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Background: People with chronic diseases of the immune system, such as multiple sclerosis (MS), are at risk for Covid-19 disease. However, more research is needed with long-term follow-up. The aim of the study was to follow up people with MS (PwMS) for up to three months after AstraZeneca vaccination for the recurrence of MS and Covid-19 infection. Method(s): This study was a case study (descriptive-analytical) of follow-up type. The study population was PwMS over 18 years of age in Kermanshah province who received both doses of the AstraZeneca vaccine. This study was conducted from August to November 2021. Sampling was done with existing methods based on the National MS Registry of Iran (NMSRI). Demographic information of patients was extracted from NMSRI. A researcher-made form was used to collect information by telephone three months after vaccination about clinical characteristics, Covid-19 infection, and recurrence of MS. Data were analyzed using SPSS-25 software. Result(s): Study participants were 40 MS patients with a mean (SD) age of 39.27 (8.8) years, including 32 (80.0%) women. A mean of 9.39 (4.6) years had passed since The patients were diagnosed with MS, and 29 (76.4%) had RR type MS. Four patients (10%) relapsed between the second dose and three months later, of whom two (50%) had sensory symptoms, one (25%) had optic nerve involvement, and one (25%) had motor symptoms and pyramidal pathway involvement. The symptoms of Covid-19 were mild in three patients (10%), while severe symptoms developed in one patient (10%) who received rituximab. Among the patients, no cases of thrombosis were observed. Infusion therapy, a leg fracture, and kidney stones were the only hospitalized cases. Conclusion(s): Covid-19 and MS relapse prevalence did not differ significantly in the three months before and after vaccination. There is a need for further studies with a longer follow-up period.Copyright © 2022 Razazian et al. Published by Tehran University of Medical Sciences.
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Introduction: Cerebral venous sinus thrombosis (CVST) is a rare but potentially debilitating thrombosis affecting 3-4 cases per million adults in the United States. Risk factors are thought similar to venous thrombosis, but there is little epidemiologic data corroborating this assertion. Concern about a possible association between the Janssen (Johnson and Johnson) and Oxford-AztraZenaca COVID-19 vaccines and cases of CVST resulted in increased global attention to this condition. Thus, large epidemiological assessment of the risk factors, treatment and outcomes of CVST are needed. Objective(s): Estimate the distributions of risk factors antecedent to CVST diagnosis, report CVST treatments in clinical practice, and potential sequelae of CVST in a large retrospective cohort of adults with CVST in the United States. Method(s): MarketScan Commercial and Medicare Supplemental administrative databases were employed to assess CVST diagnosed between 2011 and 2019 in the U.S. Validated International Classification of Disease (ICD) codes and receipt of an outpatient anticoagulant (either oral or subcutaneous anticoagulant) prescription within 30 days of the ICD code identified incident CVST. Antecedent clinical characteristics, treatments, and sequelae of CVST were identified using inpatient, outpatient, and prescription data. For outcomes, proportions and incidence with 95% confidence intervals (CIs) were calculated, stratified by sex. Result(s): We identified 1,869 CVST patients. Of these 1,314 (70%) were female, with 200 (10%) events identified as a pregnancy-related CVST. The average age was 41 years for females and 48 years for men. Among women, 24.7% were on hormonal therapy (oral contraceptive, estrogen, and progestin) prior to diagnosis. Men had a higher prevalence of comorbidities, such as diabetes (15% men vs. 9% women) and cancer (19% men vs. 10% women). Oral anticoagulant (OAC) use was the most common treatment for CVST in both men (88%) and women (85%) and did not vary by sex. Use of procedures to treat CVST, optic nerve fenestration and catheter directed thrombolysis, were 0.5% and 4.1%, respectively. The most common sequela after CVST was incidence of intracranial hypertension (Incidence: 4.2 per 100 person-years;95% CI: 3.3, 5.1) and palliedema was rare. Conclusion(s): Overall, a majority of CVST patients were women of reproductive age. Our findings suggest a potential association with both endogenous (pregnancy) and exogenous (oral contraceptives, HRT) hormones which needs further study. In our sample, CVST was managed with oral anticoagulants, regardless of sex, and intracranial hypertension was elevated following CVST. This large claims-based analysis is a descriptive insight into the risk factors and management of CVST, a rare and debilitating condition.
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Introduction: Autoimmune and inflammatory thyroid diseases have been reported following SARS-CoV-2 infection or vaccination, but thyroid eye disease (TED) post-COVID-19 infection is less common. We describe a case of TED following SAR-CoV-2 infection in a patient with a history of Graves' disease. Case Description: A 59-year-old female with history of Graves' disease status post radioiodine ablation therapy in 2002. She developed post-ablative hypothyroidism which has been stable on levothyroxine 88 mcg daily. In January 2021, the patient's husband and daughter were diagnosed with COVID-19 infection. A few days later, the patient developed an upper respiratory tract infection associated with loss of sense of smell and taste consistent with COVID-19 infection. Three days later, she developed bilateral watery eyes which progressed to eye redness, eyelid fullness, retraction, and pain with eye movement over 1-month duration. Her eye examination was significant for severe periocular soft tissue swelling, lagophthalmos and bilateral exophthalmos. The laboratory workup was consistent with normal TSH 0.388 mIU/L (0.358-3.740 mIU/L) and positive TSI 1.01 (0.0-0.55). The patient was referred to an Ophthalmologist for evaluation of TED. He noted bilateral exophthalmos, no restrictive ocular dysmotility or compressive optic neuropathy (clinical activity score 4/7 points). CT scan of orbit showed findings compatible with thyroid orbitopathy. Based on clinical activity score of 4, treatment with Teprotumumab was recommended pending insurance approval. Discussion(s): Many cases of new-onset Graves' hyperthyroidism have been reported after COVID-19, with only a few associated with TED. Our patient has been in remission for 20 years before she developed COVID-19 infection with occurence of TED.This suggests that COVID-19 infection may have played a role. SARS-CoV-2 may act through several mechanisms, including breakdown of central and peripheral tolerance, molecular mimicry between viral and self-antigens, stimulation of inflammasome with release of type I interferon. In our patient, treatment with Teprotumumab was indicated due to Graves' orbitopathy clinical activity score greater than or equal to 3. In conclusion, it is very uncommon for TED to present after COVID-19 infection. Our case reinforces the speculative hypothesis that SARS-CoV-2 virus could have triggered an autoimmune response against eye antigens. There is a need for increased awareness about the link between COVID-19 and autoimmunity to help better define the management of patients.Copyright © 2023
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Background Acute invasive fungal rhinosinusitis (AIFR) is a rare, rapidly progressive, and life-threatening infection involving the nasal cavity and paranasal sinuses. Purpose of this study is to describe imaging features of coronavirus disease-2019 (COVID-19)-associated AIFR.Methods This was a retrospective observational study. Inclusion criteria: (1) post-COVID-19 patients with fungal rhinosinusitis detected on potassium hydroxide smear or histopathology;(2) onset of symptoms (facial pain, dental pain, facial swelling or discoloration, nasal bleed, periorbital swelling, ptosis, redness of eyes, vision loss) less than 4 weeks;and (3) magnetic resonance imaging/computed tomography (MRI/CT) done within 5 days before surgery. Exclusion criteria: (1) cases of sinusitis without a history of previous COVID-19 infection;and (2) cases in whom fungal hyphae were not demonstrated on pathological examination. Noncontrast CT and dedicated MRI sequences were done initially. Site of involvement, unilateral/bilateral involvement, pattern of mucosal thickening, enhancement pattern, periantral invasion, orbital invasion, intracranial involvement, perineural spread, vascular involvement, and bony involvement were recorded. CT and MRI imaging features were compared.Results Analysis of 90 studies (CT and MRI) in 60 patients was done. Most common site of involvement was ethmoid followed by maxillary sinus. Bilateral disease was more common. Mucosal thickening with T2 hypointense septations was seen in 88.4% MRI studies. Periantral and orbital involvement was seen, respectively, in 84.6% and 55.7% cases of MRI. Intracranial involvement was noted in form of meningitis, cerebritis, abscess, infarct, hemorrhage, cavernous sinus, or perineural invasion. Vascular involvement was noted in form of vascular occlusion ( n = 3), vascular narrowing ( n = 3), and pseudoaneurysm ( n = 2). MRI was more sensitive in detecting periantral invasion, deep infratemporal fossa, cavernous sinus involvement, perineural invasion, optic nerve involvement, and vascular occlusion and narrowing, while CT was superior in identification of bony erosions.Conclusion Early recognition of AIFR in post-COVID-19 patients is important to prevent disease-related morbidity/mortality. Several rarely described findings are noted in our series of AIFR, like optic nerve involvement, pituitary fungal abscess, perineural spread, fungal aneurysms, and arteritis-related posterior circulation infarcts. MRI is superior for early detection of disease and in estimation of extent of disease, compared with CT. Imaging can help in early detection of AIFR, which has a significant impact on patient outcome.
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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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Background: PRPS1 deficiency spectrum is an X-linked condition caused by pathogenic variants in PRPS1, which encodes for the PRPP enzyme involved in the purine synthesis pathway, among other metabolic pathways. Severely affected individuals, also known as Arts syndrome, have congenital sensorineural hearing loss, optic atrophy, developmental delays, ataxia, hypotonia, and recurrent infections. Infections often precipitate worsening of symptoms and many individuals pass away in childhood. Mildly to moderately affected individuals can have isolated hearing loss, also known as DFNX1, or hearing loss with later onset ataxia and optic neuropathy concerns, also known as CMTX5. Given the importance of PRPP in the role of purine synthesis as well as other cellular processes, including formation of NAD(P), supplementation of these pathways is a logical approach for these patients. 2 Arts syndrome patients were previously supplemented with S-adenosylmethionine, starting in mid-childhood, with improvement in infection severity and frequency, as well as stabilization of other symptoms. Recently another Arts syndrome patient was supplemented with S-adenosylmethionine and nicotinamide riboside, starting in early childhood, with improvement in infection frequency and developmental gains. Here we present a now 23 month old male patient with severe PRPS1 deficiency spectrum symptoms, who was started on S-adenosylmethionine and nicotinamide riboside supplementation. Result(s): This is a 23 month old male with developmental delay, retinal dystrophy, congenital bilateral sensorineural hearing loss, and hypotonia with a PRPS1 c.383A > T / p.Asp128Val likely pathogenic variant. He does not have a history of recurrent infections, however family reports relative isolation due to the Covid-19 pandemic. He sat unsupported at 10 months, crawled at 14 months, pulled to stand at 18 months, and is nonverbal. His uric acid testing was in the low range of normal. He had normal purine testing with low normal xanthine and hypoxanthine levels. At 19 months the patient started 20 mg/kg/d S-adenosylmethionine supplementation. At 20 months this was increased to 40 mg/kg/d S-adenosylmethionine and he started on 30 mg/kg/d nicotinamide riboside supplementation. Parents reported subjective improvement in strength and endurance with supplementation. He made significant developmental gains including walking with a walker. He had done well with occasional upper respiratory infections without regression in skills, worsening hypotonia, or increased respiratory needs. Unfortunately, very recently he had a cardiac arrest secondary to respiratory failure from rhinovirus/enterovirus and H. influzenzae pneumonia, for which he remains hospitalized at this time. Conclusion(s): This is the 4th reported patient with severe PRPS1 deficiency treated with S-adenosylmethionine supplementation and the 2nd reported patient treated with nicotinamide riboside supplementation. Both supplements have a limited side effect profile and have a biochemical basis for consideration in PRPS1 deficiency. He initially did well on supplementation with improvements in strength and endurance, as well as developmental gains, however his current trajectory remains to be seen. Unfortunately, NAD/NADP, ADP/ATP, and similar markers were unavailable to us and we plan to continue clinical monitoring on supplementation. Further studies are needed to evaluate the effectiveness of S-adenosylmethionine and nicotinamide riboside supplementation in these patients.Copyright © 2023
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Purpose. The analysis of CT and MR patterns of rhinoorbitocerebral mucormycosis (ROCM) among patients with type 2 diabetes (T2D) after COVID-19. Materials and methods. The study included 11 patients with confirmed COVID-19 and invasive ROCM in the long-term period. CT examinations were performed on Revolution EVO CT scanner (GE, Russia) and MRI on Aera MR scanner 1.5 T (Siemens, Germany). Results. We report several CT and MR patterns for a series of patients: involvement of paranasal sinuses, orbits, optic nerves, large arteries;intracranial spread;involvement bones of cranial base. The features of differential diagnosis and recommendations for standard protocols are presented. Conclusion. CT of paranasal sinuses is the method of choice for suspected fungal infections. MRI is recommended if there is a suspicion of orbital, vascular, and intracranial complications or cavernous sinus extension. The combination of both methods makes it possible to showed soft tissue invasion and bony destruction to choose optimal medical tactics ROCM.Copyright © 2022 Russian Electronic Journal of Radiology. All rights reserved.
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Backgrounds: To report the first case of left optic neuritis and perineuritis associated with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) BNT162b2 mRNA vaccination. Case presentation: A 39-year-old woman was referred and admitted to our hospital due to transient left visual field abnormality with left ophthalmalgia and headache 12 days after the first vaccination dose of SARS-CoV-2 (BNT162b2). On admission (Day 2), she presented with left ophthalmalgia and headache without any other neurological deficits including the movement of eyeballs, visual field, visual acuity, or nystagmus. MRI on Day 2 suggested slight left optic neural swelling;Gadolinium-enhanced MRI on Day 4 revealed left optic perineuritis. Test for serum anti-aquaporin 4 antibody was negative, whereas anti-myelin oligodendrocyte glycoprotein (MOG) antibody was positive. She was diagnosed with left optic perineuritis after SARS-CoV-2 mRNA vaccination. Her visual disturbance never recurred and her ophthalmalgia and headache subsided only with anti-inflammatory agents. Discussion(s): Many cases of optic neuritis associated with vaccinations have been reported except for SARS-CoV-2 BNT162b2 mRNA. To our knowledge, only one neuromyelitis optica case was associated with anti-MOG antibody. Therefore, we propose that SARS-CoV-2 mRNA vaccination may induce transient optic neuritis and perineuritis, associated with anti-MOG antibody in the present case. Conclusion(s): This is the first case of left optic neuritis and perineuritis associated with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) BNT162b2 mRNA vaccination.Copyright © 2022
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BACKGROUND: As scientific knowledge continues to grow regarding coronavirus disease 2019 (COVID-19) infection, several neuro-ophthalmological manifestations have emerged, including rare reports of optic neuritis. Optic neuritis is an inflammatory demyelinating condition of the optic nerve that typically presents as subacute, unilateral vision loss and pain on eye movement. Several cases of COVID-19 infection and COVID-19 vaccination related cases of optic neuritis have been reported. We present a case of hyperacute, unilateral optic neuritis after both recent COVID-19 infection and subsequent booster vaccination. CASE PRESENTATION: Within two hours after receiving her COVID-19 booster vaccination, a 58-year-old female began experiencing bilateral eye pain, worsened by eye movements. The patient had previously contracted a mild COVID-19 infection three weeks prior to receiving her booster vaccination, confirmed by a rapid antigen test. The pain persisted in her right eye for a week at which time she presented to an ophthalmology clinic. She denied any changes to her visual acuity. Neuroimaging revealed right optic nerve enhancement, and the patient was admitted to the hospital for a course of intravenous steroids, which quickly resolved her eye pain. CONCLUSION: To our knowledge, this is the first reported case of COVID-19 related optic neuritis following both COVID-19 infection and vaccination. High clinical suspicion is needed to make the appropriate diagnosis, as cases of COVID-19 related optic neuritis may exhibit mild presentations, as was the case with our patient.
Subject(s)
COVID-19 Vaccines , COVID-19 , Optic Neuritis , Female , Humans , Middle Aged , COVID-19/prevention & control , COVID-19 Vaccines/adverse effects , Eye Pain/diagnosis , Eye Pain/etiology , Optic Neuritis/diagnosis , Optic Neuritis/etiology , Pain , Vaccination/adverse effectsABSTRACT
BACKGROUND: Our aim in this observational prospective study is to determine whether the prone position has an effect on intracranial pressure, by performing ultrasound-guided ONSD (Optic Nerve Sheath Diameter) measurements in patients with acute respiratory distress syndrome (ARDS) ventilated in the prone position. METHODS: Patients hospitalized in the intensive care unit with a diagnosis of ARDS who were placed in the prone position for 24 h during their treatment were included in the study. Standardized sedation and neuromuscular blockade were applied to all patients in the prone position. Mechanical ventilation settings were standardized. Demographic data and patients' pCO2, pO2, PaO2/FiO2, SpO2, right and left ONSD data, and complications were recorded at certain times over 24 h. RESULTS: The evaluation of 24-hour prone-position data of patients with ARDS showed no significant increase in ONSD. There was no significant difference in pCO2 values either. PaO2/FiO2 and pO2 values demonstrated significant cumulative increases at all times. Post-prone SPO2 values at the 8th hour and later were significantly higher when compared to baseline (p < 0.001). CONCLUSION: As a result of this study, it appears that the prone position does not increase intracranial pressure during the first 24 h and can be safely utilized, given the administration of appropriate sedation, neuromuscular blockade, and mechanical ventilation strategy. ONSD measurements may increase the safety of monitoring in patients ventilated in the prone position.
Subject(s)
Intracranial Hypertension , Intracranial Pressure , Prone Position , Respiratory Distress Syndrome , Humans , Intracranial Hypertension/diagnosis , Intracranial Hypertension/etiology , Intracranial Pressure/physiology , Optic Nerve/diagnostic imaging , Prospective Studies , Respiratory Distress Syndrome/diagnostic imaging , Respiratory Distress Syndrome/therapy , Respiratory Distress Syndrome/complications , UltrasonographyABSTRACT
BACKGROUND: Hypoxia and hypercapnia due to acute pulmonary failure in patients with coronavirus disease 2019 (COVID-19) can increase the intracranial pressure (ICP). ICP correlated with the optic nerve sheath diameter (ONSD) on ultrasonography and is associated with a poor prognosis. AIM: We investigated the capability of ONSD measured during admission to the intensive care unit (ICU) in patients with critical COVID-19 in predicting in-hospital mortality. METHODS: A total of 91 patients enrolled in the study were divided into two groups: survivor (n = 48) and nonsurvivor (n = 43) groups. ONSD was measured by ultrasonography within the first 3 h of ICU admission. RESULTS: The median ONSD was higher in the nonsurvivor group than in the survivor group (5.95 mm vs. 4.15 mm, p < 0.001). The multivariate Cox proportional hazard regression analysis between ONSD and in-hospital mortality (contains 26 covariates) was significant (adjusted hazard ratio, 4.12; 95% confidence interval, 1.46-11.55; p = 0.007). The ONSD cutoff for predicting mortality during ICU admission was 5 mm (area under the curve, 0.985; sensitivity, 98%; and specificity, 90%). The median survival of patients with ONSD >5 mm (43%; n = 39) was lower than those with ONSD ≤ 5 mm (57%; n = 52) (11.5 days vs 13.2 days; log-rank test p = 0.001). CONCLUSIONS: ONSD ultrasonography during ICU admission may be an important, cheap, and easy-to-apply method that can be used to predict mortality in the early period in patients with critical COVID-19.
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PURPOSE: Presence of SARS-CoV-2 RNA in human retinal biopsies (RBs) was previously reported by us. In this consecutive study, we analysed RB and optic nerve biopsies (ONBs) in deceased patients with confirmed COVID-19 assessing viral RNA load, possible virus replication and infectivity. PATIENTS AND METHODS: In this case series, 14 eyes of 14 deceased patients with COVID-19 were enucleated during autopsy. RB and ONB were subjected to molecular detection of viral RNA, virus cultivation and immunohistochemistry. SARS-CoV-2 RNA loads were compared with RNA loads in the respective throat swabs, vitreous humour and blood samples. RESULTS: SARS-CoV-2 RNA was detected in 7/14 RBs and in 10/13 ONBs. While virus isolation failed and immunohistochemistry of SARS-CoV-2 spike protein was negative, subgenomic RNA (sgRNA) was detectable (40% RB; 60% ONB). CONCLUSION: SARS-CoV-2 RNA is detectable in RB and ONB of patients with COVID-19. Presence of sgRNA could point to a SARS-CoV-2 infection of neuronal tissue, but as virus isolation failed and immunohistochemistry of SARS-CoV-2 spike protein was negative, an active infection seems unlikely.
Subject(s)
COVID-19 , SARS-CoV-2 , Genomics , Humans , Optic Nerve , RNA, Viral/analysis , RNA, Viral/genetics , Retina , SARS-CoV-2/genetics , Spike Glycoprotein, CoronavirusABSTRACT
Introduction: In patients presented with hypertensive crises, a fundoscopic assessment is necessary because once hypertensive retinopathy is discovered, a hypertensive emergency is diagnosed, and intravenous antihypertensive medication is recommended. However, direct ophthalmoscopy is relatively underutilized, especially under the social distance regulation, which may result in delayed diagnosis and treatment. The novel method, namely, smartphone-based fundoscopy offers longer working distance and shorter doctor-patient contact time, however, there is limited data regarding its feasibility and effectiveness. Objective: We aimed to gather scientific evidence on the smartphone-based fundoscopy in terms of its effectiveness, accessibility, and trainability in detecting hypertensive retinopathy among hypertensive crisis patients in emergency room settings. Methods: A literature search was conducted on PubMed, Google Scholar, and the Cochrane Library for papers published from January 2010 to November 2021. Keywords including hypertensive crisis, hypertensive retinopathy, target organ damage, fundoscopic optic examination, direct ophthalmoscope, fundus images, smartphone fundoscopy, digital fundus camera, and COVID-19 were used. Full papers published in English and s of non-English publications were all reviewed. Results: Eight studies out of 34 fulfilled our search criteria. Five observational studies confirmed the effectiveness of smartphone-based fundoscopy in obtaining fundus images adequate for interpretation compared with those from commercially available fundus cameras. Also, smartphone-based fundoscopy offers time-saving properties as it allows fundus examination to be effectively completed within 74 seconds compared to 130 seconds with a traditional direct ophthalmoscope. Two studies investigated the accessibility of smartphonebased fundoscopy and discovered that fundus images can be obtained by using 20 diopter condensing lenses with the video mode of the smartphone camera, which can be easily provided even at a primary level hospital due to their low cost. Another study reviewed the trainability of the smartphone-based fundoscopy in 137 undergraduate medical students which concluded that 75% of these students can identify the optic nerve within 20-25 minutes of face-to-face demonstration. Conclusion: With a greater diagnostic capability, accessibility, and trainability of smartphone-based fundoscopy makes it a potentially game-changing technique for detecting hypertensive retinopathy in hypertensive emergency patients, especially during the current COVID-19 pandemic, in which longer working distance and shorter doctor-patient contact time are both required.
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Introduction/Background: Antiphospholipid syndrome (APS) is a rare autoimmune multisystem disease characterised by thrombosis and pregnancy morbidity in the presence of persistently elevated titres of: lupus anticoagulant, anticardiolipin and/or anti-glycoprotein 1. It may be primary (occurring alone) or secondary (in combination with another disease, most commonly systemic lupus erythematosus (SLE)). Recent publications highlighted clinical criteria limitations for children and raised awareness of the burden and prevalence of non-criteria manifestations in this population. This case report adds further weight to the need to raise multi-specialty awareness of non-criteria manifestations to aid recognition and treatment of this rare condition with potentially severe sequelae. Description/Method: 13-year-old female with SLE diagnosed aged 8 in India with bilateral optic neuritis occurring two months later. ANA positive at diagnosis with low complement and thrombocytopenia. Treated with prednisolone and hydroxychloroquine. Patient moved to the UK aged 9;initial abnormal bloods: mildly positive ANA (ENA negative), thrombocytopenia, strong lupus anticoagulant. As serology not strongly suggestive and optic neuritis rare in lupus diagnosis questioned. Ophthalmology review confirmed bilateral optic atrophy without evidence of previous vasculitis. There was debate whether the postretinal demyelination was due to antiphospholipid syndrome or a primary demyelinating condition. Hydroxychloroquine stopped and azathioprine started. Following normal neurology investigations (brain, spine MRI/MRV/MRA) concluded if patient developed new APSrelated symptoms or worsening visual evoked potentials anticoagulation would be discussed. Patient remained stable over four years with chronic thrombocytopenia and ESR persistently elevated. Azathioprine changed to Mycophenolate mofetil (MMF) due to side effects. Routine medication monitoring bloods in 2022 showed ESR 97, CRP 78, Platelets 61. Review identified vasculitic rash on soles of both feet with palpable nodules and normal pulses. Further investigation confirmed antiphospholipid antibody triple positivity. Aspirin commenced, hydroxychloroquine restarted, MMF dose increased and rituximab administered. Left foot rash settled but right progressed with toe discolouration and numbness. Skin biopsy considered but not performed due to skin integrity concerns. Foot pulses remained present and normal. Bilateral lower limb doppler reported as normal;increased symptoms resulted in CT angiogram which revealed bilateral non-occlusive popliteal thrombus and left pulmonary embolus. Subsequent echocardiogram was normal. Patient was anticoagulated with low molecular weight heparin followed by warfarin. Vascular surgical team advocated medical management and patient received seven infusions of Iloprost followed by Sildenafil. She achieved near total resolution of skin changes to toes with only minimal loss of skin over tip of right great toe. Patient will now require long-termanticoagulation. Discussion/Results: APS was considered in initial differential diagnosis but patient did not meet current clinical criteria as no past evidence of thrombosis. Lupus anticoagulant was consistently strongly positive and anticardiolipin repeatedly negative. As anti-B2 glycoprotein 1 antibody is not routinely tested and must be verbally requested, it was only checked once (negative) prior to discovery of triple positivity. ANA reported as strongly positive at time of SLE diagnosis but reviewing original notes from India titre was 1:100 and therefore not highly convincing. ENA negative and complement and white cell count normal on repeat testing since. Therefore, it is probable that this patient has primary APS as opposed to secondary APS in association with SLE. However, it is possible that this patient may develop more symptoms of SLE over time. When this patient presented with foot rash there were high numbers of children presenting with varying severity of painful, itchy toes coined 'covid toes' due to suspected lin to SARS-CoV-2 infection. Patient had exposure history, and COVID antibody serology was difficult to interpret due to recent vaccination. Dermatology found appearance to be consistent with 'covid toes' and advised supportive treatment. The triple APS antibody positivity result provided probable aetiology. Providing evidence of thrombus was problematic with false reassurance from apparently normal lower limb arterial doppler when actually popliteal arteries were not checked in view of the presence of normal flow proximally at the groin and distally in the feet. This case highlights the need to continue to search for thrombus in presence of high titres antiphospholipid antibodies and particularly in the case of triple positivity as although patient presented with colour change to toes, she was entirely asymptomatic from her PE and her left foot improved spontaneously despite a left popliteal thrombus also being present. Key learning points/Conclusion: Non-criteria manifestation of thrombocytopenia (occurs in 25% paediatric APS patients) was present throughout and patient had past history of haematuria (a recognised renal non-criteria manifestation). A paediatric specific APS criteria including these may have resulted in earlier detection of triple antiphospholipid antibody positivity and thus earlier treatment escalation and possible avoidance of thrombus. It has been reported that a high proportion of children with positive antiphospholipid antibodies don't develop a thrombus. However, it is interesting that our patient was entirely asymptomatic from her pulmonary embolus which was an incidental finding on her CT angiogram. This prompts a discussion about how much imaging should be performed in those with high levels of persistent positive antiphospholipid antibodies. Rituximab resulted in normalisation of platelet count and ESR for the first time since initial presentation. Anticardiolipin antibodies normalised, lupus anticoagulant decreased from strong to moderate and anti- B2 glycoprotein levels decreased but remained positive. Rituximab is a recognised treatment for catastrophic antiphospholipid syndrome (CAPS) but not routinely used in APS. The consistently raised ESR in an apparently clinically well patient is a reminder to continue to search for causes of inflammation. As the CRP was largely in normal range, this demonstrates the unique value of the ESR. In view of anti-B2 glycoprotein 1 antibody requiring to be verbally requested, discussions are ongoing with the laboratory department regarding the possibility of electronic request and a comment with recommendation to check other two antiphospholipid antibodies following one positive antibody result. As a result of this case, a plan will be put in place to ensure annual screening of antiphospholipid antibodies in all juvenile SLE patients in our care. It is hoped that this case report promotes discussion amongst the paediatric rheumatology community regarding further research required for development of paediatric specific APS criteria and management.
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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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Objective: Many neurological symptoms due to central nervous system, peripheral nervous system and musculoskeletal system damage have been reported in more than a third of patients with coronavirus disease-2019 (COVID-19). Although optic neuritis has been reported in patients with COVID-19, they are extremely rare. The aim of this study was screening optic nerve involvement in COVID-19 with visual evoked potential (VEP) in asymptomatic patients without a history of visual impairment. Materials and Methods: Pattern reversal VEP measurements were made in 101 adult patients with COVID-19 without a history of visual impairment after they completed COVID-19 treatments and the quarantine period. VEPs were recorded with the 4-channel electromyography-evoked device in a dark room. P100 latencies and amplitudes were analyzed by the same neurologist. Results: A total of 34 (33.7%) patients had P100 latency prolongation. There was no significant difference in terms of gender, age or outpatient/inpatient treatment status. There was no significant correlation between the time of polymerase chain reaction diagnosis and VEP values. Conclusion: Contrary to previous studies, asymptomatic optic nerve involvement after COVID-19 was detected by VEP measurements. Prolongation of P100 latency shows the probable linkage between COVID-19 virus and angiotensin converting enzyme 2 receptors in human eyes.
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Purpose: The SARS‐CoV‐2 pandemic has affected all countries in the world and is still ongoing. Although respiratory symptoms are the main manifestation of acute infection, there is also increasing evidence that neurological and vascular symptoms occur, and it is unknown whether residuals remain after patients have recovered. We therefore set out to investigate whether ocular vascular alterations remain after patients have recovered.Methods: Patients that had recovered from COVID‐19 infection within the last 6 months before inclusion and healthy age‐ and sex‐matched controls were recruited. Main inclusion criteria for patients were confirmed positive PCR test for SARS‐CoV‐2 in the medical history and positive testing for SARS‐CoV‐2 seroprevalence while controls had no history of COVID‐19 infection. Arteriovenous (AV) difference in oxygen saturation was calculated out of retinal arterial and venous oxygen saturation, which were measured with a commercially available Dynamic Vessel Analyser (DVA, Imedos, Germany). Retinal vessel diameters and arteriovenous ratio (AVR) were assessed using the same device. In addition, mean blur rate in the tissue area of the optic nerve head (MT) was quantified using laser speckle flowgraphy (LSFG, Nidek, Japan).Results: 29 patients that had recovered from moderate to severe COVID‐19 requiring hospitalization (mean age 35 ± 17 years) and 11 control subjects (mean age 36 ± 12 years) were included in the present study. No differences between groups regarding sex or concomitant diseases in the medical history were found. Body mass index (BMI) was significantly higher in patients that had recovered from COVID‐19 (27.5 ± 5.6 vs. 24.5 ± 2.8 m2/kg, p = 0.036). AVR as well as AV difference in oxygen saturation was significantly lower in patients compared to healthy controls (p = 0.021 for AVR and p = 0.023 for AV difference in oxygen saturation). MT in the optic nerve head also was significantly lower in patients (23.4 ± 10.1 a.u.) than in control subjects (47.3 ± 26.6 a.u., p < 0.001).Conclusions: The results of this study imply that retinal metabolism is still altered in patients after recovering from COVID‐19 infection. Longitudinal studies are required to investigate whether these changes in retinal vessels as well as optic nerve head blood flow are temporary or remain.
ABSTRACT
We present a case of a 61-year-old woman with an atypical non-arteritic anterior ischemic optic neuropathy (NA-AION) as a unique manifestation of COVID-19. Furthermore, the patient worsened after Pfizer-BioNTech COVID-19 vaccine administration. Our findings suggest that NA-AION could result from microangiopathic/thrombotic events that may occur during SARS-CoV-2 infection and/or vaccination against COVID-19. This report sheds light on possible ophthalmologic complications of COVID-19.