J Neurol 2013 Oct 25;260(10):2650-2. Epub 2013 Aug 25.
Dipartimento di Gerontologia, Neuroscienze ed Ortopedia, Istituto di Neurologia, Università Cattolica del Sacro Cuore, Policlinico A.Gemelli, Largo A. Gemelli 8, 00168, Rome, Italy.
Clin Neurophysiol 2021 May 20;132(8):1757-1769. Epub 2021 May 20.
Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.
Since the term Stimulus-Induced Rhythmic, Periodic, or Ictal Discharges (SIRPIDs) was introduced into the vocabulary of electrophysiologists/neurologists, there has been an ongoing debate about its significance, as well as its correlation with outcomes. SIRPIDs are frequently seen in patients who are critically ill from various causes. The literature reflects the findings of triphasic morphology, with the generalized periodic discharge (GPD) classification in many patients with SIRPIDs: toxic/metabolic encephalopathies, septic, and hypoxemic/hypercapnic encephalopathies, but also sharp periodic complexes in Creutzfeldt-Jakob disease and advanced Alzheimer's disease. Read More
Prion 2021 Dec;15(1):94-106
Department of Neurodegeneration, Ageing and Mental Health, National Epidemiology Centre, Carlos III Health Institute, Madrid, Spain.
In Spain, human transmissible spongiform encephalopathies (TSEs) have been undergoing continuous surveillance for over 25 years. In 1995, the system was launched as an EU Concerted Action, with EU surveillance network procedures being incorporated from 2002 onwards. The aim of this report was to describe performance and outcomes of this surveillance system across the period 1993-2018. Read More
Ann Geriatr Med Res 2021 Jun 14. Epub 2021 Jun 14.
Department of Physical Medicine and Rehabilitation, Dong-Eui Medical Center, Busan, Korea.
While sporadic Creutzfeldt-Jakob disease (sCJD) typically presents with neurological symptoms such as cognitive impairment, ataxia, and myoclonus, its clinical manifestations can be diverse. We report about a 70-year-old woman with sCJD who was misdiagnosed with recurrent stroke. She initially showed hemiplegia and high-intensity signals on brain diffusion-weighted magnetic resonance imaging (DWI), which corresponded to the symptoms of a stroke. Read More
Front Aging Neurosci 2021 19;13:655667. Epub 2021 May 19.
Department of Neurology, Xuanwu Hospital, Capital Medical University, Beijing, China.
Objective: The objective of the study was to explore patterns of white matter (WM) alteration in preclinical stage familial Creutzfeldt-Jakob disease (fCJD) using diffusion tensor imaging (DTI).Methods: Seven asymptomatic carriers of the G114V mutation and six non-carriers were recruited from the same fCJD kindred and follow-up obtained from all asymptomatic carriers and two non-carriers 2 years later. Overlapping WM patterns were also explored in asymptomatic carriers and symptomatic CJD patients. Read More
Prion 2021 Dec;15(1):82-86
Department of Neurology, Gifu University Graduate School of Medicine, Gifu, Japan.
Swallowing function in long-term survivors of Creutzfeldt-Jakob disease (CJD) has not been elucidated. Herein, we report a patient with MM2-cortical-type sporadic CJD (MM2C-type sCJD) with long-term preservation of pharyngeal swallowing function using videofluoroscopic (VF) examination of swallowing. A 55-year-old woman was admitted to hospital because of dyscalculia and memory disturbance 3 years after the onset of these symptoms. Read More