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Spehl Timo S TS Department of Nuclear Medicine, Freiburg, - - 2014
Posterior cortical atrophy (PCA) is a rare neurodegenerative syndrome with visuospatial deficits. PET studies have identified hypometabolism of the occipital cortex in PCA. There is, however, a huge overlap in clinical presentation and involvement of the occipital cortex between PCA, dementia with Lewy bodies (DLB), and Alzheimer's disease (AD). Syndrome-specific ...
Lee Dong-Won DW From Hallym University Sacred Heart Hospital (D.-W.L., S.-W.E., H.-I.M., Y.J.K.), Hallym University College of Medicine, Anyang; and Sangmoo Hospital (C.O.M.), Gwangju, - - 2014
Antiphospholipid syndrome (APS) is a rare autoimmune coagulopathy with various systemic and neurologic symptoms. Ischemic stroke and TIA are common neurologic manifestations of APS, while migraine, epilepsy, chorea, and multiple sclerosis-like syndromes have also been reported.(1,2) Dementia is an unusual manifestation of APS, and the presentation of APS with corticobasal ...
Graff-Radford Jonathan - - 2014
Studying neurodegenerative diseases has greatly expanded our knowledge of language, speech and emotion as these diseases can affect areas not typically seen with stroke or tumor. Newer imaging techniques such as voxel based morphometry), fluorodeoxyglucose (F18) positron emission tomography and functional magnetic resonance imaging have allowed localization of these deficits ...
Rohan Zdenek Z From the Department of Pathology and Molecular Medicine, Thomayer Hospital, and the Department of Pathology, Third Faculty of Medicine, Charles University, Prague, Czech - - 2014
Frontotemporal lobar degenerations are clinically, genetically, and molecularly heterogeneous diseases characterized by mainly frontal and temporal atrophy and affecting behavioral, language, cognitive, and motor functions. The term frontotemporal dementia incorporates 3 distinct clinical syndromes seen in frontotemporal degenerations: behavioral variant of frontotemporal dementia, progressive nonfluent aphasia, and semantic dementia. Progressive ...
Tanaka Kanta K Department of Neurology, Tenri Hospital, - - 2014
A 67-year-old woman developed dropped head. Her neck was severely flexed, with prominent cervical paraspinal muscles, although no parkinsonism was observed. Brain MRI showed no significant findings. We considered dystonia as the cause of the dropped head and administered trihexyphenidyl, an anticholinergic. After 10 years of follow-up, remarkable psychotic symptoms, ...
Rusina Robert R *Department of Neurology, Thomayer Hospital and Institute for Postgraduate Education in Medicine, Prague, Czech Republic †Department of Neurology and Centre of Clinical Neuroscience, First Faculty of Medicine, Charles University in Prague, and General University Hospital in Prague, Czech Republic ‡Neurocenter Caregroup Rychnov nad Kněžnou, Czech Republic §Department of Neurology, Faculty of Medicine in Hradec Králové, Charles University in Prague, Czech Republic ∥Department of Pathology and Molecular Medicine, Thomayer Hospital, Prague, Czech - - 2013
We describe a patient with corticobasal syndrome in whom neuropathological examination on autopsy revealed Pick and Alzheimer diseases in comorbidity. Corticobasal degeneration is a tauopathy usually associated with asymmetric parkinsonism, parietal lobe involvement, and cognitive impairment. Corticobasal syndrome is the clinical presentation of corticobasal degeneration without neuropathological confirmation. A 66-year-old ...
Cipriani Gabriele - - 2013
The delusional misidentification syndromes (DMSs) are psychopathologic phenomena in which a patient consistently misidentifies persons, places, objects, or events. Although often described in relation to psychotic states including schzofrenia, it is, nevertheless, widely considered that these syndromes have an anatomical basis because of their frequent association with organic brain disease; ...
Clerc Marie-Therese - - 2013
A 57-year-old male with no family history was diagnosed with semantic dementia. He also showed some unusual cognitive features such as episodic memory and executive dysfunctions, spatial disorientation, and dyscalculia. Rapidly progressive cognitive and physical decline occurred. About 1.5 years later, he developed clinical features of a corticobasal syndrome. He ...
Belliard S - - 2013
Semantic dementia is a lobar atrophy syndrome, related to a degeneration of anterior temporal regions, and characterized by a very predominant impairment of semantic memory. Whereas the diagnosis is relatively easy to establish in the typical form and if the patient is seen early, the emergence of possible additional cognitive ...
Armstrong Nicole - - 2013
Background and Aims: Caregiver stress is often a serious problem when caring for a patient with frontal lobe dysfunction. Methods: A total of 102 caregivers of both patients with frontotemporal degeneration and corticobasal syndrome completed the Frontal Systems Behavior Scale (FrSBe) and the Zarit Burden Interview (ZBI). To analyze the ...
Fernández-Matarrubia M - - 2013
INTRODUCTION: Behavioural variant frontotemporal dementia (bvFTD) is the most frequent presentation in the clinical spectrum of frontotemporal dementia (FTD) and it is characterised by progressive changes in personality and conduct. Major breakthroughs in molecular biology and genetics made during the last two decades have lent us a better understanding of ...
Alcantara Marion - - 2013
Among paraneoplastic syndromes (PNS) associated with malignant hemopathies, there are few reports of PNS of the central nervous system and most of them are associated with lymphomas. Limbic encephalitis is a rare neurological syndrome classically diagnosed in the context of PNS. We report the case of a 81-year-old man who ...
Jones Emma L - - 2013
It is known that individuals with Down syndrome develop Alzheimer's disease with an early age at onset, although associated genetic risk factors have not been widely studied. We tested whether genes that increase the risk of late-onset Alzheimer's disease influence the age at onset in Down syndrome using genome-wide association ...
Graff-Radford Jonathan - - 2013
Alien limb phenomenon refers to involuntary motor activity of a limb in conjunction with the feeling of estrangement from that limb. Alien limb serves as a diagnostic feature of corticobasal syndrome. Our objective was to determine the differential diagnoses of alien limb and to determine the features in a large ...
Shinagawa Shunichiro - - 2013
Abstract Frontotemporal lobar degeneration (FTLD) is a heterogeneous syndrome characterized by a progressive decline in behaviour or language associated with degeneration of the frontal and anterior temporal lobes. FTLD can be classified into three clinical syndromes based on the early and predominant symptoms: behavioural variant frontotemporal dementia (bvFTD); semantic dementia ...
Rascovsky Katya K Department of Neurology, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania 19104, USA. - - 2013
Frontotemporal lobar degeneration (FTLD) can manifest as a spectrum of clinical syndromes, ranging from behavioural impairment to language or motor dysfunction. Recently, revised diagnostic criteria have been proposed for the behavioural and progressive aphasia syndromes associated with frontotemporal degeneration. The present review will summarize these diagnostic guidelines and highlight some ...
Crutch Sebastian J - - 2013
Posterior cortical atrophy (PCA) is a syndrome defined by focal neurodegeneration of the parietal, occipital, and occipito-temporal cortices and associated with progressive dysfunction of visual processing, praxis, numeracy and reading. The condition is most commonly caused by (and viewed as an atypical presentation of) Alzheimer's disease, although can also be ...
Lazzarino De Lorenzo Lucio G - - 2013
Bilateral infarcts of the posterior cerebral arteries are associated with a range of visual and memory deficits. In 1902, Dide and Botcazo presented a clinico-pathological case study linking visual field defects, topographical disorientation, retro-anterograde amnesia and alexia with bilateral medial occipito-temporal lesions. Based on the findings they suggested the occipital ...
Brodtmann Amy - - 2013
Paradigm shifts are occurring across the realm of amyotrophic lateral sclerosis (ALS). In the past, we instructed medical students that ALS was solely a clinical diagnosis; that careful clinical examination raised the specter of this devastating condition, and neurophysiologic testing provided confirmation. We taught that ALS was an isolated clinical ...
Fekete Robert - - 2013
Catatonia, originally described by Karl Kahlbaum in 1874, may be regarded as a set of clinical features found in a subtype of schizophrenia, but the syndrome may also stem from organic causes including vascular parkinsonism, brain masses, globus pallidus lesions, metabolic derangements, and pharmacologic agents, especially first generation antipsychotics. Catatonia ...
Chen Tien-Yu - - 2013
Gerstmann's syndrome encompasses the tetrad of finger agnosia, agraphia, acalculia, and right-left confusion. An elderly man with a history of several cardiovascular diseases was initially brought to the psychiatric outpatient department by his family because of worsening of recent memory, executive function, and mixed anxious-depressive mood. Gerstmann's syndrome without obvious ...
Larsen Timothy R - - 2013
Introduction. Wernicke's encephalopathy is a well-described syndrome characterized by the classic triad of confusion, ataxia, and ophthalmoplegia. Wernicke's encephalopathy results from thiamine (vitamin B1) deficiency. Common causes include alcoholism and gastric disorders. Wernicke's has been described in patients with acquired immune deficiency syndrome (AIDS); however, given these patients' immunosuppressed state, ...
Lovan Alyson - - 2013
A woman in her early 60s presented to our Movement Disorders Centre with a 5-year history of progressive peripheral neuropathy, gait instability with falls, blurred vision, cognitive impairment and tremors. The patient was found to have profound sensory ataxia, chronic ophthalmoplegia, dementia with significant deficits in registration and construction and ...
Nozaki Taiki - - 2013
Use of the International Society for the Study of Vascular Anomalies (ISSVA) classification system has been strongly recommended in recent years because of the need for separate therapeutic measures for patients with vascular tumors and malformations. In the ISSVA classification system, vascular tumors, which are neoplastic, are distinguished from vascular ...
Solfrizzi Vincenzo - - 2012
BACKGROUND: Frailty is a clinical syndrome generally associated with a greater risk for adverse outcomes such as falls, disability, institutionalization, and death. Cognition and dementia have already been considered as components of frailty, but the role of frailty as a possible determinant of dementia, Alzheimer's disease (AD), and vascular dementia ...
Silverstein Jeffrey H - - 2012
A number of serious clinical cognitive syndromes occur following surgery and anesthesia. Postoperative delirium is a behavioral syndrome that occurs in the perioperative period. It is diagnosed through observation and characterized by a fluctuating loss of orientation and confusion. A distinct syndrome that requires formalized neurocognitive testing is frequently referred ...
Thaipisuttikul Papan P Comprehensive Center on Brain Aging, Alzheimer Disease Center, New York University Langone Medical Center, New York, NY, - - 2013
Capgras syndrome is characterized by the recurrent, transient belief that a person has been replaced by an identical imposter. We reviewed clinical characteristics of Dementia with Lewy Bodies (DLB) patients with Capgras syndrome compared to those without Capgras. We identified 55 consecutive DLB patients (11 cases with Capgras syndrome (DLB-C) ...
Cipriani Gabriele - - 2012
Diogenes syndrome (DS) is a behavioral disorder of the elderly. Symptoms include living in extreme squalor, a neglected physical state, and unhygienic conditions. This is accompanied by a self-imposed isolation, the refusal of external help, and a tendency to accumulate unusual objects. To explore the phenomenon of DS in dementia ...
Khouloud Bouslama - - 2012
Pellagra is a nutritional disease caused by the deficiency of niacin. It is a clinical syndrome characterized by four "D's": diarrhea, dermatitis, dementia and ultimately death. We describe a case of pellagra as the initial presentation of congenital duodenal diaphragm.
Truzzi Annibal - - 2012
ABSTRACT Background: Neuropsychiatric symptoms (NPS) affect the majority of patients who have dementia. Neuropsychiatric sub-syndromes with symptoms that occur together and have common neurobiological correlates have been identified. There are scarce data regarding the comparison of the pattern of the neuropsychiatric sub-syndromes in distinct ethnical and cultural populations. We aim ...
Chen Ruoling - - 2013
Environmental tobacco smoke (ETS) has a range of adverse health effects, but its association with dementia remains unclear and with dementia syndromes unknown. We examined the dose-response relationship between ETS exposure and dementia syndromes. Using a standard method of GMS, we interviewed 5921 people aged ≥60 years in five provinces ...
Dejesus-Hernandez Mariely M Department of Neuroscience, Mayo Clinic Jacksonville, 4500 San Pablo Road, Jacksonville, FL 32224, - - 2013
The hexanucleotide expanded repeat (GGGGCC) in intron 1 of the C9orf72 gene is recognized as the most common genetic form of amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). However, as part of the clinical phenotype, some patients present with parkinsonism. The present study investigated the potential expansion or association ...
Strandberg Timo E - - 2012
The term geriatric syndrome is used to characterize multifactorial clinical conditions among older people which are not subsumed readily into disease entities, but which nevertheless predispose older people to disability and death. Commonly included are frailty, dementia, delirium, incontinence, falls, and dizziness. Geriatric syndromes are common among older people: in ...
Cipriani Gabriele - - 2012
Jealousy is a complex emotion that most people have experienced at some time in life; pathological jealousy refers primarily to an irrational state. Othello syndrome is a psychotic disorder characterized by delusion of infidelity or jealousy; it often occurs in the context of medical, psychiatric or neurological disorders. At least ...
Verghese Joe J Division of Cognitive & Motor Aging, Saul R Korey Department of Neurology, Albert Einstein College of Medicine, 1165 Morris Park Avenue, Rousso 301, Bronx, NY 10461, USA. - - 2013
Despite growing evidence of links between gait and cognition in aging, cognitive risk assessments that incorporate motoric signs have not been examined. We sought to validate a new Motoric Cognitive Risk (MCR) syndrome to identify individuals at high risk of developing dementia. We evaluated 997 community residing individuals aged 70 ...
McGinnis Scott M - - 2012
Neurodegenerative dementias are characterized by insidious onset and gradual progression of cognitive dysfunction, initially relatively focal with respect to cognitive domains and brain regions involved. Neuroimaging techniques have contributed enormously to both our understanding of large-scale network specificity in neurodegenerative syndromes and our ability to make clinical diagnoses of syndromes ...
Ness Seth - - 2012
A public-private partnership to establish biomarkers of dementia in Down's syndrome could aid the development of preventive therapies for the dementia associated with both Down's syndrome and Alzheimer's disease, based on the apparent common pathogenic role of amyloid precursor protein in the two conditions.
Conduit Sarah E - - 2012
Phosphoinositides regulate numerous cellular events via the recruitment and activation of multiple lipid-binding effector proteins. The precise temporal and spatial regulation of phosphoinositide signals by the co-ordinated activities of phosphoinositide kinases and phosphatases is essential for homeostasis and development. Mutations in two inositol polyphosphate 5-phosphatases, INPP5E and OCRL, cause the ...
Chan Lap Kei - - 2012
Meige syndrome is a rare form of segmental dystonia characterized by blepharospasm and oromandibular dystonia. A few case reports of Meige syndrome have been associated with Lewy body pathologies, and the syndrome was also proposed for inclusion in the spectrum of Lewy body disease. We report a case of an ...
Bolar Nikhita - - 2012
PURPOSE OF REVIEW: Although historically Marfan syndrome (MFS) has always been considered as a condition caused by the deficiency of a structural extracellular matrix protein, fibrillin-1, the study of Marfan mouse models and Marfan-related conditions has shifted our current understanding to a pathogenic model that involves dysregulation of the cytokine-transforming ...
Al-Qattan Mohammad M - - 2012
Protein regulation is the function of several pathways and enzyme systems in the human body. One of these pathways is the ubiquitin/SUMO pathway. The author has noted that almost all known syndromes of radial ray deficiency are related to this pathway. In this article, these syndromes are reviewed with special ...
Klüppel Michael - - 2012
Costello syndrome is a pediatric genetic disorder linked to oncogenic germline mutations in the HRAS gene. The disease is characterized by multiple developmental abnormalities, as well as predisposition to malignancies. Our recent observation that heart tissue from patients with Costello syndrome showed a loss of the glycosaminoglycan chondroitin-4-sulfate (C4S) inspired ...
Lee Wen-Sen - - 2011
A woman aged 56 years of age had a community-acquired left neck abscess and internal jugular vein thrombosis with septicemia due to extended-spectrum β-lactamase (ESBL)-producing Klebsiella pneumoniae. Even though she was treated with intravenous meropenem, the bacteremia persisted. She was complicated with multiple brain abscesses, seizure, and leucopenia. After a ...
Morgan Robert David - - 2011
A 5-year-old female with Papillon-Lefèvre syndrome presented with renal mass. A radiological diagnosis of malignancy was made; however, partial nephrectomy revealed granulomatous disease indicative of chronic infection. Although liver abscess is an emerging complication in patient with Papillon-Lefèvre syndrome, this case represents the first renal abscess described in such patients.
Hammad Eman A - - 2011
The prevalence of metabolic syndrome is increasing worldwide, and patients with metabolic syndrome have increased risk of developing cardiovascular disease and type 2 diabetes. Although specific criteria vary, the National Cholesterol Education Program Adult Treatment Panel III (NCEP/ATP III) criteria (2002) defined metabolic syndrome as the presence of 3 or ...
Agarwal Rishi - - 2011
POEMS Syndrome is a rare cause of demyelinating and axonal mixed neuropathy. Plasmacytomas are usually seen in POEMS syndrome and can be osseous or extramedullary. Plasmacytomas presenting as an abscess has not been noted earlier. Our patient presented with localized hyperpigmented patch on the back and later developed progressive weakness ...
Vila Andrea - - 2011
Klebsiella pneumoniae liver abscess syndrome (KLAS) is an emerging invasive infection caused by highly virulent community-acquired strains of K. pneumoniae displaying hypermucoviscosity. The salient features of this syndrome include the presence of bacteremia, primary monomicrobial liver abscess, and metastatic complications. A previously healthy Argentinean man presented with fever and found ...
Ramdial Pratistadevi K - - 2010
The clinicopathologic features of 4 AIDS patients with cutaneous colesional Kaposi sarcoma (KS) and cryptococcosis, a rare phenomenon, are described. Biopsies from 3 patients who were highly active antiretroviral therapy (HAART)-naive demonstrated predominant KS with a conspicuous spindle cell component and small aggregates of cryptococcal yeasts in 2 biopsies and ...
Browne Sarah K - - 2010
Anticytokine autoantibodies are an important and emerging mechanism of disease pathogenesis. We will review the clinical and laboratory features of syndromes in which immunodeficiency is caused by or associated with neutralizing anticytokine autoantibodies. A growing number of patients have been described who demonstrate unique infectious phenotypes associated with neutralizing autoantibodies ...
Feng Bin - - 2011
Hypersensitivity in inflammatory/irritable bowel syndrome is contributed to in part by changes in the receptive properties of colorectal afferent endings, likely including mechanically insensitive afferents (MIAs; silent afferents) that have the ability to acquire mechanosensitivity. The proportion and attributes of colorectal MIAs, however, have not previously been characterized. The distal ...
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