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Neurologic Clinics

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https://www.readbyqxmd.com/read/29655457/fifty-key-publications-on-myasthenia-gravis-and-related-disorders
#1
EDITORIAL
Mazen M Dimachkie, Richard J Barohn
No abstract text is available yet for this article.
May 2018: Neurologic Clinics
https://www.readbyqxmd.com/read/29655456/lambert-eaton-myasthenic-syndrome
#2
REVIEW
Vita G Kesner, Shin J Oh, Mazen M Dimachkie, Richard J Barohn
Lambert-Eaton myasthenic syndrome is a paraneoplastic or primary autoimmune neuromuscular junction disorder characterized by proximal weakness, autonomic dysfunction and ariflexia. The characteristic symptoms are thought to be caused by antibodies generated against the P/Q-type voltage-gated calcium channels present on presynaptic nerve terminals and by diminished release of acetylcholine. More than half of Lambert-Eaton myasthenic syndrome cases are associated with small cell lung carcinoma. Diagnosis is confirmed by serologic testing and electrophysiologic studies...
May 2018: Neurologic Clinics
https://www.readbyqxmd.com/read/29655455/congenital-myasthenic-syndromes
#3
REVIEW
Perry B Shieh, Shin J Oh
The congenital myasthenic syndromes (CMS) are a group of rare genetic conditions characterized by abnormal neuromuscular transmission. Typically, these conditions have been the result of a dysfunctional protein that is present in the presynaptic terminal, the synaptic cleft, or the postsynaptic terminal. Many of these syndromes present within the first few years of life with fluctuating and fatiguable weakness in a distribution similar to myasthenia gravis, although a limb-girdle distribution and late onset are also seen in certain specific types of CMS...
May 2018: Neurologic Clinics
https://www.readbyqxmd.com/read/29655454/an-update-myasthenia-gravis-and-pregnancy
#4
REVIEW
Johanna Hamel, Emma Ciafaloni
Myasthenia gravis presents a risk factor for pregnancy and delivery, and can affect the newborn. In return, pregnancy can affect the course of myasthenia and worsen the disease during pregnancy requiring treatment modifications. Treatment optimization and drug safety should be addressed before conception. Delivery is complicated by prolonged labor. Newborns can develop neonatal myasthenia gravis, a treatable and transient disease. Patients should not be discouraged to become pregnant, but provided with supportive counseling, planning, and monitoring in a multidisciplinary team involving obstetrician, anesthesiologist, pediatrician, and neurologist...
May 2018: Neurologic Clinics
https://www.readbyqxmd.com/read/29655453/measuring-clinical-treatment-response-in-myasthenia-gravis
#5
REVIEW
Carolina Barnett, Laura Herbelin, Mazen M Dimachkie, Richard J Barohn
In this article we provide an overview of health-related outcome measurement-to better understand what different outcomes used in myasthenia actually measure-and to provide some guidance when choosing measures based on the clinical context and question. In myasthenia, the most commonly used outcome measures are aimed at assessing the signs and symptoms. In this review, we provide a summary of the most commonly used outcome measures. We discuss instruments that gauge disease overall health impact, such as on disability and quality of life...
May 2018: Neurologic Clinics
https://www.readbyqxmd.com/read/29655452/treatment-of-myasthenia-gravis
#6
REVIEW
Constantine Farmakidis, Mamatha Pasnoor, Mazen M Dimachkie, Richard J Barohn
With specialized care, patients with myasthenia gravis can have very good outcomes. The mainstays of treatment are acetylcholinesterase inhibitors, and immunosuppressive and immunomodulatory therapies. There is good evidence thymectomy is beneficial in thymomatous and nonthymomatous disease. Nearly all of the drugs used for MG are considered "off-label." The 2 exceptions are acetylcholinesterase inhibitors and complement inhibition with eculizumab, which was recently approved by the US Food and Drug Administration for myasthenia gravis...
May 2018: Neurologic Clinics
https://www.readbyqxmd.com/read/29655451/muscle-specific-tyrosine-kinase-and-myasthenia-gravis-owing-to-other-antibodies
#7
REVIEW
Michael H Rivner, Mamatha Pasnoor, Mazen M Dimachkie, Richard J Barohn, Lin Mei
Around 20% of patients with myasthenia gravis are acetylcholine receptor antibody negative; muscle-specific tyrosine kinase antibodies (MuSK) were identified as the cause of myasthenia gravis in 30% to 40% of these cases. Anti MuSK myasthenia gravis is associated with specific clinical phenotypes. One is a bulbar form with fewer ocular symptoms. Others show an isolated head drop or symptoms indistinguishable from acetylcholine receptor-positive myasthenia gravis. These patients usually respond well to immunosuppressive therapy, but not as well to cholinesterase inhibitors...
May 2018: Neurologic Clinics
https://www.readbyqxmd.com/read/29655450/nature-and-action-of-antibodies-in-myasthenia-gravis
#8
REVIEW
Robert L Ruff, Robert P Lisak
This article discusses antibodies associated with immune-mediated myasthenia gravis and the pathologic action of these antibodies at the neuromuscular junctions of skeletal muscle. To explain how these antibodies act, we consider the physiology of neuromuscular transmission with emphasis on 4 features: the structure of the neuromuscular junction; the roles of postsynaptic acetylcholine receptors and voltage-gated Na+ channels and in converting the chemical signal from the nerve terminal into a propagated action potential on the muscle fiber that triggers muscle contraction; the safety factor for neuromuscular transmission; and how the safety factor is reduced in different forms of autoimmune myasthenia gravis...
May 2018: Neurologic Clinics
https://www.readbyqxmd.com/read/29655449/diagnosis-of-myasthenia-gravis
#9
REVIEW
Mamatha Pasnoor, Mazen M Dimachkie, Constantine Farmakidis, Richard J Barohn
Myasthenia gravis (MG) diagnosis is primarily clinically based. By the end of the clinical evaluation, clinicians have a sense as to whether presenting symptoms and elicited signs are weakly or strongly supportive of MG. Diagnostic tests can reaffirm the clinicians' impression. Edrophonium testing is rarely used but helpful in cases of measurable ptosis. Decremental response on slow-frequency repetitive nerve stimulation has a modest diagnostic yield in ocular MG but is helpful in generalized MG cases. The most sensitive test is single-fiber electromyography...
May 2018: Neurologic Clinics
https://www.readbyqxmd.com/read/29655448/generalized-myasthenia-gravis-classification-clinical-presentation-natural-history-and-epidemiology
#10
REVIEW
Michael K Hehir, Nicholas J Silvestri
Myasthenia gravis (MG) is a rare disease, but the most common disorder of the neuromuscular junction. It is the prototypic autoimmune disease most commonly caused by antibodies to the acetylcholine receptor (AChR) leading to characteristic fatigable weakness of the ocular, bulbar, respiratory, axial, and limb muscles. The majority of patients with MG first present with ocular symptoms. Most patients with MG will experience at least 1 exacerbation of symptoms throughout the course of their illness. This article will cover the epidemiology, clinical presentation, classification, and natural history of MG...
May 2018: Neurologic Clinics
https://www.readbyqxmd.com/read/29655447/ocular-myasthenia
#11
REVIEW
Mohammed Al-Haidar, Michael Benatar, Henry J Kaminski
Ocular myasthenia is a form of myasthenia gravis in which weakness is restricted to the ocular muscles and may produce significant visual disability. Patients present with fluctuating ptosis, diplopia, or a combination of both. Examination may show any type of ocular motility deficit ranging from isolated muscle palsy to complete ophthalmoplegia. Cogan lid twitch, enhanced ptosis, peek sign, and saccadic fatigue are specific examination findings that support the clinical diagnosis of myasthenia gravis. Confirmation of the diagnosis is challenging with autoantibody serology, and repetitive nerve stimulation studies are often negative...
May 2018: Neurologic Clinics
https://www.readbyqxmd.com/read/29655446/practical-anatomy-of-the-neuromuscular-junction-in-health-and-disease
#12
REVIEW
Hiroshi Nishimune, Kazuhiro Shigemoto
Neuromuscular junctions (NMJs) form between nerve terminals of spinal cord motor neurons and skeletal muscles, and perisynaptic Schwann cells and kranocytes cap NMJs. One muscle fiber has one NMJ, which is innervated by one motor nerve terminal. NMJs are excitatory synapses that use P/Q-type voltage-gated calcium channels to release the neurotransmitter acetylcholine. Acetylcholine receptors accumulate at the postsynaptic specialization called the end plate on the muscle fiber membrane, the sarcolemma. Proteins essential for the organization of end plates include agrin secreted from nerve terminals, Lrp4 and MuSK receptors for agrin, and Dok-7 and rapsyn cytosolic proteins in the muscle...
May 2018: Neurologic Clinics
https://www.readbyqxmd.com/read/29157406/contemporary-topics-in-multiple-sclerosis
#13
EDITORIAL
Darin T Okuda
No abstract text is available yet for this article.
February 2018: Neurologic Clinics
https://www.readbyqxmd.com/read/29157405/common-clinical-and-imaging-conditions-misdiagnosed-as-multiple-sclerosis-a-current-approach-to-the-differential-diagnosis-of-multiple-sclerosis
#14
REVIEW
Aksel Siva
Multiple Sclerosis is increasing, so is the number of misdiagnosed cases as MS. One major source of misdiagnosis is misinterpretation of nonspecific clinical and imaging findings and misapplication of MS diagnostic criteria resulting in an overdiagnosis of MS! Since nonspecific white matter abnormalities on brain MRI and other imaging findings that may mimic MS, as well as MS-nonspecific lesions that are seen in people with MS, neurologists should be aware of all possibilities and should be able to interpret the clinical and MRI findings well and by their selves! The differential diagnosis of MS includes MS variants and inflammatory astrocytopathies and other atypical inflammatory-demyelinating syndromes, as well as a number of systemic diseases with CNS involvement...
February 2018: Neurologic Clinics
https://www.readbyqxmd.com/read/29157404/anomalies-characteristic-of-central-nervous-system-demyelination-radiologically-isolated-syndrome
#15
REVIEW
Christine Lebrun, Orhun H Kantarci, Aksel Siva, Daniel Pelletier, Darin T Okuda
Radiologically isolated syndrome (RIS) was defined in 2009 for asymptomatic patients who presented incidentally identified white matter anomalies within the central nervous system suggestive of multiple sclerosis (MS). Approximately one-third of RIS subjects will have a seminal clinical demyelinating event within 5 years of the identification of their abnormal MRI. Clinical evolution mirrors relapsing remitting or progressive forms of MS. Pejorative factors for clinical conversion are male gender, age younger than 35 years, and spinal cord lesions...
February 2018: Neurologic Clinics
https://www.readbyqxmd.com/read/29157403/spinal-cord-mri-in-multiple-sclerosis
#16
REVIEW
Alexandra Muccilli, Estelle Seyman, Jiwon Oh
Spinal cord (SC) MRI in multiple sclerosis (MS) has significant usefulness in clinical and investigational settings. Conventional MRI of the SC is used in clinical practice, because it has both diagnostic and prognostic value. A number of advanced, quantitative SC MRI measures that assess the structural and functional integrity of the SC have been evaluated in investigational settings. These techniques have collectively demonstrated usefulness in providing insight into microstructural and functional changes relevant to disability in MS...
February 2018: Neurologic Clinics
https://www.readbyqxmd.com/read/29157402/effective-utilization-of-mri-in-the-diagnosis-and-management-of-multiple-sclerosis
#17
REVIEW
Antonio Giorgio, Nicola De Stefano
MRI is the most important tool for diagnosis and management of patients with multiple sclerosis (MS). MRI shows high sensitivity for detection of white matter lesions in the central nervous system and specificity for lesion dissemination in space and time. MRI is also used for tracking disease activity, prognostic evaluation, and monitoring the efficacy and safety of disease-modifying treatments. Nonconventional MRI measures (eg, brain atrophy) and quantitative measures of advanced MRI can capture features of MS beyond WM lesions but are not currently implemented in clinical practice...
February 2018: Neurologic Clinics
https://www.readbyqxmd.com/read/29157401/multiple-sclerosis-in-the-contemporary-age-understanding-the-millennial-patient-with-multiple-sclerosis-to-create-next-generation-care
#18
REVIEW
Madison R Hansen, Darin T Okuda
The average age of onset of multiple sclerosis (MS) is between 20 and 40 years of age. Therefore, most new patients diagnosed with MS within the next 10 to 15 years will be from the millennial generation, representing those born between 1982 and 2000. Certain preferences and trends of this contemporary generation will present new challenges to the MS physician and effective MS care. By first understanding these challenges, relevant and successful solutions can be created to craft a system of care that best benefits the millennial patient with MS...
February 2018: Neurologic Clinics
https://www.readbyqxmd.com/read/29157400/advanced-symptom-management-in-multiple-sclerosis
#19
REVIEW
Elizabeth Crabtree-Hartman
Meaningful symptom management can have a profound impact on quality of life. It can challenge time parameters during clinic, and therefore thought should be given to strategies that can improve efficiency and thereby make the undertaking more tenable. For any given symptom, the building blocks of care, such as vitamin D status, exercise/physical therapy, nutrition, and stress management, and ensuring disease-modifying therapy coverage, should be maximized. For each symptom, there are pharmacologic and nonpharmacologic interventions...
February 2018: Neurologic Clinics
https://www.readbyqxmd.com/read/29157399/the-dynamics-of-the-gut-microbiome-in-multiple-sclerosis-in-relation-to-disease
#20
REVIEW
Ellen M Mowry, Justin D Glenn
Multiple sclerosis (MS) is a neuroinflammatory autoimmune disease of unknown etiology, although genetic components and environmental triggers are thought to collude to commence pathogenesis. Numerous investigations are now demonstrating the role of the gut microbiota in neuroinflammation and how alterations in its content may be associated with MS disease. This article explores the studies using MS rodent models to determine the roles of gut bacteria in neuroinflammatory disease, evaluate the evidence linking gut bacterial dysbiosis and MS, and give insight into potential MS therapies targeting the gut microbiota currently under investigation...
February 2018: Neurologic Clinics
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