scholarly journals Juvenile Myasthenia Gravis

2018 ◽  
Vol 02 (01) ◽  
pp. E6-E9
Author(s):  
Adela Marina ◽  
Ulrike Schara
Keyword(s):  
Young Children ◽  
Myasthenia Gravis ◽  
Children And Adolescents ◽  
Clinical Symptoms ◽  
Clinical Signs ◽  
Autoimmune Disorder ◽  
Congenital Myasthenic Syndrome ◽  
Muscular Weakness ◽  
Specific Antibodies ◽  
Juvenile Myasthenia Gravis

AbstractJuvenile myasthenia gravis (JMG) is an autoimmune disorder of neuromuscular transmission caused by production of antibodies against the postsynaptic membrane of the neuromuscular junction. Clinical signs in young children and adolescents range from isolated ocular symptoms to general muscular weakness and respiratory insufficiency. Clinical presentation of JMG in young children and adolescents shows distinct features compared to adults. Young children may show generalized muscular weakness already during the first two years of life, and in this group specific antibodies can be only slightly increased. Because of existing therapeutic options, an early diagnosis is important. In case of negative specific antibodies and onset of the first symptoms during infancy or early childhood, the diagnosis of a congenital myasthenic syndrome (CMS) must be considered and is not always clear to differentiate. Clinical symptoms, diagnostic procedures and therapeutic strategies with consideration of specificities of this age group are discussed.

Myasthenia gravis in young children and adolescents: clinical signs and treatment options

2012 ◽  
Vol 43 (02) ◽  
Author(s):  
A Della Marina ◽  
H Trippe ◽  
S Lutz ◽  
G Shamdeen ◽  
U Schara
Keyword(s):  
Young Children ◽  
Myasthenia Gravis ◽  
Children And Adolescents ◽  
Treatment Options ◽  
Clinical Signs

G.P.71 Myasthenia gravis in young children and adolescents: Clinical symptoms and treatment options

2012 ◽  
Vol 22 (9-10) ◽  
pp. 856
Author(s):  
A. Della Marina ◽  
H. Trippe ◽  
S. Lutz ◽  
G.M. Shamdeen ◽  
U. Schara
Keyword(s):  
Young Children ◽  
Myasthenia Gravis ◽  
Children And Adolescents ◽  
Clinical Symptoms ◽  
Treatment Options

scholarly journals Diagnostic significance of clinical and para-clinical signs of community-acquired pneumonia in children

2020 ◽  
Vol 24 (2) ◽  
pp. 244-249
Author(s):  
V.Yu. Pasik
Keyword(s):  
Young Children ◽  
Sensitivity And Specificity ◽  
Clinical Symptoms ◽  
Clinical Signs ◽  
The Body ◽  
Diagnostic Methods ◽  
Statistical Hypothesis ◽  
Diagnostic Significance ◽  
Operational Characteristics ◽  
Gallbladder Dyskinesia

Annotation. Respiratory diseases are relevant in pediatric practice, which is associated with its widespread and frequent complications, especially in young children. The aim of the study was to assess the diagnostic value of clinical symptoms, laboratory and ultra-sonographic parameters in pneumonia in children of the first 3 years of life. A retrospective study of medical records of 218 children who were hospitalized in the department for young children diagnosed with pneumonia for the period from 2016 to 2018. The average age of children was 11.67±9.97 months and it was within the range from 1 month to 3 years. The ration of boys and girls was practically identical (51.8% and 48.2% accordingly). The first group included children aged under one year (the average age is 4.57±0.84 months; n=88). The second group included children aged from 1 to 3 years (the average age is 18.2±4.25 months; n=130). To characterize the information content of clinical and laboratory symptoms the study has used objective parameters defined as the operational characteristics of tests. The most important operational characteristics of diagnostic methods included: sensitivity (Se, sensitivity) and specificity (Sp, specificity). To check the statistical hypothesis on differences of absolute and relative frequencies, fractions, and ratios in two independent samples, the criteria of хі-square (χ2) was used. While detailing an anamnesis, the disease was more often related to untimely treatment and outpatient care. Various data were obtained on the absolute and relative risk, as well as the sensitivity and specificity of the localization of pneumonia depending on age. Therefore, the incidence of bilateral pneumonia was considered an indicator of risk. On admission to hospital, the body temperature of patients was 38.2±0.66°С. Most of the complaints were on the unproductive or productive cough. Besides, in some cases, shortness of breath and runny nose were mentioned. Thus, in young children with pneumonia, a diagnostically significant clinical symptom is a bilateral lung impression (82.6%), compared with right-handed (15.1%) and left-handed (2.3%), which is significantly more common in children under 1-th year of life compared with patients 1–3 years; laboratory features are probably higher levels of liver-specific enzymes – ALT and AST in children under 1 year; ultrasonographic indicators associated with the presence of pneumonia in young children include increased liver size, gallbladder deformity, the presence of sediment in the gallbladder, dyskinesia of the biliary tract, thickening of the gallbladder wall; children under 1 year of age have a risk of liver enlargement and biliary dyskinesia.

scholarly journals Myasthenia Crisis Vs Cholinergic Crisis: Challenges in Crisis Management Without Plasmapheresis or Intravenous Immunoglobulin (IVIG)

2020 ◽  
Vol 2 (2) ◽  
pp. 53
Author(s):  
Lila Tri Harjana ◽  
Hardiono Hardiono
Keyword(s):  
Mechanical Ventilation ◽  
Respiratory Failure ◽  
Myasthenia Gravis ◽  
Muscle Weakness ◽  
Spontaneous Breathing Trial ◽  
Ventilatory Support ◽  
Clinical Signs ◽  
Autoimmune Disorder ◽  
High Dose ◽  
Respiratory Compromise

Introduction: Myasthenia gravis (MG) is an acquired autoimmune disorder clinically characterized by skeletal muscle weakness & fatigability on exertion with prevalence as high as 2–7 in 10,000 and women are affected more frequently than men (~3:2). Over 12-16% of generalized MG patients experience crisis once in their lifetime. A serious complication of myasthenia gravis is respiratory failure. This may be secondary to an exacerbation of myasthenia (myasthenia crisis) or to treatment with excess doses of a cholinesterase inhibitor (cholinergic crisis). Case Report: Thirty-two years old woman refereed from a private hospital to ED for further treatment with myasthenia in crisis, after nine days of treatment in the previous ICU. Patient already in intubation with mechanical ventilation and history of the treatment of a high dose of multiple anticholinesterase drugs and steroids without plasmapheresis or immunoglobulin intravenous. During admission, diarrhea was present, with no sign of GI infection. On the third day of admission, the patient performed a Spontaneous Breathing Trial and was a success then extubated. Then two day after extubation, the patient falls to respiratory failure and need mechanical ventilation. Anticholinesterase test was performed, and it shows no improvement in clinical signs, and diagnose as Cholinergic Crisis. After re-adjustment of anticholinesterase drug with a lower dose, clinically, the respiratory condition improved, and on the 10th day of admission, the patient was succeed extubated. At 12nd days of ICU admission, patient discharge from ICU. Discussion: Myasthenia and Cholinergic Crisis is a severe and life-threatening condition characterized by generalized muscle weakness with a respiratory compromise that requires ventilatory support. Respiratory failure may be present in the cholinergic crisis without cholinergic symptoms (miosis, diarrhea, urinary incontinence, bradycardia, emesis, lacrimation, or salivation). The most important management aspect of Myasthenia patients in crisis is the recognition and treatment of myasthenia vs cholinergic crisis.

Pediatric Miller Fisher Syndrome and Ocular Myasthenia Gravis (A Reminder of Clinical Mimicry): A Case Report

2021 ◽  
Author(s):  
Zakaria Barsoum
Keyword(s):  
Myasthenia Gravis ◽  
Acetylcholine Receptors ◽  
Correct Diagnosis ◽  
Clinical Signs ◽  
Autoimmune Disorder ◽  
Miller Fisher Syndrome ◽  
Neurological Deficits ◽  
Fisher Syndrome ◽  
Ocular Myasthenia ◽  
Ocular Myasthenia Gravis

AbstractMiller Fisher syndrome (MFS) is a rare immune-mediated neuropathy that often presents with diplopia and bilateral external ophthalmoplegia. Other neurological deficits may occur such as ataxia and areflexia but not in all cases. Although MFS is a clinical diagnosis, serological confirmation is possible by identifying the anti-GQ1b antibody found in the majority of patients. Myasthenia gravis is an autoimmune disorder of the availability of acetylcholine receptors in the neuromuscular junction. Ocular myasthenia gravis is a disease subtype characterized by variable patterns of weakness of extraocular muscles, eyelid elevator, and orbicular muscle in which the initial sign in most adults and children is ptosis. We report a child with MFS who presented with clinical signs suggestive of ocular myasthenia gravis, but in whom the correct diagnosis was made on the basis of serological testing for the anti-GQ1b antibody. We aim to highlight the similarity between the two rare conditions and address the importance of early liaison with neurologists and ophthalmologists in reaching to the proper diagnosis.

scholarly journals Myasthenia Gravis Masquerading as Status Asthmaticus

2021 ◽  
Vol 2021 ◽  
pp. 1-4
Author(s):  
Neha Pirwani ◽  
Shayna Wrublik ◽  
Shashikanth Ambati
Keyword(s):  
Myasthenia Gravis ◽  
Status Asthmaticus ◽  
Pediatric Population ◽  
Invasive Mechanical Ventilation ◽  
Delayed Diagnosis ◽  
Autoimmune Disorder ◽  
Acute Onset ◽  
Diagnostic Challenge ◽  
Skeletal Musculature ◽  
Juvenile Myasthenia Gravis

Myasthenia gravis, an autoimmune disorder of neuromuscular transmission, can lead to varying degrees of weakness and fatigability of the skeletal musculature. Juvenile myasthenia gravis accounts for 10–15% of all cases of myasthenia gravis. The clinical presentation of juvenile myasthenia gravis varies tremendously, which presents itself as a diagnostic challenge for clinicians. We report a case of a 15-year-old female with mild intermittent asthma presenting with shortness of breath. Acute onset of dyspnea is a common chief complaint amongst the pediatric population with a broad differential diagnosis. Our patient was presumptively treated for status asthmaticus and required invasive mechanical ventilation. After extubating, the patient showed persistent ptosis, which led to the eventual work-up of myasthenia gravis. Upon further review, this patient had months of intermittent symptoms including ptosis and fatigue which went previously undiagnosed. This case demonstrates that dyspnea in an asthmatic can occur from nonairway processes and, if missed, may result in overtreatment of asthma or delayed diagnosis of an important neuromuscular process.

scholarly journals Juvenile Myasthenia Gravis: A Case Report and Review of Literatures

2013 ◽  
Vol 29 (1) ◽  
pp. 64-69
Author(s):  
Narayan Chandra Kundu ◽  
Moushumi Sen ◽  
KM Nazmul Islam Joy ◽  
Feroj Ahmed Quraish
Keyword(s):  
Myasthenia Gravis ◽  
Antibody Test ◽  
Autoimmune Disorder ◽  
Repetitive Nerve Stimulation ◽  
History Of ◽  
Congenital Myasthenia ◽  
Pediatric Presentation ◽  
Juvenile Myasthenia Gravis ◽  
Ice Pack ◽  
Anti Acetylcholine

Juvenile myasthenia gravis (JMG) is a rare autoimmune disorder of childhood. Pediatric presentation of MG is more common in Oriental than in Caucasian populations. JMG need to be differentiated from congenital myasthenia gravis which do not have haan autoimmune basis. An 11 years old girl presented with drooping of eye lids which was more marked at the later part of day and was gradually progressive . She had complained of double vision. She had no family history of myasthenia gravis. Ice pack test, repetitive nerve stimulation test, and anti acetylcholine receptor antibody test support the diagnosis. She was treated with pyridostigmine and was started as 30mg four times daily and increased to 60 mg/qds. Subsequently her symptoms improved gradually and she became stable. Bangladesh Journal of Neuroscience 2013; Vol. 29 (1) : 64-69

scholarly journals Myasthenia Crisis Vs Cholinergic Crisis: Challenges in Crisis Management Without Plasmapheresis or Intravenous Immunoglobulin (IVIG)

2020 ◽  
Vol 2 (2) ◽  
pp. 16
Author(s):  
Lila Tri Harjana ◽  
Hardiono Hardiono
Keyword(s):  
Mechanical Ventilation ◽  
Respiratory Failure ◽  
Myasthenia Gravis ◽  
Muscle Weakness ◽  
Spontaneous Breathing Trial ◽  
Ventilatory Support ◽  
Clinical Signs ◽  
Autoimmune Disorder ◽  
High Dose ◽  
Respiratory Compromise

Introduction: Myasthenia gravis (MG) is an acquired autoimmune disorder clinically characterized by skeletal muscle weakness & fatigability on exertion with prevalence as high as 2–7 in 10,000 and women are affected more frequently than men (~3:2). Over 12-16% of generalized MG patients experience crisis once in their lifetime. A serious complication of myasthenia gravis is respiratory failure. This may be secondary to an exacerbation of myasthenia (myasthenia crisis) or to treatment with excess doses of a cholinesterase inhibitor (cholinergic crisis). Case Report: Thirty-two years old woman refereed from a private hospital to ED for further treatment with myasthenia in crisis, after nine days of treatment in the previous ICU. Patient already in intubation with mechanical ventilation and history of the treatment of a high dose of multiple anticholinesterase drugs and steroids without plasmapheresis or immunoglobulin intravenous. During admission, diarrhea was present, with no sign of GI infection. On the third day of admission, the patient performed a Spontaneous Breathing Trial and was a success then extubated. Then two day after extubation, the patient falls to respiratory failure and need mechanical ventilation. Anticholinesterase test was performed, and it shows no improvement in clinical signs, and diagnose as Cholinergic Crisis. After re-adjustment of anticholinesterase drug with a lower dose, clinically, the respiratory condition improved, and on the 10th day of admission, the patient was succeed extubated. At 12nd days of ICU admission, patient discharge from ICU. Discussion: Myasthenia and Cholinergic Crisis is a severe and life-threatening condition characterized by generalized muscle weakness with a respiratory compromise that requires ventilatory support. Respiratory failure may be present in the cholinergic crisis without cholinergic symptoms (miosis, diarrhea, urinary incontinence, bradycardia, emesis, lacrimation, or salivation). The most important management aspect of Myasthenia patients in crisis is the recognition and treatment of myasthenia vs cholinergic crisis.

Outcome after Robotic-Assisted Thymectomy in Children and Adolescents with Acetylcholine Receptor Antibody-Positive Juvenile Myasthenia Gravis

2017 ◽  
Vol 48 (04) ◽  
pp. 315-322 ◽  
Author(s):  
Heike Kölbel ◽  
Maximilian Müllers ◽  
Olaf Kaiser ◽  
Mahmoud Ismail ◽  
Marc Swierzy ◽  
...  
Keyword(s):  
Myasthenia Gravis ◽  
Children And Adolescents ◽  
Acetylcholine Receptor ◽  
Safe Procedure ◽  
Long Term Outcomes ◽  
Robotic Assisted ◽  
The Mean ◽  
Juvenile Myasthenia Gravis

AbstractThe aim of our study was to describe the long-term outcomes after robotic-assisted thymectomy in a cohort of acetylcholine receptor (AChR)-antibody (Ab)–positive, generalized juvenile myasthenia gravis (JMG). We retrospectively analyzed a cohort of 18 patients (15 females and 3 males) who underwent robotic-assisted thymectomy. At the time of diagnosis, 12/18 patients were prepubertal; the mean age was 9.8 years at the onset of the disease. All patients received therapy with pyridostigmine; additional immunotherapy included: corticosteroid therapy in 18/18, azathioprine in 14/18 patients, mycophenolate mofetil in 4/18, and cyclosporine in 1/18 patients. Eight patients received intravenous immunoglobulin and four plasma exchange. The mean age of patients at thymectomy was 11.7 years (range: 4.2–16 years). The mean duration of postoperative stay was 2.9 days. Thymectomy was followed by gradual clinical improvement (39% patients achieved clinical remission) and dose reduction in steroid therapy in all patients during the follow-up period (mean: 27.4 months). In children and adolescents with AChR-Ab–positive JMG, thymectomy has a beneficial effect on the weaning off immunosuppressive therapy in patients with generalized symptoms and should be considered as a part of multimodal therapy. Robotic-assisted thymectomy is a safe procedure with low morbidity and a comparable clinical outcome compared with the open sternal procedure.

scholarly journals Analysis of Different Diagnostis Methods of Influenza in Horses

2018 ◽  
Vol 44 (1) ◽  
pp. 7
Author(s):  
Mihajlo Erdeljan ◽  
Ivana Davidov ◽  
Ramiz Cutuk ◽  
Dragan Rogan ◽  
Aleksandar Potkonjak
Keyword(s):  
Influenza Virus ◽  
Nucleic Acid ◽  
Clinical Symptoms ◽  
Sport Activity ◽  
Clinical Signs ◽  
Rapid Test ◽  
Nasal Discharge ◽  
Rt Pcr ◽  
Serological Tests ◽  
Specific Antibodies

Background: Equine Influenza is a serious, acute respiratory illness with characteristical clinical signs. The disease is caused by family of Orthomyxoviridae, genera Influenza virus A by two subtypes H7N7 and H3N8. Currently, there is believe that H7N7 has been replaced as a predominant subtype with the H3N8. Horse infection with influenza virus can be detected by serological tests on paired sera using HI test. Commercial rapid tests could be used for the detection of influenza virus. Recently it is widely use a PCR method as fast and more specific methods.Materials, Methods & Results: Fifty horses and one pony, age between one and 22 years have been included in experiment. Horses were of different race, sex, and age and vaccination status. Ten out of total 51 (10/51) have been regularly vaccinated against EI. Prior to initiation of these study epidemiological survey has been performed. The clinical examination has been followed by blood sampling for blood cell and serum extraction. The serums were evaluated by HI method. Nasal swabs are taken from both nostrils twice, one was frozen for virus detection by RT-qPCR while another was used for detection of EI virus by Directi-gen™ FLU A rapid test. Analysis of titers of antibody reveled that 7 horses (14%) had specific antibodies (IgG) against subtype H7N7, while 9 horses (18%) had specific antibodies against H3N8. In the same time 4 horses had specific antibodies against both subtypes. Serological data confirmed that from 48 horses (96%) had the titer of antibodies greater than 16 against H7N7, while 40 horses (80%) had the specific antibodies (IgG) against H3N8. We found quite unexpected presence of specific antibodies (IgG) for H7N7 in horses that have not been previously vaccinated with H7N7 subtype. These horses never been utilized for sport activity and there was no legal requirements for their vaccination. Could horses with specific antibodies for H7N7 be transfected from vaccinated horses we could not confirme with scientific evidence either we could not have evidence that wild and domestic birds played a significant role especially knowing that horses are dead end of further we could confirm it. Our findings unequivocally confirmed that EI virus subtype H7N7 antibodies (IgG) were present in horses that have not been vaccinated and this is serological evidence that virus H7N7 is circulating  in these geographical areas.Discussion: Fast and reliable diagnosis and isolation of suspicious horses represent the first line of defense against pandemic influenza. Recognition of clinical signs (fever, depression, sharp cough and nasal discharge) along with epizootical survey provides the basis for the early detection of infection. In some cases, cough and rapid spread of symptoms of cough in a group of horses that were either unvaccinated against influenza virus or have been in contact with influenza virus infected horses can clearly point out to the EI virus. The definitive confirmation of EI virus could be done by virus isolation on tissue culture or embrionated eggs followed by detection of virus nucleic acid by RT-PCR. Our data suggest that a substantial number of horses (90%) that have not been vaccinated or vaccinated irregularly had specific antibodies against both subtypes of EI, what suggest that those horses have been exposed to viruses sometimes during their lifetime. Additionally, despite the fact that 20% of horses had some signs of respiratory disease that resemble EI we were not able to confirm EI nucleic acid by RT-PCR while Directi-gene™ assay confirmed virus presence just in two horses. Failure to detect virus nucleic acid could be due to fact that nasal swab samples have been taken at the end of the clinical symptoms, other authors have simular PCR negative patient which displayed a significant rising titre to influenza type A.

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