[Dermato-neuro syndrome-an acute and life-threatening complication of scleromyxedema Arndt-Gottron]. / Dermato-neurologisches Syndrom ­ eine akute und lebensbedrohliche Komplikation des Skleromyxödems Arndt-Gottron.

Scleromyxedema Arndt-Gottron is the systemic variant of lichen myxedematosus in which mucin accumulation occurs in the dermis. The disease is usually chronically progressive and extracutaneous manifestations or complications are possible. The pathogenesis is unknown and the disease is usually associated with monoclonal gammopathy. High-dose intravenous immunoglobulins (IVIg) are considered to be an effective therapy. We report the case of a patient who developed dermato-neuro syndrome following an interruption of IVIg treatment and a SARS-CoV­2 infection. A similar episode occurred 2 years earlier in association with an influenza A infection. Dermato-neuro syndrome is a potentially lethal neurological complication which is characterized by fever, delirium, convulsions, and coma.

Diagnostic and therapeutic considerations in idiopathic systemic capillary leak syndrome: a case report

Background: Systemic capillary leak syndrome (SCLS) is a rare disorder which leads to severe shock. Typically, endothelial dysfunction leads to massive leakage of fluids from the intravascular compartment to the interstitial space, causing hemoconcentration, hypoalbuminemia, hypotension and potential organ failure. The syndrome may be idiopathic or triggered by disease, such as viral infections. The syndrome is often unrecognized and besides resuscitation, no effective treatments are known. Case Description: Here we describe a 46-year-old female with recurrent episodes of shock due to unrecognized SCLS, with the second episode being triggered by an asymptomatic COVID-19 infection. She was, besides resuscitation, treated with high dose vasopressors and intravenous immunoglobulins (IVIG). The case is complicated by compartment syndrome with infected muscle necrosis and eventually amputation of both lower legs. Moreover, the patient still has a chronic kidney insufficiency. In this case report we will discuss pittfalls and potential therapeutic options in SCLS treatment. Conclusions: Vasopressor use may aggravate ischemic complications in a hypovolemic condition and its use should therefore be discouraged in these patients. Cardiac output monitoring should be considered early. The use of IVIG might be beneficial in the acute phase as well as in preventing future episodes of shock. Whether the use of bevacizumab is also of value is yet unclear. © Journal of Emergency and Critical Care Medicine. All rights reserved.

A Rare Case of Miller Fisher Syndrome in a 45-Year-Old Female.

Miller Fisher syndrome (MFS) is an uncommon form of Guillain-Barré syndrome (GBS), a neurological condition that is acquired, degenerative, demyelinating, and frequently characterized by gradual, symmetrical ascending paralysis. Ophthalmoplegia, ataxia, and areflexia are common symptoms that follow a bacterial or viral infection. Here, we want to draw attention to a rare case of MFS in a 45-year-old Indian female who had dysphagia, dysphasia, ataxia, and dyskinesia while moving around. Unusually, she had no past medical history of Campylobacter jejuni infection, recent vaccinations, upper respiratory tract infections, or any sexually transmitted diseases. Since this disorder has excellent prognosis, early diagnosis and effective treatment are crucial to minimizing unnecessary medical intervention and psychological suffering.

COVID-19 and myocarditis: a review of literature.

Myocarditis has been discovered to be a significant complication of coronavirus disease 2019 (COVID-19), a condition caused by the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) virus. COVID-19 myocarditis seems to have distinct inflammatory characteristics, which make it unique to other viral etiologies. The incidence of COVID-19 myocarditis is still not clear as a wide range of figures have been quoted in the literature; however, it seems that the risk of developing myocarditis increases with more severe infection. Furthermore, the administration of the mRNA COVID-19 vaccine has been associated with the development of myocarditis, particularly after the second dose. COVID-19 myocarditis has a wide variety of presentations, ranging from dyspnea and chest pain to acute heart failure and possibly death. It is important to catch any cases of myocarditis, particularly those presenting with fulminant myocarditis which can be characterized by signs of heart failure and arrythmias. Initial work up for suspected myocarditis should include serial troponins and electrocardiograms. If myocardial damage is detected in these tests, further screening should be carried out. Cardiac magnetic resonance imagining and endomyocardial biopsy are the most useful tests for myocarditis. Treatment for COVID-19 myocarditis is still controversial; however, the use of intravenous immunoglobulins and corticosteroids in combination may be effective, particularly in cases of fulminant myocarditis. Overall, the incidence of COVID-19 myocarditis requires further research, while the use of intravenous immunoglobulins and corticosteroids in conjunction requires large randomized controlled trials to determine their efficacy.

The post-COVID syndrome

Post-COVID syndrome is a multifactorial condition characterized by a combination of neurological, autoimmune, cardiovascular factors as well as psychological stress and neuro-endocrine dysfunction. The most common complaints include shortness of breath, pain syndrome, tachycardia, flu-like syndrome, chronic fatigue, impaired attention, memory, and sleep. These symptoms can develop within 2-4 weeks after acute novel coronavirus infection and persist for many months. The principles of therapy include, first of all, a multidisciplinary approach, and treatment of autoimmune disorders, which can play a key role in the pathogenesis of post-COVID syndrome. Intravenous immunoglobulins in combination with other immunomodulatory techniques can enable millions of patients around the world to improve their quality of life and return to normal activities. © 2023 Elsevier Inc. All rights reserved.

Immune Thrombocytopenic Purpura in Patients with COVID-19.

We described three COVID-19-infected patients with profound immune thrombocytopenia causing haemorrhagic mucocutaneous complications. We conclude that an immune mechanism was responsible as common causes were excluded. Since corticoids were considered harmful in the circumstances, the patients were successfully treated with intravenous immunoglobulins without later relapse. LEARNING POINTS: The severity of haemorrhagic syndrome is not correlated with the severity of COVID-19 infection.Thrombocytopenia in mild COVID-19 infection seems to have an autoimmune mechanism.Intravenous immunoglobulins (1 g/kg) should be the first line of treatment.

Post-COVID-19 Encephalitis With Claustrum Sign Responsive to Immunomodulation.

We describe a case of encephalitis following coronavirus disease 2019 (COVID-19) in a six-and-a-half-year-old girl who presented with acute onset confusion and jerky movements of the limbs. The patient was unvaccinated for COVID-19. Subsequent magnetic resonance imaging revealed a bilateral "claustrum sign" on T2 and fluid-attenuated inversion recovery (FLAIR) images and electroencephalogram reported moderate diffuse encephalopathy. The patient tested negative for COVID-19 by polymerase chain reaction, had positive serology for COVID-19 indicating past infection, and had a negative autoimmune panel and infectious workup. She was treated on the lines of post-infectious encephalitis with immunomodulatory therapies such as high-dose intravenous steroids and intravenous immunoglobulins. She responded significantly and had complete resolution of her symptoms; therefore, further supporting the suspicion of an immune-mediated etiology. Cases of post-COVID-19 encephalitis have been reported all over the world; however, most cases are based on speculation and temporal associations and therefore more research is required to optimize treatment guidelines.

Multisystem Inflammatory Syndrome in Adults Associated With COVID-19: A Case Series on the Importance of Early Diagnosis and Corticosteroid Therapy in Prognosis.

Multisystem inflammatory syndrome in adults (MIS-A) is a rare condition that can occur after an adult has been infected with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). It can occur anywhere between two and 12 weeks after the beginning of acute coronavirus disease 2019 (COVID-19) infection and is characterized by extrapulmonary multiorgan failure. It is primarily seen in young and previously healthy individuals. The exact prevalence of MIS-A is unclear. It is likely underdiagnosed due to overlapping symptoms with severe COVID-19 and difficulty in identifying the syndrome without a preceding COVID-19 infection. The pathogenesis of MIS-A is also largely unknown but is likely caused by an immune response that is dysregulated or antibody-mediated. Treatment primarily involves corticosteroids, but severe cases may require intravenous immune globulin (IVIG). The timing of starting corticosteroid therapy is crucial, as delays can result in increased complications and a longer hospital stay.

Acute Disseminated Encephalomyelitis Following COVID-19 Infection.

Acute disseminated encephalomyelitis (ADEM) is a relatively rare, post-inflammatory, immune-mediated demyelinating central nervous system disease that is predominantly reported in pediatric populations. Following the emergence of severe acute respiratory syndrome coronavirus 2, cases of ADEM are being reported following infection with this virus. Our case report describes a male patient in his early 40s who developed severe coronavirus disease 2019 (COVID-19) that rapidly progressed to a critical disease requiring invasive mechanical ventilation and high positive end-expiratory pressure, which was complicated by extensive neurological involvement and quadriplegia. MRI of the brain showed characteristic demyelinating lesions, suggestive of ADEM. As other entities were ruled out, our patient was treated using pulse steroids and intravenous immunoglobulins. The patient showed a good response to treatment and had an overall good prognosis, despite the severity of his condition. ADEM following COVID-19 is a rare entity worldwide.

A Patient With Myelin Oligodendrocyte Glycoprotein Positive Encephalitis With Ring Enhancing Lesions on Magnetic Resonance Imaging (MRI) After COVID-19 Exposure.

Myelin oligodendrocyte glycoprotein (MOG) antibody has been associated with a wide range of neurological diseases, from neuromyelitis optica spectrum disorder to acute disseminated encephalomyelitis. However, MOG positivity with isolated encephalitis has been infrequently reported. MRI findings are usually of the demyelination type. In this case, we report on a patient with COVID-19 exposure who presented with altered mental status and multiple ring-enhancing lesions on MRI mimicking metastatic disease. Due to his unusual MRI findings and presentation, the correct diagnosis was not apparent until MOG antibody results came back positive.

Atypical Guillain-Barré Syndrome Presenting After COVID-19 Infection.

Guillain-Barré syndrome (GBS) is an acute autoimmune disease affecting the peripheral nervous system presenting as a symmetric, ascending polyneuropathy. The syndrome arises after a stimulus, such as infection or vaccination, and provokes an autoimmune response in the body. Common symptoms include rapidly progressive weakness in the extremities and generalized hyporeflexia or areflexia. However, GBS may have various presentations, which can make for a challenging diagnosis. We present a case of a 46-year-old female with asymmetric ascending weakness, paresthesias, and acute onset urinary retention occurring after Coronavirus Disease 2019 (COVID-19) infection. Of note, this patient did not present with albuminocytologic dissociation in cerebrospinal fluid (CSF) studies. The complex presentation of her symptoms prompted a diagnosis of atypical GBS. Her diagnosis was achieved through a series of diagnostic tests ruling out other etiologies, such as meningitis and spinal cord compression syndromes.

Unexpected Thrombocytopenia in a Parturient With Evans Syndrome Complicated by COVID-19 Infection.

We report the case of a 23-year-old parturient who received epidural analgesia and was subsequently diagnosed with Evans syndrome (ES). The diagnosis was made after a complete blood count (CBC) resulted in severe anemia and a platelet count of less than 10K/µL. To further complicate this case, the patient developed post-delivery pleuritic chest pain and pulmonary emboli (PE), and a chest computed tomography (CT) scan showed bilateral ground-glass lung opacities. This prompted a COVID-19 testing and ultimately confirmed infection.

Miller-Fisher syndrome associated with SARS-CoV-2: a case report.

SARS-CoV-2 infections are increasingly associated with neurological complications, including immune-mediated neuropathies. Miller-Fisher syndrome is a rare variant of Guillain-Barré syndrome characterised by the triad of ataxia, ophthalmoplegia and areflexia. Here we present a case of Miller-Fisher syndrome following COVID-19 infection. The clinical presentation was a short history of a rapidly-progressive peripheral sensorimotor neuropathy with bulbar dysfunction and facial weakness following mild COVID infection. Examination revealed global areflexia and a broad-based ataxic gait. CSF analysis revealed albuminocytological dissociation and neurophysiological testing later supported the diagnosis. The patient required high flow nasal oxygen therapy for respiratory dysfunction in a level 2 care setting and received immunological treatment with intravenous immunoglobulins. We conclude that Miller-Fisher syndrome needs to be considered in patients presenting with new sensorimotor dysfunction following SARS-COV-2 infection. Early recognition is key given the propensity to cause life-threatening respiratory failure, and early administration of immunological treatment is associated with improved prognosis.

Malignant Acute Disseminated Encephalomyelitis (ADEM) Presenting as a Stroke Alert: A Case Report.

Acute disseminated encephalomyelitis (ADEM) is a rare illness. Generally characterized by encephalopathy and non-specific, heterogeneous neurological deficits depending on the location of the demyelinated lesions, ADEM is considered a clinical diagnosis with radiological findings that may or may not have supportive features based on the temporal relationship of an inciting factor and symptom onset. Even rarer, hyperacute or malignant ADEM can be defined by rapid symptom onset followed by catastrophic brain edema and its sequelae. We present a case of a patient who presented with an acute stroke with activation of a rapid sequence care pathway (stroke alert protocol) to mobilize resources that could expedite his care to determine eligibility for thrombolysis. ADEM was the definitive diagnosis with a subsequent rapid and treatment-refractory decline.

Efficacy and Safety of Blood Derivative Therapy for Patients with COVID-19: A Systematic Review and Meta-Analysis.

Background: The outbreak of COVID-19 has resulted in more than 200 million infections and 4 million deaths. The blood derivative therapy represented by intravenous immunoglobulin (IVIG) and convalescent plasma (CP) therapy may be the promising therapeutics for COVID-19. Methods: A systematic article search was performed for eligible studies published up to August 3, 2021, through the PubMed, Embase, Cochrane Library. The included articles were screened by using rigorous inclusion and exclusion criteria. All analyses were conducted using Review Manager 5.4. Quality of studies and risk of bias were evaluated. Results: A total of 5 IVIG therapy and 13 CP therapy randomized controlled trials were included with a sample size of 13,696 subjects diagnosed with COVID-19. IVIG could reduce the mortality compared with the control group (RR 0.65, 95% CI: 0.46-0.93, p = 0.02). The use of CP did not effectively reduce the mortality (RR 0.97, 95% CI: 0.91-1.03, p = 0.38), the length of hospital stay (MD -0.47, 95% CI: -4.13 to 3.20, p = 0.80), and the mechanical ventilation use (RR = 0.98, 95% CI: 0.89-1.07, p = 0.62) of the patients with COVID-19. Treatment with IVIG or CP was not significantly associated with an increase in reported adverse events (RR 1.07, 95% CI: 0.94-1.22, p = 0.28). Conclusions: Treatment with IVIG could be effective and safe to improve survival for patients with COVID-19. But the benefit of CP in the treatment of COVID-19 is limited. The certainty of the evidence was moderate for all outcomes.

Diagnosis of Multisystem Inflammatory Syndrome in Children in a Resource-Limited Center.

Multisystem inflammatory syndrome in children (MIS-C) has become a serious disease entity following the high prevalence of coronavirus disease 2019 (COVID-19) infection with the involvement of gastrointestinal organs, kidneys, heart, and lungs. When the patient presents with mucocutaneous findings such as conjunctival injection, red lips, neurocognitive symptoms, swollen hands and lymphadenopathy, it is always highly recommended to exclude multisystem inflammatory syndrome.  As it affects multiple organs, it can result in more serious consequences. The manifestations depend largely on the organ involved. Therefore, successful management partly depends on the early diagnosis. Many treatment strategies have been put forth to tackle the disorder so far.

Focal Seizures in a Child Following COVID-19 Infection: A Case Report.

Coronavirus disease 2019 (COVID-19) has been shown to impact multiple organs, even in instances where patients did not show any symptoms. In this case report, we detail a six-year-old male child presenting with focal seizures without an antecedent history of epilepsy. The child presented with twitching movements on the right side of the face involving the oral cavity. Non-contrast brain MRI showed meningoencephalitis. He was given antibiotics, antipyretics, and antiepileptic drugs (AEDs), but his clinical condition continued to deteriorate despite treatment. Oropharyngeal and nasopharyngeal swabs tested positive for COVID-19. Thus, treatment was initiated for COVID-19 encephalitis and seizures with intravenous immune globulin (IVIG) and steroids. Frequency of seizures decreased dramatically after steroids were initiated and remained infrequent during the five days of steroid therapy. After steroids were discontinued seizures returned but were shorter, less frequent and manageable with AEDs. The child was discharged on AEDs and was seizure-free at six months of follow-up. The following case report details the disease and treatment pathway of the patient.