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Introduction: TBX1 haploinsufficiency is an inborn error of immunity with the phenotype of DiGeorge Syndrome. DiGeorge Syndrome has variable immunodeficiency associated with grade of thymic hypoplasia ranging from mild with no infections to severe requiring thymus implant. Enterovirus is an example of an opportunistic infection that can be fatal in these patients. Case Presentation: A 1 year old girl with TBX1 haploinsufficiency complicated by Tetralogy of Fallot, pulmonary atresia, high arched palate, and vesicovaginal fistula presented for elective cardiac repair surgery from another country due to failure to thrive and cyanosis. She had no prior infectious history but was on sulfamethoxazole-trimethoprim for prophylaxis. She was asymptomatic with a negative COVID test but no other infectious studies performed. Immediately postoperatively, she was febrile and nasal respiratory viral panel was positive for rhinovirus/enterovirus with increased procalcitonin and leukocytosis with left shift. She decompensated with multi-organ failure and cardiac arrest on postoperative day two. She was cannulated to veno-arterial extracorporeal membrane oxygenation (ECMO). Pre-operatively, she had a normal absolute lymphocyte count. No thymus tissue was observed in surgery. She had profound CD3 lymphopenia to 130 cells/cmm when critically ill. Enteroviral meningitis was suspected as no infectious, cardiac, or other pathology could be identified causing decompensation. Enteroviral serum polymerase chain reaction (PCR) test was negative while lumbar puncture deferred due to clinical status. She was treated with immunoglobulin. Offlabel investigational drug pocapavir was considered but deferred to patient's irreversible neurological status. The patient was disconnected from ECMO and expired. Discussion(s): Though we cannot confirm that this patient had enteroviral meningitis, invasive enteroviral infections are associated with elevated transaminases, coagulopathy, and seizures all present in our patient. There has also been reported negative serum enteroviral PCR but positive CSF enteroviral PCR in an immunodeficient patient. Additionally, this case highlights the importance of immunologic evaluation in patients with DiGeorge Syndrome and questions if asymptomatic viral screening for viruses like enterovirus should be considered pre-operatively in patients with inborn errors of immunity. This case highlights potential treatment options for invasive enteroviral infections in patients with inborn errors of immunity: high dose immunoglobulin, fluoxetine, and pocapavir.Copyright © 2023 Elsevier Inc.
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SARS-CoV-2 which first was observed in Wuhan region, China in December 2019 is affected many organs, such as central nervous system. We describe a case of a 57-year-old male patient, in hospital with the loss of consciousness, in the form of lack of verbal and visual communication. He got a seizure attack for about 3 minutes in the form of generalized tonic-clonic seizure (GTS) and admitted to the neurological department and was intubated. Since, the patient was not aware, awake, did not obey, corneal reflexes test was positive and his pupils were isochoric and reactive therefore, the primary diagnosis was cerebrovascular accident (CVA). On the second day after admission, although the brain computed tomography (CT) did not show brain lesion, but chest X-ray (CXR) revealed lung involvement. In addition, on third day the RT-PCR test for coronavirus RNA in and the cerebrospinal fluid and nasopharyngeal swap done and the result was positive for both of them. Therefore, treatment for the covid-19 was started. Result(s): Since, the treatment for the covid-19 was started with Atazanavir, Clindamycin and ceftriaxone, ten days after hospitalization, the lung involvement and general condition of patient got better and after two weeks he was released from the hospital. Conclusion(s): GTS should be considered as a neurological outcome of COVID-19 and medications against the coronavirus, such as Atazanavir, Clindamycin and ceftriaxone can recover the neurological deficits in these patients.Copyright© 2020, TMU Press.
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Purpose of study COVID-19 primarily affects the respiratory system from flu-like syndrome to acute hypoxic respiratory failure. Neurological manifestations are uncommon and can result in serious complications. We report a unique case of sudden onset of rapidly progressive encephalopathy in the setting of COVID-19. Methods used Reviewed the manifestations, clinical course, and outcome for a patient presenting with altered mental status secondary to COVID-19. Summary of results A 48-year-old with no significant past medical history presented to the emergency department complaining of severe headache for four days. His vital signs on presentation showed a blood pressure of 154/90, pulse of 114 bpm, temperature of 99.6 degreeF, and oxygen saturation of 97% on room air. Physical exam was unremarkable. Lab work showed elevated D-dimer 8,500 ng/L, Elevated ESR:42, LDH:340 and Ferritin:692. White blood count: 7.59 uL, Platelets 50 x 103 uL. Computer tomography angiography (CTA) of the chest showed bilateral multifocal pneumonia. CT Head was performed and was negative for an acute hemorrhage, hydrocephalus or territorial infarcts. Patient spiked a fever shortly after admission 103degreeF. Patient was started on Ceftriaxone and Azithromycin. Blood and urine cultures were positive for Klebsiella pneumonia. Patient was re-evaluated in the morning and was found altered with associated neck stiffness. Antibiotics were switched to cover for suspected meningitis. Neurology was consulted and recommended lumbar puncture. Within a few hours, the patient's mental status deteriorated and was found to be hypertensive with a blood pressure of 220/110. Repeat CT Head was negative. The patient was tested and found to be positive for COVID-19. Patient further decompensated within a few hours and became unresponsive, pulseless. ACLS was performed and the patient was transferred to the intensive care unit. Conclusions This case report highlights the heterogenous presentation in patients with COVID-19 and the importance of recognizing a new onset, severe headache as the only initial presentation. Headaches in some cases may precede the respiratory symptoms or may be the only manifestations in COVID-19 patients and it is crucial to be aware of the neurological complications and the rapid decompensation these patients may undergo if not recognized early.
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Background: Hospitals are one of the primary resources for disease transmission, so many guidelines were published, and neuro-surgeons were advised to postpone elective spine surgeries during the COVID-19 pandemic. Objective(s): To avoid pulmonary complications and reduce the risk of spreading the virus and contracting the disease during the COVID-19 era, we operated a group of our patients under spinal anesthesia rather than general anesthesia. Method(s): We retrospectively analyzed all patients who underwent discectomy surgery for lumbar spinal disc herniation under SA between September 2020 and 2021. Result(s): Sixty-four patients diagnosed with lumbar disc herniation underwent lumbar discectomy with SA. All patients except three were male. The mean age was 44.52 +/- 7.95 years (28 to 64 years). The mean procedure time for SA was 10 minutes. The duration of the surgery was 40 to 90 minutes per each level of disc herniation. The mean blood loss was 350 cc (200 to 600 cc). The most common involved level was L4/L5 intervertebral disc (n = 40 patients;63.5%). The mean recovery time was 20 minutes. Only three patients requested more analgesics for relief of their pain postoperatively. All patients with discectomy were discharged a day after surgery, and in the case of fusion, two days after surgery. All the patients were followed up for six months, showing no recurrence symptoms, good pain relief, satisfaction with the surgery, and no bad memory of the surgery. Conclusion(s): Spinal anesthesia is a good alternative or even the main anesthesia route for patients with lumbar disc herniation. More studies are needed to elucidate the best candidate for SA in patients with lumbar pathology.Copyright © 2023, Author(s).
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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.
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Case Report: This is a 50-year-old man that presented to the ED complaining of generalized weakness and acute loss of ability to ambulate which has been progressing for a month. Patient began having left arm and leg weakness, which started in his fingertips of his left upper extremity and soon moved proximally to upper left arm. Symptoms then progressed to right upper and lower arms. Symptoms further continued to progress making the patient bedridden. On presentation, CT head showed a C1/C2 subluxation possibly chronic without significant focal soft tissue swelling. CT cervical spine showed C1-C2 subluxation, possibly chronic. MRI of brain was unremarkable pre and postcontrast without focal findings or abnormal enhancement and showed redemonstration of the C1-C2 subluxation as described on CT scan. MRI of cervical spine showed at the level of C1 there is spinal canal stenosis. However, there is no direct pressure upon the cord/medulla. Upon evaluation, patient had significant motor weakness and required maximal assistance for movement. Patient was moreover noted to have flaccidity of muscles associated with weakness with no bulbar weakness. Patient had no difficulty in breathing or with speech. A lumbar tap was performed which showed elevated protein, WBC, and glucose. Upon further investigation, patient stated that he received his (3rd dose) of the Moderna Vaccine for Covid-19 about a month before the onset of symptoms and felt fine. Two weeks later, he began experiencing subjective fevers, diarrhea, abdominal pain, and fatigue that lasted for a week and then self-resolved. Approximately another two weeks later is when patient began noticing his neurological symptoms. Possible Guillain-Barre Syndrome post Campylobacter Jejuni (C. Jejuni) infection vs. post Covid-19 vaccine induced GBS was suspected at this point and patient was started on Intravenous Immunoglobulin (IVIG). Stool cultures were collected for C.Jejuni which came back negative. Gastrointestinal Pathogen Panel PCR Feces also came back negative. Patient was discharged to a rehab center and planned to receive another round of IVIG for 5 days. Conclusion(s): Guillain Barre Syndrome (GBS) is a rare immune-mediated neurological disorder affecting peripheral nerves and nerve roots, that presents as acute sensorimotor neuropathy starting with distal paresthesia that progresses to weakness of legs and arms, noteably, flaccid paralysis. GBS has several triggers namely infections such as C. jejuni, cytomegalovirus, M. pneumoniae, Epstien-Barr virus and Zika virus. There has also been several case reports and studies that have shown increased incidence of GBS vaccines such as influenza vaccine. Furthermore, there has been several studies that have linked GBS to COVID-19 vaccine. With COVID-19 cases continuing to persist, and increasing advocacy for vaccination against the disease, GBS should be considered as very rare but possible side effect of the vaccine.
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Background: Severe acute respiratory syndrome coronavirus 2 (SARSCoV2) has been associated with several neuro-ophthalmic manifestations. We report a case of bilateral longitudinally extensive optic perineuritis suspected due to SARSCoV2. Case Presentation: A 32-year-old woman developed headaches, photophobia, pulsatile tinnitus, and blurred vision 8 d after having a positive SARS-CoV-2 qualitative polymerase chain reaction (PCR) testing for coronavirus disease 2019 (COVID-19). She was diagnosed with and treated for idiopathic intracranial hypertension (IIH) elsewhere. Repeat evaluation at our institution showed a poor visual acuity in both eyes with Frisen grade II papilledema and cotton wool spots on fundoscopic examination. Orbital magnetic resonance imaging (MRI) showed bilateral longitudinally extensive optic nerve sheath enhancement. Repeat lumbar puncture revealed an elevated cerebrospinal fluid (CSF) opening pressure and protein, a finding that is incompatible with the diagnosis of IIH. Myelin oligodendrocyte glycoprotein, aquaporin-4 (AQP4)-IgG antibodies, and other serological tests for optic neuritis were unremarkable. Her visual acuity partially improved after corticosteroids. With the growing association of demyelinating disorders and COVID-19, unremarkable serological workup, and temporal relation of the patient's symptoms to the infection, we believe that her diagnosis is SARS-CoV-2 associated bilateral optic neuritis. Conclusion(s): There is a growing association between demyelinating disorders and COVID-19 and COVID-19 vaccination, and it is essential to recognize CSF abnormalities that are incompatible with a diagnosis of IIH, such as increased protein in our case, and may lead to an incorrect diagnosis.Copyright © 2023 The Authors. Clinical and Experimental Neuroimmunology published by John Wiley & Sons Australia, Ltd on behalf of Japanese Society for Neuroimmunology.
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Since the introduction of COVID-19 vaccine, various adverse events have been reported including injection site pain, fatigue, headaches, and myocarditis. Cranial neuropathies and optic neuritis, have been also rarely reported, however, the significance of these autoimmune manifestations after the administration of COVID-19 vaccine remain controversial. In this report we present a case of myocarditis and bilateral optic neuritis that occurred in a young healthy male patient after the administration of first dose of mRNA-1273 vaccine (Moderna).Copyright © 2022 The Author(s)
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Serotonin syndrome associated with clozapine withdrawal and concurrent selective serotonin reuptake inhibitor (SSRI) use has previously been reported. A 56-year-old female with schizophrenia was admitted for pyrexia, rigidity, and altered mental state after her second dose of clozapine restart. She had discontinued her long-term clozapine 2 weeks prior. She developed ventilatory failure, reduced consciousness, eye deviation, and worsening rigidity, requiring ICU support. Examination showed a right upper motor neurone syndrome with absent ankle reflexes. She had raised inflammatory markers and creatine kinase. Serum neuropathy, encephalitis screen, and COVID PCR were negative. Respiratory investigations were unfruitful. MRI head and spine did not show brain or cord signal change to correlate to signs. Lumbar puncture showed a quiet CSF, negative culture, viral PCR, and encephalitis antibodies. EEG showed bihemispheric background slowing. Despite clinical improvement, repeat examination showed persistent signs. She was diagnosed with serotonin syndrome after developing a bilateral tremor. Treatment with cyproheptadine correlated with an improvement in her signs, cognitive state, and EEG. Serotonin syndrome can present with reversible neuromuscular signs. With clozapine withdrawal, it can require a prolonged time course of recovery in contrast with classical serotonin syndrome. Cyprohepta- dine can cause agranulocytosis and this delays clozapine restart.
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Background The incidence of severe bacterial infections (SBIs) in infants aged ≤90 days is thought to have decreased because of widespread vaccination programs. However, relevant epidemiological data in Japan are scarce. Materials and methods This observational, single-center study investigated the epidemiology of fever in infants aged ≤90 days. SBI was defined as the presence of meningitis, urinary tract infections (UTIs), or bacteremia. Invasive bacterial infection (IBI) was defined as the presence of meningitis, bacteremic UTI, or bacteremia. We determined the incidence of UTIs, bacteremia, meningitis, SBIs, and IBIs in the following three age groups: 0-28, 29-60, and 61-90 days. We subsequently calculated the relative incidence for the groups aged 29-60 and 61-90 days, using the group aged 0-28 days as the reference group. Results Herein, 58, 124, and 166 infants were included in the 0-28 days, 29-60 days, and 61-90 days age groups, respectively. Of the total number of patients, 15.5%, 8.9%, and 16.9% in the 0-28 days, 29-60 days, and 61-90 days age groups, respectively, were diagnosed with SBI. The relative incidences were 1 for the 0-28 days group (reference group), 0.67 for the 29-60 days group (95% confidence interval [CI], 0.39-1.15), and 1.08 for the 61-90 days group (95% CI, 0.58-2.00). Of the total number of patients, 10.3%, 3.2%, and 0.6% in the 0-28 days, 29-60 days, and 61-90 days age groups, respectively, were diagnosed with IBI. Relative incidences were 1 (reference group), 0.50 (95% CI, 0.29-0.88), and 0.28 (95% CI, 0.19-0.41) for the 0-28 days, 29-60 days, and 61-90 days age groups, respectively. All cases of IBI were caused by Group B streptococcus (GBS), except for two cases of bacteremia, which were caused by Haemophilus influenzae. Conclusion The incidence of SBI was similar in the 0-28 days and 61-90 days age groups. However, the incidence of IBI decreased with increasing age. The incidence of UTIs was highest in the 61-90 days age group, and that of meningitis and bacteremia decreased with increasing age.
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We present a rare case of meningoradiculitis occurring after mRNA COVID-19 vaccination. This patient, with a history of inflammatory arthritis following rubella vaccination, presented to the emergency department 4 days after her vaccination with both central and radicular nervous system symptoms. Symptoms included pain, sensory and motor deficits in L5 roots distribution, along with signs of central irritation, such as headache, difficulty concentrating and a Babinski sign. MRI showed bilateral L5 nerve roots enhancement. Lumbar puncture showed elevated protein and IgG, and relevant serologies excluded common causes. Prednisone and physical therapy helped the patient to achieve near complete recovery nine weeks after presentation. We concluded that this patient presented meningoradiculitis probably secondary to her vaccination in a context of possible overactive immune system. While such presentations might be rare, and do not constitute a general reason to abstain from vaccination, they must be well recognized and treated.Copyright © 2022
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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
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Background: Neurological autoimmune disorders are often triggered by bacterial and viral infections, with growing evidence supporting coronavirus disease 2019 (COVID-19) infection precipitation of these disorders. COVID-19 is already implicated in causing discrete para-infectious neurological syndromes: acute disseminated encephalomyelitis (ADEM), transverse myelitis, neuromyelitis optica spectrum disorders (NMOSD), Guillain-Barre syndrome (GBS), and is also associated with encephalopathy, acute cerebrovascular disease, neuromuscular disorders, and seizures. Case Presentation: We describe a case of a 43-year-old Asian woman with chronic Hepatitis B (HBV) co-infected acutely with COVID-19, presenting with urinary retention, bilateral blindness, thoracic sensory level, and quadriparesis. Extensive workup narrowed down her diagnosis as seronegative NMOSD. She had complete resolution of symptoms after treatment with concurrent plasma exchange (PLEX), high dose corticosteroids, and emtricitabine-tenofovir. Follow-up visit showed no seroconversion at 6 months and no relapses. Conclusion(s): Our literature review highlights the likely link between COVID-19 infection and the development of neurologic autoimmune diseases. Our literature review supports a virus-triggered immune-mediated process rather than neuro-invasion. Many viral illnesses have been linked to the development of NMOSD and anti-AQP4 antibody-related myelitis. Additionally, there is limited literature linking chronic HBV infection with the development of optic neuritis and speculation thatcross-reactivity between HBsAg and myelin antigens may lead to the development of demyelinating diseases in the CNS and PNS. We observed remarkable clinical improvement after treatment with alternating days of IV methylprednisolone and therapeutic PLEX.Copyright © 2022
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Background: There has been lot of speculation around the possible side effects associated with COVID vaccination and incidence of facial palsy is one of them. Bilateral facial palsy is less likely to be idiopathic as compared to unilateral facial nerve palsy and warrants further investigations to find any secondary cause. COVID 19 infection and the vaccinations for the same are also included in the unique list of differentials. Case report: We report an interesting case of bilateral rapidly sequential facial nerve palsy following the administration of COVID vaccination that showed subsequent improvement. We provide literature review to report the current incidence of same, secondary to the vaccination as well the infection itself Case presentation: Following the introduction of COVID 19 vaccine, there have been reports of various cranial nerve involvement including lower motor neuron type facial paresis. Bilateral facial palsy is less likely to be idiopathic as compared to unilateral palsy(23% vs 70%) and requires further work up to determine the etiology before determining to be idiopathic. Unilateral facial palsy(FP) has been reported in the Phase I and II trials for Pfizer and Moderna vaccine, with a total of 7 cases reported in these initial trials. To date, there is no direct evidence that these vaccines have increased the incidence of facial palsy as compared to adverse events reported with other vaccines or compared to COVID 19 infection itself. We report a unique case of bilateral lower motor neuron type facial palsy noted in a young male within hours of receiving the vaccine that later improved with treatment. Reports of simultaneous bilateral facial palsy after vaccine are rare with only few cases reported to date in literature. Conclusion(s): In conclusion from current available literature, we would like to postulate that though there is a risk of facial nerve palsy following the vaccination, it is comparable to the risks associated with any other vaccinations and not been higher than the non-vaccinated population. The overall risk is higher with the actual COVID 19 infection itself as compared to the vaccine.Copyright © 2022
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Background: Neuromyelitis optica spectrum disorder (NMOSD) is a rare autoimmune disease, targeting the central nervous system, rarely associated with vaccination. Case report: We report a case of a 47 year-old healthy woman who presented, ten days after the first dose of SARS-CoV-2 Vaccine AstraZeneca (Vaxzevria), with back pain, tetraplegia, urinary retention, dysarthria and dysphagia. The patient was diagnosed NMOSD. She underwent intravenous-corticosteroids, vein-Immunoglobulin and plasma-exchange without significant improvement . Conclusion(s): The absence of any possible related conditions, the temporal relation with anti-SARS-CoV-2 vaccination, suggest that, in our case, NMOSD may be due to the cross reaction from Vaxzevria.Copyright © 2021 The Author(s)
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Seventy-three-year-old diabetic male was a high-risk transfer from Alaska for respiratory decompensation in the setting of progressive bulbar and proximal weakness. He was diagnosed with COVID-19 two months prior and viral mononucleosis 1 month prior to presentation. While the patient had a fall 3 months prior to presentation, and decreased mobility at home, there was abrupt onset of progressive upper/lower extremity weakness, dysphagia, and difficulties managing secretions 2 weeks prior to presentation. Initial exam was notable for MRC 3-4/5 proximal upper/lower extremity weakness, areflexia, and negative inspiratory force of 224 to 230 cm H20. A subtle periorbital heliotrope rash was documented. Lumbar puncture demonstrated albumino-cytologic dissociation (protein 142 mg/dL, 6 WBCs) and CK remained elevated (1930 U/L) despite intravenous hydration. Outside electrodiagnostic testing demonstrated a sensorimotor axonal neuropathy with questionable myopathic features on needle electromyography. Given concern for an inflammatory neuropathy and concomitant inflammatory myopathy, intravenous immunoglobulin 2G/kg and IV methylprednisolone 1G/day over 5 days was started. He was transferred for further diagnostic workup and supportive care 6 days after presentation and required intubation within 24 hours of admission. Exam showed progressive proximal and distal weakness of the extremities and general areflexia/hyporeflexia. Repeat electromyography confirmed a severe sensorimotor axonal polyneuropathy without acquired demyelinating features and normal repetitive nerve stimulation. While the patient could no longer activate muscles voluntarily, proximal muscles had increased spontaneous activity with predominant myotonia. Neuroaxis imaging was notable only for enhancement of the lumbar nerve roots. Combined vastus lateralis muscle biopsy and serologic testing confirmed a second pathologic process contributing to the patient's weakness. This case highlights the cooccurrence of 2 distinct neuropathological entities, with potential relation to a prior viral infection, and the importance of ancillary testing to guide treatment for acute causes of neuromuscular respiratory failure.
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Case Report: Mediastinal masses are rare with an incidence of 1 in 100 000 [1]. While the differential is broad, the risk of malignancy is higher in the pediatric population. Lymphomas account for about 50% of mediastinal masses [2].We present a patient with superior vena cava (SVC) syndrome from a mediastinal mass, concerning for lymphoma. After extensive work up, the mass was determined to be reactive. Case Presentation: A 5-year-old male, presented with one day of left sided face and neck swelling. Review of systemswas positive for a fewweeks of cough but notably negative for night sweats, fatigue, fever, or weight loss. Computed tomography (CT) scan (Figure 1, left) showed a heterogeneous mass, most concerning for lymphoma. Blood work was notable for lymphopenia (640 x 103/uL), elevated lactate dehydrogenase and uric acid (549 U/L and 7.1 mg/dL respectively). He tested positive for SARs- CoV2 RNA on nasopharyngeal PCR. Upon admission, he was started on methylprednisolone and allopurinol. A bone marrow biopsy and a lumbar puncture were unrevealing for immunophenotypic evidence of lymphoid neoplasm. A mediastinal biopsy showed fibrosis with patchy inflammation and inadequate number of viable cells to allow for flow cytometric analysis. A post-biopsy echocardiogram revealed a moderate sized pericardial effusion which eventually resolved. He was discharged with infectious disease and oncology follow up. Later, histoplasma and bartonella antibodies, and T spot were negative. A CT (Figure 1, right), ten days after initial presentation showed significant decrease in size of the mediastinal mass. At one month follow up, he remained clinically well with a normal chest x-ray. [Figure presented] Fig 1: A CT ten days after initial presentation showed significant decrease in size of the mediastinal mass Conclusion(s): This patient presented with SVC syndrome from a mediastinal mass that resolved with 3 days of intravenous steroids. The initial presumed diagnosis of lymphoma was ultimately inconsistent with the extensive workup, and the mass was ultimately deemed reactive. COVID-19 related mediastinal mass is not described in the literature, and although possible, remains unlikely. This case represents the importance of avoiding premature closure and keeping a broad differential diagnosis. 1. Park DR, Vallieres E. The mediastinal mass. Murray and Nadel's Textbook of Respiratory Medicine. 5th edn. Philadelphia, PA: Saunders;2010. pp. 1814-35. 2. Glick R. D., & La Quaglia M. P. (1999). Lymphomas of the anterior mediastinum. Seminars in Pediatric Surgery, 8(2),69-77.Copyright © 2023 Southern Society for Clinical Investigation.
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Background: Cerebral amyloid angiopathy related inflammation (CAA-RI) is a neuroinflammatory disease that is associated with perivascular amyloid- deposition. Case presentation: A middle-aged woman with a remote history of autoimmune disorders presented with unilateral migraine headaches, dizziness, unsteadiness, and fogginess 36 hours after administration of mRNA vaccine against SARS-CoV-2. Initially, unilateral leptomeningeal enhancement on MRI on the same side of headaches raised suspicion for leptomeningeal involvement of her known cutaneous T-cell lymphoma in remission. After two relatively unremarkable CSF analyses, she underwent a brain biopsy which showed amyloid deposits in vessels instead of lymphomatous infiltration. She was diagnosed with CAA-RI, and the headache and cognitive symptoms responded well to high-dose corticosteroids with a slow taper. Discussion/conclusion: We review the clinical literature of CAA-RI and its potential association with amyloid-related imaging abnormalities (ARIA) after administration of immunotherapy against amyloid.Copyright © 2022
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Introduction: Post-COVID-19 autoimmune encephalitis is a rare manifestation following COVID-19. Most cases have not demonstrated solid evidence regarding their pathogenesis. Some believe it to be an immune process. Case presentation: In this case report, we present a case of a young female who presented to our emergency department with visual, auditory, and olfactory hallucinations after successfully treating COVID-19 two weeks prior to this visit. On examination, her vital signs were stable, but she was agitated, distressed, and hallucinating. Neurological examinations were normal. Laboratory investigations, including autoimmune profiles, were all negative. Magnetic resonance imaging of the brain showed non-specific changes in the bilateral frontal area. Electroencephalography (EEG) showed lateralized rhythmic delta activity (LRDA) arising more from the right occipital lobes. Autoimmune psychosis was suspected due to psychosis, abnormal imaging, and abnormal EEG findings. She was given corticosteroids and antipsychotic medication. Her symptoms improved within ten days. On follow-up, she remained well without any return of psychosis. Conclusion(s): Possible autoimmune pediatric encephalitis following COVID-19 is a rare entity that has scarcely been reported. The majority of the cases were reported to have been related to stress following the infection. To establish the correct diagnosis, an extensive workup, including an autoimmune profile, lumbar puncture, magnetic resonance imaging, and electroencephalography, is recommended.Copyright © 2022 The Author(s)