scholarly journals Creutzfeldt-Jakob Disease as a Cause of Cognitive Decline and Seizures in the Elderly: Diagnostic Pointers and Strategy for Investigation

2011 ◽  
Vol 2011 ◽  
pp. 1-4 ◽  
Author(s):  
R. Williams ◽  
F. Cresswell ◽  
M. McClure ◽  
R. Lane
Keyword(s):  
Cognitive Decline ◽  
Clinical Features ◽  
The Elderly ◽  
Postmortem Brain ◽  
High Signal ◽  
Rapid Decline ◽  
Spongiform Encephalopathy ◽  
History Of ◽  
Conscious Level ◽  
Jakob Disease

Cognitive decline affects one in twenty people over the age of 65. There is often a paucity of clues as to the underlying pathology, and while the diagnosis will usually prove to be either Alzheimer’s disease or vascular dementia, there may be clinical features suggesting rarer alternatives. This case of a 71-year-old lady with a 3-month history of progressive cognitive decline illustrates clinical features suggestive of Creutzfeltd-Jakob disease such as rapid decline in conscious level and myoclonic jerking. Diagnosis was confirmed by 3 means: (1) Electroencephalogram demonstrating periodic sharp wave complexes, (2) MRI brain showing cortical ribboning and high signal in the caudate nucleus, and (3) presence of protein S100 and protein14-3-3 in the cerebrospinal fluid. Postmortem brain histology confirmed a typical spongiform encephalopathy. Establishing an underlying aetiology is dementia is important not only for prognostic reasons but in order to detect potentially reversible causes. In cases of an atypical dementing illness our proposed investigations may assist in confirming or excluding underlying Creutzfeltd-Jakob disease.

scholarly journals Kuru, the First Human Prion Disease

Viruses ◽  
2019 ◽  
Vol 11 (3) ◽  
pp. 232 ◽  
Author(s):  
Paweł Liberski ◽  
Agata Gajos ◽  
Beata Sikorska ◽  
Shirley Lindenbaum
Keyword(s):  
Electron Microscopy ◽  
Confocal Microscopy ◽  
Prion Disease ◽  
Amyloid Plaques ◽  
Human Medicine ◽  
Spongiform Encephalopathy ◽  
Biomedical Sciences ◽  
Human Prion Disease ◽  
History Of ◽  
Jakob Disease

Kuru, the first human prion disease was transmitted to chimpanzees by D. Carleton Gajdusek (1923–2008). In this review, we summarize the history of this seminal discovery, its anthropological background, epidemiology, clinical picture, neuropathology, and molecular genetics. We provide descriptions of electron microscopy and confocal microscopy of kuru amyloid plaques retrieved from a paraffin-embedded block of an old kuru case, named Kupenota. The discovery of kuru opened new vistas of human medicine and was pivotal in the subsequent transmission of Creutzfeldt–Jakob disease, as well as the relevance that bovine spongiform encephalopathy had for transmission to humans. The transmission of kuru was one of the greatest contributions to biomedical sciences of the 20th century.

scholarly journals Cardiomyopathy associated with Ceutzfeld–Jakob disease: a diagnosis of exclusion: a case report

2020 ◽  
Vol 4 (1) ◽  
pp. 1-5
Author(s):  
Osnat Itzhaki Ben Zadok ◽  
Katia Orvin ◽  
Edna Inbar ◽  
Eldad Rechavia
Keyword(s):  
Safety Concern ◽  
Brain Mri ◽  
Prnp Gene ◽  
Mortality Data ◽  
High Signal ◽  
Spongiform Encephalopathy ◽  
Diffusion Weighted Images ◽  
Jakob Disease ◽  
Magnetic Resonance Imaging Mri ◽  
Work Up

Abstract Background Creutzfeldt–Jakob disease (CJD), the most common prion disease in humans, is primarily known for its adverse neurological impact and inevitable mortality. Data regarding myocardial involvement in CJD are scarce. Case summary A 54-year-old female patient, presented with progressive effort dyspnoea, was diagnosed with unexplained non-ischaemic cardiomyopathy. An extensive cardiac work-up including cardiac magnetic resonance imaging (MRI) did not reveal any underlying aetiology. Simultaneously, the patient developed involuntary limb movements and progressive cognitive decline. Thalamic high-signal abnormalities on diffusion-weighted images were apparent on brain MRI. Based on these findings, she was subsequently referred to a neurology department, where she suddenly died the day after her admission. Brain autopsy demonstrated spongiform encephalopathy. A genetic analysis performed to her son revealed a mutation in the PRNP gene; all of these were consistent with CJD. Discussion This case describes the clinical association of CJD and cardiomyopathy and the diagnosis prion-induced cardiomyopathy by exclusion. It is not inconceivable that the coexistence of these two clinical entities may be related to genetic expression and contemporaneously deposition of infectious prions in myocardial muscle and brain tissue. Awareness of this possible association could be of important public-safety concern, and merits further collaborative cardiac-neurological work-up to elucidate this phenotype among patients with unexplained cardiomyopathy with neurological symptoms that resemble CJD.

scholarly journals Rapidly Progressing Sporadic Creutzfeldt-Jakob Disease Presenting as a Stroke

2018 ◽  
Vol 10 (3) ◽  
pp. 261-265 ◽  
Author(s):  
Maxim Oliver ◽  
Lisa Dyke ◽  
Alex Rico ◽  
Mario Madruga ◽  
Jorge Parellada ◽  
...  
Keyword(s):  
Prion Disease ◽  
Neurological Disorders ◽  
Hospice Care ◽  
Clinical Suspicion ◽  
Spongiform Encephalopathy ◽  
Medical Procedures ◽  
Human Prion Disease ◽  
Progressive Dementia ◽  
History Of ◽  
Jakob Disease

Sporadic Creutzfeldt-Jakob disease (sCJD) is a rare, fatal human prion disease that is characterized by progressive dementia and neurologic degeneration. It can mimic multiple other neurological disorders, and a high index of clinical suspicion is necessary to make a diagnosis. A 74-year-old woman with a 3-month history of a stroke and progressive neurologic deterioration was found to have sCJD. She expired within a week of her diagnosis. Autopsy revealed spongiform encephalopathy consistent with prion disease, and genetic analysis revealed 129 polymorphism and no pathologic mutation, confirming the diagnosis of nonfamilial human prion disease. No pathologic evidence of a stroke was found. Awareness of the disease by clinicians is important not only at the time of initial presentation but also during the following months. Since there is no treatment, invasive medical procedures should be limited to only those that are required for either diagnosis or hospice care.

scholarly journals PROFIL PENURUNAN FUNGSI KOGNITIF PADA LANSIA DI YAYASAN-YAYASAN MANULA DI KECAMATAN KAWANGKOAN

e-CliniC ◽  
2013 ◽  
Vol 1 (1) ◽  
Author(s):  
Rachel Mongisidi ◽  
Rizal Tumewah ◽  
Mieke A. H. N. Kembuan
Keyword(s):  
Cognitive Decline ◽  
Elderly People ◽  
Sex Education ◽  
Cognitive Functions ◽  
The Elderly ◽  
Cross Sectional Study ◽  
Cross Sectional ◽  
Number Of Children ◽  
History Of ◽  
Descriptive Survey

Background: The cognitive impairment in elderly people is the major cause of the inability to execute the daily activity and of the major reason of the happening of care-dependence. There has not been any research about the profile of cognitive functions impairment in the District of Kawangkoan. Thus, the purpose of this research was to obtain the profile of cognitive functions impairment in the District of Kawangkoan. Methods: This was a descriptive survey with the design of cross-sectional study, which rolls out, the results of MMSE, TMT A, TMT B and CDT; the age, sex, education, occupations, family history of cognitive decline, marital status, the number of children, and the history of stroke and DM, and also the smoking profile of the participants. The subjects of this research were the elderly people that were the members of the old people foundations in the District of Kawangkoan. Results: There were 61 participants of this research, consisting of four males (6.6%) and 57 (94.4%) females participants. The result of this research shows that the MMSE scores were mostly normal (72.1%), the TMT A and the TMT B scores were both mostly abnormal (95.1% and the latter 72.1%), the CDT scores mostly normal (67.2%). In all these three instruments have the absolute result that was, the elderly people with older age has more numbers of participants with cognitive functions impairment than the younger age. The result also shows that the group of subjects with higher education has less numbers of cognitive decline subjects than the group of subjects with lower education. The subjects that had a former occupation as a teacher have the normal cognitive functions as the results of all the tests. Subjects that were married and have children, and do not have a history of stroke, DM and smoking got the score of normal cognitive functions. Conclusions: The cognitive functions of elderly people based on the MMSE and CDT scores, show that most of them have a normal cognitive functions where as the result of the TMT part A and the TMT part B show the opposite result that is most of the participants have an abnormal score. Key words: Cognitive functions impairment – Elderly peopleLatar Belakang: Penurunan fungsi kognitif pada lansia merupakan penyebab terbesar terjadinya ketidakmampuan dalam melakukan aktifitas normal sehari-hari, dan juga merupakan alasan tersering yang menyebabkan terjadinya ketergantungan terhadap orang lain untuk merawat diri sendiri. Belum pernah ada penelitian tentang profil penurunan fungsi kogntif di Kec. Kawangkoan. Tujuan dari penelitian ini untuk mengetahui profil penurunan fungsi kognitif pada lansia di Kecamatan Kawangkoan. Metode: Penelitian survey deskriptif dengan rancangan penelitian potong lintang, yang memaparkan data hasil pemeriksaan MMSE, TMT A, TMT B, CDT, umur, jenis kelamin; riwayat pendidikan, pekerjaan, keluarga dengan penurunan fungsi kognitif, status pernikahan dan jumlah anak, riwayat penyakit stroke, diabetes mellitus dan merokok. Subjek penelitian adalah para lansia yang menjadi anggota dari yayasan-yayasan manula yang ada di Kec. Kawangkoan. Hasil: Terdapat 61 sampel dari total 65 subjek penelitian. Sampel terdiri dari 4 orang berjenis kelamin laki-laki (6.6%) dan 57 perempuan (94.4%). Penelitian menunjukkan hasil pemeriksaan MMSE menunjukkan 72.1% normal, TMT A 95.1% tidak normal, pemeriksaan TMT B 72.1% tidak normal dan CDT67.2% normal. Pada hasil pemeriksaan ditemukan hasil absolut pada ketiga jenis pemeriksaan ini yaitu lebih banyak terdapat penurunan fungsi kognitif pada lansia dengan umur yang lebih tua. Profil fungsi kognitif berdasarkan riwayat pendidikan menunjukkan bahwa sampel dengan pendidikan kurang dari sembilan tahun sebagian besar mengalami penurunan fungsi kogntif. Riwayat pekerjaan guru seluruhnya memiliki hasil fungsi kognitif yang normal sedangkan sampel yang riwayat pekerjaannya petani lebih banyak mengalami penurunan fungsi kognitif. Sampel yang tidak menikah dan tidak memiliki anak memiliki hasil penurunan fungsi kognitif yang dominan daripada yang menikah dan memiliki anak. Pada hasil ditemukan bahwa sampel yang memiliki riwayat stroke, DM dan merokok positif memiliki hasil penurunan fungsi kognitif yang dominan disbanding yang tidak memiliki riwayat stroke, DM dan merokok. Kesimpulan: Hasil pemeriksaan fungsi kognitif berdasarkan pemeriksaan MMSE dan CDT menunjukkan bahwa sebagian besar lansia masih memiliki fungsi kogntif yang normal sedangkan pada TMT A dan TMT B ditemukan hasil sebaliknya di mana ditemukan hasil sebagian besar mengalami penurunan fungsi kognitif. Kata Kunci: Penurunan fungsi kognitif – Lansia

scholarly journals Negative Diffusion Weighted Imaging on Magnetic Resonance Imaging of the Brain in Creutzfeldt–Jakob Disease

2020 ◽  
Vol 2020 ◽  
pp. 1-4
Author(s):  
Elijah Lackey ◽  
Deepal P. Shah-Zamora ◽  
Jodi Hawes ◽  
Andy J. Liu
Keyword(s):  
Magnetic Resonance Imaging ◽  
Magnetic Resonance ◽  
Diffusion Weighted Imaging ◽  
Caucasian Woman ◽  
Rapid Decline ◽  
Resonance Imaging ◽  
Diffusion Weighted ◽  
History Of ◽  
Jakob Disease ◽  
The Brain

A 76-year-old Caucasian woman initially presented to the Duke Memory Disorders clinic with a 9-month history of a rapid decline in cognitive, motor, and neuropsychiatric function. On initial presentation, the patient required assistance with activities of daily living. On neurological examination, she was found to have Gerstmann’s syndrome along with appendicular apraxia. A positional tremor was noted without myoclonus or fasciculations. She had a paucity of speech and was unable to write her own name. Snout and grasp reflexes were present. Episodes of inappropriate laughter were noted during the exam. She was admitted to the inpatient neurology service for further evaluation. The Diffusion Weighted Imaging sequence on Magnetic Resonance Imaging of the brain was negative for restricted diffusion. An electroencephalogram was unremarkable. Cerebrospinal fluid analysis for Real-Time Quaking-Induced Conversion assay was positive along with an elevated 14-3-3 and increased total Tau protein levels. There was no family history of Creutzfeldt–Jakob disease. The cerebral spinal fluid results were consistent with a diagnosis of Creutzfeldt–Jakob disease, despite the negative MRI brain findings.

scholarly journals Trajectories of Frailty and Cognitive Decline Among Older Mexican Americans

2020 ◽  
Vol 75 (8) ◽  
pp. 1551-1557 ◽  
Author(s):  
Bret T Howrey ◽  
Soham Al Snih ◽  
Joyce A Middleton ◽  
Kenneth J Ottenbacher
Keyword(s):  
Cognitive Decline ◽  
Mexican Americans ◽  
Church Attendance ◽  
Handgrip Strength ◽  
The Elderly ◽  
Rapid Decline ◽  
Cognitively Impaired ◽  
Increased Risk ◽  
Risk Of Falls ◽  
Rapid Cognitive Decline

Abstract Background Progressive physical frailty and cognitive decline in older adults is associated with increased risk of falls, disability, institutionalization, and mortality; however, there is considerable heterogeneity in progression over time. We identified heterogeneous frailty and cognitive decline trajectory groups and examined the specific contribution of health conditions to these trajectories among older Mexican origin adults. Methods We use a sample from the Hispanic Established Population for the Epidemiological Study of the Elderly (HEPESE) with at least two measures of frailty criteria during 18 years follow-up: slow gait, weak handgrip strength, exhaustion, and unexplained weight loss (n = 1362, mean age 72). Cognition was measured using the Mini-Mental State Examination (MMSE). Results Using group-based trajectory models we identified three frailty groups—non-frail (n = 331), moderate progressive (n = 855), and progressive high (n = 149)—and three cognitive decline groups—non-cognitively impaired (476), moderate decline (677) and rapid decline (n = 209). The probability of membership in a high-frailty group given membership in a progressive cognitive decline group was 63%, while the probability of being in a non-frail group given membership in a non-cognitively impaired group was 68%. Predictors of membership into both the progressive high frailty and rapid cognitive decline groups combined were low education and diabetes. Weekly church attendance was associated with a 66% reduction in the odds of being in the combined groups. Conclusions Interventions to reduce frailty rates and cognitive decline might focus on the management of underlying chronic disease and on increasing participation in activities outside the home.

scholarly journals The controversial Third Reich history of Hans Creutzfeldt: was he a supporter or just another adept of the “hand washing policy”?

2021 ◽  
Vol 79 (1) ◽  
pp. 84-87
Author(s):  
Paulo Eduardo Mestrinelli CARRILHO ◽  
Ricardo NITRINI
Keyword(s):  
Transmissible Spongiform Encephalopathy ◽  
Third Reich ◽  
Spongiform Encephalopathy ◽  
Nazi Ideology ◽  
History Of ◽  
Jakob Disease ◽  
The Third Reich ◽  
Initial Description ◽  
Post War ◽  
Aktion T4

ABSTRACT Creutzfeldt-Jakob disease (CJD) is a transmissible spongiform encephalopathy whose initial description is associated with two German authors, Alfons Maria Jakob and Hans Gerhard Creutzfeldt. As polemic as the issue about the Creutzfeldt’s merit in the first description of the disease, is his history during the Third Reich. Some evidence pointed to the idea that he was essentially against the Nazi ideology, though some did not. He was an official member of the SS, but his own wife was convicted by a Nazi court. Some authors have argued that Creutzfeldt helped save many patients during Aktion T4 operation, but, in fact, he could have done more. Even during the post-war period, he sent a letter to authorities reporting the name of a Nazi physician who worked as a medical reviewer at the euthanasia court, but he did not proceed any further when his letter initially failed to start an investigation.

DURAL AVF CAUSING A TREATABLE CJD LIKE COGNITIVE DYSFUNCTION

2015 ◽  
Vol 86 (11) ◽  
pp. e4.135-e4
Author(s):  
Anna Randall ◽  
Richard James Booth Ellis ◽  
Brython Hywel ◽  
Christopher Andrew McCoy ◽  
Rhys Davies ◽  
...  
Keyword(s):  
Cognitive Decline ◽  
Psychiatric Disturbance ◽  
Gradual Improvement ◽  
Av Fistula ◽  
Sagittal Sinus ◽  
Retrograde Filling ◽  
History Of ◽  
Jakob Disease ◽  
Subacute Dementia ◽  
Extrapyramidal Features

A 67-year-old woman presented with a seven-month history of progressive psychiatric disturbance and sub-acute cognitive decline mimicking Creuztfeldt-Jakob Disease.She presented initially to Psychiatry with worsening anxiety, depression, lassitude, impaired concentration and insomnia. She was given a diagnosis of agitated depression.She subsequently experienced episodes of disorientation, indecision and inability to complete routine, learned tasks as well as visual hallucinations. Initial investigations for causes of subacute dementia were unremarkable including a non-diagnostic MRI. However, five months into her presentation, there was further rapid deterioration with fluctuating consciousness and deteriorating mobility. She became bedbound, incontinent and cortically blind. She had right hemiparesis, hemineglect, extrapyramidal features, and extensor plantars.A 4D computerised tomography angiogram demonstrated a dural arteriovenous (AV) fistula with retrograde filling of superior sagittal sinus and cortical venous congestion. She underwent emergency Onyx embolisation. Over a few weeks, there was gradual improvement in her anxiety, cognition and mobility. She had a mild residual right hemiparesis.Intracranial dural AV fistulae can present with a spectrum of neurological symptoms, including cognitive decline. A lowhigh index of suspicion combined with close liaison between Neuroradiology, Neurology and Neurosurgery ensures prompt diagnosis and maximises the potential for cognitive recovery.

scholarly journals Community-acquired pneumonia and pneumococcal vaccination in the elderly

2016 ◽  
Vol 84 (2) ◽  
pp. 23-25
Author(s):  
Charles Yin ◽  
Jeffrey Law
Keyword(s):  
Streptococcus Pneumoniae ◽  
Healthcare System ◽  
Cause Of Death ◽  
Clinical Features ◽  
Pneumococcal Vaccination ◽  
The Elderly ◽  
Community Acquired Pneumonia ◽  
Pneumococcal Vaccines ◽  
Significant Challenge ◽  
History Of

Community-acquired pneumonia (CAP), most often caused by infection with the Gram-positive diplococcus Streptococcus pneumoniae, remains a leading cause of death in Canada amongst the elderly. With an aging population in Canada, CAP will soon be a significant challenge to the healthcare system in this country. In this article, we review the characteristics of CAP in the elderly, including its epidemiology, etiology and clinical features. We then provide an overview and history of pneumococcal vaccines and present current recommendations for S pneumoniae vaccination in Canada.

Informant Questionnaire on Cognitive Decline in the Elderly--Farsi Version

2019 ◽  
Author(s):  
Mahshid Foroughan ◽  
Zahra Jafari ◽  
Ida Ghaemmagham Farahani ◽  
Vahid Rashedi
Keyword(s):  
Cognitive Decline ◽  
The Elderly ◽  
Farsi Version
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