79 results on '"Creutzfeldt Jacob Disease"'
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2. Accidental aspiration of removable dental prosthesis in a patient with Creutzfeldt‐Jacob disease
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Mario Eduardo Baldo, Paulo Sérgio da Silva Santos, Gabriel Umbelino Santanna, and Regina Maria Raffaele
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medicine.medical_specialty ,business.industry ,Dental prosthesis ,Disease ,Surgery ,medicine.anatomical_structure ,Swallowing ,Creutzfeldt Jacob Disease ,Accidental ,Intensive care ,medicine ,Esophagus ,business ,General Dentistry ,TRAUMATISMOS DENTÁRIOS ,Removable partial denture - Abstract
Accidents involving dental prostheses are unusual but relevant, which may result in complications in the upper respiratory airways and esophagus. A 47-year-old male patient with Creutzfeldt-Jacob disease (CJD), completely dependent on care, was reported by his family to have a partial obstruction and intraoral bleeding after swallowing his upper Removable Partial Denture (RPD). After difficulties in the endoscopy procedure, a surgical approach by exploratory cervicotomy to remove the prosthetic device was conducted, without postoperative complications. Considering individual impairments and the patients' advanced age, the removal of dental prostheses during sleep or for patients under intensive care is a preventive strategy to reduce comorbidities in patients with unstable health conditions.
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- 2021
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3. E200k Familial Creutzfeldt-Jakob Disease Presenting with Subacute Multiple Cranial Neuropathy
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L. Saitta, Paola Mandich, Giacomo Boffa, Nicola Romano, Marina Grandis, G. L. Mancardi, Caterina Lapucci, and Flavio Nobili
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Psychiatry and Mental health ,medicine.medical_specialty ,Neurology ,Creutzfeldt Jacob Disease ,business.industry ,mental disorders ,medicine ,Familial Creutzfeldt-Jakob ,Neurology (clinical) ,Multiple cranial neuropathy ,Disease ,business ,Dermatology - Abstract
Unusual clinical presentations in patients with E200K familial Creutzfeldt-Jakob Disease (fCJD) have been rarely reported. Herein, we described a case of E200K fCJD presenting with subacute cranial multiple neuropathy, initially suspected to be paraneoplastic or due to a leptomeningeal carcinomatosis, considering the neoplastic comorbidity of the patient. Surprisingly, brain MRI was highly suggestive of CJD. Brain histological examination confirmed the diagnosis. Genetic tests led to the definite diagnosis of E200K fCJD. To the best of our knowledge, the current case provides the first report of a histologically-confirmed E200K fCJD starting with cranial multiple neuropathy and may widen the spectrum of the clinical variability of CJD, also in its genetic variant. Unusual presentations may lead, as in this case, to incorrect diagnostic hypothesis and unuseful therapeutic attempts in the first phase of the diagnostic process. Also in the genetic variant of CJD, brain MRI demonstrated a very high sensitivity to detect the typical abnormalities since the earliest phases of the disease.
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- 2019
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4. Evaluation of the disease burden of sporadic Creutzfeldt-Jacob disease with the surveillance data from 2013 to 2017 in China
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Yuan Wang, Wei Zhou, Chen Gao, Xiao-Ping Dong, Kang Xiao, Li-Na Zhang, Qi Shi, and Cao Chen
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Surveillance data ,Creutzfeldt Jacob Disease ,business.industry ,Environmental health ,Medicine ,business ,China ,Disease burden - Abstract
Background Sporadic Creutzfeldt-Jacob disease (sCJD) is the commonest type of human prion disease and cause 100% fatality. The morbidity of sCJD is about 1 to 2 cases/million/year worldwide.Methods The disease burden of sCJD and the indirect economic burdens in China were evaluated based on the data of Chinese National CJD Surveillance from 2013 to 2017. Results The Disability-Adjusted Life Year (DALY) of sCJD was measured with 753 probable sCJD patients. The total DALY of 753 sCJD cases in the past five surveillance years was 16202.62 person-years. The Years of Life Lost (YLL) was the predominant component for DALY reaching to more than 95%, while the Years Lost due to Disability (YLD) accounted for less than 5% only. The highest DALY was noticed in the group of 45-50 year-old (6541.49), followed by the groups of 60-69 year-old (6311.25). Furthermore, the indirect economic burdens of 753 sCJD patients were estimated with the official capital data. Total indirect economic burden for five years was 372.08 million RMB (1US $=6.9 RMB), with the average of 494,133 RMB per case. The patients aged 45-59 year-old were the predominant population that accounted for approximate 73% of total indirect economic burden. Although still lacking of the exact annual incidence of sCJD in China, the DALY intensity of sCJD in China could be estimated and predicted as 2.2 to 4.4 person-years /100,000 with the global morbidity of sCJD. Conclusion This is the first disease burden assay for sCJD in China, which will supply fundamental evidence for further development of medical strategy.
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- 2020
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5. Creutzfeldt-Jacob Disease, As a Rare Cause of Dementia Should Not be Forgotten: Single-Center Experience
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Şebnem Biçakci, Mehmet Balal, Meltem Demirkiran, Mehmet Taylan Peköz, Turgay Demir, Hacer Bozdemir, and Kezban Aslan
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Pediatrics ,medicine.medical_specialty ,General and Internal Medicine ,business.industry ,General Medicine ,Single Center ,medicine.disease ,Creutzfeldt Jacob Disease ,Prion diseases,Creutzfeldt-Jacob disease,Cognitive impairment,Myoclonus,Electroencephalography ,medicine ,Dementia ,Creutzfeldt-Jacob hastalığı,elektroensefalografi,kognitif bozukluk,miyoklonus,prion hastalıkları ,business ,Genel ve Dahili Tıp - Abstract
Giriş: Önemli bir halk sağlığı sorunu olan demans primer ve sekonder demans olmak üzere iki gruba ayrılmaktadır. Çok nadir olarak görülen Creutzfeldt-Jakob hastalığı (CJH), hızlı progresif seyir gösteren bir sekonder demans nedenidir. CJH insanları ve hayvanları etkileyebilen enfeksiyöz spongioform ensefalopatilerden, ölümcül bir nörodejeneratif hastalıktır. Dört formu olan hastalığın en sık görülen formu olan sporadik CJH olgularında progresif kognitif bozukluk, miyoklonus ve ataksi tipik klinik tablodur. Bu çalışmada Nörolojik yoğun bakım ünitesinde tanı alan CJH olgularının demografik, klinik ve laboratuvar bulgularını tartışmayı amaçladık. Yöntem: Retrospektif olarak planlanan bu çalışmaya 16 hasta dahil edildi. Hastaların yaş, cinsiyet, başvuru şikayetleri, semptom başlangıcından mortaliteye kadar geçen süre, nörolojik muayene, beyin manyetik rezonans görüntüleme (MRG), elektroensefalografi (EEG) ve beyin omurilik sıvısında (BOS) protein 14.3.3 durumu kaydedildi. Bulgular: Ortalama yaş 61.18 ± 9.1 (37-73) olan hastaların başvuruda en sık karşılaşılan şikayetleri sırayla bilişsel bozukluk, bilinç bozukluğu, ataksi ve yürüme bozukluğu idi. BOS’ta protein 14.3.3 hastaların % 100'ünde pozitifti. En sık saptanan EEG bulgusu periyodik keskin dalga kompleksleri, en sık saptanan beyin MRG’de kortikal veya putamen ve kaudat nükleus hiperintensitesi ve kortikal ve serebellar atrofi idi. Bir hasta varyant CJH, diğer hastalar ise sporadik form olarak değerlendirildi. Sonuç: Progresif kognitif bozukluk ve eşlik eden miyoklonus veya ataksi varlığında CJH tanısı mutlaka akılda tutulmalıdır. Tanı için beyin görüntüleme, seri EEG kayıtlamaları, BOS analizi ve yapılabilirse histopatolojik inceleme yapılmalıdır., Introduction: Dementia, which is an important public health problem, is divided into two groups as primary and secondary dementia. Creutzfeldt–Jakob disease (CJD), which is rarely seen, is a secondary cause of dementia with a rapidly progressive course. It is a fatal neurodegenerative disorder of infectious spongiform encephalopathy that can affect humans and animals. Sporadic CJD is the most common type that can present in four forms and has typical clinical presentations of progressive cognitive impairment, myoclonus, and ataxia. We aimed to discuss the demographic, clinical, and laboratory findings of CJD cases diagnosed in the neurointensive care unit. Methods: Sixteen patients were included in this retrospective study. Age, sex, complaints on admission, duration from symptom onset to mortality, neurologic examination, brain magnetic resonance imaging (MRI), electroencephalography (EEG), and the protein 14.3.3 status of the cerebrospinal fluid (CSF) were recorded. Results: The mean age was 61.18 ± 9.1 years (range, 37–73 years), and the most common complaints on admission were cognitive impairment, disturbance in consciousness, ataxia, and gait disturbance. CSF protein 14-3-3 was positive in 100% of patients. The most common findings were periodic sharp and wave complexes on EEG, cortical or putamen and caudate nucleus hyperintensity, cortical and cerebellar atrophy on MRI. One of the patients was diagnosed as variant CJD, and the others were diagnosed as the sporadic form. Conclusions: CJD should be kept in mind in patients with myoclonus or ataxia accompanied by progressive cognitive impairment. Neuroimaging, serial EEG recordings, CSF analysis, and histopathologic examination should be performed for diagnosis.
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- 2020
6. A mathematical model for Creutzfeldt Jacob Disease (CJD)
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S. M. Salman and E. Ahmed
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Quantitative Biology::Biomolecules ,education.field_of_study ,General Mathematics ,Applied Mathematics ,Population ,General Physics and Astronomy ,Statistical and Nonlinear Physics ,Fixed point ,01 natural sciences ,nervous system diseases ,010305 fluids & plasmas ,Bifurcation analysis ,Creutzfeldt Jacob Disease ,mental disorders ,0103 physical sciences ,Applied mathematics ,Fatal disease ,010306 general physics ,education ,Nonlinear Sciences::Pattern Formation and Solitons ,Bifurcation ,Mathematics - Abstract
Creutzfeldt Jakob Disease (CJD) is a fatal disease which is transmitted by the ingestion of infectious materials (mainly BSE-contaminated beef). Here a simple mathematical model of its progress is given. Local stability analysis of fixed points of the model is studied. Moreover, codimension-one bifurcation analysis of fixed points is discussed. The model has a variety of bifurcation types such as transcritical, pitchfork and flip bifurcations. Numerical simulations are performed to illustrate analytical results obtained. Despite being a simple model, it discusses a non expected behavior which is increasing the parameter a, the growth rate of the healthy prions, the disease will persist in the population.
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- 2018
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7. Epilepsia Partialis Continua as the First Presenting Symptom in Probable Sporadic Creutzfeldt-Jacob Disease: A Case Report and Literature Review
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Sheng-Hsiang Yang, Tai-Yuan Chen, and Poh-Shiow Yeh
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0301 basic medicine ,Pediatrics ,medicine.medical_specialty ,Magnetic resonance diffusion-weighted image ,Akinetic mutism ,Epilepsia partialis continua ,Case Report ,Disease ,lcsh:RC346-429 ,03 medical and health sciences ,0302 clinical medicine ,Sporadic Creutzfeldt-Jacob disease ,Creutzfeldt Jacob Disease ,mental disorders ,medicine ,Hospice care ,lcsh:Neurology. Diseases of the nervous system ,medicine.diagnostic_test ,business.industry ,Putamen ,Magnetic resonance imaging ,medicine.disease ,Simple focal motor seizures ,030104 developmental biology ,Neurology (clinical) ,medicine.symptom ,business ,Myoclonus ,030217 neurology & neurosurgery - Abstract
We present the case of a middle-aged man suffering from epilepsia partialis continua 3 weeks before the start of cognition decline, visual disturbance, and pyramidal dysfunction. The epilepsia partialis continua was difficult to control, and the underlying cause was uncertain even after thorough surveys for infection, inflammation, autoimmunity, and neoplasm. However, progressive signal intensity changes were noted over the involved cortical gyri, bilateral caudate, and putamen on serial magnetic resonance diffusion-weighted images, which were compatible with sporadic Creutzfeldt-Jacob disease. Therefore, we tested for 14-3-3 protein in the cerebrospinal fluid, and the results were positive. Multifocal myoclonus jerks, severe mental decline, akinetic mutism, and typical periodic sharp wave complexes on electroencephalogram developed late in his disease course. He died under the hospice care, and his total disease duration was approximately 5 months. This case highlights that epilepsia partialis continua can be the first presenting symptoms of sporadic Creutzfeldt-Jacob disease, and that magnetic resonance imaging abnormalities can be helpful to identify the disease.
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- 2018
8. T188K-Familial Creutzfeldt–Jacob Disease, Predominant Among Chinese, has a Reactive Pattern in CSF RT-QuIC Different from D178N-Fatal Familial Insomnia and E200K-Familial CJD
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Qi Shi, Chen Gao, Kang Xiao, Cao Chen, Yue Ma, Yuan Wang, Wei Zhou, Bao-Yun Zhang, and Xiao-Ping Dong
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Male ,Pediatrics ,medicine.medical_specialty ,Neurology ,Physiology ,Pain medicine ,MEDLINE ,Insomnia, Fatal Familial ,Creutzfeldt-Jakob Syndrome ,Asian People ,Creutzfeldt Jacob Disease ,Anesthesiology ,Humans ,Medicine ,Genetic Predisposition to Disease ,Letter to the Editor ,Aged ,Fatal familial insomnia ,business.industry ,General Neuroscience ,General Medicine ,Human physiology ,Middle Aged ,medicine.disease ,Mutation ,Female ,business - Published
- 2019
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9. Creutzfeldt-Jacob Disease with Psychiatric Presentation: Hen's Teeth in Indian Subcontinent: A Case Report
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Shivangi Mehta, Gurmukh Singh, Alisha Salhotra, and Mitesh Kumar
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Psychiatry ,medicine.medical_specialty ,business.industry ,RC435-571 ,MEDLINE ,Indian subcontinent ,Clinical Psychology ,Psychiatry and Mental health ,Creutzfeldt Jacob Disease ,Medicine ,Presentation (obstetrics) ,Letters to Editor ,business - Published
- 2019
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10. 'Creutzfeldt-Jacob disease or other rapidly progressive dementia? Diagnostic and prognostic value of EEG, brain MRI and CSF features'
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Fabio Placidi, Claudio Liguori, Francesca Izzi, Chiara Giuseppina Bonomi, Alessandro Castelli, Andrea Pagano, Natalia Manfredi, Luisa Mari, and Nicola Biagio Mercuri
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Rapidly progressive dementia ,medicine.medical_specialty ,medicine.diagnostic_test ,business.industry ,Electroencephalography ,Neurology ,Creutzfeldt Jacob Disease ,Internal medicine ,Brain mri ,medicine ,Cardiology ,Neurology (clinical) ,business ,Value (mathematics) - Published
- 2021
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11. Sporadic Creutzfeldt-Jacob disease presenting with cerebral vasoconstriction
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Laura Fusi, Giampiero Grampa, Aristotelis Karantzoulis, and Ilaria Zivi
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Pathology ,medicine.medical_specialty ,Neurology ,Creutzfeldt Jacob Disease ,business.industry ,medicine ,Neurology (clinical) ,medicine.symptom ,business ,Vasoconstriction - Published
- 2021
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12. Creutzfeldt-Jacob Disease with presence of psychomotor agitation
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Yasin Hasan Balcioglu, Mucahid Erdogan, Hayrunisa Dilek Atakli, and Cengiz Dayan
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Psychiatry and Mental health ,Pediatrics ,medicine.medical_specialty ,Psychomotor agitation ,Creutzfeldt Jacob Disease ,business.industry ,medicine ,Neurology (clinical) ,medicine.symptom ,business - Published
- 2018
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13. Endpoint Quaking-Induced Conversion: a Sensitive, Specific, and High-Throughput Method for Antemortem Diagnosis of Creutzfeldt-Jacob Disease
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Keding Cheng, J. David Knox, Robert Vendramelli, Debra Godal, Angela Sloan, Lisa Podhorodecki, and Brooks Waitt
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0301 basic medicine ,Microbiology (medical) ,Pathology ,medicine.medical_specialty ,Canada ,computer.internet_protocol ,QUIC ,Sensitivity and Specificity ,Creutzfeldt-Jakob Syndrome ,Prion Proteins ,03 medical and health sciences ,0302 clinical medicine ,Creutzfeldt Jacob Disease ,Virology ,mental disorders ,Diagnosis ,Retrospective analysis ,Medicine ,Humans ,Prion protein ,Cerebrospinal Fluid ,Retrospective Studies ,business.industry ,Diagnostic Tests, Routine ,Antemortem Diagnosis ,Protein markers ,nervous system diseases ,030104 developmental biology ,Relative fluorescence units ,Signal intensity ,business ,computer ,030217 neurology & neurosurgery - Abstract
The Prion Laboratory Section of the Public Health Agency of Canada supports heath care professionals dealing with patients suspected to have Creutzfeldt-Jakob disease (CJD) by testing cerebrospinal fluid (CSF) for protein markers of CJD. To better serve Canadian diagnostic requirements, a quaking-induced conversion (QuIC)-based assay has been added to the test panel. The QuIC tests exploit the ability of disease-associated prion protein, found in the CSF of a majority of CJD patients, to convert a recombinant prion protein (rPrP) into detectable amounts of a misfolded, aggregated form of rPrP. The rPrP aggregates interact with a specific dye, causing a measurable change in the dye's fluorescence emission spectrum. Optimal test and analysis parameters were empirically determined. Taking both practical and performance considerations into account, an endpoint QuIC (EP-QuIC) configuration was chosen. EP-QuIC uses a thermo-mixer to perform the shaking necessary to produce the quaking-induced conversions. Fluorescence readings are obtained from a microwell fluorescence reader only at the beginning and the end of EP-QuIC reactions. Samples for which the relative fluorescence unit ratio between the initial and final readings represent a ≥4 increase in signal intensity in at least two of the three replicates are classified as positive. A retrospective analysis of 91 CSF samples that included 45 confirmed cases of CJD and 46 non-CJD cases was used to estimate the performance characteristics of the EP-QuIC assay. The diagnostic sensitivity and specificity of the EP-QuIC test of this set of samples were 98 and 91%, respectively.
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- 2016
14. Prion Diseases of the Central Nervous System (Creutzfeldt-Jacob Disease)
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MI Sahadulla
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medicine.anatomical_structure ,Creutzfeldt Jacob Disease ,business.industry ,Central nervous system ,Medicine ,business ,Virology - Published
- 2018
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15. Creutzfeldt–Jacob disease mimics, or how to sort out the subacute encephalopathy patient
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Katherine Murray
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Brain Diseases ,Pathology ,medicine.medical_specialty ,Pediatrics ,Sporadic CJD ,business.industry ,Encephalopathy ,General Medicine ,Disease ,medicine.disease ,Creutzfeldt-Jakob Syndrome ,nervous system diseases ,Diagnosis, Differential ,Central nervous system disease ,Creutzfeldt Jacob Disease ,mental disorders ,Humans ,Medicine ,Neurology (clinical) ,Differential diagnosis ,business - Abstract
Sporadic Creutzfeldt–Jacob disease (CJD) is a rare untreatable neurodegenerative disease which every neurologist will occasionally encounter during their career. However, it is likely to appear on their differential diagnosis list significantly more frequently. Numerous conditions can present with subacute encephalopathy which might be sporadic CJD and this article explores these diagnoses. It includes the commonest sporadic CJD mimics which are neurodegenerative, and highlights the relatively rare treatable mimics which must not be missed. It discusses relevant investigations, including serum antibodies, CSF, electroencephalography and MR brain imaging, and strategies when preliminary investigations fail to support sporadic CJD but no alternative diagnosis is readily apparent.
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- 2011
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16. Accumulation of L-type Bovine Prions in Peripheral Nerve Tissues
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Yujing Shu, Tetsutaro Sata, Sadao Onoe, Kentaro Masujin, Shirou Mohri, Yoshifumi Iwamaru, Ken'ichi Hagiwara, Takashi Yokoyama, Shigeo Fukuda, Yuichi Matsuura, Yuichi Murayama, Kazuo Kasai, Yoshio Yamakawa, Yoshihisa Shimizu, Hiroyuki Okada, Morikazu Imamura, and Megumi Kurachi
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Microbiology (medical) ,Pathology ,medicine.medical_specialty ,Prions ,Epidemiology ,animal diseases ,Bovine spongiform encephalopathy ,Central nervous system ,lcsh:Medicine ,Biology ,atypical bovine spongiform encephalopathy ,lcsh:Infectious and parasitic diseases ,prion ,Pathogenesis ,Creutzfeldt Jacob Disease ,Peripheral nerve ,mental disorders ,distribution ,medicine ,transmissibility ,Animals ,lcsh:RC109-216 ,Peripheral Nerves ,L-type ,lcsh:R ,Dispatch ,food and beverages ,nerve tissues ,medicine.disease ,Virology ,zoonoses ,nervous system diseases ,Encephalopathy, Bovine Spongiform ,Atypical bovine spongiform encephalopathy ,Infectious Diseases ,medicine.anatomical_structure ,cattle ,Creutzfeldt-Jacob disease ,neurodegenerative disorder - Abstract
We recently reported the intraspecies transmission of L-type atypical bovine spongiform encephalopathy (BSE). To clarify the peripheral pathogenesis of L-type BSE, we studied prion distribution in nerve and lymphoid tissues obtained from experimentally challenged cattle. As with classical BSE prions, L-type BSE prions accumulated in central and peripheral nerve tissues.
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- 2010
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17. Hallucinatory palinopsia and paroxysmal oscillopsia as initial manifestations of sporadic Creutzfeldt-Jakob disease: A case study.
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Lahiri D, Dubey S, Ray BK, and Ardila A
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- Brain, Hallucinations, Humans, Magnetic Resonance Imaging, Male, Middle Aged, Vision Disorders, Creutzfeldt-Jakob Syndrome complications
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Background: Heidenhain variant of Cruetzfeldt Jacob Disease is a rare phenotype of the disease. Early and isolated visual symptoms characterize this particular variant of CJD. Other typical symptoms pertaining to muti-axial neurological involvement usually appear in following weeks to months. Commonly reported visual difficulties in Heidenhain variant are visual dimness, restricted field of vision, agnosias and spatial difficulties. We report here a case of Heidenhain variant that presented with very unusual symptoms of palinopsia and oscillopsia., Case Presentation: A 62-year-old male patient presented with symptoms of prolonged afterimages following removal of visual stimulus. It was later on accompanied by intermittent sense of unstable visual scene. He underwent surgery in suspicion of cataratcogenous vision loss but with no improvement in symptoms. Additionally he developed symptoms of cerebellar ataxia, cognitive decline and multifocal myoclonus in subsequent weeks. On the basis of suggestive MRI findings in brain, typical EEG changes and a positive result of 14-3-3 protein in CSF, he was eventually diagnosed as sCJD., Conclusion: This case adds to the tally of handful reports of Heidenhain variant CJD in literature, particularly from India. Two atypical initial symptoms, namely hallucinatory palinopsia and paroxysmal oscillopsia were observed in the index case. Possible explanations of such phenomena in CJD have been explored in light of the available studies., (Copyright © 2019 Elsevier Ltd. All rights reserved.)
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- 2020
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18. Sporadic Creutzfeldt-Jacob Disease Presenting With Symptoms of Corticobasal Degeneration: A Case Report
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Yuksel Erdal, Ufuk Emre, and Arife Çimen Atalar
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0301 basic medicine ,Pathology ,medicine.medical_specialty ,Pediatrics ,Ataxia ,business.industry ,General symptoms ,Akinetic mutism ,Progressive dementia ,Disease ,030105 genetics & heredity ,medicine.disease ,nervous system diseases ,03 medical and health sciences ,0302 clinical medicine ,Creutzfeldt Jacob Disease ,mental disorders ,medicine ,Corticobasal degeneration ,medicine.symptom ,business ,Myoclonus ,030217 neurology & neurosurgery - Abstract
Creutzfeldt–Jacob disease (CJD) is a rare neurodegenerative disease with a rapid progressive course. Clinically, general symptoms include progressive dementia, ataxia, myoclonus and akinetic mutism, in addition to other presentations such as alien hand. Here, we describe a patient with symptoms resembling corticobasal degeneration (CBD) who was diagnosed with CJD at follow up.
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- 2016
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19. Emerging Patterns of Diffusion, Perfusion-Weighted MRI and Spectroscopy in Creutzfeldt-Jacob Disease
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Luigi Maria Fantozzi, P. Tisei, Alessandro Bozzao, and Andrea Romano
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Pathology ,medicine.medical_specialty ,business.industry ,Caudate nucleus ,General Medicine ,nervous system diseases ,030218 nuclear medicine & medical imaging ,03 medical and health sciences ,0302 clinical medicine ,medicine.anatomical_structure ,Perfusion Weighted MRI ,Creutzfeldt Jacob Disease ,Cerebral cortex ,Basal ganglia ,Medicine ,Radiology, Nuclear Medicine and imaging ,In patient ,Neurology (clinical) ,business ,Perfusion ,030217 neurology & neurosurgery ,Diffusion MRI - Abstract
Sporadic Creutzfeldt-Jacob disease (CJD) is a transmissible, progressive, fatal spongiform encephalopathy. Routine MR imaging sequences may show abnormalities in the basal ganglia and cerebral cortex. Recently, several reports claimed that diffusion weighted MRI (DWI) could demonstrate early brain lesions in CJD patients when conventional MR images are normal on T2-weighted sequences. We evaluated the usefulness of DWI, perfusion-weighted MRI (PWI) and spectroscopy to confirm the clinical diagnosis and assess lesion progression in two patients with suspected CJD. We noted a diffuse hypoperfusion in the basal ganglia where ADC values were reduced but spectroscopy values were normal. A strong hypoperfusion was observed in the right head of the caudate nucleus in patient n° 2 where spectroscopy values were abnormal. A typical distribution of hypoperfusion followed the posterior progression of disease. We suggest the hypoperfusion in the areas presenting restricted diffusion probably reflects spongiform degeneration and moderate mass effects from cytotoxic edema.
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- 2007
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20. The Compensation of the Victims of the Creutzfeldt-Jacob Disease in the United Kingdom
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Andrea Boggio
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Kingdom ,Politics ,Creutzfeldt Jacob Disease ,business.industry ,Law ,Political science ,Compensation (psychology) ,Medicine ,General Medicine ,business ,Affect (psychology) ,Law and economics - Abstract
Establishing no-fault compensatory schemes is problematic from both a political and legal point of view. In this essay, I analyse the process that led to the compensation of the victims of Creutzfeldt-Jacob Disease and its variant. The paper shows that, although the diseases present many similarities, the two processes took very different paths because of the different, political environments in which they took place. Moreover, several possible lessons can be drawn from the compensation of CJD victims, which can potentially affect the establishment of future no-fault schemes in the United Kingdom.
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- 2005
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21. Spongiform encephalopathy, cannibalism and Neanderthals extinction
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Brunetto Chiarelli
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education.field_of_study ,Extinction ,Neanderthal ,animal diseases ,Population ,Cannibalism ,Zoology ,Biology ,Creutzfeldt Jacob Disease ,Anthropology ,biology.animal ,Spongiform encephalopathy ,education ,Demography - Abstract
There are paleoecological evidences that Neanderthals ate the brains of deers and goats as well that of their own deceased. This would expose each individual and the population to the risks of contracting the Creutzfeldt Jacob disease. Those who consumed the remains of infected individual’s would then contract the disease and eventually infect others. If this is the case then the Neanderthal extinction could be attributed to spongiform encephalopathy and not to the cultural supremacy of the anatomically modern man.
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- 2004
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22. The pathophysiology of variant creutzfeldt-jacob disease: the hypotheses behind concerns for blood components and products
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John Burthem and David J. Roberts
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Prions ,business.industry ,Blood Component Transfusion ,Hematology ,Creutzfeldt-Jakob Syndrome ,Pathophysiology ,Creutzfeldt Jacob Disease ,Immunology ,Humans ,Medicine ,Blood safety ,Infectious risk ,Prion protein ,business ,Neuroscience - Published
- 2003
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23. New Variant Creutzfeldt-Jacob Disease Presenting With Catatonia: A Rare Presentation
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Narayana Manjunatha, Lokesh Singh Shekhawat, Santosh Kumar, Prerna Gupta, and Santosh Ramdurg
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Psychiatry and Mental health ,medicine.medical_specialty ,Creutzfeldt Jacob Disease ,business.industry ,Catatonia ,Medicine ,Neurology (clinical) ,New variant ,Presentation (obstetrics) ,business ,medicine.disease ,Dermatology - Published
- 2012
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24. Diffusion-weighted imaging: As the first diagnostic clue to Creutzfeldt Jacob disease
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Sanjaya Viswamitra, BT Pushpa, E V Joshy, Sunitha P Kumaran, and Kanchan Gupta
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Clinical settings ,Case Report ,Inversion recovery ,Fluid-attenuated inversion recovery ,030218 nuclear medicine & medical imaging ,lcsh:RC321-571 ,03 medical and health sciences ,0302 clinical medicine ,Creutzfeldt Jacob Disease ,mental disorders ,diffusion weighted imaging ,Medicine ,Cognitive decline ,lcsh:Neurosciences. Biological psychiatry. Neuropsychiatry ,medicine.diagnostic_test ,business.industry ,General Neuroscience ,Magnetic resonance imaging ,Hyperintensity ,nervous system diseases ,Creutzfeldt-Jacob disease ,Neurology (clinical) ,business ,Nuclear medicine ,030217 neurology & neurosurgery ,Diffusion MRI - Abstract
We describe a case report where the diagnosis of sporadic Creutzfeldt-Jakob disease(CJD) was suspected by the magnetic resonance imaging (MRI) radiologist in a 75-year-old lady who presented with rapid cognitive decline. MRI revealed cortical T2 and FLAIR(fluid attenuation and inversion recovery) hyperintensities in bilateral fronto-parietal and temporo-occipital locations and showed significant restriction on diffusion-weighted images(DWI). In this case report, we discuss the role of MRI to suspect the diagnosis of CJD in appropriate clinical settings.
- Published
- 2012
25. Expression patterns of antioxidant proteins in brains of patients with sporadic Creutzfeldt-Jacob disease
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Kurt Krapfenbauer, Gert Lubec, Michael Fountoulakis, Eva Mitrova, and Byong Chul Yoo
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Male ,Cerebellum ,Frontal cortex ,Antioxidant ,medicine.medical_treatment ,Blotting, Western ,Clinical Biochemistry ,Gene Expression ,Nerve Tissue Proteins ,Biology ,medicine.disease_cause ,Biochemistry ,Antioxidants ,Creutzfeldt-Jakob Syndrome ,Analytical Chemistry ,Western blot ,Creutzfeldt Jacob Disease ,medicine ,Humans ,Electrophoresis, Gel, Two-Dimensional ,Aged ,Gel electrophoresis ,medicine.diagnostic_test ,Peroxiredoxins ,Middle Aged ,Molecular biology ,medicine.anatomical_structure ,Peroxidases ,Case-Control Studies ,Spectrometry, Mass, Matrix-Assisted Laser Desorption-Ionization ,Female ,Peroxiredoxin ,Oxidative stress - Abstract
Using two-dimensional gel electrophoresis (2-DE) and Western blot analysis, we were able to identify and quantify six antioxidant proteins, peroxiredoxin (Prx) I, Prx II, Prx III, 1-Cys Prx, putative peroxisomal antioxidant enzyme (PLP), and mitochondrial Mn superoxide dismutase (Mn-SOD) in two individual brain regions, cerebellum and frontal cortex of patients with sporadic Creutzfeldt-Jacob (sCJD). Among six antioxidant proteins, 1 -Cys Prx showed significant increase (P < 0.05) in sCJD frontal cortex whereas Prx I was decreased (P < 0.01). In cerebellum, levels of all antioxidant proteins studied were comparable to those of controls. Our findings provide evidence for the link between aberrant expression of antioxidant proteins, 1-Cys Prx and Prx I and CJD neuropathogenesis and we discuss the neuropathological meaning of these dysregulated antioxidant proteins in sCJD brain.
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- 2002
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26. Differential generator mechanisms of giant somatosensory evoked potentials and periodic synchronous dischargrs in creutzfeldt-jacob disease
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T. Ishihara, A. Demura, K. Yamamoto, Masako Kinoshita, K. Park, I. Okabe, T. Moriuchi, S. Kajikawa, K. Enoki, and M. Taniguchi
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Physics ,Generator (computer programming) ,Neurology ,Creutzfeldt Jacob Disease ,Neurology (clinical) ,Giant somatosensory evoked potentials ,Neuroscience ,Differential (mathematics) - Published
- 2017
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27. Novel Spiroplasma sp. Isolated From CWD Is an Extreme Bacterial Thermoacidophile That Survives Autoclaving, Boiling, Formalin Treatment, and Significant Gamma Irradiation.
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Bastian FO, Lynch J, and Wang WH
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- Animals, Animals, Wild microbiology, Anti-Bacterial Agents administration & dosage, Cells, Cultured, Formaldehyde administration & dosage, Gamma Rays, Spiroplasma ultrastructure, Prion Diseases microbiology, Spiroplasma isolation & purification, Spiroplasma pathogenicity, Wasting Disease, Chronic microbiology
- Abstract
Rapid spreading of chronic wasting disease (CWD) in wildlife and captive cervid populations has exposed lack of progress in dealing with the transmissible spongiform encephalopathies (TSE) of man and animals. Since the TSE transmissible agent was resistant to extremes in environmental and chemical treatments, focus was on an unconventional agent including the prion theory. Recent breakthrough research has revealed consistent isolation of a novel Spiroplasma sp. from TSE-affected tissues that propagates in cell-free media and on agar. Here, we developed a live culture assay to test whether the CWD spiroplasma isolate possessed unconventional biologic properties akin to those of the transmissible agent of TSE. The CWD spiroplasma isolate survived boiling for 1 hour, standard liquid autoclaving, 10% formalin treatment overnight, and gamma irradiation of 20 kGy. The CWD spiroplasma isolate is an acidophile, growing best at pH 2. The biologic resistance of the CWD spiroplasma isolate may be due to unusual phage-like viruses found in the bacterial pellet or to DNA-protein binding. Because the CWD spiroplasma isolate has biologic properties consistent with the causal agent of the TSEs, TSE research focus should be redirected to development of diagnostic tests and preventive vaccines for control of CWD based upon the bacterium., (© 2019 American Association of Neuropathologists, Inc. All rights reserved.)
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- 2019
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28. Aphasia or Dementia: The Cautionary Tale of Dr. JJ
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O. M. Reinmuth and Audrey L. Holland
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Male ,Psychotherapist ,Anomia ,Interpersonal communication ,Neuropsychological Tests ,LPN and LVN ,medicine.disease ,Creutzfeldt-Jakob Syndrome ,Developmental psychology ,Diagnosis, Differential ,Speech and Hearing ,Creutzfeldt Jacob Disease ,Alzheimer Disease ,Aphasia ,mental disorders ,medicine ,Humans ,Dementia ,Differential diagnosis ,medicine.symptom ,Psychology ,Aged - Abstract
This case study involves the differential diagnosis of aphasia versus dementia. However, the case of Dr. JJ is instructive from a number of aspects, presented here as "cautionary notes" which are intended to remind clinicians of sometimes-forgotten interpersonal issues in the diagnosis of disorders of speech and language. This case also is intended to illustrate that merely distinguishing between dementia and aphasia is sometimes inadequate, particularly when potentially transmittable (and in some cases, curable) dementias might be involved.
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- 1998
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29. The different faces of Creutzfeldt-Jacob disease CJD in psychiatry
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Alzbeta Juven-Wetzler, Joseph Zohar, and Anat Abudy
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Adult ,Male ,medicine.medical_specialty ,Psychosis ,Disease ,Creutzfeldt-Jakob Syndrome ,New onset ,Diagnosis, Differential ,Psychiatric treatments ,Creutzfeldt Jacob Disease ,mental disorders ,Medicine ,Dementia ,Humans ,Medical diagnosis ,Psychiatry ,Depression (differential diagnoses) ,Aged ,business.industry ,Mental Disorders ,Middle Aged ,medicine.disease ,nervous system diseases ,Psychiatry and Mental health ,Female ,business - Abstract
Objective Creutzfeldt-Jacob Disease (CJD) is a rapidly progressive spongiform disease of the central nervous system. Psychiatric symptoms, though considered rare, can be the presenting symptoms of CJD and impose diagnosis difficulties. We reviewed prospectively our database to identify the frequency of psychiatric symptoms as identifying symptoms among our community. Methods We included all patients in Sheba Medical Center who were diagnosed with CJD between the years 2006 and 2012. Data were collected retrospectively. Results Twenty-three patients with CJD were admitted to our hospital during this 6-year period. Among them, 10 (44%) were diagnosed first as "psychiatric patients" due to psychiatric presenting symptoms. Conclusion In our series, the frequency of misleading psychiatric symptom was 44%. Clinicians should therefore include CJD in their differential diagnoses of new onset dementia, particularly when associated psychosis and depression symptoms persist and worsen, despite standard psychiatric treatments.
- Published
- 2013
30. Creutzfeldt-jacob disease; a case report with radiological and cerebrospinal fluid findings
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Orhan Yilmaz
- Subjects
Pathology ,medicine.medical_specialty ,Neuropsychology and Physiological Psychology ,Cerebrospinal fluid ,Creutzfeldt Jacob Disease ,business.industry ,Physiology (medical) ,General Neuroscience ,Radiological weapon ,Medicine ,business - Published
- 2016
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31. Centripetal nystagmus in a case of Creutzfeldt-Jacob disease
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C. Helmchen and Ulrich Büttner
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medicine.medical_specialty ,genetic structures ,Nystagmus ,ECCENTRIC FIXATION ,Audiology ,medicine.disease ,eye diseases ,nervous system diseases ,Ophthalmology ,Creutzfeldt Jacob Disease ,mental disorders ,medicine ,Dementia ,Neurology (clinical) ,Cerebellar signs ,medicine.symptom ,Psychology ,Myoclonus - Abstract
Acquired forms of gaze-holding nystagmus usually produce centrifugal nystagmus. The authors report about a 63-year-old-patient with a rapidly deteriorating syndrome of cerebellar signs, dementia and myoclonus suggesting Creutzfeldt-Jakob disease (CJD), who developed the unusual sign of bilateral horizontal and vertical centripetal nystagmus with sustained eccentric fixation early in the disease. To our knowledge, this is the first report of centripetal nystagmus in CJD.
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- 1995
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32. Sheep and goat BSE propagate more efficiently than cattle BSE in human PrP transgenic mice
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Hubert Laude, Juan María Torres, Fabienne Reine, Laetitia Herzog, Juan Carlos Espinosa, Alfonso Gutiérrez-Adán, Emilie Jaumain, Vincent Béringue, Danielle Padilla, Belén Pintado, Olivier Andreoletti, Instituto Nacional de Investigación y Tecnología Agraria y Alimentaria = National Institute for Agricultural and Food Research and Technology (INIA), Unité de recherche Virologie et Immunologie Moléculaires (VIM (UR 0892)), Institut National de la Recherche Agronomique (INRA), Interactions hôtes-agents pathogènes [Toulouse] (IHAP), Institut National de la Recherche Agronomique (INRA)-Ecole Nationale Vétérinaire de Toulouse (ENVT), Institut National Polytechnique (Toulouse) (Toulouse INP), Université Fédérale Toulouse Midi-Pyrénées-Université Fédérale Toulouse Midi-Pyrénées-Institut National Polytechnique (Toulouse) (Toulouse INP), Université Fédérale Toulouse Midi-Pyrénées-Université Fédérale Toulouse Midi-Pyrénées, Departamento de reproduccion animal, and Instituto Nacional de Investigaciones Agrarias
- Subjects
[SDV]Life Sciences [q-bio] ,animal diseases ,Creutzfeldt-Jakob Syndrome ,BSE ,Mice ,0302 clinical medicine ,Small ruminant ,TSE ,Biology (General) ,0303 health sciences ,Goats ,food and beverages ,New variant ,Meat and bone meal ,3. Good health ,Encephalopathy, Bovine Spongiform ,Research Article ,Genetically modified mouse ,Prions ,QH301-705.5 ,Bovine spongiform encephalopathy ,Immunology ,Mice, Transgenic ,Biology ,Microbiology ,03 medical and health sciences ,Creutzfeldt Jacob Disease ,Infectious Diseases/Prion Diseases ,Virology ,mental disorders ,Genetics ,medicine ,Animals ,Humans ,human ,Allele ,Epidemics ,Molecular Biology ,030304 developmental biology ,Sheep ,RC581-607 ,medicine.disease ,nervous system diseases ,cattle ,sheep ,Parasitology ,Cattle ,Immunologic diseases. Allergy ,030217 neurology & neurosurgery - Abstract
A new variant of Creutzfeldt Jacob Disease (vCJD) was identified in humans and linked to the consumption of Bovine Spongiform Encephalopathy (BSE)-infected meat products. Recycling of ruminant tissue in meat and bone meal (MBM) has been proposed as origin of the BSE epidemic. During this epidemic, sheep and goats have been exposed to BSE-contaminated MBM. It is well known that sheep can be experimentally infected with BSE and two field BSE-like cases have been reported in goats. In this work we evaluated the human susceptibility to small ruminants-passaged BSE prions by inoculating two different transgenic mouse lines expressing the methionine (Met) allele of human PrP at codon 129 (tg650 and tg340) with several sheep and goat BSE isolates and compared their transmission characteristics with those of cattle BSE. While the molecular and neuropathological transmission features were undistinguishable and similar to those obtained after transmission of vCJD in both transgenic mouse lines, sheep and goat BSE isolates showed higher transmission efficiency on serial passaging compared to cattle BSE. We found that this higher transmission efficiency was strongly influenced by the ovine PrP sequence, rather than by other host species-specific factors. Although extrapolation of results from prion transmission studies by using transgenic mice has to be done very carefully, especially when human susceptibility to prions is analyzed, our results clearly indicate that Met129 homozygous individuals might be susceptible to a sheep or goat BSE agent at a higher degree than to cattle BSE, and that these agents might transmit with molecular and neuropathological properties indistinguishable from those of vCJD. Our results suggest that the possibility of a small ruminant BSE prion as vCJD causal agent could not be ruled out, and that the risk for humans of a potential goat and/or sheep BSE agent should not be underestimated., Author Summary Prion diseases, also referred as transmissible spongiform encephalopathies, are fatal neurodegenerative diseases caused by proteinaceous infectious particles denominated “prions.” Prion diseases acquired their first real public relevance with the outbreak of bovine spongiform encephalopathy (BSE) (“mad cow disease”) in the United Kingdom in the 80s and its link with the appearance of a new, variant form of Creutzfeldt-Jakob disease in humans. Recycling of ruminant tissues in meat and bone meal has been proposed as origin of the BSE epidemic. During this episode, sheep and goats have also been exposed to BSE-contaminated meal, so transmission to this species may have occurred. We analyzed the human susceptibility to sheep and goat passaged-BSE prions by using transgenic mice expressing human prion protein (PrP). When different sheep and goat BSE isolates were inoculated in these transgenic mice, higher susceptibility than that observed for cattle BSE was detected and the disease manifestation was similar to that observed in mice inoculated with the new variant of Creutzfeldt-Jakob disease. Our findings suggest that humans are at least equally, and might be even more, susceptible to a sheep or goat BSE agent compared to a cattle BSE one.
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- 2011
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33. Parkinsonism in early Creutzfeldt–Jacob disease: Possible pre- and post-synaptic mechanism
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Young Eun Kim, Jong Min Kim, Ho Young Lee, Seok Jin Choi, Sang Eun Kim, and Han Yeong Jeong
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Neurology ,Creutzfeldt Jacob Disease ,medicine.diagnostic_test ,Mechanism (biology) ,business.industry ,Parkinsonism ,medicine ,Neurology (clinical) ,Electroencephalography ,medicine.disease ,business ,Neuroscience ,Pre and post - Published
- 2014
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34. Sporadik Creutzfeldt-Jacob Hastalığında Supranükleer Bakış Parezisi
- Author
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Hakan Gurvit, Hasmet Hanagasi, Alpsan Gökmen Mh, Murat Emre, and Gür Özmen S
- Subjects
Psychiatry and Mental health ,Pathology ,medicine.medical_specialty ,Creutzfeldt Jacob Disease ,business.industry ,General Neuroscience ,medicine ,Abnormality ,business ,Gaze - Published
- 2014
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35. Creutzfeldt–Jacob Disease
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Pierluigi Gambetti, Gianfranco Puoti, Ignazio Cali, Wen-Quan Zou, and Qingzhong Kong
- Subjects
Pathology ,medicine.medical_specialty ,Ataxia ,animal diseases ,Disease ,Biology ,medicine.disease ,Virology ,nervous system diseases ,Cerebrospinal fluid ,Creutzfeldt Jacob Disease ,Genotype ,medicine ,Etiology ,Dementia ,medicine.symptom ,Myoclonus - Abstract
Sporadic Creutzfeldt–Jakob disease (sCJD) is the most common type of a group of fatal illnesses named transmissible spongiform encephalopathies or prion diseases, which have the unique features of being sporadic, genetically determined or acquired by infection. The three etiologies are associated with the same central pathogenic mechanism: a normal cellular protein, named prion protein, becomes insoluble, pathogenic and infectious by undergoing a change in conformation. sCJD is very heterogeneous due to the unique effect on the disease phenotype of the patient's prion protein genotype and characteristics of the abnormal prion protein. Based on these molecular features, five subtypes of sCJD with distinctive clinical and histopathological features have been identified. Increase in amount of 14–3–3 and/or tau in the cerebrospinal fluid and especially MRI are helpful in the diagnosis. Despite broad effort, no effective treatment is available.
- Published
- 2010
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36. Chemical disinfection of virus‐contaminated surfaces
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V. Susan Springthorpe and Syed A. Sattar
- Subjects
Creutzfeldt Jacob Disease ,Disinfectant ,Human immunodeficiency virus (HIV) ,medicine ,Infective Dose ,Scrapie agent ,Contamination ,Biology ,medicine.disease_cause ,Pollution ,Virus ,Chemical disinfection ,Microbiology - Abstract
Chemical disinfection is widely practiced as a means of controlling and preventing the spread of infectious diseases. Although disinfection of bacteria has been widely studied, much less attention has been paid to the virucidal potential of commonly used disinfectants in spite of the low infective dose of many human pathogenic viruses. This review considers what is known about the disinfection of viruses and the virucidal properties of different classes of disinfectant chemicals. It focuses on virus disinfection from a practical viewpoint and also critically evaluates the testing techniques currently used for examining the efficacy of disinfectant products.
- Published
- 1990
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37. Alzheimer's disease
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James D. Gunton, Daniel L. Pagan, and Andrey Shiryayev
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Crystallography ,Parkinson's disease ,Creutzfeldt Jacob Disease ,Huntington's disease ,Chemistry ,Condensation ,medicine ,Synaptic efficacy ,Protein folding ,Disease ,medicine.disease ,Molecular biology ,Biochemistry of Alzheimer's disease - Published
- 2007
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38. Child health and parental obligations
- Author
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Jo Bridgeman
- Subjects
Creutzfeldt Jacob Disease ,business.industry ,Law ,Health care ,Whooping-cough vaccine ,Medicine ,business ,Public funding ,Family law ,Child health - Published
- 2007
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39. Sporadic onset Creutzfeldt-Jacob disease: interesting MRI observations
- Author
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Sanjib Sinha, H. S. Chandrasekhar, Joy Vijayan, A B Taly, and KS Praveen
- Subjects
Pathology ,medicine.medical_specialty ,Myoclonic Jerk ,Fluid-attenuated inversion recovery ,Creutzfeldt-Jakob Syndrome ,Creutzfeldt Jacob Disease ,mental disorders ,medicine ,Humans ,Rapidly progressive dementia ,medicine.diagnostic_test ,business.industry ,Putamen ,Magnetic resonance imaging ,Electroencephalography ,Middle Aged ,Magnetic Resonance Imaging ,Neurology ,Gait abnormality ,Dementia ,Female ,Neurology (clinical) ,Radiology ,medicine.symptom ,Abnormality ,business - Abstract
We describe a 60-year-old woman with probable sporadic Creutzfeldt-Jacob disease (CJD) who manifested with two months history of rapidly progressive dementia and abnormal behavior, speech and gait abnormality, excessive sleepiness and myoclonic jerks. Scalp EEG showed diffuse slowing of background activity to delta range and triphasic sharp wave complexes occurring periodically twice in one-second interval. Magnetic resonance Imaging (MRI) of brain revealed high signal intensity on T2 weighted image (T2WI) and fluid attenuated inversion recovery sequences in the caudate and putamen bilaterally. Diffusion weighted images showed bilateral symmetric hyperintense signals in the caudate and putamen. The role of MRI in the diagnosis of CJD is discussed.
- Published
- 2006
40. Variant Creutzfeldt-Jacob disease - the availability of a screening test will be the beginning, not the end, of difficult transfusion-related issues
- Author
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Sandler Sg
- Subjects
Pathology ,medicine.medical_specialty ,Screening test ,business.industry ,Medical screening ,Transfusion Reaction ,Blood Donors ,Hematology ,Truth Disclosure ,Creutzfeldt-Jakob Syndrome ,United States ,Creutzfeldt Jacob Disease ,Risk Factors ,Genotype ,medicine ,Humans ,Mass Screening ,Genetic variability ,business ,Intensive care medicine - Published
- 2006
41. The utility of EEG in dementia: a clinical perspective
- Author
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Dimitrios Adamis, Adrian Treloar, and Sunita Sahu
- Subjects
medicine.medical_specialty ,AIDS Dementia Complex ,MEDLINE ,Audiology ,Electroencephalography ,Creutzfeldt-Jakob Syndrome ,Quantitative eeg ,Diagnosis, Differential ,Functional brain ,Creutzfeldt Jacob Disease ,medicine ,Dementia ,Humans ,Psychiatry ,Geriatrics ,medicine.diagnostic_test ,business.industry ,Perspective (graphical) ,Parkinson Disease ,medicine.disease ,Prognosis ,Psychiatry and Mental health ,Huntington Disease ,Treatment Outcome ,Cholinesterase Inhibitors ,Geriatrics and Gerontology ,business - Abstract
Background Despite being simple and cheap, the EEG is not often used in clinical practice. Methodology Literature search using PUBMED and Medline. Results Quantitative EEG can help to identify mild dementia and mild cognitive impairment and can increase diagnostic accuracy when used with other imaging techniques. EEG helps differentiate organic from functional brain disease and predict response to cholinesterase inhibitors and is central in the diagnosis of Creutzfeldt Jacob disease. The accuracy of EEG may be greater than that of CT or MRI scans alone. Discussion Quantitative EEG may save on specialist interpretation time and enable more routine use of EEG in diagnosis and care. More widespread use of EEG's is indicated. Agreement on the parameters that are best measured on qEEG is still awaited. Copyright © 2005 John Wiley & Sons, Ltd.
- Published
- 2005
42. How Heterogeneous Can the Clinical Presentation for Creutzfeldt-Jacob Disease Be?
- Author
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Roberto Mello, Isabela A. Mota, José Eriton Gomes da Cunha, Silvana Sobreira, and João Ricardo Mendes de Oliveira
- Subjects
Psychiatry and Mental health ,Pediatrics ,medicine.medical_specialty ,Presentation ,Creutzfeldt Jacob Disease ,business.industry ,media_common.quotation_subject ,Medicine ,Neurology (clinical) ,business ,media_common - Published
- 2013
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43. Alcoholic fixation of blood to surgical instruments-a possible factor in the surgical transmission of CJD?
- Author
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A Renfrew, Karen Fernie, G Heneaghan, and F.G.R. Prior
- Subjects
Microbiology (medical) ,Pathology ,medicine.medical_specialty ,Electrodiagnosis ,animal diseases ,Creutzfeldt-Jakob Syndrome ,Microbiology ,2-Propanol ,Fixatives ,Creutzfeldt Jacob Disease ,medicine ,Blood-Borne Pathogens ,Humans ,Fixation (histology) ,Disinfection methods ,biology ,medicine.diagnostic_test ,Ethanol ,business.industry ,General Medicine ,Stainless Steel ,Surgical Instruments ,nervous system diseases ,Disinfection ,Infectious Diseases ,Blood ,Alcohols ,Surgical instrument ,biology.protein ,Equipment Contamination ,Spongiform encephalopathy ,Prion Proteins ,Protein A ,business - Abstract
While developing a new protein removal test for the quality control of surgical instrument cleaning, it was noted that alcohol firmly binds blood to stainless steel. Creutzfeldt-Jacob disease is one of the transmissible spongiform encephalopathies (TSE) that has been transmitted between humans and chimpanzees by electroencephalogram electrodes, previously 'sterilized' using ethanol and formaldehyde. Although ethanol has a bactericidal action, it also binds protein to metal. Prion proteins found in TSE are thought to be the causal agents of spongiform disease and it is likely that these proteins are also bound to the stainless steel of surgical instruments by alcohols. Where spongiform disease is a possibility, alcohol, and probably formaldehyde, should not be used to decontaminate neurosurgical instruments.
- Published
- 2003
44. A discrepancy between Tc-99m HMPAO SPECT and Tc-99m ECD SPECT in Creutzfeldt-Jacob disease
- Author
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Toru Ishida, Naohiro Tsuyuguchi, Ichiro Sunada, and Shinichi Sakamoto
- Subjects
Male ,Single-photon emission computed tomography ,Creutzfeldt-Jakob Syndrome ,Diagnosis, Differential ,Technetium Tc 99m Exametazime ,Creutzfeldt Jacob Disease ,Physiology (medical) ,mental disorders ,Medicine ,Humans ,Cysteine ,Tc-99m HMPAO ,Tomography, Emission-Computed, Single-Photon ,medicine.diagnostic_test ,business.industry ,Tc-99m-ECD ,General Medicine ,Organotechnetium Compounds ,Middle Aged ,Neurology ,Surgery ,Neurology (clinical) ,Ethyl cysteinate dimer ,Radiopharmaceuticals ,business ,Nuclear medicine ,Perfusion - Abstract
We observed a discrepancy between the perfusion patterns seen in single photon emission computed tomography (SPECT) images obtained using technetium-99m hexamethyl propylene amine oxime (HMPAO) and SPECT images obtained using technetium-99m ethyl cysteinate dimer (ECD) in an 84-year-old man with Creutzfeldt-Jacob disease (CJD). HMPAO-SPECT demonstrated a reduction in perfusion in the parieto-temporal regions, especially the left temporal area. However, ECD-SPECT revealed a significant reduction in the bifrontal regions. At autopsy, the characteristic pathological findings of CJD, spongiform vacuolation, were most prominent in the bifrontal regions. Thus, the clinical features and the pathological findings were more closely correlated with the ECD-SPECT images than the HMPAO-SPECT images.
- Published
- 2003
45. A unique case of sporadic Creutzfeldt-Jacob disease presenting as progressive supranuclear palsy
- Author
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Teruyuki Hirano, Tetsuyuki Kitamoto, Tatsuhumi Murakami, Eiichiro Uyama, Shuji Mita, Munehisa Shimamura, and Makoto Uchino
- Subjects
Pathology ,medicine.medical_specialty ,Frontal cortex ,Severity of Illness Index ,Creutzfeldt-Jakob Syndrome ,Progressive supranuclear palsy ,Central nervous system disease ,Diagnosis, Differential ,Degenerative disease ,Radiologic sign ,Creutzfeldt Jacob Disease ,mental disorders ,Internal Medicine ,medicine ,Humans ,Tomography, Emission-Computed, Single-Photon ,business.industry ,Biopsy, Needle ,General Medicine ,Middle Aged ,medicine.disease ,Immunohistochemistry ,Magnetic Resonance Imaging ,nervous system diseases ,Needle biopsy ,Female ,Supranuclear Palsy, Progressive ,medicine.symptom ,business ,Myoclonus ,Follow-Up Studies - Abstract
We report a Japanese case of sporadic Creutzfeldt-Jakob disease (CJD) presenting as progressive supranuclear palsy. For 2 years after onset, neurological deficits had slowly progressed but neither myoclonus nor periodic synchronous discharge was observed. Diffusion-weighted image (DWI) showed unique high signal lesions in the bilateral frontal cortex, left parietooccipital and occipital cortices, but there was nearly no change eight months later. Needle biopsy revealed deposition of prion protein of a patchy/perivacuolar type with spongiform degeneration. Thus, the phenotype of sporadic CJD seems variable and DWI should be performed, even in atypical cases lacking the characteristics of CJD.
- Published
- 2003
46. Rapidly Progressive Dementia and Creutzfeldt-Jacob Disease: A Case Report
- Author
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Soydan Ince, Sefer Günaydin, Özlem Çokar, Zulfikar Memis, Belgin Petek Balcı, and Birgül Baştan
- Subjects
Rapidly progressive dementia ,Aging ,Pediatrics ,medicine.medical_specialty ,Creutzfeldt Jacob Disease ,business.industry ,General Neuroscience ,Medicine ,Neurology (clinical) ,Geriatrics and Gerontology ,business ,Developmental Biology - Published
- 2014
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47. Phenothiazines: potential management of Creutzfeldt-Jacob disease and its variants
- Author
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Leonard Amaral and Jette E. Kristiansen
- Subjects
Microbiology (medical) ,Tioridazina ,business.industry ,Prions ,Autopsy ,General Medicine ,Disease ,New variant ,Creutzfeldt-Jakob Syndrome ,nervous system diseases ,Neuroblastoma cell ,Infectious Diseases ,Creutzfeldt Jacob Disease ,Phenothiazines ,Clinical diagnosis ,Immunology ,Tumor Cells, Cultured ,Medicine ,Humans ,Pharmacology (medical) ,business ,Antibacterial agent - Abstract
Creutzfeldt-Jakob disease acquired from bovines (nvCJD) has been responsible for nearly 100 deaths in the UK and thousands more may die in the years to come. New variant CJD (nvCJD) is incurable and although clinical diagnosis is becoming more precise, the diagnosis is only certain at autopsy. Phenothiazine derivatives inhibit production of prions, the disease causing agent, in cultured neuroblastoma cells, and an advanced case of nvCJD was recently brought to remission by the use of these agents in combination with an antimalarial. In this review we present direct and circumstantial evidence in support of a model describing the manner by which the intracellular antimicrobial activity of phenothiazines might cause the destruction of intracellular prions.
- Published
- 2001
48. Plasma-Based Sterilization
- Author
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OLD DOMINION UNIV NORFOLK VA DEPT OF ELECTRICAL AND COMPUTER ENGINEERING, Laroussi, Mounir, OLD DOMINION UNIV NORFOLK VA DEPT OF ELECTRICAL AND COMPUTER ENGINEERING, and Laroussi, Mounir
- Abstract
Rapid, safe, and effective sterilization is of the utmost importance when it comes to protecting the public in general and hospital patients in particular. Today, public health institutions face unprecedented challenges due to the advent of heat sensitive reusable medical tools and due to the appearance of heat resistant microorganisms such as prion, the protein which causes Creutzfeldt-Jacob disease, more commonly known as "mad cow disease". Conventional sterilization methods such as autoclaving and Ethylene Oxide (EtO) are inadequate in these cases. Non-equilibrium "cold" plasmas have recently been shown to be a very promising alternative, potentially capable of overcoming the above-mentioned challenges. In addition to not damaging the articles to be sterilized, cold plasmas proved to be very effective due to the synergistic effects of free radicals and UV photons, which interact with the cells of microorganisms on the atomic and molecular levels., Pub. in International Conference on Phenomena in Ionized Gases (26th), v4 p11-12, July 2003. This article is from ADA421147 International Conference on Phenomena in Ionized Gases (26th) Held in Greifswald, Germany on 15-20 July 2003. Proceedings, Volume 4
- Published
- 2003
49. Ophthalmologische Erstmanifestation einer Creutzfeldt-Jacob-Erkrankung
- Author
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A.-C. Haller, B. Schroeder, and W. Wiegand
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Ophthalmology ,medicine.medical_specialty ,Creutzfeldt Jacob Disease ,business.industry ,medicine ,business ,Dermatology - Published
- 2009
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50. Hemidystonia as initial manifestation of sporadic Creutzfeldt-Jakob disease
- Author
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Didier Hannequin, Annie Laquerrière, Lucie Guyant-Maréchal, E. Gérardin, D. Maltete, Olivier Martinaud, B Mihout, Service de neurologie [Rouen], CHU Rouen, Normandie Université (NU)-Normandie Université (NU), Génétique médicale et fonctionnelle du cancer et des maladies neuropsychiatriques, Université de Rouen Normandie (UNIROUEN), Normandie Université (NU)-Normandie Université (NU)-Institut National de la Santé et de la Recherche Médicale (INSERM), Service d'imagerie médicale [CHU Rouen], Hôpital Charles Nicolle [Rouen], Normandie Université (NU)-Normandie Université (NU)-CHU Rouen, Normandie Université (NU)-Normandie Université (NU)-Université de Rouen Normandie (UNIROUEN), Normandie Université (NU), Service d'Anatomie et Cytologie Pathologique [CHU Rouen], Breton, Céline, and Hôpital Charles Nicolle [Rouen]-CHU Rouen
- Subjects
Pathology ,medicine.medical_specialty ,MESH: Dystonia ,MESH: Cognition ,03 medical and health sciences ,0302 clinical medicine ,Creutzfeldt Jacob Disease ,Basal ganglia ,medicine ,MESH: Neurologic Examination ,MESH: Functional Laterality ,[SDV.NEU] Life Sciences [q-bio]/Neurons and Cognition [q-bio.NC] ,Motor activity ,ComputingMilieux_MISCELLANEOUS ,030304 developmental biology ,Dystonia ,0303 health sciences ,MESH: Humans ,business.industry ,Disease progression ,MESH: Creutzfeldt-Jakob Syndrome ,Sporadic Creutzfeldt-Jakob disease ,Creutzfeldt-Jakob Syndrome ,medicine.disease ,MESH: Motor Activity ,Neurology ,MESH: Disease Progression ,[SDV.NEU]Life Sciences [q-bio]/Neurons and Cognition [q-bio.NC] ,Neurology (clinical) ,business ,MESH: Female ,030217 neurology & neurosurgery - Abstract
International audience
- Published
- 2006
- Full Text
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