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Case Report: Cryptococcosis is an opportunistic infection caused by the encapsulated yeast Cryptococcus, with C. neoformans and C. gattii being the most common species to cause human disease. Immunocompromised individuals are predisposed to infections with C. neoformans, which has known predilection to CNS and pulmonary lymph nodes. We present a unique case of disseminated cryptococcosis in the setting of end-stage renal disease (ESRD), cirrhosis, tumor necrosis factor inhibitor use and steroid use for COVID19. Method(s): A single-patient case report was conducted after IRB approval. Case Presentation: A 55-year-old woman with uncontrolled diabetes, lupus, rheumatoid arthritis on adalimumab, hepatitis C status post boceprevir, cirrhosis, former IV drug use, and ESRD on hemodialysis via bovine arterial-venous fistula graft presented with worsening dyspnea, cough, and altered mental status. Three months prior, patient was admitted to an outside hospital for COVID19, complicated by pulmonary embolism status post anticoagulation therapy. Patient was treated with an unknown steroid regimen, which was continued by a second outside facility when symptoms failed to improve. Patient then presented to our facility 24 hours after discharge due to continued symptoms. On admission, patient was noted to have altered mentation and hypoxia with pulmonary edema on chest x-ray and was urgently hemodialyzed. Further work-up was obtained due to non-resolving symptoms, including blood and sputum cultures, cocci serology and QuantiFERON gold. CT chest revealed bilateral consolidations. Patient was started on antibiotics for presumed hospital-acquired pneumonia. During the hospital stay, preliminarily blood cultures grew yeast and patient was started on Micafungin. However, Micafungin was changed to Liposomal Amphotericin B as ovoid structures seen on gram stain could not confirm nor rule out cryptococcus. Subsequent bronchial wash and bronchoalveolar lavage cultures, as well as final blood cultures resulted Cryptococcus neoformans. Serum cryptococcus antigen returned reactive, titer 1:512. Antibiotics were discontinued and Isavuconazonium was started with Liposomal Amphotericin B. Due to recurrent headaches, lumbar puncture was obtained and revealed lymphocytic pleocytosis without cryptococcal antigenicity. Patient completed 14 days of Liposomal Amphotericin B and Isavuconazole with continuation of Isavuconazole upon discharge. Conclusion(s): Disseminated cryptococcosis in non-HIV patients is rare in the modern HIV era. Clinicians should be aware and include it in their differential of any patient with multiple risk factors for opportunistic infection. In patients with cirrhosis and ESRD, treatment is limited given altered pharmacokinetics. Studies have shown improved survival with the addition of Isavuconazole in patients with disseminated cryptococcosis with CNS involvement in the setting of chronic liver disease and ESRD.
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Background: Large-scale vaccination against the novel coronavirus (COVID-19) occurred globally at an unprecedented pace. Sporadic cases of autoimmune encephalitis (AE) have been reported following COVID-19 vaccination, mainly in adults. Case report: A 14-year-old girl developed altered mental status and was brought to our emergency department because of a seizure 19 days after receiving the third dose of COVID-19 vaccination. She was treated with steroid pulse therapy and fully recovered. The diagnosis of probable autoantibody-negative AE was finally made. Conclusion(s): This case met the criteria for probable autoantibody-negative AE in children, as well as adults. Because of the temporal association and absence of another identifiable cause, her conditions may have been triggered by the COVID-19 vaccination. To our knowledge, this is the first published pediatric case of autoantibody-negative but probable AE following COVID-19 vaccination.Copyright © 2023 The Authors
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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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Case Report: Although the coronavirus disease 2019 (COVID-19) affects the respiratory system, neurological complications in children have been reported. Neurological manifestations in children with acute COVID-19 infection are rare and range from headaches, transverse myelitis, strokes, and encephalitis which presents as a part of Multisystem Inflammatory Syndrome in Children (MIS-C). However, encephalitis presenting post-COVID-19 in the absence of MIS-C in children has not been described. Case presentation: A 9-year-old Hispanic female with no past medical history presented with altered mental status and seizures. Associated symptoms prior to seizures included worsening headaches and vomiting. Initial labs were significant for an elevated erythrocyte sedimentation rate of 32 mm/hr, C-reactive protein of 2 mg/dL, and white blood cell (WBC) count of 28 000 cells/mcl with neutrophilia. Comprehensive metabolic panel was normal. Computed tomography of the head and urine drug screen were normal. Magnetic resonance imaging of the brain demonstrated diffusion restriction in the left frontal lobe as well as mild leptomeningeal enhancement concerning for meningoencephalitis. Lumbar puncture (LP) showed pleocytosis (WBC 169 cells/mcl, 76% neutrophils), elevated glucose 77 mg/dl, normal protein 56 mg/dl, and elevated myelin basic protein indicative of a demyelinating disease. Infectious workup was significant for a positive COVID-19 immunoglobulin (Ig) G (19.66), positive Mycoplasma pneumoniae (M. pneumoniae) IgM (0.87 units/L), with an equivocal IgG (0.11 units/L). Autoimmune workup was negative. She received dexamethasone 0.15 mg/kg/dose for 1 day, followed by methylprednisolone (10 mg/kg/dose) for 3 days and oral prednisone for 5 days resulting in significant improvement. Although CSF cultures returned negative, she received a 7-day course of doxycycline for a possible coexisting M. pneumoniae infection. Repeat LP showed improving pleocytosis, and lymphocytic predominance. Discussion: In this case report, rapid neurological recovery after administration of corticosteroids in the presence of positive COVID-19 IgG and demyelinating disease was suggestive of encephalitis presenting post- COVID-19 infection. Although M. pneumoniae can present with neurological symptoms (e.g., encephalitis), repeat titers at follow-up after recovery did not show the expected 4-fold increase in IgG, making it less likely the cause of this presentation. The proposed pathophysiology of COVID-19-mediated encephalitis includes direct invasion of the nervous system, immune-mediated cytokine response, and molecular mimicry between coronaviruses and neuronal proteins causing demyelination. The mainstay treatment includes immunomodulators such as corticosteroids, Intravenous Immunoglobulin, monoclonal antibodies (eg., rituximab), or plasma exchange. Conclusion: COVID-19 infection should be considered when evaluating a patient with meningoencephalitis or post-infectious encephalitis.
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Background: Autoimmune Syndrome Induced by Adjuvants, or ASIA, suggests certain environmental exposures, including vaccination can cause hyperstimulation of the innate and adaptive immune system leading to production of autoantibodies in a genetically predisposed individual. A diagnosis of exclusion, proposed diagnostic criteria suggested ASIA if specified major and minor criteria are fulfilled. Suspicion for ASIA was raised in our patient due to identified exposure accompanied by typical manifestations not explained by another cause. Case: A 71-year- old Filipino female with controlled hypertension and diabetes, came in due to progressive right eye pain, supraorbital headache, ptosis and limitation of extra-ocular movements for 3 weeks. No blurring of vision, color vision changes, or visual field cuts. She didn't have other systemic features but received 2 doses of inactivated COVID-19 vaccine 1 month (1st) and 1 day (2nd) prior to the symptom onset. The left eye was unremarkable. ESR was elevated (109) with normal CRP. ANA was 1:80 with a speckled pattern. The complements were normal and lupus confirmatory panel was negative. CSF studies showed slightly elevated protein and glucose with no pleiocytosis, IgG level was normal with negative oligoclonal panel and cultures. EMG-NCV showed acute partial incomplete bilateral facial neuropathy. Cranial MRI/MRA showed chronic lacunar infarct in the right corona radiata. The MRI of the orbits showed right optic nerve enhancement with hyperintense nerve sheath compatible with optic neuritis. She underwent pulse IV steroid therapy (Methylprednisolone 1 g) for 3 days and was maintained on oral steroid 1 mg/kg/day. There was minimal improvement of symptoms for which she received intravenous immunoglobulin for 5 days. Her symptoms gradually improved upon discharge. Conclusion(s): Identification of the possible autoimmunity from adjuvants is not to discourage vaccination but rather raise awareness of the need for further studies to screen who might be at risk and to prepare or even develop alternatives, such as vaccines with a different type of adjuvant.
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Case Report: Acute transverse myelitis (TM) is a rare inflammatory disease that typically presents asweakness, sensory alterations, and bowel or bladder dysfunction. Among the causes of TM are infections, paraneoplastic syndromes, or autoimmune conditions of CNS. Postinfectious TM can develop secondary to a viral or bacterial infection. SARS-CoV-2 is a recently discovered viral illness, and sequelae due to COVID-19 infection are still being studied. There is scarce literature relating the two conditions, and it is imperative to raise awareness. A 72-year-old man with hypertension and GERD, completely independent in ADL, was brought to the ED with sudden onset of bilateral lower extremity weakness. He reported symptoms started with difficulty climbing stairs that rapidly progressed to inability to ambulate independently and were associated with bilateral thigh soreness. Nine days prior, he developed fever and generalized malaise, and two days later, SARS-CoV-2 PCR and Ag tests were positive. He received azithromycin, Paxlovid, and dexamethasone as treatment. Upon evaluation, the patient was afebrile and hemodynamically stable. Neurological examination was remarkable for spasticity and hyperreflexia at bilateral lower limbs, clonus, preserved motor strength with adequate sensation to soft touch, and intact vibration and proprioception in all extremities. Cranial nerves were intact. These findings were consistent with an upper motor neuron lesion. On imaging, the Head CT scan was unremarkable. Thoracic/Lumbar Spine MRI was significant for distal thoracic and conus areas with central homogeneous brightness compatible with nonspecific myelitis. Laboratories showed leukocytosis without neutrophilia or bandemia, thrombocytosis, and elevated CRP. HIV and RPR tests were negative. A lumbar puncture for CSF analysiswas remarkable for mild monocytic pleocytosis (7 cell/muL), an increased level of total proteins (56 mg/dL), and normal glucose (57 mg/dL). CSF culture and gram stain were negative. CSF cytology yielded few lymphocytes and few monocytes and was negative for malignant cells. The meningoencephalitis panel was negative. Based on these findings, a clinical diagnosis of postinfectious myelitis secondary to COVID-19was made. The patient was treated with intravenous Methylprednisolone 1 g daily for five days. On follow-up, lower extremity weakness resolved completely, and he resumed his daily physical activities. Patients with COVID-19 infection can present with neurologic manifestations such as headache, myalgias, dizziness, dysgeusia, and anosmia. This case hopes to raise awareness of less commonly known neurological manifestations of SARS-CoV-2 infection and how the early recognition of symptoms can help expedite the diagnosis and treatment of the condition to avoid long-term sequelae. [Figure presented] Copyright © 2023 Southern Society for Clinical Investigation.
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Case Report: A 4-year-old African American male presented to an outside emergency department (ED) following sudden inability to move left upper extremity. Past medical history was unremarkable and routine vaccinations were up to date. Radiograph of affected extremity ruled out fractures and patient was discharged to follow up with primary care physician. Two days later mother brought him to our ED due to persistent left upper extremity paralysis, poor appetite, and subjective fever. On exam his left arm was warm and tender to dull and sharp touch;he had definite loss of active movement, hypotonia and absence of deep tendon reflexes. The patient had winging of left scapula and could not shrug left shoulder. MRI of cervical and thoracic spine showed enlargement of spinal cord from C2-C6 level with gray matter hyperintensity, slightly asymmetric to the left. Laboratory studies showed leukocytosis (14 000/mcL) and CSF studies showed pleocytosis of 89 WBC/mcL (93.3% mononuclear cells and 6.7% polymorphonuclear cells), 0 RBCs, normal glucose and protein, and a negative CSF meningoencephalitis multiplex PCR panel. Due to high suspicion of demyelinating or autoimmune condition he was treated with high dose steroids and IVIG. Subsequently neuromyelitis optica was ruled out as aquaporin-4 receptor antibodies (AB) and myelin oligodendrocyte glycoprotein AB were normal. CSF myelin basic protein and oligoclonal bands were absent ruling out demyelinating disorders. CSF arboviruses IgM and West Nile IgM were negative. He showed minimal improvement in left upper extremity movement but repeat spinal cord MRI one week later showed improved cord thickness with less hyperintensity. Respiratory multiplex PCR was negative including enteroviruses. Repeat CSF studies after IVIG showed increased IgG index and IgG synthesis suggestive of recent spinal cord infection, consistent with acute flaccid myelitis (AFM). Pre-IVIG blood PCR was invalid for enteroviruses due to PCR inhibitors found in the sample. Blood post-IVIG was negative for mycoplasma IgM, West Nile IgM, and arboviruses IgM. Enterovirus panel titers (post-IVIG) were positive for coxsackie A (1:32), coxsackie B type 4 (1:80) and 5 (1:320), echovirus type 11 (1:160) and 30 (1:80) as well as positive for poliovirus type 1 and 3. These titers could not distinguish acute infection from patient's immunity or false-positives as a result of IVIG. He was discharged with outpatient follow-up visits with neurology, infectious disease, occupational and physical therapy, showing only mild improvement after discharge. Discussion(s):With the anticipated resurgence of AFM after the peak of COVID-19 pandemic, our case illustrates the need to consider this diagnostic possibility in patients with flaccid paralysis. It is important to remember CSF IgG synthesis is not affected by IVIG. In addition when treatment plans include IVIG, appropriate samples should be collected before IVIG to facilitate accurate work-up for infectious diseases. Copyright © 2023 Southern Society for Clinical Investigation.
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SESSION TITLE: Issues After COVID-19 Vaccination Case Posters SESSION TYPE: Case Report Posters PRESENTED ON: 10/19/2022 12:45 pm - 01:45 pm INTRODUCTION: Thymoma-associated autoimmune encephalitis (TAAE) is an understudied and overlooked diagnosis in patients presenting with abrupt altered mental status. Described as inflammation of brain tissue, autoimmune encephalitis is seen in 5-10 cases per 100,000 across all age groups per year. A rare subtype involves neuronal surface antibodies to alpha-amino-3-hydroxyl-5-methyl-4isoxazolepropionic acid receptors (AMPA-R) encephalitis is seen even less commonly. Given the "unicorn” nature of presenting cases and difficulty of diagnosis, prompt identification and treatment are critical as prolonged courses without treatment are irreversible and deadly. CASE PRESENTATION: A 47-year-old male with no past medical history presented 3 days after a Johnson & Johnson coronavirus-2019 (COVID-19) booster vaccine due to worsening acute altered mental status over the past week. He complained of episodes of fever & chills prior to this. The patient's wife reported abrupt changes in memory and personality. Upon admission, the patient had a Glasgow Coma Scale of 4. The patient was intubated and transferred to the intensive care unit. Intravenous (IV) vancomycin, ceftriaxone and acyclovir was initiated for meningitis. Computed tomography (CT) scan of the head without contrast was unremarkable. Magnetic resonance imaging (MRI) showed enhancements of the right anterior and medial temporal lobe suggesting encephalitis. Cerebrospinal fluid analysis (CSF) revealed lymphocytic pleocytosis. A CT scan of the chest, abdomen and pelvis showed an anterior mediastinal mass measured 1.8 x 2.3 cm (Figure 1). FilmArray Meningitis polymerase chain reaction was negative as well as Herpes Simplex Virus (HSV) 1 and 2. Autoimmune encephalitis antibody was positive for Anti-AMPAR. Pulse dose steroids and intravenous immunoglobulin were initiated but failed. Rituximab was initiated and cardiothoracic surgery completed a thymectomy. DISCUSSION: TAAE is a rare disease, permanently debilitating, and deadly if unrecognized or treatment is delayed. Autoimmune encephalitis is an umbrella disease process seen in 0.00005% of patients per year. AMPA-R positive encephalitis is even less commonly seen with only 22 cases reported between the years 2009 and 2014 [1]. A rapidly progressive cognitive decline or psychiatric disorders are early features of this disease.Our patient had prodromal symptoms of fever and cognitive decline days after receiving his COVID-19 booster vaccine. CONCLUSIONS: Post-vaccine encephalomyelitis has been described in other settings[2]. This patient was free of symptoms prior to the COVID-19 vaccine booster, and demonstrated altered mental status hours after receiving it. This furthers the possibility of an association of the COVID-19 booster vaccine, development of encephalitis, and in this case a thymoma. Despite this, conclusions can not be made on the account of one report, but introduces a new area of focus to study. Reference #1: Höftberger, R., van Sonderen, A., Leypoldt, F., Houghton, D., Geschwind, M., Gelfand, J., Paredes, M., Sabater, L., Saiz, A., Titulaer, M. J., Graus, F., & Dalmau, J. (2015). Encephalitis and AMPA receptor antibodies: Novel findings in a case series of 22 patients. Neurology, 84(24), 2403–2412. https://doi.org/10.1212/WNL.0000000000001682 Reference #2: Huynh, W., Cordato, D. J., Kehdi, E., Masters, L. T., & Dedousis, C. (2008). Post-vaccination encephalomyelitis: literature review and illustrative case. Journal of clinical neuroscience : official journal of the Neurosurgical Society of Australasia, 15(12), 1315–1322. https://doi.org/10.1016/j.jocn.2008.05.002 DISCLOSURES: No relevant relationships by Matthew Frank No relevant relationships by Justin Ilagan No relevant relationships by Danielle Mahon No relevant relationships by Danielle Mahon No relevant relationships by Harshini Sahani No relevant relationships by Kameron Tavakolian No relevant relationship by Ndausung Udongwo
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Since countries commenced COVID-19 vaccination around the world, many vaccine-related adverse effects have been reported. Among them, short-term memory loss with autoimmune encephalitis (AE) was reported as a rare adverse effect. Since case numbers are limited, this brief report may draw the attention of the medical community to this uncommon adverse effect and serve as a reference for future vaccine improvement. However, given the high risk of adverse outcomes when infected with SARS-CoV-2 and the clearly favorable safety/tolerability profile of existing vaccines, vaccination is still recommended.
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Background and Objective: The novel coronavirus SARS-CoV-2 has become a global health emergency. Methods: We report two patients with similar atypical neurological presentation of novel coronavirus SARS-CoV-2 infection admitted in May 2021. Results: A 60-year-old man and a 44-year-old woman were admitted for acute left hemiparesis, respectively unusual headache with photo-, phonophobia. The diagnosis of COVID-19 was made by a positive nasopharyngeal swab RT-PCR. The initial MRI showed for both patients an aspect of unilateral ventriculitis associated with periventricular vasogenic edema and serpiginous periventricular hypo signal in SWI. Both presented in the cerebrospinal fluid, neutrophil pleocytosis WBC 354/mm3, neutrophils 91% and lymphocytes 6% for the first patient and WBC 3000/mm3, neutrophils 91%, lymphocytes 0% for the second patients, associated with hypoglycorrhachia 2,5 mmol/L compared to 7,5 mmol/L glycemia and 2,7 mmol/l compared to 5,9 mmol/L glycemia, respectively. Empirical antibiotic treatment was started, but due to lack of argument for an infection on two consecutively lumbar punctures, the treatment was stopped. Favorable evolution was observed on corticosteroid treatment. The follow-up MRI showed a net reduction of the initial findings for both patients. Conclusion: The presented cases seem to be comparable with the first case of ventriculitis in a patient with SARS-CoV-2 reported by Moriguchi et al in 2020. The particularity of our cases is given by the serpiginous hypo signal in SWI, that was not described in the case report mentioned before. The favorable clinical and radiologic evolution on corticosteroid treatment suggests a probable inflammatory mechanism secondary of COVID.
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Objective: We aim to report clinical characteristics of an extremely rare case of myelitis with Guillain-Barré syndrome (GBS) and cerebellar ataxia (CA) after COVID-19 infection. Background: There have been many reports about neurological complications following the world pandemic of COVID-19. We found about 100 GBS, 50 myelitis, and 10 CA cases after COVID-19 infection. To best our knowledge, this is the first report of myelitis with GBS and CA accompanied by multiple autoantibodies. Design/Methods: NA Results: A 60-year-old man with fever and cough was diagnosed with mild COVID-19 infection. Fourteen days later from the onset, he developed gait disturbance and fell frequently. On hospitalization, he exhibited fever, hypoxemia, mild consciousness disturbance, flaccid paraplegia, mild numbness and severe deep sensory disturbance in the lower limbs, bladder and bowel disturbance, mild muscle weakness in the fingers, myoclonus in the extremities, and CA. The PCR of COVID-19 was negative. Blood investigations showed elevated inflammatory markers with dehydration, rhabdomyolysis, and hypercoagulation. Cerebrospinal fluid (CSF) analysis presented mild pleocytosis and elevated protein without anti-COVID-19 antibodies. Contrast-enhanced CT showed massive pulmonary embolisms and deep venous thromboses. Brain SPECT showed cerebellar hypoperfusion despite no abnormalities in brain MRI. Spine MRI revealed longitudinal hyperintense lesions mainly in the dorsal white matter, compatible with myelitis. Additional investigations of autoantibodies realized anti-GM3, TPI, GluR, and NMDAR IgG antibodies in serum, and anti-GluR and NMDAR IgG antibodies with increased granzyme B in CSF. Treatments of corticosteroid and intravenous immunoglobulin resulted in complete recovery to consciousness disturbance, muscle weakness of fingers, myoclonus, and CA, while paraparesis with deep sensory and bladder and bowel disturbance remained. Conclusions: We highlight the possibility of the coexistence of several post-infectious autoimmune neurological complications in patients of COVID-19. It is important to search autoantibodies carefully corresponding to clinical manifestations for appropriate treatments and understanding of pathophysiology.
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Objective: To investigate the clinical features of the two cases presenting neurological syndrome after receiving COVID-19 vaccination, who were diagnosed with mild encephalitis/encephalopathy with a reversible splenial lesion (MERS). Background: Neurological symptoms can occur after COVID-19 mRNA vaccination. However, its etiology hasn't been fully revealed. Design/Methods: A 23-year-old previously healthy man (Patient 1) and a 33-year-old woman with a history of depression (Patient 2) developed neurological symptoms approximately one week after receipt of the first standard dose (0.3 mL, intramuscular injection) of COVID-19 mRNA vaccination (Coronavirus Modified Uridine RNA Vaccine (SARS-CoV-2)) (Day 1) and deteriorated over the next week. Clinical course, laboratory and MRI findings were serially analyzed. Results: Patient 1 presented with headache, low-grade fever and memory disturbance (Day 3). Intravenous acyclovir and meropenem were administered under a possible diagnosis of aseptic meningitis but not effective. Patient 2 presented with visual disturbance, headache, dysarthria, a left forearm tremor, dysesthesia of the mouth and distal limbs, and visual agnosia (Day 10). In both patients, reverse transcription polymerase chain reaction test results for severe acute respiratory syndrome coronavirus 2 were negative. Complete blood cell count, blood-chemistry including electrolytes and antibody titers, and cerebrospinal fluid test findings were unremarkable initially. However, second cerebrospinal fluid test of Patient 1 (Day 8) showed pleocytosis (942 cells/μL, normal ≤ 5 cells/μL) and elevated protein levels (181 mg/dL, normal 10-40 mg/dL). Brain MRI on Day 17 in Patient 1 and Day 15 in Patient 2 after receiving the vaccination showed high signal intensity lesions at the midline of the splenium of the corpus callosum. Based on the typical imaging features, the patients were diagnosed with MERS. Intravenous methylprednisolone therapy (1,000 mg/day for 3 days) improved their symptoms and MRI lesion disappeared. Conclusions: MERS should be considered in patients with neurological manifestation after COVID-19 vaccination, even though symptoms were mild and nonspecific.
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Objective: Evaluate SARS-CoV-2 RNA and inflammatory cytokines and chemokines in the CSF of patients with acute COVID-19 and neurologic symptoms, and to compare these to controls and patients with known neurotropic pathogens. Background: Neurologic symptoms have been described in 30-60% of hospitalized patients with Coronavirus Disease 2019 (COVID-19). However, little is known about CSF profiles in these patients. Design/Methods: CSF from twenty-seven consecutive patients with COVID-19 and neurological symptoms was assayed for SARS-CoV-2 RNA using quantitative reverse transcription PCR (RT-qPCR) and unbiased metagenomic sequencing. Assays for blood brain barrier (BBB) breakdown (CSF:serum albumin ratio (Q-Alb)), and proinflammatory cytokines and chemokines (IL-6, IL-8, IL-15, IL-16, monocyte chemoattractant protein -1 (MCP-1) and monocyte inhibitory protein - 1β (MIP-1β)) were performed in 23 patients and compared to CSF from patients with HIV-1 (16 virally suppressed, 5 unsuppressed), West Nile virus (WNV) (n=4) and 16 healthy controls (HC). Results: Median CSF cell count for COVID-19 patients was 1 white blood cell/μL;two patients were infected with a second pathogen (Neisseria, Cryptococcus neoformans). No CSF samples had detectable SARS-CoV-2 RNA by either detection method. In patients with COVID-19 only, CSF IL-6, IL-8, IL-15, and MIP-1β levels were higher than HC and suppressed HIV (corrected-p < 0.05). MCP-1 and MIP-1β levels were higher, while IL-6, IL-8, IL-15 were similar in COVID-19 compared to WNV patients. Q-Alb correlated with all proinflammatory markers, with IL-6, IL-8, and MIP-1β (r≥0.6, p<0.01) demonstrating the strongest associations. Conclusions: Lack of SARS-CoV-2 RNA in CSF is consistent with pre-existing literature. Evidence of intrathecal proinflammatory markers in a subset of COVID-19 patients with BBB breakdown despite minimal CSF pleocytosis is atypical for neurotropic pathogens.
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Objective: To report a case of antibody-positive neuromyelitis optica (NMO) after COVID-19 vaccination. Background: Neuromyelitis optica spectrum disorder is a rare demyelinating disorder of the central nervous system characterized by longitudinally extensive transverse myelitis (LETM) and severe, sometimes bilateral optic neuritis. The majority of cases have serologic or CSF antibody for aquaporin-4 (AQP4). Design/Methods: Case report Results: A 19 year old woman with no prior medical history presented with two days of progressive, severe weakness and sensory changes first in the arms, then legs. On the morning of presentation, she woke with urinary incontinence. She had received COVID-19 vaccination (Moderna) fifteen days preceding her onset of symptoms. Examination revealed sinus tachycardia, MRC grade 3-4/5 power in the arms, 0/5 in the legs with approximately T4 sensory level. Cervical spine MRI revealed T2 prolongation in the spinal cord extending from the cervicomedullary junction to the conus medullaris. CSF revealed neutrophilic pleocytosis with increased IgG synthesis rate and positive CSF AQP4 antibody;serum AQP4 and MOG antibodies were negative. Bilateral, saddle pulmonary emboli were discovered shortly after admission. Her NMO was treated with high-dose intravenous methylprednisolone, plasmapheresis, and rituximab. Conclusions: This case describes a severe, new presentation of antibody-positive neuromyelitis optica following vaccination against COVID-19.
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Objective: N/A Background: COVID-19 infection has been shown to be associated with a number of neurologic sequelae in the post-infection period. There have been rare cases of autoimmune neurologic disease associated with COVID-19 infection. Here we present a case of myelin oligodendrocyte glycoprotein (MOG)-associated encephalomyelitis two months after COVID-19 infection. Design/Methods: N/A Results: A 44-year old female presented to the hospital with progressive decline in mentation and fevers for 1 week. Three months prior, she tested positive for COVID-19 by nasopharyngeal PCR testing, had mild symptoms and recovered at home. On presentation, neurologic examination showed sensory level at T8, lower extremity hyperreflexia. and gait instability. MRI of the neuroaxis showed bilateral white matter lesions in the brain and longitudinal cord lesions at multiple cervical and thoracic spinal levels. CSF showed lymphocyte-predominant pleocytosis. Patient was diagnosed with encephalomyelitis and started on plasma exchange and high dose steroids on alternating days with improvement in her symptoms. Patient was positive for serum MOG antibody. She was discharged on a prolonged prednisone taper. Conclusions: This case highlights the importance of testing for MOG antibody disease in patients presenting with findings of brain and spinal cord lesions after COVID-19 infection. The course of MOG-associated antibody disease in post-COVID patients is unknown and warrants further investigation.
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Objective: To describe cases of transverse myelitis (TM) associated with mild COVID-19 in adults. Background: Post-infectious TM is described after various infections but is not as well-known after COVID-19. Design/Methods: We present a series of 2 cases who developed TM after infection with SARS-CoV-2. Case 1: 55-year-old male with coronary artery disease presented with worsening paraparesis over 4 months, T4 sensory level and urinary retention, starting two weeks after mild COVID-19 illness. MRI showed T2 hyperintensity extending from the lower medulla to T3 spinal cord. CSF analysis revealed elevated protein and pleocytosis. His functional status improved after plasma exchange. Subsequently, his symptoms worsened and was treated with multiple courses of glucocorticoids. He recently started Rituximab and continues to have leg weakness with urinary retention. Case 2: 66-year-old male with diabetes mellitus presented in a wheelchair with rapidly progressive paraparesis over 10 days, starting six weeks after mild COVID-19 illness. He was initially diagnosed with GBS and received IVIG with no improvement. MRI revealed T2 hyperintensity in the lateral corticospinal tracts of cervical and thoracic spinal cord. Somatosensory evoked potential testing showed mild bilateral demyelinating lesions involving the dorsal columns between the C6-parietal cortex. CSF analysis was normal. Plasma exchange therapy provided minimal improvement. He remains wheelchair bound with urinary urgency. In both cases, other causes of TM including neuromyelitis optica, myelin oligodendrocyte associated disease, neurosarcoidosis and paraneoplastic myelopathy were ruled out. Conclusions: SARS-CoV2 may cause a post-infectious TM. While causation remains difficult to prove, our cases suggest TM was precipitated by COVID-19 given the temporal association and no other identified etiology. Our cases continued to have significant neurologic deficits likely due to delayed diagnosis. These cases add to the growing body of evidence of neurologic complications associated with COVID-19. Further studies are needed to establish the incidence and outcomes of post-infectious TM after COVID-19.
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Objective: N/A Background: Neurological injuries from severe acute respiratory syndrome coronavirus 2 (COVID-19) are becoming recognized in the central and peripheral nervous systems. Pathophysiology processes include a hyperinflammatory immune response due to the affinity of COVID-19 to human ACE2 receptors and multi-organ failure. CASE REPORT: This report highlights a unique presentation of a rapidly progressive, hyperacute to chronic, necrotizing transverse myelitis with associated COVID-19 long haul syndrome in a 31-year-old Venezuelan obese woman without other vascular risk factors. There was a preceding symptomatic COVID-19 infection. Symptoms included headaches, dysgeusia, asymmetric ascending proximal more than distal paresis, lumbago with dysesthesias, autonomic dysautonomia, hyperreflexia with clonus, and severe functional mobility impairment. Results: Neuraxis imaging showed a longitudinally extensive transverse myelitis (LETM) with T7-T9 enhancement and expansion throughout the thoracic spine (T4-T11). CSF studies showed lymphocytic pleocytosis with elevated protein. Aggressive empiric treatment included 1G of IV methylprednisolone, plasmapheresis (PLEX), and high-dose cyclophosphamide given the life-threatening progression of clinical symptoms. Repeat imaging post-treatment showed expansion of lesions to the cervical cord with sparing of the brainstem, stabilizing after cyclophosphamide initiation. Clinically, there was partial recovery of upper extremity sensation and strength. A T5-T6 tissue biopsy showed evidence of necrotizing myelitis with extensive neutrophil and lymphocyte infiltration. Given clinical progression, a repeat round of immunosuppression, including a monoclonal complement antibody, was pursued. Post-rehabilitation, the patient's symptoms improved above the thoracic spine but not below it. Conclusions: To our knowledge, this is the most severe form of COVID-19 associated necrotizing myelitis ever reported. Future research may clarify the molecular pathways that trigger neurological injury in patients with severe COVID-19 infection-associated spinal cord complications and increase therapeutic options.
ABSTRACT
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.