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Motor neuron synapses with muscle fiber via electrical impulse transmission and neurotransmitter release, forming neuromuscular junctions , motor neuron, neuroscience
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Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder of upper and lower motor neurons that results in progressive motor impairment. ALS is the most common disease of motor neurons with an annual incidence of approximately 1.7–2.5 per 100,000 people. It is a terminal condition with a typical life expectancy of 2–5 years from symptom onset. […]

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Coverage from: EAN 2024

Myasthenia gravis (MG) is an autoimmune disorder where antibodies disrupt the neuromuscular junction, causing muscle weakness that worsens with activity. MG subgroups are based on muscle weakness location, age, antibody type, and thymus pathology, with some patients experiencing severe, treatment-resistant symptoms. Biomarkers can indicate prognosis. First-line treatments include pyridostigmine for symptomatic relief and immunosuppressants like prednisolone and azathioprine. Thymectomy is recommended for certain patients. Second-line treatments include mycophenolate, rituximab, and others, with new therapies like complement and FcRn inhibitors showing promise. Intravenous immunoglobulin and plasma exchange are used for acute exacerbations. Supportive therapy, including adapted exercise, is crucial. In refractory cases, comorbidities and diagnosis accuracy should be reconsidered.

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Patrick Schindler, Friedemann Paul

Neuromyelitis optica spectrum disorders (NMOSD) are a group of relapsing autoimmune diseases of the central nervous system. The clinical hallmarks of NMOSD are myelitis and optic neuritis; however, a wider clinical spectrum has been recognized.1 The majority of patients with ...

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Neuromyelitis optica spectrum disorder (NMOSD) is a rare inflammatory autoimmune disease of the central nervous system (CNS) with a worldwide distribution.1 The first clinical description of NMOSD was made a century ago by Devic and Gault, who documented patients with ...

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Case study Patient information A 42-year-old woman presented in the emergency department with acute onset whole-body myoclonic jerks for 1 day. On enquiry, the patient’s parents advised that she had a history of depression over the past 15 years. Intermittently, family ...

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Acute disseminated encephalomyelitis (ADEM), first characterized in 1931,1 is a non-specific clinical syndrome of polyfocal central nervous system (CNS) inflammatory demyelination; it is characterized by encephalopathy and large, poorly demarcated cerebral white matter lesions.2,3 Myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease (MOGAD), ...

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Over the past two decades, monoclonal antibodies targeting the surface antigen CD20 have emerged as highly effective disease-modifying therapies (DMTs) for multiple sclerosis (MS).1 The major mechanism of action of these therapies is via B-cell depletion, as CD20 is expressed ...

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Aaron W Abrams, Amanda March, Daphne Porat

Paediatric-onset multiple sclerosis (POMS) is the symptom onset and diagnosis of multiple sclerosis (MS) before the age of 18.1 Diagnostic criteria were revised by the International Pediatric MS Study Group in 2013. Compared to patients with adult-onset MS (AOMS), young patients are ...

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Saša A Živkovic‘, Tawfiq Al-Lahham

The clinical use of cancer immunotherapy with immune checkpoint inhibitors (ICI) has transformed cancer management and added another effective treatment option for different types of malignancies.1–3 In 2018, the Nobel Prize for medicine and physiology was awarded for the discovery of ...

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Saranya B Gomathy, Ayush Agarwal, Venugopalan Y Vishnu

Myasthenia gravis (MG) is an autoimmune disorder caused by autoantibodies against the myoneural junction, which lead to impaired neuromuscular transmission. These antibodies act at the post-synaptic membrane, commonly against the nicotinic acetylcholine receptor (AChR) but in some cases, antibodies to ...

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Neuromyelitis optica spectrum disorder (NMOSD) is an inflammatory disease of the central nervous system (CNS) and is known to cause recurrent episodes of optic neuritis and transverse myelitis. Myelitis is radiologically referred to as longitudinal extensive transverse myelitis on spinal ...

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Alexandra Zirra, Cris S Constantinescu

In the past 4 months, a novel coronavirus originating in Wuhan, China, SARS-CoV-2 (severe acute respiratory syndrome coronavirus 2), has affected more than 200 countries. As of mid-June 2020, there are over 7 million confirmed cases and more than 425,888 deaths worldwide caused by the novel ...

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Neuromyelitis optica spectrum disorder (NMOSD) refers to a family of inflammatory central nervous system (CNS) diseases in which patients accrue disability through severe episodes of demyelination with typical manifestations including involvement of visual pathways (e.g., optic neuritis) and spinal ...

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Multiple sclerosis (MS) has traditionally been considered a primarily T-cell-mediated autoimmune disease of the central nervous system (CNS); this is based on data from animal models,1 the presence of activated T lymphocytes in MS plaques that outnumber B cells and ...

Considering the impact COVID-19 could have on those with multiple sclerosis, we were delighted to speak with Dr Asaff Harel (Lennox Hill Hospital, New York, NY, USA) on the subject. Questions 1. How does multiple sclerosis (MS) increase the risk from ...

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Myasthenia gravis (MG) leads to muscle weakness, which increases with repetitive use of muscles; in the morning, many patients are symptom-free.1,2 Diplopia, ptosis and weakness in other muscles innervated by cranial nerves, are typical. The muscle weakness is, in most ...

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Chronic inflammatory demyelinating polyneuropathy (CIDP) is a rare but challenging neuroimmunologic peripheral nerve disease with a chronic progressive or relapsing-remitting course.1,2 Effective maintenance therapies are critical for managing the condition, maintaining a response, and preventing relapse.3 In CIDP, symptoms result ...

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Chronic inflammatory demyelinating polyneuropathy (CIDP) is an immune-mediated disorder of the peripheral nervous system, of unknown etiology.1 The condition usually presents as weakness in the arms and legs, balance/gait impairment, tingling, numbness, and loss of tendon reflexes.2–4 It can ...

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Myasthenia gravis (MG) is a relatively rare autoimmune disease, caused by an antibody-mediated blockade of neuromuscular transmission and resulting in skeletal muscle weakness. MG is characterised by fluctuating muscle weakness that worsens with activity and improves on resting. Over half ...

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