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Seminars in Neurology

Sarah F Wesley, Damien Ferguson
Rapidly progressive dementia (RPD) or cognitive decline is a common presenting complaint in neurology. While primary dementia is often a concern, other forms of reversible dementia must be thoroughly considered. This article focuses on the growing field of autoimmune encephalitis (AE) as it pertains to the differential diagnostic considerations in a work-up for RPD. Understanding clues in the history and examination is the first step in identifying patients with a potential autoimmune cause for RPD. While testing for infectious and toxic-metabolic etiologies is commonly preformed, it is necessary to consider early ancillary testing for AE in appropriate cases of RPD...
April 2019: Seminars in Neurology
Mine Sezgin, Basar Bilgic, Sule Tinaz, Murat Emre
Dementia with Lewy bodies (DLB) and Parkinson's disease dementia (PD-D) are Lewy body-related neurodegenerative disorders sharing common clinical and neuropathological findings. The clinical features of both conditions include cognitive impairment, behavioral symptoms, autonomic dysfunction, sleep disorders, and parkinsonism. The cognitive profile of both disorders is characterized by particularly severe deficits in executive and visuospatial functions as well as attention. Clinical differentiation between DLB and PD-D is based on an arbitrary distinction between the time of onset of parkinsonism and cognitive symptoms; extrapyramidal symptoms precede dementia in PD-D, whereas it coincides with or follows dementia within 1 year in DLB...
April 2019: Seminars in Neurology
Farwa Ali, Keith Josephs
Tauopathies are rare neurodegenerative disorders related to microtubule-associated protein tau, which functions to stabilize microtubules. Pathological changes caused by overexpression or hyperphosphorylation of tau lead to the disengagement of tau from microtubules and accumulation of toxic intracellular inclusions. Tau pathology is the underlying mechanism for several sporadic and genetic disorders. These are collectively known as tauopathies. Each tauopathy is differentiated from others by its neuropathological features such as the presence of specific isoforms of tau, type of cellular inclusions, and the regions of the brain affected...
April 2019: Seminars in Neurology
Harri Sivasathiaseelan, Charles R Marshall, Jennifer L Agustus, Elia Benhamou, Rebecca L Bond, Janneke E P van Leeuwen, Chris J D Hardy, Jonathan D Rohrer, Jason D Warren
Frontotemporal dementias are a clinically, neuroanatomically, and pathologically diverse group of diseases that collectively constitute an important cause of young-onset dementia. Clinically, frontotemporal dementias characteristically strike capacities that define us as individuals, presenting broadly as disorders of social behavior or language. Neurobiologically, these diseases can be regarded as "molecular nexopathies," a paradigm for selective targeting and destruction of brain networks by pathogenic proteins...
April 2019: Seminars in Neurology
Matt B Paradise, Perminder S Sachdev
The term vascular cognitive disorder (VCD) refers to a heterogeneous group of disorders in which the primary feature is cognitive impairment attributable to cerebrovascular disease (CVD). This includes not only vascular dementia (VaD) but also cognitive impairment of insufficient severity to meet diagnostic criteria for dementia. VCD is recognized as the second most common cause of dementia after Alzheimer's disease (AD), but prevalence rates vary widely according to the diagnostic criteria employed. There have been recent attempts to standardize diagnostic criteria...
April 2019: Seminars in Neurology
Alireza Atri
The foundation of current Alzheimer's disease (AD) treatment involves pharmacological and nonpharmacological management and care planning predicated on patient-centered psychoeducation, shared goal-setting, and decision-making forged by a strong triadic relationship between clinician and the patient-caregiver dyad. Food and Drug Administration (FDA) approved AD medications, cholinesterase-inhibitors (ChEIs), and the N-methyl-d-aspartate (NMDA) antagonist memantine, when utilized as part of a comprehensive care plan, while generally considered symptomatic medications, can provide modest "disease course-modifying" effects by enhancing cognition, and reducing loss of independence...
April 2019: Seminars in Neurology
Nicolas Villain, Bruno Dubois
Alzheimer's disease (AD) is the commonest neurodegenerative disease and the most frequent cause of dementia. It affects 30 million people worldwide. Current research criteria focus on biomarkers' status for amyloid and tau using positron emission tomography and cerebrospinal fluid analysis, independent of clinical status. Current epidemiological data, which mostly rely on biomarker-undetermined AD cases, have highlighted ApoE4 and age as the main risk factors. Rare autosomal dominant mutations also account for a small fraction of early-onset AD...
April 2019: Seminars in Neurology
Arash Salardini
In the last few years, an improved understanding of dementia biomarkers has significantly increased the diagnostic accuracy for dementias. The National Institutes of Health Biomarkers Definitions Working Group defines a biomarker as "a characteristic that is objectively measured and evaluated as an indicator of normal biological processes, pathogenic processes, or pharmacologic responses to a therapeutic intervention." In the field of dementia, a biomarker is a biological measure pointing to a specific dementing pathology...
April 2019: Seminars in Neurology
Sowmya Mahalingam, Ming-Kai Chen
Dementia is a global health issue, the burden of which will worsen with an increasingly aging population. Alzheimer's disease (AD) is the most common dementia, with 50 to 60% of all dementias attributable to AD alone, while the rest are mostly due to frontotemporal lobar dementia, dementia with Lewy bodies, Parkinson's disease dementia, and vascular dementia. Diagnosis of dementias is made clinically with the aid of other testing modalities including neuroimaging. While the role of imaging has traditionally been to exclude reversible causes of dementia, positron emission tomography (PET) with 18-fluorine fluorodeoxyglucose and magnetic resonance imaging now are increasingly used more for definitive diagnosis of dementia in the prodromal stages and to aid with formulating the differential diagnoses...
April 2019: Seminars in Neurology
Taha Qarni, Arash Salardini
Mild cognitive impairment (MCI) represents an intermediate stage between normal cognition and dementia. Individuals with MCI are at increased risk of conversion to dementia, and the rate of progression of MCI to dementia is dependent on age, gender, and education. MCI may be diagnosed using neuropsychological criteria using cut-offs representing decrements in cognition, or using criteria to assess for a decline in functional status. The ability to determine the status of dementia-related biomarkers has allowed for better staging and prognostication in different forms of MCI...
April 2019: Seminars in Neurology
Annya Tisher, Arash Salardini
Treatment of dementias represents an important but relatively neglected part of neurological care of the elderly population. Individual therapeutic interventions may make only small changes to the quality of life of individuals afflicted with dementia, but when used in combination these interventions synergize and can make a significant difference. Additionally, given the societal scale of the problem of dementia care, the overall impact, in economic and sociological terms, of such therapies is of consequence...
April 2019: Seminars in Neurology
Arash Salardini
Dementia is a state of cognitive dysfunction which leads to functional decline. It is a syndrome caused by several medical and neurological causes, but most cases of dementia are due to "primary dementias." Primary dementias are neurological diseases whose manifestations are predominantly cognitive. Most primary dementias are caused by neurodegenerative proteinopathies where an accumulation of misfolded proteins leads to neuronal loss, neuroinflammation and glial reaction. Each proteinopathy is characterized by the type of protein implicated in its pathophysiology...
April 2019: Seminars in Neurology
Arash Salardini
No abstract text is available yet for this article.
April 2019: Seminars in Neurology
David M Greer
No abstract text is available yet for this article.
April 2019: Seminars in Neurology
Sarah M Schlein, Paul G Marcolini, Evie G Marcolini
The study of wilderness medicine is within the scope of medical care in the austere environment and addresses medicine as practiced in the setting of delayed access to definitive medical care, hostile environment, limited equipment, and inherent risks to the patient and/or rescuers. Part of this topic includes the care of patients with neurologic illness and/or injury.We will address the five most important skills of a wilderness medicine professional: decision making, prevention, preparation, protocol development, and education by applying the principles to select common neurologic problems that occur in the extended environment: traumatic brain injury, dehydration, hyponatremia, heat illness, hypothermia, spine injury, and lightning injury...
February 2019: Seminars in Neurology
Suraj Rajan, Bonnie Kaas, Emile Moukheiber
Many acute and potentially life-threatening medical conditions have hyperkinetic or hypokinetic movement disorders as their hallmark. Here we review the clinical phenomenology, and diagnostic principles of neuroleptic malignant syndrome, malignant catatonia, serotonin syndrome, Parkinsonism hyperpyrexia, acute parkinsonism, acute chorea-ballism, drug-induced dystonia, and status dystonicus. In the absence of definitive lab tests and imaging, only a high index of clinical suspicion, awareness of at-risk populations, and variations in clinical presentation can help with diagnosis...
February 2019: Seminars in Neurology
Christyn Edmundson, Shawn J Bird
Neuromuscular emergencies may be defined as disorders or exacerbation of diseases of the peripheral nervous system that are rapidly progressive and potentially life-threatening. Such disorders can affect any level of the peripheral nervous system, from the muscle to the anterior horn cell. While their clinical manifestations may vary, severe morbidity and mortality is most frequently the result of neuromuscular respiratory failure. Some disorders, such as Guillain-Barré syndrome, provide the additional threat of severe, and potentially irreversible, nerve loss...
February 2019: Seminars in Neurology
Jordan R Anderson, Vihang Nakhate, Christopher D Stephen, David L Perez
Functional neurological disorders (FND) are complex and prevalent neuropsychiatric conditions. Importantly, some patients with FND develop acute onset symptoms requiring emergency department (ED) evaluations. Historically, FND was a "rule-out" diagnosis, making assessment and management in the ED difficult. While the rapid triage of potential neurological emergencies remains the initial task, advancements have altered the approach to FND. FND is now a "rule-in" diagnosis based on validated neurological examination signs and semiological features...
February 2019: Seminars in Neurology
Michael J Bradshaw, Arun Venkatesan
Infection of the central nervous system is often a life-threatening emergency. In many cases, the clinician faces an unknown pathogen and must rely upon clinical acumen and a thorough, systematic diagnostic investigation to establish a diagnosis and initiate appropriate treatment. Because patients typically present with a syndrome, such as temporal lobe encephalitis, rather than a known pathogen (e.g., herpes simplex virus 1 encephalitis), we describe diagnostic considerations in the context of their neuroanatomic tropisms and patterns of disease...
February 2019: Seminars in Neurology
Anna M Bank, Carl W Bazil
Seizure- and epilepsy-related complications are a common cause of emergency medical evaluation, accounting for 5% of 911 calls and 1% of emergency department visits. Emergency physicians and neurologists must be able to recognize and treat seizure- and epilepsy-related emergencies. This review describes the emergency evaluation and management of new onset seizures, breakthrough seizures in patients with known epilepsy, status epilepticus, acute symptomatic seizures, and acute adverse effects of antiepileptic drugs...
February 2019: Seminars in Neurology
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