scholarly journals Case Report: Paradoxical Inflammatory Response Syndrome in a Previously Healthy, HIV-Negative, Pediatric Patient With Cryptococcus gatii Meningitis

2021 ◽  
Vol 9 ◽  
Author(s):  
Jessica H. Cheng ◽  
Ritu Cheema ◽  
Peter R. Williamson ◽  
Victoria R. Dimitriades
Keyword(s):  
Inflammatory Response ◽  
Pediatric Patient ◽  
Immunological Response ◽  
Adolescent Male ◽  
Healthy Adolescent ◽  
Immune Mediated ◽  
Microbiological Control ◽  
The Central Nervous System ◽  
Post Infectious

The immunological response of patients with cryptococcal meningitis (CM), particularly those not known to be immunocompromised, has generated an increased interest recently. Although CM is an infection with significant rates of morbidity and mortality, its sequelae may also include a post-infectious inflammatory response syndrome (PIIRS) in patients who have already achieved microbiological control. PIIRS can cause substantial immune-mediated damage to the central nervous system resulting in long-term neurological disability or even death. Steroids have been used successfully in the management of PIIRS in adults. In this report, we present the case of a previously healthy adolescent male with Cryptococcus gattii meningitis who experienced neurological deterioration due to PIIRS after the initiation of antifungal therapy. Immunological workup did not demonstrate any frank underlying immunodeficiencies, and genetic primary immunodeficiency screening was unremarkable. He was treated with steroids and recovered clinically; however, intermittent inflammatory episodes needed to be managed through several flares of symptoms. In the setting of the current literature, we discuss the management and monitoring of PIIRS in a pediatric patient, along with considerations of targeted future therapies.

scholarly journals Autoimmune Hemolytic Anemia in the Pediatric Setting

2021 ◽  
Vol 10 (2) ◽  
pp. 216
Author(s):  
Aikaterini Voulgaridou ◽  
Theodosia A. Kalfa
Keyword(s):  
Hemolytic Anemia ◽  
Autoimmune Hemolytic Anemia ◽  
Positive Outcome ◽  
Cold Temperatures ◽  
Negative Results ◽  
Splenic Macrophages ◽  
Immune Mediated ◽  
Post Infectious ◽  
Paroxysmal Cold Hemoglobinuria

Autoimmune hemolytic anemia (AIHA) is a rare disease in children, presenting with variable severity. Most commonly, warm-reactive IgG antibodies bind erythrocytes at 37 °C and induce opsonization and phagocytosis mainly by the splenic macrophages, causing warm AIHA (w-AIHA). Post-infectious cold-reactive antibodies can also lead to hemolysis following the patient’s exposure to cold temperatures, causing cold agglutinin syndrome (CAS) due to IgM autoantibodies, or paroxysmal cold hemoglobinuria (PCH) due to atypical IgG autoantibodies which bind their target RBC antigen and fix complement at 4 °C. Cold-reactive antibodies mainly induce intravascular hemolysis after complement activation. Direct antiglobulin test (DAT) is the gold standard for AIHA diagnosis; however, DAT negative results are seen in up to 11% of warm AIHA, highlighting the need to pursue further evaluation in cases with a phenotype compatible with immune-mediated hemolytic anemia despite negative DAT. Prompt supportive care, initiation of treatment with steroids for w-AIHA, and transfusion if necessary for symptomatic or fast-evolving anemia is crucial for a positive outcome. w-AIHA in children is often secondary to underlying immune dysregulation syndromes and thus, screening for such disorders is recommended at presentation, before initiating treatment with immunosuppressants, to determine prognosis and optimize long-term management potentially with novel targeted medications.

scholarly journals MRI-Negative Myelitis Associated With Myelin Oligodendrocyte Glycoprotein Antibody Spectrum Demyelinating Disease

2019 ◽  
Vol 6 ◽  
pp. 2329048X1983047 ◽  
Author(s):  
Carlos A. Pérez ◽  
Stephanie Garcia-Tarodo ◽  
Regina Troxell
Keyword(s):  
Demyelinating Disease ◽  
Disease Recurrence ◽  
Myelin Oligodendrocyte Glycoprotein ◽  
Spectrum Disorder ◽  
Antibody Testing ◽  
Immune Mediated ◽  
Clinical Presentations ◽  
The Central Nervous System

Myelin oligodendrocyte glycoprotein is expressed in the central nervous system on the surface of oligodendrocytes and is associated with a broad range of adult and pediatric demyelinating phenotypes. The entire spectrum of clinical and radiologic features of myelin oligodendrocyte glycoprotein antibody spectrum disorder remains to be fully elucidated. We describe the case of a 9-year-old boy with immune-mediated myelitis undetectable by conventional magnetic resonance imaging in the context of relapsing anti-myelin oligodendrocyte glycoprotein spectrum disorder. Despite the severe clinical presentation, his symptoms improved significantly following treatment with corticosteroids. Because timely diagnosis and treatment is imperative to prevent disease recurrence and reduce long-term morbidity, serum anti-myelin oligodendrocyte glycoprotein antibody testing should be considered in all children with acute demyelinating syndromes and unusual clinical presentations—including seizures—both at presentation and at follow-up.

scholarly journals The Role of Transcranial Magnetic Stimulation as a Diagnostic Tool for Multiple Sclerosis

2018 ◽  
Author(s):  
Shahid Bashir ◽  
Muhannad M. Alsharidah ◽  
Sarah S.Alseneidi ◽  
Afnan A. Alkharan ◽  
Ali Hamza
Keyword(s):  
Multiple Sclerosis ◽  
Transcranial Magnetic Stimulation ◽  
Disease Activity ◽  
Magnetic Stimulation ◽  
Severity Of Illness ◽  
Chronic Inflammatory Disease ◽  
Synaptic Interactions ◽  
Immune Mediated ◽  
The Central Nervous System

Multiple sclerosis (MS) is an immune-mediated, chronic inflammatory disease of the central nervous system (CNS), characterized by demyelination, axonal degeneration, and cognitive impairment. It also has an important impact on the quality of life of patients and their family members. An estimated 2,500,000 people in the world have multiple sclerosis. Neurophysiological parameters, like sensitivity to demyelination and the strength of excitatory and inhibitory synaptic interactions in the cerebral cortex, can be identified through transcranial magnetic stimulation (TMS) in patients affected by multiple sclerosis (MS). These parameters can be valid and objective parameters that can be correlated with the progression of MS, and can provide reliable indices for the severity of illness and the efficacy of drugs used to treat it. The discovery of specific and detailed neurophysiological parameters as surrogate end points for disease activity could represent an important step in clinical trials. Changes in cortical connectivity have already been demonstrated in MS, but in clinical practice, other measures are usually used to evaluate disease activity. We speculate that TMS may be more effective in identifying disease progression that leads to long-term disability, compared to standard surrogate markers, due to the fact that it represents a direct measure of synaptic transmission(s) in MS.

Isotretinoin induced nail fold pyogenic granuloma resolution with combination therapy: A case report and review of the literature

2019 ◽  
Vol 3 (4) ◽  
pp. 275-278
Author(s):  
Jonathan Bellew ◽  
Chad Taylor ◽  
Jaldeep Daulat ◽  
Vernon Mackey
Keyword(s):  
Pyogenic Granuloma ◽  
Causative Factor ◽  
Adolescent Male ◽  
Review Of The Literature ◽  
First Line ◽  
Healthy Adolescent ◽  
Therapeutic Modalities ◽  
Children And Young Adults ◽  
Intralesional Steroids ◽  
Isotretinoin Therapy

Pyogenic granulomas are vascular hyperplasias presenting as red papules, polyps, or nodules on the gingiva, fingers, lips, face and tongue of children and young adults.  Most commonly they are associated with trauma, but systemic retinoids have rarely been implicated as a causative factor in their appearance.  We present a case of spontaneous eruption of multiple pyogenic granulomas of the bilateral periungal fingers in an otherwise healthy adolescent male undergoing isotretinoin therapy for severe nodulocystic acne. These pyogenic granulomas did not resolve spontaneously with discontinuation of isotretinoin, or first line therapeutic modalities. Their resolution did occur with administration of intralesional steroids and ablation with silver nitrate.

Herbal Medicine for Glioblastoma: Current and Future Prospects

2020 ◽  
Vol 16 (8) ◽  
pp. 1022-1043
Author(s):  
Imran Khan ◽  
Sadaf Mahfooz ◽  
Mustafa A. Hatiboglu
Keyword(s):  
Cell Proliferation ◽  
Survival Rates ◽  
Standard Of Care ◽  
Natural Compounds ◽  
Treatment Modalities ◽  
Normal Brain ◽  
The Central Nervous System ◽  
Cycle Regulation ◽  
Immense Potential

Background: Glioblastoma is one of the most aggressive and devastating tumours of the central nervous system with short survival time. Glioblastoma usually shows fast cell proliferation and invasion of normal brain tissue causing poor prognosis. The present standard of care in patients with glioblastoma includes surgery followed by radiotherapy and temozolomide (TMZ) based chemotherapy. Unfortunately, these approaches are not sufficient to lead a favorable prognosis and survival rates. As the current approaches do not provide a long-term benefit in those patients, new alternative treatments including natural compounds, have drawn attention. Due to their natural origin, they are associated with minimum cellular toxicity towards normal cells and it has become one of the most attractive approaches to treat tumours by natural compounds or phytochemicals. Objective: In the present review, the role of natural compounds or phytochemicals in the treatment of glioblastoma describing their efficacy on various aspects of glioblastoma pathophysiology such as cell proliferation, apoptosis, cell cycle regulation, cellular signaling pathways, chemoresistance and their role in combinatorial therapeutic approaches was described. Methods: Peer-reviewed literature was extracted using Pubmed, EMBASE Ovid and Google Scholar to be reviewed in the present article. Conclusion: Preclinical data available in the literature suggest that phytochemicals hold immense potential to be translated into treatment modalities. However, further clinical studies with conclusive results are required to implement phytochemicals in treatment modalities.

Case Report: Delayed Alemtuzumab-Induced Concurrent Neutropenia and Thrombocytopenia in Relapsing-Remitting Multiple Sclerosis

2021 ◽  
pp. 089719002110212
Author(s):  
Akaansha Ganju ◽  
James C. Stock ◽  
Kim Jordan
Keyword(s):  
Multiple Sclerosis ◽  
Case Reports ◽  
Severe Neutropenia ◽  
Cell Counts ◽  
Immune Mediated ◽  
Relapsing Remitting ◽  
Remitting Multiple Sclerosis ◽  
Study Participants ◽  
Relapsing Remitting Multiple Sclerosis

Alemtuzumab is an anti-CD52 monoclonal antibody used to treat relapsing-remitting multiple sclerosis following failure of second-line medications. It is administered intravenously in 2 treatment sequences 1 year apart. This drug is frequently associated with mild infusion reactions within days of administration, increased infection risk, and long term adverse events from secondary autoimmunity. Alemtuzumab-induced serious immune-mediated thrombocytopenia (ITP) is well-reported and occurred in 1.0-2.2% of participants in initial phase 2 and 3 trials for multiple sclerosis. Significant neutropenia, however, is rare and was only observed in 0.1% of study participants. Delayed neutropenia and/or ITP is thought to occur from secondary autoimmunity. Few case reports have described severe neutropenia occurring beyond 2 months of last alemtuzumab dose. We present an unusual case of delayed combined neutropenia and thrombocytopenia that occurred 15 months after the second infusion of alemtuzumab. The patient was asymptomatic and presented following discovery of neutropenia and thrombocytopenia during routine laboratory studies. The patient responded to steroids initially and was discharged, although outpatient cell counts subsequently revealed recurrent neutropenia and ITP. The adverse drug reaction probability (Naranjo) scale was completed and showed probable likelihood that the adverse event was alemtuzumab-related. Long term screening for delayed hematologic abnormalities, at least 4 years after initial dose, is necessary when using alemtuzumab. Greater research is needed to understand the mechanism of drug-associated neutropenia.

scholarly journals Self-buffering capacity of a human sulfatase for central nervous system delivery

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Yi Wen ◽  
Nazila Salamat-Miller ◽  
Keethkumar Jain ◽  
Katherine Taylor
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Buffering Capacity ◽  
The Self ◽  
High Concentration ◽  
Formulation Design ◽  
The Central Nervous System ◽  
Therapeutic Enzymes ◽  
Intrathecal Space

AbstractDirect delivery of therapeutic enzymes to the Central Nervous System requires stringent formulation design. Not only should the formulation design consider the delicate balance of existing ions, proteins, and osmolality in the cerebrospinal fluid, it must also provide long term efficacy and stability for the enzyme. One fundamental approach to this predicament is designing formulations with no buffering species. In this study, we report a high concentration, saline-based formulation for a human sulfatase for its delivery into the intrathecal space. A high concentration formulation (≤ 40 mg/mL) was developed through a series of systematic studies that demonstrated the feasibility of a self-buffered formulation for this molecule. The self-buffering capacity phenomenon was found to be a product of both the protein itself and potentially the residual phosphates associated with the protein. To date, the self-buffered formulation for this molecule has been stable for up to 4 years when stored at 5 ± 3 °C, with no changes either in the pH values or other quality attributes of the molecule. The high concentration self-buffered protein formulation was also observed to be stable when exposed to multiple freeze–thaw cycles and was robust during in-use and agitation studies.

scholarly journals Acute Disseminated Encephalomyelitis (ADEM): A Demyelinating Disease with Specific Morphological Features

2021 ◽  
pp. 106689692199356
Author(s):  
Fleur Cordier ◽  
Lars Velthof ◽  
David Creytens ◽  
Jo Van Dorpe
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Differential Diagnosis ◽  
Clinical Case ◽  
Demyelinating Disease ◽  
Acute Disseminated Encephalomyelitis ◽  
Demyelinating Diseases ◽  
Demyelinating Disorder ◽  
Immune Mediated ◽  
The Central Nervous System

Acute disseminated encephalomyelitis (ADEM) is a rare immune-mediated inflammatory and demyelinating disorder of the central nervous system. Its characteristic perivenular demyelination and inflammation aid in the differential diagnosis with other inflammatory demyelinating diseases. Here, we present a clinical case of ADEM, summarize its histological hallmarks, and discuss pitfalls concerning the most important neuropathological differential diagnoses.

scholarly journals Nonepileptic Myoclonus in COVID-19: Case Report

2021 ◽  
pp. 194187442110043
Author(s):  
Henly Hewan ◽  
Annie Yang ◽  
Aparna Vaddiparti ◽  
Benison Keung
Keyword(s):  
Case Report ◽  
Critically Ill ◽  
Recovery Phase ◽  
Critically Ill Patient ◽  
High Dose ◽  
The Novel ◽  
Neurological Manifestations ◽  
Immune Mediated ◽  
Novel Coronavirus ◽  
Post Infectious

In late 2019, the novel coronavirus, SARS-CoV-2, and the disease it causes, COVID-19, was identified. Since then many different neurological manifestations of COVID-19 have been well reported. Movement abnormalities have been rarely described. We report here a critically ill patient with COVID-19 who developed generalized myoclonus during the recovery phase of the infection. Myoclonus was associated with cyclical fevers and decreased alertness. Movements were refractory to conventional anti-epileptic therapies. There was concern that myoclonus could be part of a post-infectious immune-mediated syndrome. The patient improved fully with a 4-day course of high-dose steroids. Our experience highlights a rare, generalized myoclonus syndrome associated with COVID-19 that may be immune-mediated and is responsive to treatment.

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