The Experts below are selected from a list of 1617 Experts worldwide ranked by ideXlab platform
David C Dorman - One of the best experts on this subject based on the ideXlab platform.
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Extrapyramidal System neurotoxicity animal models
Handbook of Clinical Neurology, 2015Co-Authors: David C DormanAbstract:The central nervous System's Extrapyramidal System provides involuntary motor control to the muscles of the head, neck, and limbs. Toxicants that affect the Extrapyramidal System are generally clinically characterized by impaired motor control, which is usually the result of basal ganglionic dysfunction. A variety of Extrapyramidal syndromes are recognized in humans and include Parkinson's disease, secondary parkinsonism, other degenerative diseases of the basal ganglia, and clinical syndromes that result in dystonia, dyskinesia, essential tremor, and other forms of tremor and chorea. This chapter briefly reviews the anatomy of the Extrapyramidal System and discusses several naturally occurring and experimental models that target the mammalian (nonhuman) Extrapyramidal System. Topics discussed include Extrapyramidal syndromes associated with antipsychotic drugs, carbon monoxide, reserpine, cyanide, rotenone, paraquat, 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP), and manganese. In most cases, animals are used as experimental models to improve our understanding of the toxicity and pathogenesis of these agents. Another agent discussed in this chapter, yellowstar thistle poisoning in horses, however, represents an important spontaneous cause of parkinsonism that naturally occurs in animals. The central focus of the chapter is on animal models, especially the concordance between clinical signs, neurochemical changes, and neuropathology between animals and people.
J Buscombe - One of the best experts on this subject based on the ideXlab platform.
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REVIEWARTICLE The Role of Functional Dopamine-Transporter SPECT Imaging in Parkinsonian Syndromes, Part 1
2016Co-Authors: Thomas C Booth, M Nathan, Adam D Waldman, Anthony H V Schapira, -m. A. Quigley, J BuscombeAbstract:SUMMARY: As we defeat infectious diseases and cancer, one of the greatest medical challenges facing us in the mid-21st century will be the increasing prevalence of degenerative disease. Those diseases, which affect movement and cognition, can be the most debilitating. Dysfunction of the Extrapyramidal System results in increasing motor disability often manifest as tremor, bradykinesia, and rigidity. The common pathologic pathway of these diseases, collectively described as parkinsonian syndromes, such as Parkinson disease, multiple System atrophy, progressive supranuclear palsy, corticobasal degeneration, and dementia with Lewy bodies, is degeneration of the presynaptic dopaminergic pathways in the basal ganglia. Conventional MR imaging is insensitive, especially in early disease, so functional imaging has become the primary method used to differentiate a true parkinsonian syndrome from vascular parkinsonism, drug-induced changes, or essential tremor. Unusually for a modern functional imaging technique, the method most widely used in European clinics depends on SPECT and not PET. This SPECT technique (described in the first of 2 parts) commonly reports dopamine-transporter function, with decreasing striatal uptake demonstrating increasingly severe disease. ABBREVIATIONS: DaT dopamine transporters; 18F fluorine 18; 18F-DOPA 6-[18F]fluoro-L-3,4-dihydroxyphenylalanine; 123I iodine 123; 123I-FP-CIT 123I-ioflupane (N--fluoropropyl- CIT); PD idiopathic Parkinson disease; SWEDD scans without evidence of dopaminergic deficit Parkinsonian syndromes are a group of movement disorderscharacterized by tremor, bradykinesia, and rigidity. They are most frequently due to primary neurodegenerative disease, resultin
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the role of functional dopamine transporter spect imaging in parkinsonian syndromes part 1
American Journal of Neuroradiology, 2015Co-Authors: Thomas C Booth, M Nathan, Adam D Waldman, Annmarie Quigley, Anthony H V Schapira, J BuscombeAbstract:SUMMARY: As we defeat infectious diseases and cancer, one of the greatest medical challenges facing us in the mid-21st century will be the increasing prevalence of degenerative disease. Those diseases, which affect movement and cognition, can be the most debilitating. Dysfunction of the Extrapyramidal System results in increasing motor disability often manifest as tremor, bradykinesia, and rigidity. The common pathologic pathway of these diseases, collectively described as parkinsonian syndromes, such as Parkinson disease, multiple System atrophy, progressive supranuclear palsy, corticobasal degeneration, and dementia with Lewy bodies, is degeneration of the presynaptic dopaminergic pathways in the basal ganglia. Conventional MR imaging is insensitive, especially in early disease, so functional imaging has become the primary method used to differentiate a true parkinsonian syndrome from vascular parkinsonism, drug-induced changes, or essential tremor. Unusually for a modern functional imaging technique, the method most widely used in European clinics depends on SPECT and not PET. This SPECT technique (described in the first of 2 parts) commonly reports dopamine-transporter function, with decreasing striatal uptake demonstrating increasingly severe disease.
Thomas C Booth - One of the best experts on this subject based on the ideXlab platform.
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REVIEWARTICLE The Role of Functional Dopamine-Transporter SPECT Imaging in Parkinsonian Syndromes, Part 1
2016Co-Authors: Thomas C Booth, M Nathan, Adam D Waldman, Anthony H V Schapira, -m. A. Quigley, J BuscombeAbstract:SUMMARY: As we defeat infectious diseases and cancer, one of the greatest medical challenges facing us in the mid-21st century will be the increasing prevalence of degenerative disease. Those diseases, which affect movement and cognition, can be the most debilitating. Dysfunction of the Extrapyramidal System results in increasing motor disability often manifest as tremor, bradykinesia, and rigidity. The common pathologic pathway of these diseases, collectively described as parkinsonian syndromes, such as Parkinson disease, multiple System atrophy, progressive supranuclear palsy, corticobasal degeneration, and dementia with Lewy bodies, is degeneration of the presynaptic dopaminergic pathways in the basal ganglia. Conventional MR imaging is insensitive, especially in early disease, so functional imaging has become the primary method used to differentiate a true parkinsonian syndrome from vascular parkinsonism, drug-induced changes, or essential tremor. Unusually for a modern functional imaging technique, the method most widely used in European clinics depends on SPECT and not PET. This SPECT technique (described in the first of 2 parts) commonly reports dopamine-transporter function, with decreasing striatal uptake demonstrating increasingly severe disease. ABBREVIATIONS: DaT dopamine transporters; 18F fluorine 18; 18F-DOPA 6-[18F]fluoro-L-3,4-dihydroxyphenylalanine; 123I iodine 123; 123I-FP-CIT 123I-ioflupane (N--fluoropropyl- CIT); PD idiopathic Parkinson disease; SWEDD scans without evidence of dopaminergic deficit Parkinsonian syndromes are a group of movement disorderscharacterized by tremor, bradykinesia, and rigidity. They are most frequently due to primary neurodegenerative disease, resultin
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the role of functional dopamine transporter spect imaging in parkinsonian syndromes part 1
American Journal of Neuroradiology, 2015Co-Authors: Thomas C Booth, M Nathan, Adam D Waldman, Annmarie Quigley, Anthony H V Schapira, J BuscombeAbstract:SUMMARY: As we defeat infectious diseases and cancer, one of the greatest medical challenges facing us in the mid-21st century will be the increasing prevalence of degenerative disease. Those diseases, which affect movement and cognition, can be the most debilitating. Dysfunction of the Extrapyramidal System results in increasing motor disability often manifest as tremor, bradykinesia, and rigidity. The common pathologic pathway of these diseases, collectively described as parkinsonian syndromes, such as Parkinson disease, multiple System atrophy, progressive supranuclear palsy, corticobasal degeneration, and dementia with Lewy bodies, is degeneration of the presynaptic dopaminergic pathways in the basal ganglia. Conventional MR imaging is insensitive, especially in early disease, so functional imaging has become the primary method used to differentiate a true parkinsonian syndrome from vascular parkinsonism, drug-induced changes, or essential tremor. Unusually for a modern functional imaging technique, the method most widely used in European clinics depends on SPECT and not PET. This SPECT technique (described in the first of 2 parts) commonly reports dopamine-transporter function, with decreasing striatal uptake demonstrating increasingly severe disease.
Jolanta Dorszewska - One of the best experts on this subject based on the ideXlab platform.
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genetics of parkinson s disease and other diseases of the Extrapyramidal System
Current Genomics, 2014Co-Authors: Jolanta DorszewskaAbstract:Advancements in science and technology in the second half of the twentieth century have led to increased life expectancy, which has contributed to the increased number of cases typical for old age diseases, including Parkinson's disease. Estimates suggest that, in the aging population, the number of patients with Parkinson’s disease will maintain an upward trend. Parkinson's disease is a disorder of the Extrapyramidal System. Other diseases causing Extrapyramidal disorders, with the exception of Parkinson’s disease, are called atypical parkinsonism or parkinsonism plus. These diseases include: multiple System atrophy, MSA; progressive supranuclear palsy, PSP; corticobasal degeneration, CBD; dementia with Lewy bodies, DLB. Their diagnosis, especially in the initial stage of the disease, is not clear. Parkinson's disease is one of the most common degenerative diseases of the central nervous System. It is known that the intravital diagnosis of Parkinson's disease is difficult. Certain diagnosis of this disease is only possible after postmortem neuropathological examination of the brain for lesions typical for this disorder. Lack of early diagnosis of Parkinson's disease can make it difficult to treat effectively, contributing to the progression of the disease, and may further lead to reduced quality of life for these patients. Since the 1960s, the primary drug used to treat Parkinson's disease is L-dopa. Unfortunately, the effectiveness of L-dopa therapy in patients with Parkinson’s disease often decreases with progression of the disease and is associated with the appearance of numerous side effects. Side effects of L-dopa treatment include: dyskinesia, atherosclerosis, depression and dementia. Around the world, research is underway to find an effective therapy for patients with Parkinson's disease, including drugs that are neuroprotective and inhibit the degenerative process within the substantia nigra in the midbrain. At the same time, it appears that elucidating the currently unknown pathological processes of this disease may lead to more effective diagnosis and pharmacotherapy. Currently, it is believed that genetic study may be an essential element of intravital diagnosis of patients with Parkinson's disease and the elucidation of the mechanism of interaction between the genes associated with the pathogenesis of this disease probably helps explain unfamiliar pathways of selective damage to dopaminergic neurons in Parkinson's disease patients.
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genetic variants in diseases of the Extrapyramidal System
Current Genomics, 2014Co-Authors: Anna Oczkowska, Wojciech Kozubski, Margarita Lianeri, Jolanta DorszewskaAbstract:Knowledge on the genetics of movement disorders has advanced significantly in recent years. It is now recognized that disorders of the basal ganglia have genetic basis and it is suggested that molecular genetic data will provide clues to the pathophysiology of normal and abnormal motor control. Progress in molecular genetic studies, leading to the detection of genetic mutations and loci, has contributed to the understanding of mechanisms of neurodegeneration and has helped clarify the pathogenesis of some neurodegenerative diseases. Molecular studies have also found application in the diagnosis of neurodegenerative diseases, increasing the range of genetic counseling and enabling a more accurate diagno-sis. It seems that understanding pathogenic processes and the significant role of genetics has led to many experiments that may in the future will result in more effective treatment of such diseases as Parkinson’s or Huntington’s. Currently used molecular diagnostics based on DNA analysis can identify 9 neurodegenerative diseases, including spinal cerebellar ataxia inherited in an autosomal dominant manner, dentate-rubro-pallido-luysian atrophy, Friedreich’s disease, ataxia with ocu-lomotorapraxia, Huntington's disease, dystonia type 1, Wilson’s disease, and some cases of Parkinson's disease.
Laila M. Sherief - One of the best experts on this subject based on the ideXlab platform.
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Idiopathic Hypoparathyroidism With Extensive Intracranial Calcification in Children: First Report From Saudi Arabia
Medicine, 2017Co-Authors: Naglaa M. Kamal, Hamed A. Alghamdi, Abdulrahman Halabi, Ayman A Bakkar, Ali Algarni, Abdullah Al-harbi, Abdulla A. Alharthi, Rawan A Alharbi, Laila M. SheriefAbstract:Rationale Pediatric idiopathic hypoparathyroidism with extensive intracranial calcifications outside the basal ganglia (BG) is extremely rare with less than 10 cases worldwide. Patient concerns An 11-year-old Saudi male child presented with tetany with otherwise normal neurological and other body System examination diagnoses severe hypocalcemia for differential diagnosis. Interventions Further investigations revealed hyperphosphatemia and undetectable serum intact parathyroid hormone. Brain computed tomography revealed BG and extensive brain calcifications. He has no dysmorphic features, vitiligo, mucocuataneous manifestations, or hair loss. He had normal hemoglobin, electroencephalogram, and skeletal survey, with negative autoantibodies to alpha and omega interferons and negative genetic testing for Glial Cell Missing 2 (GCM2) and calcium-sensing receptors (CaSRs) excluding known causes of hypoparathyroidism. Outcomes This case presents a rare entity of idiopathic hypoparathyroidism with extensive intracranial calcification, not only in BG but also outside the Extrapyramidal System with normal mentality, development, pubertal achievement, and neurological examination. To our knowledge, this is the first report from Saudi Arabia in pediatrics. Lessons Idiopathic hypoparathyroidism is a diagnosis of exclusion after ruling out all known causes of hypoparathyroidism. It is associated with BG calcifications, but extensive intracranial calcifications outside the BG are extremely rare.
