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When patients with Creutzfeldt-Jakob disease are misdiagnosed as having nonconvulsive status epilepticus.
Epilepsia
January 2025
Epilepsy Unit, Hôpital Gui de Chauliac, Montpellier, France.
Contemporary studies report nonconvulsive status epilepticus (NCSE) in Creutzfeldt-Jakob disease (CJD), based on benzodiazepine (BZP)-responsive epileptiform discharges on the electroencephalogram (EEG), with the following false syllogism: (1) intravenous (IV) administration of BZPs usually suppress ictal activity in NCSE; (2) in CJD, periodic sharp wave complexes (PSWCs) are suppressed by IV BZPs; (3) therefore, these patients have NCSE. This is a simplistic and invalid conclusion, because authors of 20th-century science reports have clearly shown that IV BZPs, short-acting barbiturates, and drugs with no antiseizure effects, such as chloral hydrate and IV naloxone, suppress PSWCs, but patients fall asleep with no clinical improvement. In contrast, IV methylphenidate transiently improves both the EEG and clinical states.
View Article and Find Full Text PDFBackground: Patients with the precipitous onset of cognitive symptoms (i.e., suspected rapid progressive dementia [RPD]) may continue to decline (true RPD) or may stabilize or improve (non-RPD).
View Article and Find Full Text PDFDetermination of prion proteins in the diagnosis of Creutzfeldt-Jakob disease using RT-QuIC: A case report from northeastern Colombia.
Biomedica
November 2024
Departmento de Radiología, Hospital Central de la Policía, Bogotá, D. C., Colombia.
Creutzfeldt-Jakob disease is a rare neurodegenerative disease caused by prions. We present the case of a woman in the seventh decade of life with rapidly progressive dementia and myoclonus. Her brain magnetic resonance imaging revealed lesions in the basal nuclei, and the electroencephalogram showed periodic bilateral epileptiform discharges.
View Article and Find Full Text PDFApparent Diffusion Coefficient Measurements. A Reliable Tool for the Diagnosis of Creutzfeldt-Jakob Disease.
Arch Med Res
October 2024
Genetics Department, National Institute of Neurology and Neurosurgery, Manuel Velasco Suarez, Mexico City, Mexico. Electronic address:
Background And Purpose: Creutzfeldt-Jakob disease (CJD) is a rare, rapidly fatal neurodegenerative disorder. The gold standard test for a positive diagnosis of definite CJD is histopathological confirmation, usually post-mortem; however, an autopsy study is not always feasible in all health settings.
Materials And Methods: We performed a cross-sectional analysis of a retrospectively enrolled cohort of patients with suspected prion disease between 2008 and 2019.
An Atypical Case of Creutzfeldt-Jakob Syndrome Presenting with Cacosmia and Amyloid Positivity.
J Alzheimers Dis Rep
July 2024
Department of Clinical Research in Neurology, Center for Neurodegenerative Diseases and the Aging Brain, "Pia Fondazione Cardinale G. Panico", University of Bari 'Aldo Moro', Tricase, Italy.
This report presents a challenging case of Creutzfeldt-Jakob Disease (CJD), a rare and rapidly progressing neurological disorder. The patient exhibited diverse and progressive neuro-psychiatric symptoms, including memory impairment, behavioral changes, and hallucinations associated with cacosmia. The diagnosis of CJD is complicated due to its variable clinical presentation, limited awareness, and the need for tissue pathology confirmation.
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