was also a heterozygous carrier for the OTC deletion, reporting periods of meat avoidance and anorexia. She was single, lived alone, and complained of work-related stress, mainly as she worked from home during the COVID-19 pandemic as an IT consultant. The patient presented at our clinic in emergency for psychomotor agitation, slurred speech, complex auditory and visual hallucinations, and mystical delusional ideas. Furthermore, one week before her presentation, she started fasting because of her Christian orthodox religious beliefs (before Easter celebration), but she also complained of insomnia, fatigue, and tachycardia. The patient reported being vaccinated with the first dose of Pfizer's SARS-CoV-2 vaccine one week before the presentation. Results: Laboratory tests showed iron-deficient anemia and ketonuria;hepatic function was normal. Thyroid function was also normal, but anti thyroperoxidase antibodies were elevated. Serum ammonia levels were normal, and urinary orotic acid levels were within normal range. The result of head CT was unremarkable. Neurological examination was normal. She was started on 10 mg i.m. Haloperidol per day, but given the possibility of inducing hyperammonemia in urea cycle disorders patients, she was switched to Risperidone 6 mg/day, which was gradually reduced to 3 mg/day. Also, she was started on a protein-restricted diet. On the second and third days of admission, she was partially disoriented and somnolent but showed no signs of metabolic encephalopathy;therefore, metabolic treatment was not initiated. On the sixth day, she was almost completely recovered, with no psychotic symptoms. After the remission of psychotic symptoms, the neuropsychological evaluation showed significant cognitive deficits: executive functions (impaired performance on Tower of London task), deficits of focused and distributed attention, and decreased immediate verbal memory, even though the patient had received higher education, being at the top of her class during her studies. Given that metabolic profiles were normal, we discuss the complex interactions between autoimmune disorders, genetic factors, precipitating factors, and psychosocial factors that could have contributed to the psychotic episode. Conclusion: Clinicians should consider various factors that can influence the psychological state of a patient, paying attention to atypical factors or symptoms. Also, regarding the treatment of psychiatric symptoms in patients with urea cycle disorders with a normal metabolic profile, psychiatrists must avoid certain medications (haloperidol, valproate) that can worsen the patient's status. No conflict of interest