Abstract:
:Myotonic dystrophies and channelopathies are rare but important causes of muscle diseases which may present with myotonia, episodic attacks of weakness, fixed muscle weakness, and atrophy or their combination. Here, the authors provide an overview of these disorders and describe their clinical and pathophysiological features, diagnostic methods, and management.
journal_name
Semin Neuroljournal_title
Seminars in neurologyauthors
Quinn C,Salajegheh MKdoi
10.1055/s-0035-1558979subject
Has Abstractpub_date
2015-08-01 00:00:00pages
360-8issue
4eissn
0271-8235issn
1098-9021journal_volume
35pub_type
杂志文章,评审abstract::Natalizumab is a monoclonal therapeutic antibody that inhibits migration of lymphocytes into the central nervous system (CNS) by blocking integrins. Several clinical trials have shown an excellent efficacy in the treatment of relapsing remitting multiple sclerosis. This efficacy is also underlined by postmarketing dat...
journal_title:Seminars in neurology
pub_type: 杂志文章,评审
doi:10.1055/s-0033-1343793
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abstract::I argue that there is no mind-brain problem but rather that there are irreducible subjective-objective problems. These include the difference between "inside" and "outside" perspectives on neural states, the creation of subjective neural states with objectified outside objects, and awareness of the self as an object i...
journal_title:Seminars in neurology
pub_type: 杂志文章,评审
doi:10.1055/s-2008-1040917
更新日期:1997-06-01 00:00:00
abstract::Disorders of gait and balance are common in medicine and often lead to referral for neurologic evaluation. Because the maintenance of balance and normal gait are mediated by complex neurologic pathways as well as musculoskeletal, metabolic, and behavioral considerations, the list of possible contributing causes is ver...
journal_title:Seminars in neurology
pub_type: 杂志文章,评审
doi:10.1055/s-0033-1354601
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abstract::Evaluation of patients with cranial neuropathies requires an understanding of brainstem anatomy and nerve pathways. Advances in neuroimaging, particularly high spatial resolution magnetic resonance imaging (MRI), have enabled visualization of these tiny structures and their related pathology. This review provides an a...
journal_title:Seminars in neurology
pub_type: 杂志文章,评审
doi:10.1055/s-0032-1331807
更新日期:2012-09-01 00:00:00
abstract::Neurothermography is a new, clinically useful method of detecting autonomic sympathetic peripheral nerve dysfunction. Body symmetry of the skin temperature as maintained by symmetrically functional peripheral nerves is the key to detecting a normal state. Persistent 1 degree C temperature asymmetry in the pattern of a...
journal_title:Seminars in neurology
pub_type: 杂志文章,评审
doi:10.1055/s-2008-1041432
更新日期:1987-12-01 00:00:00
abstract::Improved disease control is critical for enhancing the lives of those living with multiple sclerosis. With specific immunologic targets, monoclonal antibody (mAb) treatments are highly effective options for relapsing forms of multiple sclerosis. The mechanism, efficacy, and current safety profiles are detailed for the...
journal_title:Seminars in neurology
pub_type: 杂志文章,评审
doi:10.1055/s-0036-1579695
更新日期:2016-04-01 00:00:00
abstract::Leptomeningeal metastasis occurs in ~5% of all patients with cancer and is the third most common metastatic complication of the central nervous system. Staging of leptomeningeal metastasis includes contrast-enhanced brain and spine magnetic resonance imaging and radionuclide cerebrospinal fluid (CSF) flow study. Treat...
journal_title:Seminars in neurology
pub_type: 杂志文章
doi:10.1055/s-0030-1255220
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abstract::Epilepsy is one of the most common neurological conditions encountered worldwide. The development of newer antiepileptic drugs (AEDs) has expanded over recent years, and the use of such drugs for indications other than epilepsy has also broadened. These factors insure that the majority of health care providers will be...
journal_title:Seminars in neurology
pub_type: 杂志文章,评审
doi:10.1055/s-2002-36645
更新日期:2002-09-01 00:00:00
abstract::With age, the presence of multiple neuropathologies in a single individual becomes increasingly common. Given that traumatic brain injury and the repetitive head impacts (RHIs) that occur in contact sports have been associated with the development of many neurodegenerative diseases, including chronic traumatic encepha...
journal_title:Seminars in neurology
pub_type: 杂志文章
doi:10.1055/s-0040-1713628
更新日期:2020-08-01 00:00:00
abstract::Status epilepticus is a neurological emergency with an outcome that is highly associated with the initial pharmacotherapy management that must be administered in a timely fashion. Beyond first-line therapy of status epilepticus, treatment is not guided by robust evidence. Optimal pharmacotherapy selection for individu...
journal_title:Seminars in neurology
pub_type: 杂志文章
doi:10.1055/s-0040-1718721
更新日期:2020-12-01 00:00:00
abstract::Sergei Sergeevich Prokofiev (1891-1953) was one of the great Russian composers. He died, at age 61, of a supposed intracerebral brain hemorrhage. During the 8 years before his death, he suffered from episodic headaches, nausea and dizziness. This article reviews the historical information about Prokofiev's illness lea...
journal_title:Seminars in neurology
pub_type: 传,历史文章,杂志文章
doi:
更新日期:1999-01-01 00:00:00
abstract::Chronic insomnia afflicts millions of people in the United States. It is associated with decreased quality of life, accidents, several comorbid conditions, increased morbidity, and substantial direct and indirect costs. Chronic insomnia is a multifactorial and individualized disorder. Its etiology may be cognitive or ...
journal_title:Seminars in neurology
pub_type: 杂志文章
doi:10.1055/s-0029-1237125
更新日期:2009-09-01 00:00:00
abstract::Patients who suffer from neuromuscular diseases often have complications from respiratory insufficiencies. Some neuromuscular diseases, for example Landry Guillain-Barré syndrome, may only require temporary tracheal intubation; patients with other neuromuscular diseases, such as amyotrophic lateral sclerosis, may deci...
journal_title:Seminars in neurology
pub_type: 杂志文章,评审
doi:10.1055/s-2003-40756
更新日期:2003-03-01 00:00:00
abstract::Neuroimaging is commonly utilized in the evaluation of post-cardiac arrest patients, providing a unique ability to visualize and quantify structural brain injury that can complement clinical and electrophysiologic data. Despite its lack of validation, we would advocate that neuroimaging is a valuable prognostication t...
journal_title:Seminars in neurology
pub_type: 杂志文章,评审
doi:10.1055/s-0036-1594253
更新日期:2017-02-01 00:00:00
abstract::Although there are several animal models of Lyme disease, only the rhesus monkey model exhibits all of the key manifestations of the disease. After infection with Borrelia burgdorferi, rhesus monkeys develop signs of early localized, early disseminated, and chronic Lyme disease. Specific features include erythema migr...
journal_title:Seminars in neurology
pub_type: 杂志文章,评审
doi:10.1055/s-2008-1040913
更新日期:1997-03-01 00:00:00
abstract::Obstructive sleep apnea (OSA) is a very common condition in patients with stroke and is found in over half of stroke patients. There is a complex relationship between OSA and stroke, attributable to shared risk factors. There are numerous mechanisms by which OSA may contribute to increased stroke risk, including promo...
journal_title:Seminars in neurology
pub_type: 杂志文章,评审
doi:10.1055/s-2006-933315
更新日期:2006-02-01 00:00:00
abstract::The management of severe brain injury requires a comprehensive approach in which imaging is an indispensable complement to the clinical and physiological information acquired at the bedside. Neuroimaging methods are routinely used in the diagnosis and prognosis of a broad spectrum of patients with acute neurological d...
journal_title:Seminars in neurology
pub_type: 杂志文章,评审
doi:10.1055/s-0028-1105977
更新日期:2008-11-01 00:00:00
abstract::Cognitive complaints are common in any adult neurology practice, and may present in a variety of ways other than classic "memory loss." In this article, the author reviews a simple approach to a symptom-based differential diagnosis, including recognition of impairment in several key cognitive domains as well as associ...
journal_title:Seminars in neurology
pub_type: 杂志文章,评审
doi:10.1055/s-0031-1271314
更新日期:2011-02-01 00:00:00
abstract::Pregnancy and the postpartum period confer an increased risk of stroke. The risk appears to be increased for all strokes during the puerperium and for hemorrhagic strokes during pregnancy. During pregnancy, physiologic systems are under increased stress to accommodate the growing fetus, which results in substantial he...
journal_title:Seminars in neurology
pub_type: 杂志文章,评审
doi:10.1055/s-0037-1608940
更新日期:2017-12-01 00:00:00
abstract::Insomnia is the most common sleep complaint. Insomnia is not a disease itself but mostly a clinical sign of an underlying disease. Degenerative and vascular diseases involving the central nervous system (CNS) may impair sleep either as a result of the brain lesion or because of illness-related discomfort (motor immobi...
journal_title:Seminars in neurology
pub_type: 杂志文章,评审
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abstract::The field of neurocritical care has evolved tremendously in recent years, a development explained vastly by the advent of neurophysiological monitoring. From basic intracranial pressure measurements to brain tissue oxygenation, microdialysis, cerebral blood flow (CBF), and surface and intracortical electroencephalogra...
journal_title:Seminars in neurology
pub_type: 杂志文章,评审
doi:10.1055/s-0036-1592171
更新日期:2016-12-01 00:00:00
abstract::Fungal central nervous system infections present in a myriad of clinical manifestation and remain a significant diagnostic challenge. Presenting symptoms, cerebrospinal fluid analysis, conventional fungal serum markers and imaging correlates of disease are seldom sensitive or specific; fungal culture growth or histopa...
journal_title:Seminars in neurology
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abstract::Vaccines have undoubtedly been a medical milestone, preventing immeasurable morbidity and mortality from infectious diseases worldwide. Modern vaccines have tremendously reduced the global impact of numerous infections; they have succeeded in eliminating smallpox completely. However, the nature by which vaccines confe...
journal_title:Seminars in neurology
pub_type: 杂志文章,评审
doi:10.1055/s-0031-1287655
更新日期:2011-07-01 00:00:00
abstract::Neuropathic pain management is challenging, and typically requires a multifaceted approach. There are several treatment options, both pharmacologic and nonpharmacologic. Of the available pharmacologic agents, those with the strongest supporting evidence include tricyclic antidepressants, serotonin-norepinephrine reupt...
journal_title:Seminars in neurology
pub_type: 杂志文章,评审
doi:10.1055/s-0038-1673679
更新日期:2018-12-01 00:00:00
abstract::Synucleinopathies, with and without dementia, encompass a wide range of diseases including Parkinson's disease, multiple system atrophy, rapid eye movement (REM) sleep behavior disorder, and dementia with Lewy bodies (DLB). DLB is a neurodegenerative disorder resulting in slowly progressive and unrelenting dementia un...
journal_title:Seminars in neurology
pub_type: 杂志文章,评审
doi:10.1055/s-2006-956754
更新日期:2007-02-01 00:00:00
abstract::Epidemiological and individual case studies indicate that genetic factors play a significant role in the genesis of Alzheimer Disease (AD). To date, molecular genetic studies in families multiply affected with AD have identified three genes (Presenilin 1-PS1, Presenilin 2-PS2, and beta-amyloid precursor protein--betaA...
journal_title:Seminars in neurology
pub_type: 杂志文章,评审
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abstract::Hypoxic-ischemic encephalopathy (HIE) is a manifestation of perinatal asphyxial insult that continues to evolve over days to weeks following the initial injury. Therapeutic hypothermia has demonstrated that a proportion of this secondary brain injury may indeed be preventable. However, therapeutic hypothermia has also...
journal_title:Seminars in neurology
pub_type: 杂志文章
doi:10.1055/s-0040-1702939
更新日期:2020-06-01 00:00:00
abstract::Stroke in the distribution of the posterior circulation may present as acute onset spontaneous vertigo and imbalance. Although vertigo due to posterior circulation stroke is usually associated with other neurologic symptoms or signs, small infarcts in the cerebellum or brainstem can present with vertigo without other ...
journal_title:Seminars in neurology
pub_type: 杂志文章,评审
doi:10.1055/s-0033-1354600
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abstract::As the population ages, the prevalence of many neurologic diseases is increasing. At the same time, older patients are undergoing more surgical procedures. This confluence of events puts neurohospitalists in a unique position to provide both pre- and postoperative guidance to minimize complications, improve clinical o...
journal_title:Seminars in neurology
pub_type: 杂志文章,评审
doi:10.1055/s-0035-1564302
更新日期:2015-12-01 00:00:00
abstract::Chronic traumatic encephalopathy (CTE) is a neurodegenerative disease associated with repetitive head impacts (RHI), such as those received in contact/collision sports, blast injury in military veterans, and domestic violence. Currently, CTE can only be diagnosed following death. Although the clinical features of form...
journal_title:Seminars in neurology
pub_type: 杂志文章
doi:10.1055/s-0040-1713624
更新日期:2020-08-01 00:00:00