Pediatric Acute Onset Neuropsychiatric Syndrome Associated with Epstein-barr Infection in Child with Noonan Syndrome

Author(s):  
Giuseppe Pietro Tisi
2017 ◽  
Vol 41 (S1) ◽  
pp. S456-S456
Author(s):  
G.P. Tisi ◽  
M. Marzolini ◽  
G. Biffi

IntroductionPediatric acute onset neuropsychiatric syndrome is associated with various infections (i.e. Streptococcus, Mycoplasma pneumoniae).ObjectivesWe describe a case of PANS associated with mononucleosis, in a patient with Noonan syndrome.AimsTo report a case of EBV-related PANS.MethodsA 13-year-old patient, diagnosed with Noonan syndrome, was referred to the pediatric unit of our hospital in August 2016 because of aggressive behavior and suicidal ideation. He had no personal or family history of psychiatric disorder. His parents and him denied substance abuse. His symptoms had begun abruptly one month prior to our evaluation, after watching an internet video, and consisted in intrusive thoughts and images associated with mental compulsions. Suicidal thoughts and verbal aggressiveness emerged because he felt overwhelmed by these symptoms.ResultsHe was initially treated with sertraline 25 mg, and subsequently switched to aripiprazole because of increased anxiety. Throat cultures and anti-streptolysin titer (ASO) were negative, as well as Ig(M) and Ig(G) antibodies for M. pneumoniae. Erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) were also negative. Epstein–Barr virus Ig(M) and Ig(G) were positive. He continued therapy with aripiprazole 10 mg after hospital discharge with partial benefit.ConclusionsEpstein–Barr virus infection has been reported to precede various neuropsychological disorders, but to the best of our knowledge, rare cases of PANS following mononucleosis have been described in literature. In our case, psychopharmacological treatment for OCD symptoms was the only treatment performed and led to a partial remission of symptoms.Disclosure of interestThe authors have not supplied their declaration of competing interest.


2021 ◽  
Vol 14 (8) ◽  
pp. e238078
Author(s):  
Anjely Pulparampil Sebastian ◽  
Arini Basu ◽  
Nandini Mitta ◽  
Dominic Benjamin

Transverse myelitis is a rare neurological complication seen with varicella-zoster virus (VZV) infection, which is common among immunocompromised hosts. It can occur during the primary VZV infection or reactivation of latent infection. It is a complication that requires prompt diagnosis and treatment. The present case is that of a 28-year-old immunocompetent man, who presented with fever, rash and acute-onset spastic paraparesis with bladder involvement. Causes such as herpes simplex 1 and 2, cytomegalovirus, enterovirus and Epstein-Barr virus infection were ruled out. On evaluation, he was diagnosed with acute primary disseminated VZV infection with parainfectious transverse myelitis, based on positive cerebrospinal fluid multiplex PCR (PCR) and serum VZV IgM antibodies. He was treated with intravenous acyclovir and steroids, with which he improved significantly.


2020 ◽  
Vol 13 (1) ◽  
pp. e233190
Author(s):  
George Luis Leonor Lopez ◽  
Sandra Viviana Chaparro ◽  
Nicholas Brozzi ◽  
Amit Badiye

An 18-year-old male patient presented to the emergency department complaining of new onset chest pain, fever and orthopnoea. Initial workup was remarkable for elevated troponin, diffuse ST-segment elevation on ECG and chest X-ray with enlarged cardiac silhouette. Transthoracic echocardiogram (TTE) demonstrates severe biventricular concentric hypertrophy and pericardial effusion. Also, Coxsackie virus A and B titres were positive, concerning for a classic viral pericarditis. However, despite medical management, the patient became dyspnoeic and hypotensive. Impending cardiac tamponade was observed on repeat TTE, and pericardiocentesis was performed, complicated by pulseless electrical activity cardiac arrest, and ultimately patient requiring venoarterial extracorporeal membrane oxygenation support. Emergent endomyocardial biopsy showed no inflammatory process, and a skin biopsy of a small lesion in the right arm showed unexpected diagnosis of Epstein-Barr virus (+) natural killer/T-cell lymphoma. On initiation of chemotherapy, clinical improvement was observed as evidenced by improving ejection fraction, resolution of pericardial effusion and gradual decrease in myocardial hypertrophy.


2021 ◽  
Vol 2 (3) ◽  
pp. 126-131
Author(s):  
DA Kinderlehrer

In 1994, Susan Swedo and colleagues described children who developed mental health issues following infection with Group A Streptococcus (GAS) infections, and in a subsequent report coined the term Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal Infections (PANDAS) [1,2]. In short order it was discovered that multiple microbes have the potential of triggering mental health issues in children and adolescents, and the nomenclature was updated to Pediatric Acute-Onset Neuropsychiatric Syndrome (PANS) [3]. The microbes that thus far have been associated with PANS include herpes simplex virus, influenza A virus, varicella virus, HIV, recurrent sinusitis, Epstein-Barr virus, the common cold, Mycoplasma pneumoniae and Bartonella henselae [4-6].


2021 ◽  
pp. 1-7
Author(s):  
Eduardo Orrego-González ◽  
Carlos Martin-Restrepo ◽  
Alberto Velez-Van-Meerbeke

Noonan syndrome with multiple lentigines (NSML), previously known as LEOPARD syndrome, is a rare autosomal dominant disorder with an unknown prevalence. Characteristics of this disease include cutaneous, neurologic, and cardiologic abnormalities. In this case report, we present a 12-year-old girl who was admitted to the emergency department for acute-onset left weakness, unsteady gait, nausea, and vomiting. Her physical exam notably showed left side upper motor neuron signs and dysmetria. CT scan revealed an acute hemorrhage of the right thalamus. Physical exam exhibited several craniofacial dysmorphisms and lentigines. The genetic test revealed a heterozygous missense mutation in the protein tyrosine phosphatase non-receptor type 11 (<i>PTPN11</i>) gene and a variant of unknown significance of the <i>MYH11</i> gene. To the best of our knowledge, this is the first case of a patient with NSML presenting an intracerebral hemorrhage.


Author(s):  
B. G. Uzman ◽  
M. M. Kasac ◽  
H. Saito ◽  
A. Krishan

In conjunction with the cultivation and transplantation of cells from human tumors by the Programs of Microbiology and Immunogenetics, virus surveillance by electron microscopy has been routinely employed. Of particular interest in this regard have been 3 cell lines cultured from lymph nodes or spleen of 2 patients with Hodgkin's disease and 1 patient with Letterer-Siwe's disease. Each of these cell lines when transplanted in Syrian hamster neonates conditioned with anti-lymphocyte serum grew as serially transplantable tumors; from such transplants of the 3 cell lines cell cultures were retrieved.Herpes type virus particles (Figs. 1, 2, 3) were found in the primary cultures of all three lines, in frozen thawed aliquots of same, and in cultures retrieved from their tumors growing by serial transplantation in hamsters. No virus was detected in sections of 25 of the serially transplanted tumors. However, in 10 such tumors there were repeated instances of tubular arrays in the cisternae of the endoplasmic reticulum (Fig. 4). On serologic examination the herpes virus was shown to be the Epstein-Barr virus.


Author(s):  
C. M. Payne ◽  
P. M. Tennican

In the normal peripheral circulation there exists a sub-population of lymphocytes which is ultrastructurally distinct. This lymphocyte is identified under the electron microscope by the presence of cytoplasmic microtubular-like inclusions called parallel tubular arrays (PTA) (Figure 1), and contains Fc-receptors for cytophilic antibody. In this study, lymphocytes containing PTA (PTA-lymphocytes) were quantitated from serial peripheral blood specimens obtained from two patients with Epstein -Barr Virus mononucleosis and two patients with cytomegalovirus mononucleosis. This data was then correlated with the clinical state of the patient.It was determined that both the percentage and absolute number of PTA- lymphocytes was highest during the acute phase of the illness. In follow-up specimens, three of the four patients' absolute lymphocyte count fell to within normal limits before the absolute PTA-lymphocyte count.In one patient who was followed for almost a year, the absolute PTA- lymphocyte count was consistently elevated (Figure 2). The estimation of absolute PTA-lymphocyte counts was determined to be valid after a morphometric analysis of the cellular areas occupied by PTA during the acute and convalescent phases of the disease revealed no statistical differences.


Author(s):  
R. Stephens ◽  
K. Traul ◽  
D. Woolf ◽  
P. Gaudreau

A number of antigens have been found associated with persistent EBV infections of lymphoblastoid cells. Identification and localization of these antigens were principally by immunofluorescence (IF) techniques using sera from patients with nasopharyngeal carcinoma (NPC), Burkitt lymphoma (BL), and infectious mononucleosis (IM). Our study was mainly with three of the EBV related antigens, a) virus capsid antigen (VCA), b) membrane antigen (MA), and c) early antigens (EA) using immunoperoxidase (IP) techniques with electron microscopy (EM) to elucidate the sites of reactivity with EBV and EBV infected cells.Prior to labeling with horseradish peroxidase (HRP), sera from NPC, IM, and BL cases were characterized for various reactivities by the indirect IF technique. Modifications of the direct IP procedure described by Shabo and the indirect IP procedure of Leduc were made to enhance penetration of the cells and preservation of antigen reactivity.


2000 ◽  
Vol 111 (1) ◽  
pp. 239-246 ◽  
Author(s):  
Kenny I. K. Lei ◽  
Lisa Y.S. Chan ◽  
Wing Y. Chan ◽  
Philip J. Johnson ◽  
Y. M. Dennis Lo

1996 ◽  
Vol 21 (2) ◽  
pp. 123-126
Author(s):  
U. BALDARI ◽  
A. ASCARI RACCAGNI ◽  
B. CELLI ◽  
M. GIOVANNA RIGHINI

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