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Background: X-Linked Moesin-Associated Immune Deficiency (X-MAID) is a rare severe combined immunodeficiency (SCID) subtype that can present at any age due to its variability. Depending on severity, patients demonstrate failure to thrive, recurrent bacterial and viral infections, and increased susceptibility to varicella zoster. It has been characterized by marked lymphopenia with hypogammaglobulinemia and impaired T-cell migration and proliferation. Case Presentation: This is a report of a Cuban 7-year-old male with poor weight gain and facial dysmorphia. He had a history of recurrent bacterial gastrointestinal infections and pneumonia beginning at 4 months of age. He additionally had 4-6 upper respiratory tract and ear infections annually. While still living in Cuba, he was admitted for a profound EBV infection in the setting of significant leukopenia. A bone marrow biopsy confirmed no malignancy. After he moved to the United States, his laboratory work-up revealed marked leukopenia with low absolute neutrophil and lymphocyte count with low T and B cells, very low immunoglobulin levels IgG, IgA, and IgM, and poor vaccination responses to streptococcus pneumonia, varicella zoster, and SARS-CoV-2. Genetic testing revealed a missense pathogenic variant c.511C>T (p.Arg171Trp) in the moesin (MSN) gene associated with X-MAID. He was managed with Bactrim and acyclovir prophylaxis, and immunoglobulin replacement therapy, and considered for hematopoietic stem cell transplantation. Discussion(s): Diagnosis of X-MAID should be considered in patients with recurrent infections and profound lymphopenia. As with SCID, early diagnosis and intervention is of utmost importance to prevent morbidity and mortality. This case demonstrates the importance of genetic testing in identifying this disease as it may prompt an immunologist to consider HSCT if conservative management is suboptimal. In the current literature, HSCT appears promising, but the long-term outcomes have yet to be described.Copyright © 2023 Elsevier Inc.
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Introduccion: La prevalencia de esofagitis infecciosa en individuos inmunocompetentes es inusual. El principal agente etiologico es el VHS 1, afectando en la mayoria de los casos a menores de 40 anos con odinofagia intensa y disfagia como sintomas principales. Se desconoce si la infeccion por SARS-CoV-2 o la administracion de su vacuna son factores predisponentes. Objetivos: Determinar las principales caracteristicas clinicas, diagnosticas, endoscopicas y la respuesta al tratamiento de pacientes diagnosticados de esofagitis virica desde el inicio de la pandemia. Metodos: Se incluye una serie de casos de pacientes inmunocompetentes con esofagitis virica en el Complejo Asistencial Universitario de Leon desde marzo 2020 hasta enero 2022. Resultados: Un total de cinco pacientes fueron diagnosticados de esofagitis virica. Los datos personales, clinicos, diagnosticos y terapeuticos de todos los casos se recogen en la tabla. Conclusiones: La esofagitis virica en pacientes inmunocompetentes es infrecuente, lo que conlleva su infradiagnostico. Se deben sospechar ante un cuadro agudo de odinofagia intensa. En nuestra serie el diagnostico histologico fue compatible solo en el 40%. Al tratarse de una infeccion autolimitada en individuos inmunocompetentes, existen discrepancias entre administrar aciclovir o unicamente tratar los sintomas. La infeccion por SARS-CoV-2 o la administracion de sus vacunas podrian actuar como factores predisponentes, aunque se necesitan mas estudios para establecer dicha relacion. [Table presented]Copyright © 2023 Elsevier Espana, S.L.U. Todos los derechos reservados.
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Objectives: A 14-year-old female patient presents with marked haemorrhagic, adherent crusting of the upper and lower lip and enoral vesicles and erosions. Two weeks before, she had suffered from a respiratory tract infection. She did not take antibiotics but ibuprofen. One week later, she described a swelling and crusting of the upper and lower lips. Urogenital mucosa was also erosive. There was no ocular involvement. Another week later, cocard-like single lesions with partly central blister formation developed. A flaccid blister of 15 mm in diameter was detected in the left ear helix. In total, there was a limited cutaneous involvement of <10% BSA. The girl was admitted to the paediatric clinic. Method(s): Due to mucocutaneous eruptions, bullous lesions and multimucosal involvement, we assumed a Steven-Johnson syndrome or reactive infectious mucocutaneous eruption (RIME). Intravenous rehydration and prophylactic administration of cefotaxime and aciclovir were given. She was balanced and given analgesia with novalgin. The recent increased intake of ibuprofen was discontinued. Local therapy included mometasone cream and serasept dressings. During the inpatient stay, the general condition stabilised and the skin efflorescence's showed a clear regression. Result(s): The microbiological smears for COVID-19, HSV, VZV, mycoplasma, and chlamydia were negative. Discussion(s): As adult classifications for blistering severe cutaneous adverse reactions are limited applicable in children, Ramien et al. proposed revised paediatric-focused clinical criteria 2021. They leave traditional definitions of EEM, SJS and TEN. But they distinguish erythema multiforme (EM) for classic targets with/without mucosal involvement, RIME for cases with mucosal predominance and a respiratory infection trigger, and drug-induced epidermal necrolysis (DEN) for cases caused by medications. (Ramien BJD 2021) There are no current guidelines for RIME therapy. A reasonable management approach includes symptomatic therapy, treatment of identifiable infectious triggers (if possible), consulting urologists, ophthalmologists and gynaecologists (if necessary), immunosuppression, and psychological support. (Ramien ClinExpDermatol 2021).
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Clinical presentation of COVID-19 infection can be variable in the current pandemic even in patients presenting to the clinic with a mild history of upper respiratory complaints. Various cutaneous manifestations have been noticed in COVID-19 patients with herpes zoster (HZ) being one among them. HZ is an infection that results when varicella zoster virus reactivates from its latent state in the posterior dorsal root ganglion. Here, we aim to expand our knowledge by reporting three cases of associated zoster infection in COVID-19 patients admitted to our intensive care unit in view of respiratory complaints. All the three patients admitted, had revealed lymphocytopenia at the time of HZ diagnosis, and were managed conservatively throughout the course. In all the cases, acyclovir/valacyclovir led to the resolution of lesions in 10 days. No postherpetic sequelae were observed. We here suggest that the clinical presentation of HZ at the time of the current pandemic should be considered as an alarming sign for a latent subclinical SARS CoV-2 infection and thorough follow-up of such patients be adopted.Copyright © 2021 Bali Journal of Anesthesiology. All rights reserved.
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Background/Aims Advances in rational drug design and recent clinical trials are leading to emergence of a range of novel therapies for SLE and therapeutic options in clinical practice are expected to broaden rapidly. The optimal real-world place of emerging and established agents will be guided by understanding their differential efficacy on specific SLE manifestations as well as efficacy for more resistant disease. Anifrolumab, a type-I interferon receptor blocking monoclonal antibody, showed efficacy in SLE in phase III trials with a notable effect on mucocutaneous disease although specific lesion subtypes and chroncicity were not explored. Severe refractory mucocutaneous SLE such as scarring discoid lesions are an important and common clinical challenge in current practice. We therefore prospectively evaluated the real-world efficacy and quality of life impact of anifolumab for active mucocutaneous SLE, recalcitrant to multiple biologic and immunosuppressant therapies. Methods Seven patients commenced anifrolumab (300mg by monthly iv infusion) following application to the manufacturer's early access programme (NCT 04750057). Prior biologic therapies were discontinued at least 5 half-lives in advance. Mucocutaneous disease activity was captured by Cutaneous Lupus Disease Area and Severity Index (CLASI) activity score and medical photography. Patient reported health-related quality of life comprising the Dermatology Life Quality Index (DLQI);Lupus-QoL and EQ5D-5L were evaluated at baseline, three and six months. Results Seven female patients with active mucocutaneous SLE (Discoid LE n=5, chilblain LE n=1, subacute cutaneous LE n=1) and median disease duration of 17 years were evaluated. Median baseline CLASI activity score was 17 (range 10-26;higher scores indicating severe disease). Median number of previously failed therapies was 7 and included rituximab in 6/7, belimumab in 2/7 and thalidomide in 4/7. Rapid resolution of scale and erythema in DLE was established within 1 month of anifrolumab treatment. Improvements to chilblain lupus were evident by three months. CLASI activity score was improved >=75% in all patients at 3 months. Clinical responses were associated with significant improvements in DLQI (p<0.001) and EQ5D-VAS (p=0.002) by three months. Lupus-QoL trended toward improvement across all domains but most strongly for fatigue (p=0.01) and pain (p=0.002) by 6 months. One patient discontinued treatment after 4 months due to polydermatomal shingles complicated by sensorineural hearing loss. Infection coincided with background prednisolone dose >15mg daily, recent COVID-19 infection and new on-treatment hypogammaglobulinaemia (IgG <5g/L). Prolonged aciclovir treatment was required for lesion resolution. Conclusion We report rapid real-world efficacy and quality of life impact of anifrolumab on highly refractory mucocutaneous SLE, which exceeded that anticipated from existing clinical trial data. Findings suggest a unique role for emerging interferon targeting therapies in management of mucocutaneous SLE but emphasize need for enhanced VZV precautions among higher risk patients.
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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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Background Management of patients with multiple sclerosis (MS) and evidence of disease activity during treatment with cladribine tablets represents a challenging point. Objectives To report a patient with highly active multiple sclerosis (HAMS) who has been early switched from cladribine to alemtuzumab owing to tumultuous clinical and radiological activity Methods A single retrospective case report. Results. Treatment with alemtuzumab has led to a complete suppression of disease activity without any evidence of infections or acquired autoimmune diseases. Conclusion Our report suggests that an early switch from cladribine to alemtuzumab, may be safe and efficacious in selected HAMS cases.Copyright © 2022 The Authors
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Case report Background: Neurological adverse effects (NAE) as headache and dizziness are commonly reported with COVID-19 vaccines but are transient and self-limited. However, few serious NAE have been recently described which can be fatal. Here we report two rare cases of encephalitis related to COVID-19vaccination BNT162b2 (Pfizer) and mRNA-1273 (Moderna) and the inherent challenges in their diagnosis. Method(s): We report two cases of acute encephalitis notified to the department of pharmacovigilance in the University Hospital of Monastir Results: Case n'1: Three weeks after receiving her first dose of mRNA-1273, a 35-year- old female, with a medical history of hypothyroidism and eczema was admitted to the intensive care unit as she had confusion and a febrile tonic-clonic seizure complicated with a status epilepticus and dysautonomia. CSF investigations were nonspecific, and the MRI head did not detect any abnormality. Common causes were excluded by an extensive workup (neoplastic, neuro-vascular, autoimmune and infectious causes). She received cefotaxime and acyclovir without any recovery. However, a spectacular recovery was noticed when receiving methylprednisolone. Case n'2: Three days after receiving her first dose of BNT162b2, a-40- year- old female, with a medical history of rheumatoid arthritis was admitted to the medical care unit as she had experienced a three-day history of headache, memory disturbance, severe cognitive disorders and 4 febrile tonic-clonic seizures. MRI head showed signs of bitemporal encephalitis and CSF investigations was with no findings. Extensive laboratory studies ran out alternative causes as neoplastic, autoimmune and infectious diseases. A twenty-one- day acyclovir regimen was administrated with no recovery. As the cognitive deficit is getting more severe, she got intravenous immunoglobulin therapy with a spectacular improvement. Conclusion(s): Based on the Naranjo Algorithm, this adverse NAR can be possibly (score = 6) induced by COVID-19 vaccines. The dramatic improvement after receiving either corticoids or immunoglobulin therapy supports an immune-mediated mechanism behind this acute presentation. Cases of acute encephalitis secondary to H1N1 influenza and poliomyelitis vaccines have been previously reported but those related to COVID-19 vaccines are still not yet elucidated due to the unproven causality. Further prospective studies are needed to evaluate the causal association between vaccine and NAE occurring vaccination.
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Antibody deficiencies constitute the majority of primary immunodeficiencies in adults. These patients have a well-established increased risk of bacterial infections but there is a lack of knowledge regarding the relative risks upon contracting COVID-19. In this monocentric study the disease course of COVID-19 in 1 patient with Good's syndrome and in 13 patients with common variable immunodeficiency (CVID) is described. The severity of disease ranged from very mild to severe. Several patients required hospitalization and immunomodulatory treatment but all survived. Although viral infections are not a typical feature of humoral immunodeficiencies we recommend that vigilance is increased in the management of patients with Good's syndrome and CVID during the COVID-19 pandemic.Copyright © 2021
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Background: Literature describing triggers of GFAP astrocytopathy (GFAP-A) is limited. We report a case of GFAP-A in a patient with recent messenger ribonucleic acid (mRNA) severe acute respiratory syndrome corona virus 2 (SARS-CoV-2) vaccination and discuss the possible pathogenesis. Case description: A 45-year-old gentleman presented with features of meningoencephalitis 31 days after the first dose and 4 days after the second dose of mRNA SARS-CoV-2 vaccination. He sequentially developed brainstem/cerebellar, autonomic and cord dysfunction. Cerebrospinal fluid was positive for GFAP autoantibody. Clinical improvement occurred after intravenous methylprednisolone and immunoglobulins. Conclusion(s): Although we are uncertain of a causal link of GFAP-A to mRNA vaccine, indirect activation of an underlying dysregulated immune milieu is plausible.Copyright © 2021 The Author(s)
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Introduction: Varicella zoster virus is a highly infectious alpha-herpesvirus, pathogenic only to humans. The primary infection of varicella zoster virus causes chickenpox, which is contagious and primarily infects children and adolescents in India. Following the primary infection, the virus remains dormant in sensory root ganglia. Activation of the dormant virus in later stages of life causes herpes zoster infection which may vary from subclinical infection to typical zoster, scattered vesicles, zoster sine herpete or disseminated zoster, which depends on the individual's immune status. Case report: In this case series, we present two patients with herpes zoster involving the mandibular branch of the trigeminal nerve. Cytology revealed characteristic features of the infection including nuclear moulding, multinucleated giant cells and ballooning degeneration. Conclusion(s): More recently, patients presenting with herpes zoster have been reported to have sub-clinical Covid-19 infection, suggesting a possibility that herpes zoster might be an indicator for latent Covid-19. Timely detection and treatment of this infection can reduce the risk of post herpetic neuralgia.Copyright © 2022 Sciendo. All rights reserved.
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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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This study aimed to review zinc's effectiveness as an antivirus in treating herpes simplex virus infection. The authors use international journals published from 2000-2022, and use search engines such as Google Scholar, PubMed, and Science Direct with the keywords "zinc and herpes simplex virus". The herpes simplex virus that often causes symptoms in humans are HSV type 1 and type 2. The lesions appear as vesicles which then rupture into ulcers. Zinc is one of the most abundant nutrients or metals in the human body besides iron. Studies about the effects of zinc on HSV have shown that it has the function of inhibiting the viral life cycle. HSV attaches to the host cells to replicate and synthesize new viral proteins. Zinc can inhibit this process by depositing on the surface of the virion and inactivating the enzymatic function which is required for the attachment to the host cell, disrupting the surface glycoprotein of the viral membrane so it could not adhere and carry out the next life cycle, it can also inhibit the function of DNA polymerase that works for viral replication in the host cell. This article showed that zinc has effectiveness as an antivirus against the herpes simplex virus, therefore, patients infected with HSV can be treated with zinc as an alternative to an antivirus drug.Copyright © 2022 The Authors. Published by Innovare Academic Sciences Pvt Ltd.
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Background: The SARS-CoV-2 invades the cells by attachment of virus spike proteins (S1, S2) to cell membrane and engages angiotensin-converting enzyme 2 (ACE2), which is highly expressed in the epithelium of cerebral vasculature. Here, we describe a patient with encephalitis following SARS-CoV-2 infection. Case presentation: A 77-year-old male patient presented with mild cough and coryza lasting for eight days without a prior history of underlying disease or neurologic disorder. Oxygen saturation (SatO2) was decreased and behavioral changes, confusion, and headaches were started within three days prior to admission. Bilateral ground glass opacifications and consolidations were noted on chest CT scan. Lymphopenia, highly elevated D-Dimer and ferritin were noted in laboratory results. Brain CT and MRI showed no changes regarding encephalitis. Cerebrospinal fluid was collected as the symptoms persisted. The results of SARS-CoV-2 RNA RT-PCR from CSF and nasopharyngeal samples were positive. The combination therapy with remdesivir, interferon beta-1alpha and methylprednisolone was started. Due to deterioration of the patient's status and SatO2, he was admitted to the ICU and intubated. Tocilizumab, dexamethasone, and mannitol were started. The patient was extubated on the 16th day of ICU admission. The patient's level of consciousness and SatO2 were improved. He was discharged from the hospital a week later. Conclusion(s): RT-PCR of CSF sample along with brain imaging can help with diagnosis when encephalitis due to SARS-CoV-2 is suspected. However, no changes regarding encephalitis may be seen on brain CT or MRI. Combination therapy with antivirals, interferon beta, corticosteroids, and tocilizumab can help patients recover in these conditions.Copyright © 2022 NRITLD, National Research Institute of Tuberculosis and Lung Disease, Iran.
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Introduction: Since 2019, COVID-19 pneumonia caused by SARS-CoV-2 virus has led to a worldwide pandemic. Since then, various neurological manifestations of COVID-19 pneumonia have been reported. Neurological manifestations include headache, anosmia, seizures, and altered mental status. In some cases, it presents as stroke, encephalitis, and neuropathy. Artery of Percheron (AOP) is a variant in the posterior circulation. Here, a single artery arises from the posterior cerebral artery p1 segment. It supplies bilateral thalamus with or without midbrain. Thrombosis in this artery leads to clinical symptoms like reduced level of consciousness, altered mental status, and memory impairment. Case Report: Here, we present a case who presented with fever and altered sensorium without any focal neurological deficits and without known risk factors for stroke. His COVID-19 PCR was positive. He was initially diagnosed as COVID-19 pneumonia with encephalitis and was started on treatment for the same. His initial CT brain and lumbar puncture were normal. The next day, when MRI brain with and without contrast was done, the thalamic stroke due to AOP infarction was diagnosed and appropriate treatment for stroke was initiated. Discussion(s): Many patients miss the window for thrombolysis because of variable presentation in clinical symptoms with negative imaging. It is also difficult to assess the time of onset of stroke in this varied presentation. Our patient had fever and cough for 2 days and had altered mental status since the morning of admission. During hospital stay, he developed bilateral third nerve palsy. This case also highlights the importance of detailed evaluation in COVID-19 patients with neurological complaints. This helps to avoid delays in treatment and to improve clinical outcomes. As our knowledge of COVID-19 and its varied neurological manifestations evolve, we need to be prepared for more atypical presentation to facilitate timely interventions.Copyright © 2022 The Author(s). Published by S. Karger AG, Basel.
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Background: Dimethyl fumarate (DMF) is an approved treatment for multiple sclerosis (MS). Due to its efficacy and safety profile, DMF is the most prescribed oral medication for relapsing remitting (RR) MS. Given the long-term course of treatment with DMF in MS, continuous surveillance of opportunistic infections is fundamental. Case presentation: We report the occurrence of facial herpes zoster (HZ) associated with MS disease reactivation in a person with RRMS after 6 years of DMF therapy. Case report: A 44-year-old woman with RRMS developed right temple pain and blisters over the right cheek, suggestive of facial HZ. A normal lymphocyte count with however relatively lower proportions of CD8+ T cells and higher percentages of natural killer cells were detected in blood. The patient failed oral treatment and required hospitalization for intravenous acyclovir. She eventually developed symptoms of an MS exacerbation featured by lower extremities weakness and urinary retention. Conclusion(s): Our case highlights the importance of counseling patients on the possibility of HZ reactivation even in the setting of a normal lymphocyte count, the risk of MS exacerbation possibly associated with HZ occurrence and the importance of timely vaccination.Copyright © 2022
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Significant progress in understanding cancer pathogenesis, it remains one of the leading causes of death after cardiovascular diseases. Similarly viral infections have emerged from wildlife or re-emerged, generating serious threats to the global health. As a result, there is an urgent need for the development of novel, more effective anticancer and antiviral therapeutics. Scientists, medicinal chemists and researchers are continuously finding novel targets, mechanisms and molecules against theses severe and dangerous infections. Therefore, ongoing extensively study and research emphasizes 1,3,4 thiadiazole pharmacophore have versatile pharmacological actions. Due to mesoionic behaviour of 1,3,4 thiadiazole pharmacophore allows to enter and easily cross biological membrane which allow to interact various biological proteins. In this review study an attempt has been made of various mechanisms involved in cancer and viral prevalence with updated studies done so far. This review study also findings the role of 1,3,4 thiadiazole motif in the management of various cancers and viral infection. This study also highlighting research statics on clinical trials and various patents containing 1,3,4 thiadiazole derivatives. © 2022 The Author(s)