scholarly journals Juvenile myasthenia

2011 ◽  
Vol 64 (5-6) ◽  
pp. 295-298
Author(s):  
Marija Knezevic-Pogancev

Introduction. Juvenile myasthenia is a chronic autoimmune neuromuscular disease characterized by varying degrees of fluctuating, painless muscle weakness and rapid fatigue of any muscles under voluntary control. Juvenile myasthenia is a form of myasthenia appearing in adolescent age, representing 10% to 15% of all cases of myasthenia gravis. Juvenile myasthenia is presented by a defect in the transmission of nerve impulses to muscles, resulting from a breakdown in the normal communication between nerves and muscles. In myasthenia, antibodies produced by the body?s own immune system block, alter, or destroy the receptors for acetylcholine. Juvenile myasthenia is neither directly inherited nor is it contagious. Signs and Symptoms. The first noticeable symptoms may be eye muscle weakness, difficulty in swallowing, or slurred speech. Juvenile myasthenia usually affects muscles innervated by the cranial nerves (face, lips, tongue, neck and throat), but it can affect any muscle group. Symptoms vary in type and severity with typical periods of exacerbation interspersed with periods of remission. When the muscles necessary for breathing are affected, a patient is said to be in a myasthenic crisis, which is a life-threatening situation. Disease Outcome and Treatment. Juvenile myasthenia produces sporadic but progressive weakness and abnormal fatigability of striated (skeletal) muscles, exacerbated by exercise and repeated movement, but improved by rest and anticholinesterase drugs. Juvenile myasthenia follows an unpredictable course of recurring exacerbations and periodic remissions. With current therapies, however, most cases of juvenile myasthenia are not as serious as the name implies. Although there is no known cure, drug treatment has improved prognosis and allows patients to lead relatively normal lives, except during exacerbations.

2008 ◽  
Vol 47 (172) ◽  
Author(s):  
Buddhi Prasad Paudyal

Acute poisoning by organophosphorus (OP) compounds is a major global clinical problem, withthousands of deaths occurring every year. Most of these pesticide poisoning and subsequentdeaths occur in developing countries following a deliberate self ingestion of the poison. Metacid(Methyl parathion) and Nuvan (Dichlorovos) are commonly ingested OP pesticides; Dimethoate,Profenofos, and Chlorpyrifos are other less frequently ingested compounds in Nepal. The toxicityof these OP pesticides is due to the irreversible inhibition of acetylcholinesterase (AChE) enzymeleading to accumulation of acetylcholine and subsequent over-activation of cholinergic receptorsin various parts of the body. Acutely, these patients present with cholinergic crisis; intermediatesyndrome and delayed polyneuropathy are other sequel of this form of poisoning. The diagnosisdepends on the history of exposure to these pesticides, characteristic manifestations of toxicityand improvements of the signs and symptoms after administration of atropine. The supportivetreatment of OP poisoning includes the same basic principles of management of any acutelypoisoned patient i.e., rapid initial management of airways, breathing, and circulation. Gastriclavage and activated charcoal are routinely used decontamination procedures, but their valuehas not been conclusively proven in this poisoning. Atropine is the mainstay of therapy, andcan reverse the life threatening features of this acute poisoning. However, there are no clearcut guidelines on the dose and duration of atropine therapy in OP poisoning. Cholinesterasereactivators, by regenerating AChE, can reverse both the nicotinic and muscarinic effects;however, this benefit has not been translated well in clinical trials. All these facts highlight thatthere are many unanswered questions and controversies in the management of OP poisoningand there is an urgent need for research on this aspect of this common and deadly poisoning.Key Words: poisoning, organophosphorus insecticides, decontamination, antidotes


2016 ◽  
Vol 50 (4) ◽  
pp. 199-200
Author(s):  
Yuvraj S Cheema ◽  
Parveen Gupta ◽  
Sahil Attri ◽  
Vipin Jamdagni ◽  
Deven Mittal

ABSTRACT Polymyositis is an uncommon inflammatory disease that causes muscle weakness affecting both sides of the body, typically the proximal muscles. Most commonly, it affects adults in their 30s, 40s, or 50s. Signs and symptoms usually develop gradually, over weeks or months. Raised creatinine phosphokinase (CPK) levels are commonly seen during the acute phase. The electromyogram characteristically shows a myopathic pattern. The affected muscles histopathologically demonstrate endomysial inflammation, lymphocytic infiltration, zonalmyofibrillar loss, and perifascicular atrophy. We report a male patient with a classical presentation of polymyositis. How to cite this article Cheema YS, Gupta P, Attri S, Jamdagni V, Mittal D. Polymyositis. J Postgrad Med Edu Res 2016;50(4):199-200.


2020 ◽  
Vol 13 (7) ◽  
pp. e235385 ◽  
Author(s):  
Naohi Isse ◽  
Masashi Hashimoto

A 72-year-old Japanese man treated with omeprazole for 11 years was admitted due to loss of consciousness and muscle weakness. Wolff-Parkinson-White syndrome-induced tachycardia was considered as the cause of syncope. His blood examination revealed rhabdomyolysis, hypokalaemia, hypomagnesaemia, hypocalcaemia, hyperlactacidaemia, hyperammonaemia and high-anion-gap metabolic acidosis. Hypomagnesaemia could be caused by magnesium malabsorption due to omeprazole use. Hypocalcaemia might be caused by the inhibitory effect of hypomagnesemia on the parathyroid gland hormone secretion. Hyperammonaemia might be caused by two reasons: (1) renal ammonium production induced by hypokalaemia; (2) inhibition of ammonium secretion by omeprazole. Both hypocalcaemia and hypokalaemia might cause chronic elevation of serum creatinine phosphokinase which ended up with rhabdomyolysis. Correction of serum electrolytes rapidly improved his muscle weakness. Discontinuation of omeprazole no longer caused these abnormalities. A physician should be aware of unexplained signs and symptoms of patients using proton-pump inhibitors to avoid life-threatening electrolyte and physiologic disturbances.


2021 ◽  
Vol 10 (35) ◽  
pp. 3040-3046
Author(s):  
Nandalal Girijalal Toshniwal ◽  
Shubhangi Amit Mani ◽  
Nilesh Mote ◽  
Ashwini Ramesh Nalkar

Obstructive sleep apnoea (OSA) is a sleep associated breathing disorder and it affects the health and quality of life of individuals suffering from it. Orthodontists should be well aware of the symptoms of this disorder and competent enough to recognize its signs and symptoms. Orthodontics is well suited for the treatment of OSA patients due to their expertise and knowledge regarding growth and development of orofacial and dentofacial structures as well as orthopaedic, orthodontic, and surgical correction of the jaws and other supporting tissues. There are basically two types of sleep apnoea- Central sleep apnoea and obstructive sleep apnoea where obstructive sleep apnoea is the more common one. This disorder can be life threatening as the oxygen supply to various parts of the body is substantially reduced. Obstructive sleep apnoea is caused by an interplay between a variety of factors, including sleep related loss of muscle tone in the tissues supplied by the glossopharyngeal nerve, anatomical obstruction of the nasal passages, large tonsils, large tongue, a retrognathic mandible, obesity, alcohol, sedative medication, and allergies. Sleep apnoea can be caused due to many factors and many treatment modalities have been employed to correct this disorder including mandibular advancement appliances, polysomnographs, and surgical intervention. It can be treated using surgery, continuous positive airway pressure and oral appliances therapy. This article highlights the role the orthodontist plays in the diagnosis and treatment planning of OSA patients. KEY WORDS Orthodontics, Obstructive Sleep Apnoea, Sleep, Snoring


2020 ◽  
Vol 477 (14) ◽  
pp. 2679-2696
Author(s):  
Riddhi Trivedi ◽  
Kalyani Barve

The intestinal microbial flora has risen to be one of the important etiological factors in the development of diseases like colorectal cancer, obesity, diabetes, inflammatory bowel disease, anxiety and Parkinson's. The emergence of the association between bacterial flora and lungs led to the discovery of the gut–lung axis. Dysbiosis of several species of colonic bacteria such as Firmicutes and Bacteroidetes and transfer of these bacteria from gut to lungs via lymphatic and systemic circulation are associated with several respiratory diseases such as lung cancer, asthma, tuberculosis, cystic fibrosis, etc. Current therapies for dysbiosis include use of probiotics, prebiotics and synbiotics to restore the balance between various species of beneficial bacteria. Various approaches like nanotechnology and microencapsulation have been explored to increase the permeability and viability of probiotics in the body. The need of the day is comprehensive study of mechanisms behind dysbiosis, translocation of microbiota from gut to lung through various channels and new technology for evaluating treatment to correct this dysbiosis which in turn can be used to manage various respiratory diseases. Microfluidics and organ on chip model are emerging technologies that can satisfy these needs. This review gives an overview of colonic commensals in lung pathology and novel systems that help in alleviating symptoms of lung diseases. We have also hypothesized new models to help in understanding bacterial pathways involved in the gut–lung axis as well as act as a futuristic approach in finding treatment of respiratory diseases caused by dysbiosis.


2019 ◽  
Vol 1 (2) ◽  
pp. V1
Author(s):  
Sima Sayyahmelli ◽  
Jian Ruan ◽  
Bryan Wheeler ◽  
Mustafa K. Başkaya

Primary glioblastoma multiforme tumors of the medulla oblongata are rare, especially in the adult population. Perhaps due to this rarity, we are not aware of any previous reports addressing the resection of these tumors or their clinical outcomes.In this surgical video, we present a 43-year-old man with a 1-month history of left-sided paresthesia. The paresthesia initiated in the left hand, along with weakness and reduced fine motor control, and then spread to the entire left side of the body. He had recent weight loss, imbalance, difficulty in swallowing, and hoarseness in his voice. He also had a diminished gag reflex, and significant atrophy of the right side of the tongue with an accompanying deviation of the uvula and fasciculations of the tongue. MRI showed an infiltrative expansile mass within the medulla with peripheral enhancement and central necrosis. In T2/FLAIR sequences, a hyperintense signal extended superiorly into the left inferior aspect of the pons and left inferior cerebellar peduncle and inferiorly into the upper cervical cord.The decision was made to proceed with surgical resection. The patient underwent a midline suboccipital craniotomy with C1 laminectomy for surgical resection of this infiltrative expansile intrinsic mass in the medulla oblongata, with concurrent monitoring of motor and somatosensory evoked potentials and monitoring of lower cranial nerves IX, X, XI, and XII. A gross-total resection of the enhancing portion of the tumor was performed, along with a subtotal resection of the nonenhancing portion. The surgery and postoperative course were uneventful. Histopathology revealed a grade IV astrocytoma. The patient received radiation therapy.In this surgical video, we demonstrate important steps for the microsurgical resection of this challenging glioblastoma multiforme of the medulla oblongata.The video can be found here: https://youtu.be/QHbOVxdxbeU.


Author(s):  
Yetunde A. Aluko

This paper supports the hypothesis that corruption and non-delivery of services in key sectors such as health have gender-specific poverty consequences. The study utilized qualitative micro-level information about the structures of corruption and its impact on poor women. Respondents expressed their perceptions on the occurrence of corrupt practices in public health care system and its wider impact on society. The findings revealed that the impact of corruption is felt disproportionately by women and the poor, who are most dependent on public services, and have no alternative even when facing corrupt practices in a life threatening situation, such as complicated birth delivery. Pregnant women denied access to doctors tended to deliver at home, which increased the likelihood of complications and maternal and child mortality. Medical supplies meant for public hospitals are sold to private clinics who charge more for drugs and supplies. There is need to strengthen sectoral oversight mechanisms and transparency as well as increase women’s voices in service delivery.


2020 ◽  
Author(s):  
Tarso Accorsi ◽  
Karine De Amicis Lima ◽  
Alexandra Brigido ◽  
Deborah Belfort ◽  
Fabio Habrum ◽  
...  

BACKGROUND Lightweight portable ultrasound is widely available, especially in inaccessible geographical areas. It demonstrates effectiveness and diagnosis improvement even in field conditions but no precise information about protocols, acquisition time, image interpretation, and the relevance in changing medical conduct exists. The COVID-19 pandemic implied many severe cases and the rapid construction of field hospitals with massive general practitioner (GP) recruitment. OBJECTIVE This prospective and descriptive study aimed to evaluate the feasibility of telemedicine guidance using a standardized multi-organ sonographic assessment protocol in untrained GPs during a COVID-19 emergency in a field hospital. METHODS Eleven COVID-19 in-patients presenting life-threatening complications, attended by local staff who spontaneously requested on-time teleconsultation, were enrolled. All untrained doctors successfully positioned the transducer and obtained key images guided by a remote doctor via telemedicine, with remote interpretation of the findings. RESULTS Only four (36%) general practitioners obtained the appropriate key heart image on the left parasternal long axis window, and three (27%) had an image interpreted remotely on-time. The evaluation time ranged from seven to 42 minutes, with a mean of 22.7 + 12. CONCLUSIONS Telemedicine is effective in guiding GPs to perform portable ultrasound in life-threatening situations, showing effectiveness in conducting decisions.


2021 ◽  
Vol 51 (1) ◽  
pp. 10-15
Author(s):  
Kenneth V Iserson ◽  
Sri Devi Jagjit ◽  
Balram Doodnauth

Acute thoracic aortic dissection is an uncommon, although not rare, life-threatening condition. With protean signs and symptoms that often suggest more common cardiac or pulmonary conditions, it can be difficult to diagnose. Ultrasound has proven useful in making the correct diagnosis. This case demonstrates that training gained using standard ultrasound machines can be easily and successfully adapted to newer handheld ultrasound devices. The examination technique using the handheld device is illustrated with photos and a video.


2021 ◽  
Vol 22 (8) ◽  
pp. 4110
Author(s):  
Gerhild Euler ◽  
Jens Kockskämper ◽  
Rainer Schulz ◽  
Mariana S. Parahuleva

Heart failure (HF) and atrial fibrillation (AF) are two major life-threatening diseases worldwide. Causes and mechanisms are incompletely understood, yet current therapies are unable to stop disease progression. In this review, we focus on the contribution of the transcriptional modulator, Jun dimerization protein 2 (JDP2), and on HF and AF development. In recent years, JDP2 has been identified as a potential prognostic marker for HF development after myocardial infarction. This close correlation to the disease development suggests that JDP2 may be involved in initiation and progression of HF as well as in cardiac dysfunction. Although no studies have been done in humans yet, studies on genetically modified mice impressively show involvement of JDP2 in HF and AF, making it an interesting therapeutic target.


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