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Journal articles on the topic 'LEMS; Myasthenia gravis; Neurological disorders'
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1
Altintas, Ayse, Justina Dargvainiene, Christiane Schneider-Gold, Nasrin Asgari, Ilya Ayzenberg, Andrea I. Ciplea, Ralf Junker, Frank Leypoldt, Klaus-Peter Wandinger, and Kerstin Hellwig. "Gender issues of antibody-mediated diseases in neurology: (NMOSD/autoimmune encephalitis/MG)." Therapeutic Advances in Neurological Disorders 13 (January 2020): 175628642094980. http://dx.doi.org/10.1177/1756286420949808.
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Neuromyelitis optica spectrum disorder (NMOSD), autoimmune encephalitis (AE), myasthenia gravis (MG) and Lambert-Eaton myasthenic syndrome (LEMS) are antibody-mediated neurological diseases. They have mostly female predominance, affecting many women during childbearing age. Interactions between the underlying disease (or necessary treatment) and pregnancy can occur in every of these illnesses. Herein, we present the characteristics of NMOSD, AE, MG and LEMS in general, and review published data regarding the influence of the different diseases on fertility, pregnancy, puerperium, treatment strategy during pregnancy and post-partum period, and menopause but also male factors. We summarise key elements that should be borne in mind when confronted with such cases.
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Arneth, Borros M. "Neuronal Antibodies and Associated Syndromes." Autoimmune Diseases 2019 (July 9, 2019): 1–9. http://dx.doi.org/10.1155/2019/2135423.
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Introduction. Multiple well-recognized conditions, such as Lambert–Eaton myasthenic syndrome (LEMS) and myasthenia gravis (MG), have been associated with neuronal antibodies. Materials and Methods. A search was performed using Embase, PubMed, and CINAHL. An initial search of each database was conducted using keywords and terms related to the aim of the current review. Additional articles were obtained by examining the reference lists and citations in the selected records. Results. The studies identified through the search process used different designs and methods to explore neuronal antibodies and associated syndromes. Previous studies have shown that neurological and psychiatric disorders can be mediated and influenced by various antibodies. The identification of autoantibodies can help with the accurate diagnosis of conditions and commencement of early treatment. Discussion. A review of selected studies identified in the literature implicated that classic anti-neuronal antibodies, such as anti-Ri and anti-Hu, play a role in the development of neurological diseases. More recent studies have indicated that other novel antibodies act on neuronal cell surface antigens to contribute to the development of neurological disorders. Conclusion. Existing research provides evidence revealing a spectrum of antibodies linked to the development and progression of neurological diseases. However, further antibody testing and studies should be performed to validate the relationship between conditions and antibodies.
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Grand'Maison, François. "Methods of Testing Neuromuscular Transmission in the Intensive Care Unit." Canadian Journal of Neurological Sciences / Journal Canadien des Sciences Neurologiques 25, S1 (February 1998): S36—S39. http://dx.doi.org/10.1017/s0317167100034715.
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AbstractAll disorders of neuromuscular transmission (NMT) may cause ventilatory failure, albeit rarely. Respiratory muscle weakness is occasionally the presenting feature of myasthenia gravis (MG), the Lambert-Eaton myasthenic syndrome (LEMS), hypermagnesemia and botulism. Chronic MG, congenital myasthenic syndromes and LEMS may be acutely exacerbated by various intercurrent conditions and by drugs which interfere with NMT. Finally, in the ICU, difficulty in weaning from the ventilator may be caused by prolonged use of neuromuscular blocking agents. Electrophysiological studies of NMT disorders in the intensive care unit have rarely been reported. Nevertheless, the available data indicates that the electrodiagnosis of severe NMT disorders can be misleading. With severe NMT defects, the electrophysiological distinction between post-synaptic and pre-synaptic disorders is blurred and the differential diagnosis with myopathies may be difficult. A clinically suspected NMT disorder should therefore not be ruled out when electrodiagnosis fails to demonstrate the expected abnormalities.
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Amanat, Man, Mona Salehi, and Nima Rezaei. "Neurological and psychiatric disorders in psoriasis." Reviews in the Neurosciences 29, no. 7 (September 25, 2018): 805–13. http://dx.doi.org/10.1515/revneuro-2017-0108.
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Abstract Psoriasis used to be known as a skin disorder; however, it can now be considered as a systemic disease with the involvement of multiple organs. Neurological and psychiatric disorders are some of the associated problems that can be observed in patients with psoriasis. Stroke, multiple sclerosis, seizure, migraine, restless leg syndrome, Parkinson’s disease, Guillain-Barré syndrome, and myasthenia gravis are the reported neurological diseases, while depression, bipolar mood disorder, anxiety, psychosis, cognitive impairment, personality disorders, sexual disorders, sleep disturbance, and eating disorders are the recognized psychiatric presentations in patients with psoriasis. Herein, the neurological and psychiatric disorders of psoriasis are described.
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Newsom-Davis, John. "Autoantibody-Mediated Channelopathies at the Neuromuscular Junction." Neuroscientist 3, no. 5 (September 1997): 337–46. http://dx.doi.org/10.1177/107385849700300515.
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The neuromuscular junction is vulnerable to antibody-mediated autoimmune attack, probably because it lacks the protection of the blood-brain barrier. This review focuses on three disorders: myasthenia gravis (MG) and the Lambert-Eaton myasthenic syndrome (LEMS), in both of which there is fatiguable muscle weakness, and acquired neuromyotonia (ANMT), in which hyperexcitable peripheral nerves lead to continuous muscle fiber activity and sometimes parasthesias. Each can occur as a paraneoplastic disorder (thymoma in MG and ANMT, and small cell lung cancer in LEMS). The clinical abnormalities are improved following plasmapheresis (which removes circulating antibodies), and injection of experimental animals with immunoglobulins of patients transfers the pathophysiological changes. The ion channel targets in these three disorders are the muscle acetylcholine receptor (a ligand-gated cation channel) in MG, nerve terminal and autonomic voltage-gated calcium channels in LEMS, and peripheral nerve voltage-gated potassium channels in ANMT. The autoantibody attack results in a reduced number of functional channels. Each of the autoantibodies can be detected in serum by immunoassay. These discoveries have allowed new approaches to treatment and suggest that there may be other undiscovered antibody-mediated ion channelopathies. NEUROSCIENTIST 3:337–346, 1997
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Dhallu, Manjeet Singh, Ahmed Baiomi, Madhavi Biyyam, and Sridhar Chilimuri. "Perioperative Management of Neurological Conditions." Health Services Insights 10 (January 1, 2017): 117863291771194. http://dx.doi.org/10.1177/1178632917711942.
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Perioperative care of the patients with neurological diseases can be challenging. Most important consideration is the management and understanding of pathophysiology of these disorders and evaluation of new neurological changes that occur perioperatively. Perioperative generally refers to 3 phases of surgery: preoperative, intraoperative, and postoperative. We have tried to address few commonly encountered neurological conditions in clinical practice, such as delirium, stroke, epilepsy, myasthenia gravis, and Parkinson disease. In this article, we emphasize on early diagnosis and management strategies of neurological disorders in the perioperative period to minimize morbidity and mortality of patients.
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Osman, Chinar, Rachel Jennings, Khaled El-Ghariani, and Ashwin Pinto. "Plasma exchange in neurological disease." Practical Neurology 20, no. 2 (July 12, 2019): 92–99. http://dx.doi.org/10.1136/practneurol-2019-002336.
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Plasma exchange is a highly efficient technique to remove circulating autoantibodies and other humoral factors rapidly from the vascular compartment. It was the first effective acute treatment for peripheral disorders such as Guillain-Barré syndrome and myasthenia gravis before intravenous immunoglobulin became available. The recent recognition of rapidly progressive severe antibody-mediated central nervous system disorders, such as neuromyelitis optica spectrum disorders and anti-N-methyl-D-aspartate-receptor encephalitis, has renewed interest in using plasma exchange for their acute treatment also. In this review we explain the principles and technical aspects of plasma exchange, review its current indications, and discuss the implications for its provision in the UK.
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Souza, Paulo Victor Sgobbi de, Gabriel Novaes de Rezende Batistella, Valéria Cavalcante Lino, Wladimir Bocca Vieira de Rezende Pinto, Marcelo Annes, and Acary Souza Bulle Oliveira. "Clinical and genetic basis of congenital myasthenic syndromes." Arquivos de Neuro-Psiquiatria 74, no. 9 (September 2016): 750–60. http://dx.doi.org/10.1590/0004-282x20160106.
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ABSTRACT Neuromuscular junction disorders represent a wide group of neurological diseases characterized by weakness, fatigability and variable degrees of appendicular, ocular and bulbar musculature involvement. Its main group of disorders includes autoimmune conditions, such as autoimmune acquired myasthenia gravis and Lambert-Eaton syndrome. However, an important group of diseases include congenital myasthenic syndromes with a genetic and sometimes hereditary basis that resemble and mimick many of the classic myasthenia neurological manifestations, but also have different presentations, which makes them a complex clinical, therapeutic and diagnostic challenge for most clinicians. We conducted a wide review of congenital myasthenic syndromes in their clinical, genetic and therapeutic aspects.
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Sinanović, Osman, Sanela Zukić, Adnan Burina, Nermina Pirić, Renata Hodžić, Mirza Atić, Mirna Alečković-Halilović, and Enisa Mešić. "Plasmapheresis in neurological disorders: six years experience from University Clinical center Tuzla." F1000Research 6 (July 26, 2017): 1234. http://dx.doi.org/10.12688/f1000research.11841.1.
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Background: Therapeutic plasma exchange (TPE) is an extracorporeal blood purification technique that is designed to remove substances with a large molecular weight. The TPE procedure includes removal of antibodies, alloantibodies, immune complexes, monoclonal protein, toxins or cytokines, and involves the replenishment of a specific plasma factor. The aim of the study was to describe the clinical response to TPE in various neurological patients, and to assess the clinical response to this therapy. Methods: The study was retrospective. We analyzed the medical records of 77 patients who were treated at the Department of Neurology, University Clinical Center (UCC) Tuzla from 2011 to 2016. Results: 83 therapeutic plasma exchanges were performed in the 77 patients. There was a slight predominance of male patients (54.5%), with an average age of 51±15.9 years. The most common underlying neurological diseases were Guillain–Barré syndrome (GBS) (37.7%), then chronic inflammatory demyelinating polyneuropathy (CIDP) (23.4%), multiple sclerosis (MS) (11.7%) and myasthenia gravis (10.4%). Less frequent neurological diseases that were encountered were paraneoplastic polyneuropathies (5.2%), neuromyelitis optica (also known as Devic’s disease) (3.9%), motor neuron disease (3.9%), polymyositis (2.6%) and multifocal motor neuropathy (1.2%). Conclusions: Six years experience of therapeutic plasma exchange in neurological patients in our department have shown that, following evidence-based guidelines for plasmapheresis, the procedure was most effective in patients with GBS, CIDP and myasthenia gravis.
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Luckman, Steven P., Nils Erik Gilhus, and Fredrik Romi. "Matrix Metalloproteinase-3 in Myasthenia Gravis Compared to Other Neurological Disorders and Healthy Controls." Autoimmune Diseases 2011 (2011): 1–4. http://dx.doi.org/10.4061/2011/151258.
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MMP-3 is capable of degrading a variety of proteins, including agrin, which plays a critical role in neuromuscular signaling by controlling acetylcholine receptor clustering. High MMP-3 levels in a proportion of myasthenia gravis (MG) patients have been reported. A pathogenic role of MMP-3 in other neurological disorders has been suggested but not proven. We have therefore examined the levels of MMP-3 in 124 MG patients and compared them to 59 multiple sclerosis (MS) patients, 74 epilepsy patients, 33 acute stroke patients, and 90 healthy controls. 15.3% of the patients in the MG group were MMP-3-positive (defined as higher than cutoff value 48 ng/mL) with very high mean MMP-3 concentration (79.9 ng/mL), whereas the proportion of MMP-3 positive patients in the MS (3.4%), epilepsy (6.7%), stroke (0%), and the control group (4.4%) was significantly lower. Mean MMP-3 concentration in the total MG group (25.5 ng/mL) was significantly higher than in the MS (16.6 ng/mL) and stroke (11.7 ng/mL) groups, but did not differ significantly from the epilepsy (19.4 ng/mL) and the control group (23.4 ng/mL). MMP-3 may have a specific pathogenic effect in MG in addition to being associated with autoimmune diseases in general.
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Mangaraj, Swayamsidha, Arun Kumar Choudhury, Binoy Kumar Mohanty, and Anoj Kumar Baliarsinha. "Neurological manifestations of Graves’ disease: A case report and review of the literature." Journal of Neurosciences in Rural Practice 7, no. 01 (January 2016): 153–56. http://dx.doi.org/10.4103/0976-3147.165393.
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ABSTRACTGraves’ disease (GD) is characterized by a hyperfunctioning thyroid gland due to stimulation of the thyroid-stimulating hormone receptor by autoantibodies directed against it. Apart from thyrotoxicosis, other clinical manifestations include ophthalmopathy, dermopathy, and rarely acropachy. GD is an organ-specific autoimmune disorder, and hence is associated with various other autoimmune disorders. Myasthenia gravis (MG) is one such disease, which is seen with patients of GD and vice versa. Though the association of GD and myasthenia is known, subtle manifestations of latter can be frequently missed in routine clinical practice. The coexistence of GD and ocular MG poses a significant diagnostic dilemma to treating physicians. The ocular manifestations of myasthenia can be easily missed in case of GD and falsely attributed to thyroid associated ophthalmopathy due to closely mimicking presentations of both. Hence, a high degree of the clinical vigil is necessary in such cases to appreciate their presence. We present a similar case which exemplifies the above said that the clinical challenge in diagnosing coexistent GD and ocular myasthenia.
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Nikitin, S. S., and L. M. Boriskina. "Immunoglobulins in neurological practice: a review of the literature." Neuromuscular Diseases 9, no. 1 (April 24, 2019): 32–51. http://dx.doi.org/10.17650/2222-8721-2019-9-1-32-51.
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Today intravenous immunoglobulins are used increasingly in the management of patients with neurological conditions. The efficacy and safety of intravenous immunoglobulins treatment in chronic inflammatory demyelinating polyradiculoneuropathy, Guillain–Barre syndrome and multifocal motor neuropathy have been established in randomized controlled trials and declared in systematic reviews. There are discussions about the dose, timing, duration and necessity of repeated infusions in these disorders. The intravenous immunoglobulins treatment is an option in myasthenia gravis crisis and exacerbations the disease, stiff-person syndrome, a second-line therapy in dermatomyositis and some patients with polymyositis. The use of intravenous immunoglobulins in patients with multiple sclerosis, inclusion body myositis, resistant epilepsy is not finally proved. The review discussed the data of immunoglobulins efficacy in neurological disorders based on informative studies with an emphasis on the main criteria for choosing a drug for effective high-dose intravenous immunotherapy.
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Kreis, O. A., T. M. Alekseeva, Yu V. Gavrilov, P. O. Valko, and Yu Valko. "Diagnosis of sleepiness, fatigue and depression in patients with myasthenia gravis." Neuromuscular Diseases 10, no. 4 (December 29, 2020): 27–37. http://dx.doi.org/10.17650/2222-8721-2020-10-4-27-37.
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Introduction. Examination of excessive daytime sleepiness, fatigue and depression in patients with myasthenia gravis is important for differential diagnosis of other disorders, and adds to a comprehensive clinical assessment.Objective. The aim is a comprehensive assessment of sleepiness, fatigue and depression and evaluation of the impact of autoimmune comorbidity on these symptoms in myasthenia gravis patients, using newly validated Russian versions of international questionnaires. The present article aims at familiarizing a wider Russian-speaking audience of specialists in the field of neuromuscular disease and sleep medicine with the main findings of our previously published work.Materials and methods. The study included 73 patients with MG and 230 control subjects. For sleepiness, fatigue and depression evaluation were used: Fatigue Severity Scale (FSS), Fatigue Impact Scale (FIS) (cognitive / physical / psychosocial subscales), Epworth Sleepiness Scale (ESS), Beck Depression Inventory (BDI) (cognitive-affective and somatic domains), Spielberger–Khanin State Trait Anxiety Inventory (STAI).Results. The Fatigue Severity Scale and Fatigue Impact Scale showed good psychometric properties and can be used to identify distinct aspects of fatigue in patients with myasthenia gravis. The studied patient cohort revealed clinically significant fatigue (69.9 %), excessive daytime sleepiness (15.1 %), moderate to severe depression (20.5 %), a high level of personal (64.4 %) and situational anxiety (27.4 %). Among 13 patients with myasthenia gravis and additional autoimmune comorbidity, there were no significant differences in the severity of sleepiness, fatigue and depression compared with the main group.Conclusion. The use of self-reported scale of sleepiness, fatigue and depression combined with careful clinical-neurological characterization adds to a more comprehensive view of the patient. The identification of sleepiness, fatigue and depression can guide therapeutic decisions and contributes to a better patient care. The presence of concomitant autoimmune pathology in patients with myasthenia gravis does not seem to increase the severity of sleepiness, fatigue and depression.
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Koźmiński, Przemysław, Paweł Krzysztof Halik, Raphael Chesori, and Ewa Gniazdowska. "Overview of Dual-Acting Drug Methotrexate in Different Neurological Diseases, Autoimmune Pathologies and Cancers." International Journal of Molecular Sciences 21, no. 10 (May 14, 2020): 3483. http://dx.doi.org/10.3390/ijms21103483.
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Methotrexate, a structural analogue of folic acid, is one of the most effective and extensively used drugs for treating many kinds of cancer or severe and resistant forms of autoimmune diseases. In this paper, we take an overview of the present state of knowledge with regards to complex mechanisms of methotrexate action and its applications as immunosuppressive drug or chemotherapeutic agent in oncological combination therapy. In addition, the issue of the potential benefits of methotrexate in the development of neurological disorders in Alzheimer’s disease or myasthenia gravis will be discussed.
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Aszalós, Zsuzsa. "Some neurological and psychiatric complications in the disorders of the thyroid gland." Orvosi Hetilap 148, no. 7 (February 1, 2007): 303–10. http://dx.doi.org/10.1556/oh.2007.27988.
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A pajzsmirigyhormonok alapvető fontosságúak a központi idegrendszer perinatális fejlődéséhez és a felnőtt agy működéséhez, elsősorban a specifikus célgének transzkripciójának szabályozásával. Fokozzák a kortikális szerotoninerg transzmissziót, szerepet játszanak a noradrenerg funkciók szabályozásában, és érintik a GABA-funkciót. A csecsemő- és kisgyermekkori hypothyreosis mentális retardációhoz vezet, felnőttkorban a demencia és a depresszió a leggyakoribb, de előfordulhat myxoedemás kóma, cerebellaris és agyidegtünetek, megnő a hajlam a cerebrovascularis kórképekre. A periféria részéről polyneuropathia, carpalis alagút syndroma, myalgia gyakori; ritka szövődmény a myokymia. Csaknem minden hyperthyreotikus betegnél előfordulnak minor pszichiátriai tünetek, ritkábban alakul ki pszichózis, demencia, konfúziós állapot, depresszió, apátiás thyreotoxicosis, thyreotoxikus krízis, epilepszia, pyramistünetek vagy chorea. A periféria részvételét krónikus thyreotoxikus myopathia, infiltratív ophthalmomyopathia, myasthenia gravis, thyreotoxikus periódikus hypokalaemiás bénulás és polyneuropathia jelezheti. A figyelemhiányos hiperaktivitásban szenvedők jelentős részénél generalizált pajzsmirigyhormon-rezisztenciát igazoltak. Magas antithyroid antitest titer jellemzi az akut-szubakut indulású, remissziókkal és relapszusokkal jellemezhető, súlyos, életveszélyes, de szteroidra jól reagáló, autoimmun, ritkán előforduló Hashimoto-encephalopathiát.
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Totzeck, Andreas, Elakiya Ramakrishnan, Melina Schlag, Benjamin Stolte, Kathrin Kizina, Saskia Bolz, Andreas Thimm, et al. "Gut bacterial microbiota in patients with myasthenia gravis: results from the MYBIOM study." Therapeutic Advances in Neurological Disorders 14 (January 2021): 175628642110356. http://dx.doi.org/10.1177/17562864211035657.
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Background: Myasthenia gravis (MG) is an autoimmune neuromuscular disease, with gut microbiota considered to be a pathogenetic factor. Previous pilot studies have found differences in the gut microbiota of patients with MG and healthy individuals. To determine whether gut microbiota has a pathogenetic role in MG, we compared the gut microbiota of patients with MG with that of patients with non-inflammatory and inflammatory neurological disorders of the peripheral nervous system (primary endpoint) and healthy volunteers (secondary endpoint). Methods: Faecal samples were collected from patients with MG ( n = 41), non-inflammatory neurological disorder (NIND, n = 18), chronic inflammatory demyelinating polyradiculoneuropathy (CIDP, n = 6) and healthy volunteers ( n = 12). DNA was isolated from these samples, and the variable regions of the 16S rRNA gene were sequenced and statistically analysed. Results: No differences were found in alpha- and beta-diversity indices computed between the MG, NIND and CIDP groups, indicating an unaltered bacterial diversity and structure of the microbial community. However, the alpha-diversity indices, namely Shannon, Chao 1 and abundance-based coverage estimators, were significantly reduced between the MG group and healthy volunteers. Deltaproteobacteria and Faecalibacterium were abundant within the faecal microbiota of patients with MG compared with controls with non-inflammatory diseases. Conclusion: Although the overall diversity and structure of the gut microbiota did not differ between the MG, NIND and CIDP groups, the significant difference in the abundance of Deltaproteobacteria and Faecalibacterium supports the possible role of gut microbiota as a contributor to pathogenesis of MG. Further studies are needed to confirm these findings and to develop possible treatment strategies.
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Lezak, Muriel D. "Neurology and Neuropathology in Living Color." Journal of the International Neuropsychological Society 4, no. 6 (November 1998): 690. http://dx.doi.org/10.1017/s1355617798246169.
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This small (15.5 × 23.5 cm) book summarizes in a clear, well-organized format almost all of the most common conditions, features, and concerns of neurological practice. A chapter on the neurological examination precedes the next 14 chapters which cover (2) “Headache and Facial Pain”; (3) “Loss of Consciousness and Coma” (beginning with epilepsy and ending with sleep apnea, and coma); (4) “Cranial Nerve Dysfunction” (1 through 12 with special emphasis on oculomotor disorders); (5) “Vertigo, Dizziness and Ataxia” (including a brief discussion of psychiatric issues); (6) “Disorders of Higher Cortical Function” (just about all of them); (7) “Cerebrovascular Disease” (with largest sections on infarction, small vessel disease, and subarachnoid hemorrhage); (8) “Parkinson's Disease and Other Extrapyramidal Disorders”; (9) “Cerebral Tumour and Hydrocephalus”; (10) “Multiple Sclerosis” (more pages—12—on MS than any other condition); (11) “Central Nervous System Infection” (HIV is covered under the “Retrovirus” subsection); (12) “Motor Neuron Disease and Peripheral Nerve Disorders”; (13) “Spinal Cord Disorders”; (14) “Myasthenia Gravis and Muscle Disease”; and (15) “The Neurology of Cancer, Systemic Disease and Psychiatry.”
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Grisold, Wolfgang, Bruno Giometto, Stefan Oberndorfer, and Roberta Vitaliani. "Paraneoplastic Neurologic Syndromes—An Update on Current Understanding and Future Perspectives." US Neurology 06, no. 02 (2010): 53. http://dx.doi.org/10.17925/usn.2010.06.02.53.
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Paraneoplastic neurological syndromes (PNS) are remote effects of tumors on the nervous system. They can strike at single or at multiple sites of the central nervous system (CNS) and the peripheral nervous system, and often appear before the detection of cancer. PNS can be disabling and debilitating and may be either an additional burden to cancer or the cause of death. The PNS Euronetwork group has collected a series of approximately 1,000 patients in several European centers. This study is the largest systematic series of patients with PNS and, for the first time, can answer questions about the most frequent PNS, their detailed symptoms, associated antibodies, and the types of underlying tumors. The clinical course and laboratory findings for many PNS suggest an autoimmune pathogenesis; however, research into this heterogeneous immunological relationship has been evolving over recent decades. The classic syndromes are antigen-target-oriented syndromes such as myasthenia gravis (MG), Lambert-Eaton myasthenic syndrome (LEMS), and ion channel-mediated diseases. Onconeuronal antibodies constitute a large group of PNS, characterized by the appearance of specific antibodies, defined clinical signs and often an association with specific tumors. In recent years, a new group of antibodies directed at surface antigens as receptors has been identified. Finally, there is a long list of ‘other’ PNS, which are evident to clinicians but which have no pathogenetic explanation. Examples include the mild terminal neuropathies and sarcopenia in cancer patients. In addition to the emerging classification based on pathophysiology, other new syndromes and symptoms have appeared, including apnea in brainstem encephalitis, a neuropsychiatric spectrum of limbic encephalitis, and increased knowledge about LEMS. Two important aspects warrant attention: some PNS respond to therapy and not all paraneoplastic-like syndromes are tumor related. This view is based on the current understanding of immune pathogenesis and on the enlarged spectrum of PNS.
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Grisold, Wolfgang, Bruno Giometto, Stefan Oberndorfer, and Roberta Vitaliani. "Paraneoplastic Neurologic Syndromes - An Update on Current Understanding and Future Perspectives." European Neurological Review 5, no. 2 (2010): 73. http://dx.doi.org/10.17925/enr.2010.05.02.73.
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Paraneoplastic neurological syndromes (PNS) are remote effects of tumours on the nervous system. They can strike at single or at multiple sites of the central nervous system (CNS) and the peripheral nervous system, and often appear before the detection of cancer. PNS can be disabling and debilitating and may be either an additional burden to cancer or the cause of death. The PNS Euronetwork group has collected a series of approximately 1,000 patients in several European centres. This study is the largest systematic series of patients with PNS and, for the first time, can answer questions about the most frequent PNS, their detailed symptoms, associated antibodies and the types of underlying tumours. The clinical course and laboratory findings for many PNS suggest an autoimmune pathogenesis; however research into this heterogeneous immunological relationship has been evolving over recent decades. The classical syndromes are antigen-target-oriented syndromes such as myasthenia gravis (MG), Lambert-Eaton myasthenic syndrome (LEMS) and ion channelmediated diseases. Onconeuronal antibodies constitute a large group of PNS, characterised by the appearance of specific antibodies, defined clinical signs and often an association with specific tumours. In recent years, a new group of antibodies directed at surface antigens as receptors has been identified. Finally there is a long list of ‘other’ PNS, which are evident to clinicians but which have no pathogenetic explanation. Examples include the mild terminal neuropathies and sarcopoenia in cancer patients. In addition to the emerging classification based on pathophysiology, other new syndromes and symptoms have appeared, including apnoea in brainstem encephalitis, a neuropsychiatric spectrum of limbic encephalitis and increased knowledge about LEMS. Two important aspects warrant attention: some PNS respond to therapy and not all paraneoplastic-like syndromes are tumour related. This view is based on the current understanding of immune pathogenesis and on the enlarged spectrum of PNS.
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Howard, RD, and RS Howard. "FP1-7 Neurology – the european specialty." Journal of Neurology, Neurosurgery & Psychiatry 90, no. 3 (February 14, 2019): e23.2-e23. http://dx.doi.org/10.1136/jnnp-2019-abn.73.
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ObjectivesThe case records at Queen Square afford a unique opportunity to understand the evolution of neurological diagnosis between 1870 and 1920. This period witnessed a shift from the descriptive and observational to a sophisticated and detailed understanding of discrete disease patterns.ResultsBy 1870, the introduction of microscopic pathological analysis of the nervous system had led to the recognition of disease processes including tumour, haemorrhage, infarction, meningitis and sclerosis. However, the most frequent diagnoses remained symptomatic (epilepsy, movement and functional disorders, visual loss and paralysis). Syphilis, paralysis agitans, and poliomyelitis were amongst the few specific diagnoses. The final decade of the 19th century saw the description of disease patterns, particularly in France, Germany and the US. These included disseminated and amyotrophic lateral sclerosis, myasthenia gravis and latterly eponymous disorders and signs attributed to Dejerine, Duchenne, Sanger Brown, Huntington, Marie, Romberg, Wernicke, Babinski and Charcot as well as Bright, Jackson, Gowers and Wilson in the UK.ConclusionsThe case record diagnoses, as well as the writings of Gowers and others, show how rapidly neurological thought evolved over 50 years. Queen Square was able to systematise neurological disease but was also remarkably responsive in understanding, accepting and adopting the neurological advances made across Europe.
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Gavriilaki, Maria, Vasilios K. Kimiskidis, and Eleni Gavriilaki. "Precision Medicine in Neurology: The Inspirational Paradigm of Complement Therapeutics." Pharmaceuticals 13, no. 11 (October 26, 2020): 341. http://dx.doi.org/10.3390/ph13110341.
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Precision medicine has emerged as a central element of healthcare science. Complement, a component of innate immunity known for centuries, has been implicated in the pathophysiology of numerous incurable neurological diseases, emerging as a potential therapeutic target and predictive biomarker. In parallel, the innovative application of the first complement inhibitor in clinical practice as an approved treatment of myasthenia gravis (MG) and neuromyelitis optica spectrum disorders (NMOSD) related with specific antibodies raised hope for the implementation of personalized therapies in detrimental neurological diseases. A thorough literature search was conducted through May 2020 at MEDLINE, EMBASE, Cochrane Library and ClinicalTrials.gov databases based on medical terms (MeSH)” complement system proteins” and “neurologic disease”. Complement’s role in pathophysiology, monitoring of disease activity and therapy has been investigated in MG, multiple sclerosis, NMOSD, spinal muscular atrophy, amyotrophic lateral sclerosis, Parkinson, Alzheimer, Huntington disease, Guillain–Barré syndrome, chronic inflammatory demyelinating polyneuropathy, stroke, and epilepsy. Given the complexity of complement diagnostics and therapeutics, this state-of-the-art review aims to provide a brief description of the complement system for the neurologist, an overview of novel complement inhibitors and updates of complement studies in a wide range of neurological disorders.
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Ara, Ferdous, Mohammad Sayeed Hassan, Md Abdullah Yusuf, Zubaida Nasreen, Aminul Islam, Md Badrul Alam, and Quazi Deen Mohammad. "Complications of Therapeutic Plasma Exchange in Patient with Neurological Disorders." Journal of National Institute of Neurosciences Bangladesh 3, no. 2 (May 26, 2018): 69–74. http://dx.doi.org/10.3329/jninb.v3i2.36766.
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Background: Therapeutic plasma exchange (TPE) is a procedure used in neurological disorders where autoimmunity plays a major role. Though it is a relatively safe procedure, severe complications may occur.Objective: The aim of the present study was to analyze the incidence of TPE related complications in patients with different neurological disorders.Methodology: This prospective study was conducted in 91 patients, in department of Transfusion Medicine at National Institute of Neurosciences and Hospital (NINS) from February 2014 to January 2017 for a period of three (03) years. All admitted patients with neurological disorders advised for TPE were included in this study.Results: Age range was from 13 to 70 years with a mean age of 37.14±13.79 years. 67(74%) were men and 24(26.0%) were women. The most common diagnosis was Guillain-Barré Syndrome (GBS) in 79.1 % (n=72) cases followed by myasthenia gravis (MG) in 13.2% (n=12), chronic inflammatory demyelinating polyneuropathy (CIDP) in 6.6 % (n= 6), multifocal motor neuropathy (MMN) in 1.1% (n=1) cases. Total number of 332 TPE sessions was performed with the median of 4 sessions per patient (range 1 to 9). Total 99 (29.8%) adverse reactions occurred in 332 TPE sessions. The most common adverse effect was hypotension (n=35, 10.54 %) followed by allergic reactions (n=29, 8.73 %), access problem (n= 12, 3.61 %) and vomiting (n=8, 2.41 %). Most of the adverse reactions were mild and improved spontaneously or by using simple medications. Out of 332 TPE sessions, 330(99.4%) procedures were completed successfully after managing the adverse reactions. TPE were discontinued in 2 occasions (0.6% of procedures) due to inadequate venous access. There was no mortality related to TPE procedure.Conclusion: In spite of few complications, TPE can be considered as a safe method of treatment in neurologic disorders when carried out with all necessary precautions.Journal of National Institute of Neurosciences Bangladesh, 2017;3(2): 69-74
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Nieto-Aristizábal, Ivana, Álvaro J. Vivas, Pablo Ruiz-Montaño, Cristian C. Aragón, Iván Posso-Osorio, Jairo Quiñones, Julián Alejandro Rivillas, and Gabriel J. Tobón. "Therapeutic Plasma Exchange as a Treatment for Autoimmune Neurological Disease." Autoimmune Diseases 2020 (July 31, 2020): 1–6. http://dx.doi.org/10.1155/2020/3484659.
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Introduction. Therapeutic plasma exchange (TPE) is commonly used as treatment of certain autoimmune neurological diseases (ANDs), and its main objective is the removal of pathogenic autoantibodies. Our aim was to describe the clinical profile and the experience with the usage of TPE in patients with ANDs at our institution. Methods. This is an observational retrospective study, including medical records of patients with diagnosis of ANDs who received TPE, between 2011 and 2018. Characteristics of TPE, such as number of cycles, type of replacement solution, and adverse effects, were evaluated. The modified Rankin Scale (mRS) was applied to measure the clinical response after the therapy. Results. 187 patients were included with the following diagnoses: myasthenia gravis (MG), n = 70 (37%); Guillain–Barré syndrome (GBS), n = 53 (28.3%), neuromyelitis optica spectrum disorders (NMOSD), n = 35 (18.7%); chronic inflammatory demyelinating polyneuropathy (CIDP), n = 23 (12.2%); and autoimmune encephalitis (AE), n = 6 (3.2%). The most used types of replacement solution were albumin (n = 131, 70%) and succinylated gelatin (n = 45, 24%). All patients received a median of five cycles (IQR 5-5). Hypotension and hydroelectrolytic disorders were the main complications. After TPE, 99 patients (52.9%) showed improvement in the mRS scores and a statistical significance (p<0.05) was seen between the admission score and after TPE for every diagnosis except for CIDP. Conclusion. TPE has an adequate safety profile, and improvement in functionality in treated patients reflects its effectiveness.
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Kayed, Rakez, George R. Jackson, D. Mark Estes, and Alan D. T. Barrett. "Alzheimers Disease: Review of Emerging Treatment Role for Intravenous Immunoglobulins." Journal of Central Nervous System Disease 3 (January 2011): JCNSD.S5018. http://dx.doi.org/10.4137/jcnsd.s5018.
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Alzheimer's disease (AD) is the most common neurodegenerative disorder. Currently available therapies are symptomatic but do not alter underlying disease progression. Immunotherapeutic approaches such as anti Aβ peptide active vaccination trials have had limited success to date. Intravenous immunoblobulin (IVIg) is widely used in immune-mediated neurological disorders such myasthenia gravis and Guillain-Barre syndrome. These preparations have been obtained from the pooled plasma of healthy human donors and contain natural anti-amyloid antibodies and are well tolerated. A small pilot study of passive immunotherapy using IVIg has suggested cognitive improvement. A multicenter phase III trial is ongoing and will determine whether or not this treatment can ameliorate cognitive deficits in mild-to-moderate AD. Here, we briefly review the pathogenic role of amyloid and tau in AD, as well as immunotherapeutic efforts to date. We also summarize what is known about naturally occurring anti-Aβ and tau antibodies in IVIg with a view toward explaining potential mechanisms underlying their therapeutic effects.
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Mirabile, Aurora, Elena Brioschi, Monika Ducceschi, Sheila Piva, Chiara Lazzari, Alessandra Bulotta, Maria Viganò, Giovanna Petrella, Luca Gianni, and Vanesa Gregorc. "PD-1 Inhibitors-Related Neurological Toxicities in Patients with Non-Small-Cell Lung Cancer: A Literature Review." Cancers 11, no. 3 (March 1, 2019): 296. http://dx.doi.org/10.3390/cancers11030296.
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The advent of immune checkpoint inhibitors gave rise to a new era in oncology and general medicine. The increasing use of programmed death-1 (PD-1) inhibitors in non-small cell lung cancer and in other malignancies means clinicians have to face up to new challenges in managing immune-related adverse events (irAEs), which often resemble autoimmune diseases. Neurological irAEs represent an emerging toxicity related to immunotherapy, and it is mandatory to know how to monitor, recognize, and manage them, since they can rapidly lead to patient death if untreated. Guidelines for the diagnosis and treatment of these irAEs have been recently published but sharing some of the most unusual clinical cases is crucial, in our opinion, to improve awareness and to optimize the approach for these patients. A literature review on the diagnosis and treatment of immune-related neurotoxicity’s has been conducted starting from the report of four cases of neurological irAEs regarding cases of polyneuropathy, myasthenia gravis, Bell’s palsy, and encephalopathy, all of which occurred in oncological patients receiving PD-1 inhibitors (pembrolizumab and nivolumab) for the treatment of non-oncogene addicted advanced non-small cell lung cancer. The exclusion of other differential diagnoses and the correlation between the suspension of immunotherapy and improvement of symptoms suggest that immunotherapy could be the cause of the neurological disorders reported.
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Gómez Vilda, Pedro. "Biomedical applications of voice and speech processing." Loquens 4, no. 1 (November 15, 2017): 035. http://dx.doi.org/10.3989/loquens.2016.035.
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Neurological deterioration presents different variants depending on their classification criterion, which may be their anatomic localization or their disease clinical features, although there is not a clear cut between both. Anatomically this ample group of disorders may affect the central nervous system (brain and spinal cord), or the peripheral nervous system. Clinically, the neurodegenerative disorders are classified as affecting cognitive functions or neuromotor capabilities. In the group of neurodegenerative diseases of the central nervous system, Alzheimer’s disease (AD) or Fronto-Temporal Dementia (FTD) are to be found, whereas in the second group certain pathologies as Parkinson’s Disease (PD), Amyotrophic Lateral Sclerosis (ALS), Huntington’s Disease (HD) or myasthenia gravis (MG) are among the most frequent ones, although “the number of neurodegenerative diseases is currently estimated to be a few hundred” (Przedborski et al., 2003). All these pathologies produce correlates in speech at different levels: in fluency, in prosody, in articulation or in phonation. Speech technologies offer computer solutions to evaluate objectively detected anomalies in each level, adding statistical robustness, which makes them suitable for their clinical and rehabilitative application. The present issue is devoted to briefly review the characteristics of the diseases mentioned before, defining the foundations of the correlate features present in each one. Some computer solutions available in detecting and monitoring illness progress are reviewed in the contributions of different research groups working in this field.
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Chowdhury, Rajib Nayan, Md Enayet Hussain, Md Nahidul Islam, Mostafa Hosen, AFM Al Masum Khan, Md Ferdous Mian, ATM Hasibul Hasan, Md Badrul Alam Mondal, and Quazi Deen Mohammad. "An audit of patients referred for nerve conduction study in a tertiary care hospital in Bangladesh." Journal of Dhaka Medical College 23, no. 1 (March 26, 2015): 102–8. http://dx.doi.org/10.3329/jdmc.v23i1.22703.
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Background: To examine the pattern and burden of neurologic disorders at electrophysiology lab of a tertiary care centre. Methodology: This retrospective chart review was carried out from the records and notes of electrophysiology lab in National Institute of Neurosciences and Hospital (NINS) from January to December 2013. A total of 1372 patients were evaluated with nerve conduction study (NCS) and electromyography (EMG) during this period. Result: Majority of the patients (67.6%) presented after forty with a mean age at presentation of 48.11±17.3 years. The male patients (55.2%) predominated. Carpal tunnel syndrome (CTS) was the most common condition (19.2%) observed, followed by different form of polyneuropathy namely Guillain Barre Syndrome (GBS) (6.04% with 50% being Acute inflammatory demyelinating polyneuropathy (AIDP), chronic inflammatory demyelinating polyneuropathy (CIDP) (3.27%), sensory motor polyneuropathy 3.13% and multifocal acquired motor axonopathy (MAMA) 2.55%. Though plexopathy and radiculopathy were rare (1.09 and 0.94% respectively), anterior horn cell disease was not that uncommon (8.73%). Disorders of muscle and neuromuscular junction (myasthenia gravis) were seen in 5.1% and 1.89% patient. Other various conditions (e.g. stroke, cerebral palsy, myelopathy) were observed in 10.05%. NCS and EMG were found to be normal in 270 patients (19.6%). Conclusion: Wide ranges of neurological problems are often referred to electrophysiology lab. Where ever the facilities and expert hands are available, these tests can help in diagnosing and classifying these cases. DOI: http://dx.doi.org/10.3329/jdmc.v23i1.22703 J Dhaka Medical College, Vol. 23, No.1, April, 2014, Page 102-108
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Chadha, Juskaran, Randy L. Levine, and Omer Ilyas. "IVIG-Induced Abrupt Hemolysis in Neurological Conditions." Blood 134, Supplement_1 (November 13, 2019): 4991. http://dx.doi.org/10.1182/blood-2019-124508.
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Background Immunoglobulin (IVIG) is used to treat autoimmune conditions, but there are reports of brisk hemolysis within 48 hours (hrs) of treatment due to anti-A isohemogglutinins(1). Despite these reports, hemolysis remains an unrecognized side effect of IVIG. Methods We presented a series of 3 cases of IVIG-induced hemolysis in patients with autoimmune neurological disorders. In the investigative phase, we traced the cases to a common IVIG lot number. The sample was tested to determine the anti-A titer levels. Case Studies (See Table 1) Case 1 75-year-old man presented with SOB and dysarthria from myasthenia gravis (MG). He received IVIG for 4 days. He developed a hemolytic anemia with 3 g drop in hemoglobin (Hb) 48 hours later. He needed a pRBC transfusion and folic acid. Case 2 59-year-old female with history of MG treated with IVIG at another hospital until 3 months earlier in crisis, with SOB and dysphagia. She received IVIG for 5 days and rituximab. She improved and was discharged, but returned to the ER 7 days later with SOB. Her Hb fell to 8.0 g/dL from 13 g/dL on last admission. She required a pRBC transfusion, folic acid, and vitamin B12 with improvement of SOB. Case 3 20-year-old female admitted for lower extremity weakness, diagnosed with presumed syndrome (GBS). She received IVIG for 4 days. On the 5th day, her Hb fell from 15 g/dL to 9 g/dL. She began prednisone, folic acid, and vitamin B12 with improvement in her Hb. Conclusions Although acute hemolysis is well described in the literature, it is under recognized, as exemplified by the first two cases. Their initial SOB was due to MG, so when SOB recurred, they were misdiagnosed with recurrent MG. A hemolytic anemia was later suspected, and a work up revealed a positive DAT. The initial eluate was negative against type O panel cells, suggesting a drug related hemolysis. It was only when the eluate was tested against type A cells that the etiology became clear. The third patient's hemolytic reaction was then rapidly identified. These cases remind us to consider IVIG induced anti-A hemolysis in patients who are blood type A and AB, and to evaluate the eluate against the appropriate reagent cells. These patients should receive specific IVIG that is low in anti-A isohemagglutinins. Since the second patient did not hemolyze from earlier exposure to IVIG, she likely received a low titer product. Disclosures No relevant conflicts of interest to declare.
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McCulloch, Katherine A., Yingchuan B. Qi, Seika Takayanagi-Kiya, Yishi Jin, and Salvatore J. Cherra. "Novel Mutations in Synaptic Transmission Genes Suppress Neuronal Hyperexcitation in Caenorhabditis elegans." G3 Genes|Genomes|Genetics 7, no. 7 (July 1, 2017): 2055–63. http://dx.doi.org/10.1534/g3.117.042598.
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Abstract Acetylcholine (ACh) receptors (AChR) regulate neural circuit activity in multiple contexts. In humans, mutations in ionotropic acetylcholine receptor (iAChR) genes can cause neurological disorders, including myasthenia gravis and epilepsy. In Caenorhabditis elegans, iAChRs play multiple roles in the locomotor circuit. The cholinergic motor neurons express an ACR-2-containing pentameric AChR (ACR-2R) comprised of ACR-2, ACR-3, ACR-12, UNC-38, and UNC-63 subunits. A gain-of-function mutation in the non-α subunit gene acr-2 [acr-2(gf)] causes defective locomotion as well as spontaneous convulsions. Previous studies of genetic suppressors of acr-2(gf) have provided insights into ACR-2R composition and assembly. Here, to further understand how the ACR-2R regulates neuronal activity, we expanded the suppressor screen for acr-2(gf)-induced convulsions. The majority of these suppressor mutations affect genes that play critical roles in synaptic transmission, including two novel mutations in the vesicular ACh transporter unc-17. In addition, we identified a role for a conserved major facilitator superfamily domain (MFSD) protein, mfsd-6, in regulating neural circuit activity. We further defined a role for the sphingosine (SPH) kinase (Sphk) sphk-1 in cholinergic neuron activity, independent of previously known signaling pathways. Overall, the genes identified in our study suggest that optimal modulation of synaptic activity is balanced by the differential activities of multiple pathways, and the novel alleles provide valuable reagents to further dissect neuronal mechanisms regulating the locomotor circuit.
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Zhang, Lin, Bronwyn Jenkins, Richard Stark, and Elspeth Hutton. "061 Training in headache in australia, new zealand and asia." Journal of Neurology, Neurosurgery & Psychiatry 90, e7 (July 2019): A20.1—A20. http://dx.doi.org/10.1136/jnnp-2019-anzan.53.
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IntroductionMigraine is the leading cause of age-adjusted neurological disability in Australia, but little is known about headache training in our region. We aimed to assess the quantity of teaching in headache subjects during undergraduate and postgraduate years.MethodThis is a cross-sectional survey study where questionnaires were sent to 137 delegates from Australia, New Zealand and Asia, prior to the Headache Master School in Sydney in August 2018. The Main outcome measured are recalled number of hours of teaching in undergraduate year and postgraduate years in: 1) Migraine; 2) Trigeminal autonomic cephalalgias (TACs); 3) Asthma; 4) Myasthenia gravis (MG).ResultsThe questionnaire response rate was 73% (100 of 137), of which 29 delegates were within 10 years of completing their undergraduate degree and 98 were neurologists. In undergraduate training, there was much greater quantity of teaching in asthma than migraine (Z=5.007, p<0.000) despite both being high-prevalent (asthma 11%, migraine 15–20%) conditions. Similarly, for diseases of medium-to-low prevalence, there was less training in TACs (1/1000), compared to MG (1.2/10,000) (Z=6.196, p<0.000). These major differences in training were also seen in postgraduate years even though overall headache teaching was greater in postgraduate than undergraduate training (p<0.000).ConclusionsDespite the high prevalence and morbidity of headache disorders, they receive less attention in training than conditions with similar prevalence. We propose that headache training opportunities should be improved in our region, particularly in the undergraduate course and preceptorships or fellowships in postgraduate years.
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Seetharaman, Kavita. "Thyrotoxic Periodic Paralysis." Journal of the Endocrine Society 5, Supplement_1 (May 1, 2021): A965—A966. http://dx.doi.org/10.1210/jendso/bvab048.1973.
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Abstract Thyrotoxic Periodic Paralysis (TPP) is an emergency associated with flaccid paralysis in which the paralysis is reversible with prompt potassium replacement and the attacks are terminated when hyperthyroidism is cured. Timely diagnosis and treatment are therefore prudent. While managing patients with flaccid paralysis, physicians should be aware of TPP as potential etiology and investigate history to identify the triggering factors and provide timely and cautious treatment with replacement of potassium, further addressing permanent approaches to treating thyrotoxicosis to prevent future recurrences of TPP. We report a clinical scenario of a patient who experienced TPP.22-year-old male, laying down at home at around 1:45pm developed sudden onset bilateral lower extremity weakness and was unable to stand up. His weakness was associated with thigh pain with exertion. He was eventually able to walk a few steps, but then fell onto his knees. He reported having a carbohydrate rich lunch at noon. Notably, patient underwent surgery under general anesthesia for a deviated nasal septum the day before. His neurological examination was remarkable for giveaway proximal lower extremity weakness involving only select muscle groups. Examination and CT/CTA of head and neck findings was not consistent with acute stroke. Myopathy secondary to electrolyte imbalance, drug/toxin, infection, or inflammatory disorders, myelopathy and myasthenia gravis were considered in the differential. Labs revealed suppressed TSH <0.01uIU/mL, elevated FT4 of 6ng/dL and low serum potassium of 2.6mmol/L. CT scan of the neck revealed enlarged thyroid gland and thymic enlargement. Patient was given intravenous bolus of potassium chloride 20 mEq and serum potassium normalized in 4 hours to 3.8mmol/L. His lower extremity weakness resolved within 3hours of potassium replacement. The next day his serum potassium was 5.2mmol/L. His thyroid stimulating immunoglobulin index was elevated at 1.4 (normal <1.3). He was started on propranolol, methimazole and advised to return to the endocrinology clinic to discuss permanent treatment options for hyperthyroidism to prevent TPP. Reviewing his prior medical history, he was diagnosed with Graves’ disease 5 months prior to the emergency room evaluation and was started on methimazole. He took methimazole for 2 months and did not return to follow up until the occurrence of TPP. He did not experience symptoms of hyperthyroidism in the interim. Discussion: TPP is a rare disorder with a prevalence of 0.1-0.2% in North America. TPP commonly affects Asian and Latin Americans males. The episodes of TPP are influenced by genetic, environmental, and ethnic factors. Common environmental triggers include carbohydrate rich meals, rest after intense physical exertion, fever, infection, trauma, emotional stress, and smoking. Thymic hyperplasia has also been reported to be associated with hypokalemic periodic paralysis. Clinical Presentation: The motor weakness tends to affect proximal lower extremities as noted in our patient’s presentation and is usually associated with hyporeflexia and is painless. Cardiac arrhythmias due to hypokalemia are rare, though ventricular fibrillation have been reported, and respiratory failure requiring mechanical ventilation is a possibility. Pathophysiology: TPP has been thought to be a channelopathy associated with increased Na+–K+ ATPase activity and loss of function mutation of the Kir 2.6 potassium efflux channel resulting in intracellular pooling of potassium and transient hypokalemia. The attacks are stimulated by thyroid hormone excess and/or hyperadrenergic activity and hyperinsulinemia, most commonly due to carbohydrate load or intravenous fluids containing dextrose. Treatment should include:1.Potassium supplementation to reverse paralysis and prevent life threatening cardiac arrhythmias. Care must be taken to not over replace potassium, as it could result in hyperkalemia when potassium channels revert to functioning normally.2.Propranolol, a non-selective betablocker works by blocking the thyroid hormone mediated adrenergic overstimulation of the Na-K ATPase channel, there by limiting the intracellular pooling of potassium.3.Definitive treatment of thyrotoxicosis should be offered. Studies comparing various modalities of treatment of thyrotoxicosis in the setting of TPP indicate permanent treatment with either radioactive iodine or thyroidectomy are often successful in preventing recurrence of TPP, as relapse events are higher in treatment with antithyroid drugs. Caution should be taken to prevent surge of thyroid hormone release following radioactive iodine treatment, as this can trigger TPP. Higher doses of radioactive iodine might be required to render hypothyroidism and prevent TPP recurrences. There is one case report of hypokalemic periodic paralysis associated with thymic hyperplasia that was treated with thymectomy. Our patient had multiple triggers leading to periodic paralysis, including a prior untreated Graves’ disease due to non-adherence to treatment during COVID-19 pandemic, general anesthesia for nasal septal surgery the day before presenting with TPP, having a carbohydrate rich meal one hour before the episode, and thymic enlargement on neck CT scan. He responded well to potassium replacement, and propranolol. He was started on methimazole and offered permanent treatment options to address hyperthyroidism. References: 1.K Shizume1, Y Shishiba, K Kuma, S Noguchi, J Tajiri, K Ito, J Y Noh. Comparison of the incidence of association of periodic paralysis and hyperthyroidism in Japan in 1957 and 1991. Endocrinol Jpn1992 Jun;39(3):315-8, doi: 10.1507/endocrj1954.39.315 2. R C Griggs, J Resnick, W K Engel. Intravenous treatment of hypokalemic periodic paralysis. Arch Neurol 1983 Sep;40(9):539-40.3. Maciel, R., Lindsey, S. & Dias da Silva, M. Novel etiopathophysiological aspects of thyrotoxic periodic paralysis. Nat Rev Endocrinol7, 657–667 (2011). https://doi.org/10.1038/nrendo.2011.58 4. Chang RY, Lang BH, Chan AC, Wong KP. Evaluating the efficacy of primary treatment for graves’ disease complicated by thyrotoxic periodic paralysis. Int J Endocrinol. 2014; 2014:949068 doi:10.1155/2014/949068 5.Yang R, Jurkat-Rott K, Cao J, et al. Hypokalemic Periodic Paralysis Induced by Thymic Hyperplasia and Relieved by Thymectomy. JAMA Neurol. 2013;70(11):1436–1439. doi:10.1001/jamaneurol.2013.3918
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Hawson, Frederick Y. "The Assessment of Oropharyngeal Dysphagia in Adults." Philippine Journal of Otolaryngology-Head and Neck Surgery 24, no. 2 (November 29, 2009): 43–45. http://dx.doi.org/10.32412/pjohns.v24i2.695.
Full textAbstract:
One of the more important and critical referrals that otolaryngologists can receive from colleagues in internal medicine, family medicine and geriatrics is the assessment of swallowing problems or dysphagia of their patients.
The term “dysphagia” is derived from two Greek words which literally mean difficulty in swallowing. Swallowing is a complex series of precisely coordinated voluntary and involuntary muscular movements in the mouth, pharynx and esophagus that serves to deliver food from the oral cavity into the stomach. Normal swallowing consists of three phases: oral preparatory, pharyngeal and esophageal. One normal swallow of a bolus of food should only take less than one second to reach the esophagus.
Dysphagia may manifest as difficulty managing secretions, drooling, delayed swallowing, coughing or choking with the swallow, a wet gurgly voice, and multiple swallow attempts. The complaint of dysphagia in an elderly patient should not be attributed to normal aging alone, but should be considered an alarm symptom that requires immediate definition of the exact cause and initiation of appropriate therapy.1
Dysphagia and Aspiration Pneumonia2,3
Dysphagic patients who aspirate have a seven-fold risk for acquiring pneumonia. In patients with an acute stroke, 40-70% have dysphagia. Of these, aspiration occurs in 40-50%. 50-75% of patients with degenerative diseases of the central nervous system (e.g. Alzheimer’s disease) also have dysphagia. Thus, people older than 75 years old have a six time higher risk of contracting aspiration pneumonia than younger individuals.
Factors that increase the risk of aspiration pneumonia in dysphagia patients include volume of aspirate, oropharyngeal colonization with pathogens such as Staphylococcus aureus, Klebsiella sp. or E. coli (due to decreased salivary clearance and poor oral hygiene) and poor nutritional status (that leads to decreased immunity).
Oropharyngeal Dysphagia
Dysphagia is typically distinguished into two types based on the phase of swallowing affected. Dysphagia secondary to a lesion above the esophagus is called orophayrngeal dysphagia. Dysphagia involving the upper esophageal sphincter to the stomach is considered esophageal dysphagia. This discussion will concentrate on oropharyngeal dysphagia.
Oral dysfunction causes drooling, food spillage, difficulty initiating a swallow, piecemeal swallows, and articulation problems. Pharyngeal dysfunction gives a sensation of food “getting stuck” immediately upon swallowing, regurgitation into the nose, coughing or choking while eating, and vocal problems. Difficulty is localized to the cervical region, usually involving liquids. In contrast, patients with esophageal dysphagia usually describe the onset of symptoms several seconds after initiating swallow. Difficulty is localized to the suprasternal notch or behind the sternum, usually involving solids.1
Oropharyngeal dysphagia is of unique clinical significance. Affected patients often have impaired ability to verbalize their discomfort or to cooperate with evaluation and therapy because of their neurological conditions. This dysphagia is usually not only a local problem, but just one aspect of a systemic disease syndrome. Diagnosis is a challenge because the problem is usually not obviously visible. Management therefore requires a coordinated team approach involving several medical and allied medical professionals. Aside from otolaryngologists, neurologists, radiologists, gastroenterologists, oncologists, rehabilitation medicine specialists and speech-language pathologists will have their specific roles.4
Oropharyngeal dysphagia can be locally caused by poor dentition, mucosal lesions, problems in salivary production, or by a number of neuromuscular disease syndromes. The central nervous system is commonly involved, as with cerebro-vascular accidents (usually in the brainstem), Parkinson’s disease, multiple sclerosis, amyotrophic lateral sclerosis, and brainstem tumors. The peripheral nervous system can also be involved, as with poliomyelitis or myasthenia gravis.. Local structural lesions may be inflammatory, neoplastic, compressive or post-surgical in nature. There can also be hypertensive or hypotensive motility disorders of the upper esophageal sphincter. 5,6
Physical Examination
A comprehensive physical examination should be part of the initial evaluation of all patients with oropharyngeal dysphagia. Examination of the oral cavity, head and neck, and supraclavicular region may reveal apparent problems that cause the dysphagia.
Neurological examination, which includes testing of all cranial nerves, especially those involved in swallowing (sensory components of CS V, IX, X and motor components of CN V, VII, X, XI and XII), may also detect disorders with more subtle physical findings of the various neuromuscular syndromes that could cause dysphagia. 7
Diagnostic Testing
Classic barium-swallow radiography is the most basic diagnostic test for dysphagia, though more useful for esophageal problems.
While esophageal manometry is more useful for esophageal dysphagia, it may also be helpful for patients who have oropharyngeal dysphagia with inconclusive results from other examinations. It is especially useful in cases in which surgical myotomy is being considered. 7
Videofluoroscopic evaluation of swallowing (VFES) gives a real-time and detailed analysis of swallowing mechanics from the oral to the esophageal stages, making this the gold standard of swallowing examinations. However, its prohibitive cost and the non-portability make it impractical for several patient settings, most particularly critical patients in Intensive Care Units.
Fiberoptic endoscopic evaluation of swallowing (FEES) is the diagnostic procedure performed mainly by otolaryngologists, and will be the discussed in some detail here.
Fiberoptic Endoscopic Evaluation of Swallowing (FEES)
Flexible Rhinopharyngoscopy is the preferred technique for examining the pharynx because anatomic structures are visualized without interfering with normal physiology of respiration and phonation.8 FEES is an extension technique done to examine swallowing events for both diagnostic and rehabilitative purposes 9 and was first described by Susan Langmore in 1988.10 FEES involves assessment of swallowing function for food and liquid, as well as the response to therapeutic interventions.
Before any food testing, velopharyngeal closure, anatomy of the tongue base and hypopharynx, vocal fold movement, status of pharyngeal musculature and patients’ ability to swallow saliva and secretions are first noted. If the equipment is available, sensory testing by eliciting the laryngeal adductor reflex (LAR), using calibrated air pulses delivered to the epithelium innervated by the internal branch of the superior laryngeal nerve, should also be performed.11 Being an involuntary reflex, this information is important when dealing with patients with impaired cognition.8,11
Food samples colored with green food dye (for better visibility) are typically presented in sequence: pureed food, honey thick liquid, nectar thick liquid, thin liquid, mechanical soft food, and regular food. The examiner may also include items from the patient’s current diet. 11
The patient may only swallow when asked to do so. Any premature spillage into the hypopharynx should be noted as this is frequently associated with laryngeal penetration. The oropharyngeal stage is not endoscopically visible because the tip of the endoscope will contact the base of the tongue, the epiglottis and the bolus itself when the swallowing reflex starts (swallowing white-out). During this stage, laryngeal penetration of food may be suspected if there are indirect signs like coughing or food in the laryngeal vestibule. After each swallow, it is likewise important to note the amount and location of residual food in the hypopharynx. When the patient talks or moves his head, these may also penetrate the larynx. 8,12,13
The most frequent adverse effect reported for FEES is discomfort. Topical anesthesia in the nose is not usually employed as it may affect the swallowing mechanism. Other adverse reactions such as changes in heart rate, epistaxis, laryngospasm and vasovagal response may be risky to the patient, but these events are not common. 7,10
Because of its ease of use, portability and lower cost, FEES is now the first choice method of swallowing investigation in Europe. The detection of aspiration of the bolus into the airways (even silent aspiration13) and the presence of bolus residue in the pharynx in FEES correlates very well with VFES, the gold standard. 8
Based on FEES results, the clinician can recommend resumption of oral feeding (with specified food consistencies) or shifting to non-oral options such as the nasogastric tube or percutaneous endoscopic gastrostomy. In either case, he can also recommend the initiation of swallowing rehabilitation therapy if deemed necessary.
A Medical Position Statement of the American Gastroenterological Association enumerates the following steps in the Management of Oropharyngeal Dysphagia, all of which are within the scope of Otolaryngologists:4
Ascertain whether oropharyngeal dysphagia is likely
Identify structural etiologies of oropharyrngeal dysfunction
Ascertain the functional integrity of the oropharyrngeal swallow
Evaluate the risk of aspiration pneumonia
Determine if the pattern of dysphagia is amenable to therapy
Otolaryngologists should be actively involved in the management of critically ill patients via a standardized endoscopy protocol,12 making it routine to perform FEES procedures on these patients, in order to make the best diagnostic decisions about their dysphagia, preventing aspiration pneumonia and its potentially fatal consequences.
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"A brief discussion on the diagnosis and management of Paraneoplastic Neurological Syndromes associated with ovarian cáncer. The importance of not thinking only about what we see." Research Journal of Neurology and Neuro Disorders, October 8, 2020, 01–06. http://dx.doi.org/10.36811/rjnnd.2020.110003.
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In relation to a recently published case [13] of a patient diagnosed with serous ovarian cancer which began as a paraneoplastic neurological syndrome, myasthenia gravis, here the importance of an early diagnosis of this type and its multidisciplinary handling is discussed. We want to highlight the early identification of the primary tumor and its treatment, in order to avoid the progression of these disorders which can develop into severe, incapacitating, or even fatal, neurological impairments. Keywords: Paraneoplastic Neurological Syndromes; Myasthenia gravis; Ovarian Cancer; Gynecological cancer
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Szklener, Katarzyna, and Slawomir Mandziuk. "Neurological manifestation of cancer – paraneoplastic syndromes." Current Issues in Pharmacy and Medical Sciences, July 8, 2021. http://dx.doi.org/10.2478/cipms-2021-0018.
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Abstract Neurological paraneoplastic syndromes (NPS) belong to a heterogeneous group of disorders affecting the nervous system. NPS occur as a result of immunological reaction to the tumor. As a result, the isolated syndrome is formed and manifests itself in many different ways, for example: limbic encephalitis, ataxia, dominant cerebellar degeneration, psychiatric disturbances, myasthenia gravis or diffuse encephalomyelitis. Detection of NPS is solely based on the presence of specific anti-neural antibodies. Although NPS had been previously considered unresponsive to therapy, some research has shown that there are effective therapies, including cancer- and immunotherapy targeted therapies.
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Pike-Lee, Tiffany, Sana Syed, Mary Alissa Willis, and Yuebing Li. "Pneumocystis jirovecii pneumonia in neurological disorders: Is prophylaxis necessary?" Neurology: Clinical Practice, August 28, 2020, 10.1212/CPJ.0000000000000923. http://dx.doi.org/10.1212/cpj.0000000000000923.
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AbstractBackground:The incidence of Pneumocystis jirovecii pneumonia (PJP) in patients with underlying neurological conditions is not well established, and the necessity of PJP prophylaxis for immunocompromised patients with neurological disorders is uncertain.Methods:Single-center retrospective analysis of non-HIV PJP patients at a tertiary referral center from 2007-2016 to determine the incidence of PJP in patients with primary neurological disordersResults:The study included 142 non-HIV PJP patients. Twenty patients had primary neurological diagnoses and 122 had non-neurological conditions. Associated neurological diagnoses included brain malignancies (N=16), myasthenia gravis (MG) (N=2), myopathy (N=2), neuromyelitis optica (NMO) (N=1) and central nervous system vasculitis (N=1). Estimated incidences of PJP were 0.7% for NMO and 0.3% for MG patients for the 10-year study period, while 4.6% of NMO and 3.8% of MG patients were placed on PJP prophylaxis. A survey of 24 neurologists who prescribe immunotherapy or chemotherapy confirmed an infrequent occurrence of PJP in their practice. Malignancy or parenchymal organ failure was present in 90% of neurological PJP patients and coexisting infections occurred in 45%.Conclusion:The overall incidence of PJP in patients with non-neoplastic neurological disorders is exceedingly low, raising doubt about the value of routine PJP prophylaxis in neurological patients outside neuro-oncology. PJP infection occurs frequently in patients with malignancy or parenchymal organ failure, indicating that overall health status may serve as a predisposing factor for PJP.
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Afzali, Mahdieh, Shahram Oveisgharan, Sahebeh Rajabkhah, and Siamak Abdi. "Complications of therapeutic plasma exchange in patients with neurological disorders." Iranian Journal of Neurology, June 20, 2020. http://dx.doi.org/10.18502/ijnl.v19i1.3282.
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Background: Therapeutic plasma exchange (TPE) is the treatment of choice for many neurologic disorders. The safety of this procedure is a major concern for physicians. The aim of this study was to determine the complications of TPE in patients with neurologic disorders at a tertiary referral hospital.
Methods: This retrospective cross-sectional study evaluated patients with various neurologic disorders receiving TPE in neurology department of Shariati Hospital, Tehran, Iran. Major and minor complications related to TPE were recorded.
Results: Clinical information records of 417 TPE sessions (88 patients) were available. Mean age of patients was 40.0 ± 15.8 years. Underlying diseases included central demyelinating disorders, myasthenia gravis (MG), chronic neuropathy, Guillain-Barre syndrome (GBS), and autoimmune encephalopathy in 34.1%, 33.0%, 17.0%, 14.8%, and 1.1% of patients, respectively. Major complications occurred in 15.9% of patients and 37.5% of patients accounted for minor complications. Among major adverse effects, thrombosis, infection, and lifethreatening complications were seen more commonly in patients with central vascular access (P = 0.005, P = 0.003, and P = 0.010, respectively).
Conclusion: TPE complications were seen more commonly in patients with central vascular access. Therefore, use of peripheral vascular access and vigilant patient monitoring by trained health providers can reduce its complications.
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Zografou, C., A. G. Vakrakou, and P. Stathopoulos. "Short- and Long-Lived Autoantibody-Secreting Cells in Autoimmune Neurological Disorders." Frontiers in Immunology 12 (June 17, 2021). http://dx.doi.org/10.3389/fimmu.2021.686466.
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As B cells differentiate into antibody-secreting cells (ASCs), short-lived plasmablasts (SLPBs) are produced by a primary extrafollicular response, followed by the generation of memory B cells and long-lived plasma cells (LLPCs) in germinal centers (GCs). Generation of IgG4 antibodies is T helper type 2 (Th2) and IL-4, -13, and -10-driven and can occur parallel to IgE, in response to chronic stimulation by allergens and helminths. Although IgG4 antibodies are non-crosslinking and have limited ability to mobilize complement and cellular cytotoxicity, when self-tolerance is lost, they can disrupt ligand-receptor binding and cause a wide range of autoimmune disorders including neurological autoimmunity. In myasthenia gravis with predominantly IgG4 autoantibodies against muscle-specific kinase (MuSK), it has been observed that one-time CD20+ B cell depletion with rituximab commonly leads to long-term remission and a marked reduction in autoantibody titer, pointing to a short-lived nature of autoantibody-secreting cells. This is also observed in other predominantly IgG4 autoantibody-mediated neurological disorders, such as chronic inflammatory demyelinating polyneuropathy and autoimmune encephalitis with autoantibodies against the Ranvier paranode and juxtaparanode, respectively, and extends beyond neurological autoimmunity as well. Although IgG1 autoantibody-mediated neurological disorders can also respond well to rituximab induction therapy in combination with an autoantibody titer drop, remission tends to be less long-lasting and cases where titers are refractory tend to occur more often than in IgG4 autoimmunity. Moreover, presence of GC-like structures in the thymus of myasthenic patients with predominantly IgG1 autoantibodies against the acetylcholine receptor and in ovarian teratomas of autoimmune encephalitis patients with predominantly IgG1 autoantibodies against the N‐methyl‐d‐aspartate receptor (NMDAR) confers increased the ability to generate LLPCs. Here, we review available information on the short-and long-lived nature of ASCs in IgG1 and IgG4 autoantibody-mediated neurological disorders and highlight common mechanisms as well as differences, all of which can inform therapeutic strategies and personalized medical approaches.
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Warnecke, Tobias, Bendix Labeit, Jens Schroeder, Alexander Reckels, Sigrid Ahring, Sriramya Lapa, Inga Claus, Paul Muhle, Sonja Suntrup-Krueger, and Rainer Dziewas. "Neurogenic Dysphagia: A Systematic Review and Proposal of a Classification System." Neurology, December 14, 2020, 10.1212/WNL.0000000000011350. http://dx.doi.org/10.1212/wnl.0000000000011350.
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Objective:Introduction and validation of a phenotypic classification of neurogenic dysphagia based on flexible endoscopic evaluation of swallowing (FEES).Methods:A systematic literature review was conducted, searching MEDLINE from inception to 05/2020 for FEES findings in neurological diseases of interest. Based on a retrospective analysis of FEES-videos in neurological diseases and considering the results from the review, a classification of neurogenic dysphagia was developed distinguishing different phenotypes. The classification was validated using 1012 randomly selected FEES-videos of patients with various neurological disorders. Chi-square-tests were used to compare the distribution of dysphagia phenotypes between the underlying neurological disorders.Results:159 articles were identified of which 59 were included in the qualitative synthesis. Seven dysphagia phenotypes were identified: (1) “Premature bolus spillage” and (2) “delayed swallowing reflex” occurred mainly in stroke patients, (3) “predominance of residue in the valleculae” was most common in Parkinson's disease, (4) “predominance of residue in the piriform sinus” occurred only in myositis, motoneuron disease and brainstem stroke patients, (5) “pharyngolaryngeal movement disorder” was found in atypical Parkinsonian syndromes and stroke patients, (6) “fatigable swallowing weakness” was common in patients with myasthenia gravis, and (7) “complex disorder” with a heterogeneous dysphagia pattern was the leading mechanism in amyotrophic later sclerosis. The interrater reliability showed a strong agreement (kappa = 0.84).Conclusion:Neurogenic dysphagia is not a mere symptom, but a multi-etiological syndrome with different phenotypic patterns depending on the underlying disease. Dysphagia phenotypes can facilitate differential diagnosis in patients with dysphagia of unclear etiology.
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Stathopoulos, Panos, and Marinos C. Dalakas. "Autoimmune Neurogenic Dysphagia." Dysphagia, July 5, 2021. http://dx.doi.org/10.1007/s00455-021-10338-9.
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AbstractAutoimmune neurogenic dysphagia refers to manifestation of dysphagia due to autoimmune diseases affecting muscle, neuromuscular junction, nerves, roots, brainstem, or cortex. Dysphagia is either part of the evolving clinical symptomatology of an underlying neurological autoimmunity or occurs as a sole manifestation, acutely or insidiously. This opinion article reviews the autoimmune neurological causes of dysphagia, highlights clinical clues and laboratory testing that facilitate early diagnosis, especially when dysphagia is the presenting symptom, and outlines the most effective immunotherapeutic approaches. Dysphagia is common in inflammatory myopathies, most prominently in inclusion body myositis, and is frequent in myasthenia gravis, occurring early in bulbar-onset disease or during the course of progressive, generalized disease. Acute-onset dysphagia is often seen in Guillain–Barre syndrome variants and slowly progressive dysphagia in paraneoplastic neuropathies highlighted by the presence of specific autoantibodies. The most common causes of CNS autoimmune dysphagia are demyelinating and inflammatory lesions in the brainstem, occurring in patients with multiple sclerosis and neuromyelitis optica spectrum disorders. Less common, but often overlooked, is dysphagia in stiff-person syndrome especially in conjunction with cerebellar ataxia and high anti-GAD autoantibodies, and in gastrointestinal dysmotility syndromes associated with autoantibodies against the ganglionic acetyl-choline receptor. In the setting of many neurological autoimmunities, acute-onset or progressive dysphagia is a potentially treatable condition, requiring increased awareness for prompt diagnosis and early immunotherapy initiation.
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Baruca, Mateja, and Anton Grad. "Obravnava novoodkritih bolnikov z miastenijo gravis v regionalni bolnišnici med leti 2003–2013." Slovenian Medical Journal 86 (June 5, 2017). http://dx.doi.org/10.6016/zdravvestn.1937.
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Background: Myasthenia gravis (MG) is the most common autoimmune disorders of the neuromuscular junction. Advances in intensive care in conjunction with the advent of novel treatments have made MG one of the most treatable neurological disorders. The incidence and prevalence rates of MG have increased over time due to greater disease awareness, better diagnostic methods and ageing of the population. Due to these facts, the number of MG patients evaluated at the secondarylevel regional hospitals has increased. So far, no data on the evaluation of MG in Slovenia have been systematically collected. The aim and scope of this article is to present the analysis of the evaluation of newly diagnosed MG patients at the General hospital of Isola for the time period between 1 January 2003 and 31 December 2013.Methods: We reviewed medical files of MG patients diagnosed at the General hospital of Isola in the above mentioned time period and collected data on patients’ demographic and clinical characteristics in addition to examining their clinical course and treatment outcome.Results: Twenty-nine patients were identified (14 women, 15 men). Te mean age at disease onset was 59.4 ± 21.2 years for women and 56.3 ± 19.8 years for men. In the study population, 58.6 % of the patients were acetylcholine receptor antibody positive. Besides MG, patients most often had thyroid and other chronic diseases. Most patients (68.9 %) presented with extraocular disease symptoms, which progressed to generalized disease in 65 % of cases. Myasthenic crisis occurred in 10.3 % of patients. In 68.9 % of cases, the patients were treated exclusively with symptomatic therapy. Tymectomy was performed in 13.7 % of cases. On treatment, 13.7 % achieved a stable complete remission of MG symptoms, in 55 % the condition substantially improved, in 20.6 % improvement was only partial, and in 6.8 % of patients the condition remained unchanged. The fiveyear mortality of the study population, estimated according to the Kaplan-Meier method was 13.5 %.Conclusions: With the exception of a higher mortality rate, the patients’ characteristics and clinical course of the disease were similar to data reported in other epidemiological studies. The patients’ higher mortality rate was considered to be a consequence of the lack of intensive treatment in patients with myasthenic crisis due to the unawareness of the reversibility of the condition with an adequate treatment.
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Sivapalan, Pradeesh, Charlotte Suppli Ulrik, Rasmus Dahlin Bojesen, Therese Sophie Lapperre, Josefin Viktoria Eklöf, Kjell Erik Julius Håkansson, Andrea Browatzki, et al. "Proactive Prophylaxis With Azithromycin and HydroxyChloroquine in Hospitalised Patients With COVID-19 (ProPAC-COVID): A structured summary of a study protocol for a randomised controlled trial." Trials 21, no. 1 (June 10, 2020). http://dx.doi.org/10.1186/s13063-020-04409-9.
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Abstract Objectives The aim of this randomised GCP-controlled trial is to clarify whether combination therapy with the antibiotic azithromycin and hydroxychloroquine via anti-inflammation/immune modulation, antiviral efficacy and pre-emptive treatment of supra-infections can shorten hospitalisation duration for patients with COVID-19 (measured as "days alive and out of hospital" as the primary outcome), reduce the risk of non- invasive ventilation, treatment in the intensive care unit and death. Trial design This is a multi-centre, randomised, Placebo-controlled, 2-arm ratio 1:1, parallel group double-blind study. Participants 226 participants are recruited at the trial sites/hospitals, where the study will take place in Denmark: Aalborg, Bispebjerg, Gentofte, Herlev, Hillerød, Hvidovre, Odense and Slagelse hospitals. Inclusion criteria: • Patient admitted to Danish emergency departments, respiratory medicine departments or internal medicine departments • Age≥ 18 years • Hospitalized ≤48 hours • Positive COVID-19 test / diagnosis during the hospitalization (confirmed). • Men or non-fertile women. Fertile women* must not be pregnant, i.e. negative pregnancy test must be available at inclusion • Informed consent signed by the patient *Defined as after menarche and until postmenopausal (no menstruation for 12 months) Exclusion criteria: • At the time of recruitment, the patient uses >5 LO2/min (equivalent to 40% FiO2 if measured) • Known intolerance/allergy to azithromycin or hydroxychloroquine or hypersensitivity to quinine or 4-aminoquinoline derivatives • Neurogenic hearing loss • Psoriasis • Retinopathy • Maculopathy • Visual field changes • Breastfeeding • Severe liver diseases other than amoebiasis (INR> 1.5 spontaneously) • Severe gastrointestinal, neurological and hematological disorders (investigator-assessed) • eGFR <45 ml/min/1.73 m2 • Clinically significant cardiac conduction disorders/arrhythmias or prolonged QTc interval (QTc (f) of> 480/470 ms). • Myasthenia gravis • Treatment with digoxin* • Glucose-6-phosphate dehydrogenase deficiency • Porphyria • Hypoglycaemia (Blood glucose at any time since hospitalization of <3.0 mmol/L) • Severe mental illness which significantly impedes cooperation • Severe linguistic problems that significantly hinder cooperation • Treatment with ergot alkaloids *The patient must not be treated with digoxin for the duration of the intervention. For atrial fibrillation/flutter, select according to the Cardiovascular National Treatment Guide (NBV): Calcium antagonist, Beta blocker, direct current (DC) conversion or amiodarone. In case of urgent need for digoxin treatment (contraindication for the aforementioned equal alternatives), the test drug should be paused, and ECG should be taken daily. Intervention and comparator Control group: The control group will receive the standard treatment + placebo for both types of intervention medication at all times. If part or all the intervention therapy being investigated becomes standard treatment during the study, this may also be offered to the control group. Intervention group: The patients in the intervention group will also receive standard care. Immediately after randomisation to the intervention group, the patient will begin treatment with: Azithromycin: Day 1-3: 500 mg x 1 Day 4-15: 250 mg x 1 If the patient is unable to take the medication orally by themselves, the medication will, if possible, be administered by either stomach-feeding tube, or alternatively, temporary be changed to clarithromycin 500 mg x 2 (this only in agreement with either study coordinator Pradeesh Sivapalan or principal investigator Jens-Ulrik Stæhr Jensen). This will also be done in the control group if necessary. The patient will switch back to azithromycin when possible. Hydroxychloroquine: Furthermore, the patient will be treated with hydroxychloroquine as follows: Day 1-15: 200 mg x 2 Main outcomes • Number of days alive and discharged from hospital within 14 days (summarises both whether the patient is alive and discharged from hospital) ("Days alive and out of hospital") Randomisation The sponsor (Chronic Obstructive Pulmonary Disease Trial Network, COP:TRIN) generates a randomisation sequence. Randomisation will be in blocks of unknown size and the final allocation will be via an encrypted website (REDCap). There will be stratification for age (>70 years vs. <=70 years), site of recruitment and whether the patient has any of the following chronic lung diseases: COPD, asthma, bronchiectasis, interstitial lung disease (Yes vs. No). Blinding (masking) Participants and study personnel will both be blinded, i.e. neither will know which group the participant is allocated to. Numbers to be randomised (sample size) This study requires 226 patients randomised 1:1 with 113 in each group. Trial Status Protocol version 1.8, from April 16, 2020. Recruitment is ongoing (first patient recruited April 6, 2020; final patient expected to be recruited October 31, 2020). Trial registration ClinicalTrials.gov Identifier: NCT04322396 (registered March 26, 2020) Full protocol The full protocol is attached as an additional file, accessible from the Trials website (Additional file 1). In the interest in expediting dissemination of this material, the familiar formatting has been eliminated; this Letter serves as a summary of the key elements of the full protocol. The study protocol has been reported in accordance with the Standard Protocol Items: Recommendations for Clinical Interventional Trials (SPIRIT) guidelines (Additional file 2).