scholarly journals When is a stroke not a stroke? An unusual mimic presenting to AMU

2016 ◽  
Vol 15 (1) ◽  
pp. 33-36
Author(s):  
Shyamal Patel ◽  
◽  
Nicholas Smallwood ◽  
Hyacinth-John Abu ◽  
Youssif Abousleiman ◽  
...  
Keyword(s):  
Prion Disease ◽  
Signs And Symptoms ◽  
Progressive Dementia ◽  
Medical Unit ◽  
Early Palliative Care ◽  
Acute Medical Unit ◽  
Abnormal Movements ◽  
Jakob Disease ◽  
Eeg Changes ◽  
Mri Changes

Introduction: Creutzfeldt-Jakob disease (CJD) is a rare prion disease classically manifesting with rapidly progressive dementia, abnormal movements and typical electroencephalographic (EEG) changes. Case Report: A 74 year-old Caucasian man was recently discharged from another centre diagnosed with a stroke. He re-presented to our acute medical unit with worsening symptoms, and stroke remained the working diagnosis. Collateral history revealed a progressive cognitive decline and unilateral myoclonus. Further investigations supported the diagnosis of probable CJD, confirmed by the national CJD surveillance centre. Discussion: Signs and symptoms atypical of stroke should raise the possibility of alternative diagnoses, including prion disease. CJD can present with unilateral symptoms, EEG and MRI changes. Early diagnosis prevents unnecessary investigations and treatments, allowing early palliative care input, where appropriate.

Rapidly Progressive Dementia and Coma

2019 ◽  
pp. 704-712
Author(s):  
Prasuna Kamireddi ◽  
Jason L. Siegel ◽  
Dennis W. Dickson
Keyword(s):  
Intensive Care Unit ◽  
Functional Ability ◽  
Clinical Signs ◽  
Signs And Symptoms ◽  
Cognitive Domain ◽  
Progressive Dementia ◽  
Common Causes ◽  
Jakob Disease ◽  
Clinical Signs And Symptoms ◽  
Definition Of

In most patients with dementia, the clinical signs and symptoms progress gradually over many years. However, neurointensivists may encounter patients who have rapidly progressive dementia (RPD). Often these patients need to be admitted to the intensive care unit for management of status epilepticus, agitation, or ventilation in coma. Although the prototype of RPD is Creutzfeldt-Jakob disease, this chapter reviews other common causes of RPD. An established definition of RPD does not exist, but in this chapter RPD refers to the loss of more than 1 cognitive domain and functional ability, usually occurring over a few months.

scholarly journals Impaired transmissibility of atypical prions from genetic CJDG114V

2018 ◽  
Vol 4 (4) ◽  
pp. e253 ◽  
Author(s):  
Ignazio Cali ◽  
Fadi Mikhail ◽  
Kefeng Qin ◽  
Crystal Gregory ◽  
Ani Solanki ◽  
...  
Keyword(s):  
Prion Disease ◽  
Protein Misfolding ◽  
Proteinase K ◽  
Progressive Dementia ◽  
Replication Studies ◽  
Jakob Disease ◽  
Transmission Studies

ObjectiveTo describe the clinicopathologic, molecular, and transmissible characteristics of genetic prion disease in a young man carrying the PRNP-G114V variant.MethodsWe performed genetic, histologic, and molecular studies, combined with in vivo transmission studies and in vitro replication studies, to characterize this genetic prion disease.ResultsA 24-year-old American man of Polish descent developed progressive dementia, aphasia, and ataxia, leading to his death 5 years later. Histologic features included widespread spongiform degeneration, gliosis, and infrequent PrP plaque-like deposits within the cerebellum and putamen, best classifying this as a Creutzfeldt-Jakob disease (CJD) subtype. Molecular typing of proteinase K-resistant PrP (resPrPSc) revealed a mixture of type 1 (∼21 kDa) and type 2 (∼19 kDa) conformations with only 2, rather than the usual 3, PrPSc glycoforms. Brain homogenates from the proband failed to transmit prion disease to transgenic Tg(HuPrP) mice that overexpress human PrP and are typically susceptible to sporadic and genetic forms of CJD. When subjected to protein misfolding cyclic amplification, the PrPSc type 2 (∼19 kDa) was selectively amplified.ConclusionsThe features of genetic CJDG114V suggest that residue 114 within the highly conserved palindromic region (113-AGAAAAGA-120) plays an important role in prion conformation and propagation.

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 A rare case of sporadic Creutzfeldt-Jakob disease in an 83 years old female

2021 ◽  
Vol 8 (2) ◽  
pp. 279
Author(s):  
Medo M. Kuotsu ◽  
Arjun Bal Kallupurakkal ◽  
Nyamnyei Konyak ◽  
Keisham Jaya Chanu ◽  
Vikie-o Khruomo ◽  
...  
Keyword(s):  
Prion Disease ◽  
Alpha Helix ◽  
Cellular Prion Protein ◽  
Progressive Dementia ◽  
Extrapyramidal Signs ◽  
Control And Prevention ◽  
Weighted Magnetic Resonance Imaging ◽  
Jakob Disease ◽  
Magnetic Resonance Imaging Mri ◽  
Electroencephalogram Eeg

Sporadic Creutzfeldt-Jakob disease is a rare invariably fatal neurodegenerative prion disease. Prion disease are associated with the conversion of alpha-helix rich cellular prion protein (PrPC) into a beta-structure rich insoluble conformer scrapie isoform (PrPSc) thought to be infectious isoform. Here we present a case of an 83 years old woman with findings of rapidly progressive dementia, cognitive disturbance, myoclonic jerks and extrapyramidal signs (cogwheel rigidity). Following a series of clinical and diagnostic (diffusion-weighted magnetic resonance imaging (MRI) brain, electroencephalogram (EEG)) examination she was diagnosed with sporadic Creutzfeldt-Jakob disease based on Centers for disease control and prevention (CDC) criteria.

scholarly journals RNA editing alterations define manifestation of prion diseases

2019 ◽  
Vol 116 (39) ◽  
pp. 19727-19735 ◽  
Author(s):  
Eirini Kanata ◽  
Franc Llorens ◽  
Dimitra Dafou ◽  
Athanasios Dimitriadis ◽  
Katrin Thüne ◽  
...  
Keyword(s):  
Disease Progression ◽  
Rna Editing ◽  
Prion Disease ◽  
Prion Diseases ◽  
Rapid Progression ◽  
Human Prion Disease ◽  
Progressive Dementia ◽  
Molecular Alterations ◽  
Jakob Disease ◽  
Subtype A

Prion diseases are fatal neurodegenerative disorders caused by misfolding of the normal prion protein into an infectious cellular pathogen. Clinically characterized by rapidly progressive dementia and accounting for 85% of human prion disease cases, sporadic Creutzfeldt–Jakob disease (sCJD) is the prevalent human prion disease. Although sCJD neuropathological hallmarks are well-known, associated molecular alterations are elusive due to rapid progression and absence of preclinical stages. To investigate transcriptome alterations during disease progression, we utilized tg340-PRNP129MM mice infected with postmortem material from sCJD patients of the most susceptible genotype (MM1 subtype), a sCJD model that faithfully recapitulates the molecular and pathological alterations of the human disease. Here we report that transcriptomic analyses from brain cortex in the context of disease progression, reveal epitranscriptomic alterations (specifically altered RNA edited pathway profiles, eg., ER stress, lysosome) that are characteristic and possibly protective mainly for preclinical and clinical disease stages. Our results implicate regulatory epitranscriptomic mechanisms in prion disease neuropathogenesis, whereby RNA-editing targets in a humanized sCJD mouse model were confirmed in pathological human autopsy material.

scholarly journals Case based review: Toxicology on the Acute Medical Unit

2019 ◽  
Vol 18 (1) ◽  
pp. 23-32
Author(s):  
A Achilleos ◽  
◽  
Oliver Collas ◽  
Nicholas Murch ◽  
◽  
...  
Keyword(s):  
Hospital Admissions ◽  
Clinical Course ◽  
Signs And Symptoms ◽  
Organic Substances ◽  
Front Line ◽  
Medical Unit ◽  
Acute Medical Unit ◽  
Clinical Vignettes ◽  
The Uk ◽  
Case Based

Accidental and intentional poisoning from prescribed, illicit and organic substances remains a major cause of morbidity and mortality worldwide and accounts for just under 1% of the total number of NHS hospital admissions, or around 170,000, a year in the UK. A knowledge of the constellation of signs and symptoms that constitute specific poisonings (referred to as toxidromes) may enable early empirical decontamination, antidote administration, enhanced elimination and supportive care, and may also help to predict the clinical course. This paper presents a series of clinical vignettes to demonstrate emerging presentations in toxicology to help inform the practice of Acute Physicians, who alongside colleagues in Emergency Medicine and Critical Care, are at the front line of diagnosing and treating poisoned patients.

scholarly journals Acute medical unit clinical skills lab

2019 ◽  
Vol 6 (Suppl 1) ◽  
pp. 140-140
Author(s):  
Sarb Clare ◽  
Joe Wheeler
Keyword(s):  
Clinical Skills ◽  
Medical Unit ◽  
Acute Medical Unit ◽  
Skills Lab

scholarly journals Causes of discharge delays from the acute medical unit (AMU) in a tertiary level teaching hospital, Brunei Darussalam

2021 ◽  
pp. 201010582110061
Author(s):  
Dayang Nur Hilmiyah binti Awang Husaini ◽  
Justin Fook Siong Keasberry ◽  
Khadizah Haji Abdul Mumin ◽  
Hanif Abdul Rahman
Keyword(s):  
Observational Study ◽  
Discharge Planning ◽  
Patient Factors ◽  
Medical Unit ◽  
Prolonged Length ◽  
Acute Medical Unit ◽  
Brunei Darussalam ◽  
Medical Issues ◽  
Hospital Factors ◽  
Prolonged Hospital

Background: Many patients admitted to the acute medical unit experience a prolonged length of stay in hospital due to discharge delays. Consequently, this may impact the patients, healthcare institution and national economy in terms of patient safety, decreased hospital capacity, lost patient workdays and financial performance. Objectives: The main aim of this observational study was to identify the causes of discharge delays among acute medical unit patients admitted in the Raja Isteri Pengiran Anak Saleha Hospital, Brunei. Methods: A retrospective observational study, with data of patients admitted to the acute medical unit collected from Brunei Health Information Systems between September and December 2018. Statistical analyses were performed to obtain relevant results and any statistically significant associations. Results: A total of 357 patients were admitted to the acute medical unit over the 4-month period; 218 patients (61.1%) experienced discharge delays. Of these 218 patients, 158 patients (72.5%) encountered discharge delays mainly due to intrinsic patient factors, while the discharge delays in 88 patients (40.4%) were attributed to hospital factors. The main reason for discharge delays for patient factors was slow recovery among 67 patients (30.7%), whereas for hospital factors it was the weekend limitation of services available in 23 patients (10.6%). Conclusions: There were various causes of discharge delays identified among the 218 acute medical unit patients who experienced discharge delays. Older patients with frailty, polypharmacy and complex medical issues were more likely to have a prolonged hospital stay in the acute medical unit. Stringent inclusion criteria, increasing discharge planning as well as an effective multidisciplinary approach will aid in reducing discharge delays from the acute medical unit.

Prevalence of signs of dysphagia and associated risk factors in geriatric patients admitted to an acute medical unit

2021 ◽  
Vol 41 ◽  
pp. 208-216 ◽  
Author(s):  
Maria Dissing Olesen ◽  
Robert Mariusz Modlinski ◽  
Simon Hosbond Poulsen ◽  
Pernille Mølgaard Rosenvinge ◽  
Henrik Højgaard Rasmussen ◽  
...  
Keyword(s):  
Risk Factors ◽  
Geriatric Patients ◽  
Medical Unit ◽  
Acute Medical Unit ◽  
Associated Risk Factors
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