scholarly journals A case of Creutzfeldt-Jakob disease: diagnostic dilemmas of a rapidly fatal disease

2013 ◽  
Vol 5 (2) ◽  
pp. 10 ◽  
Author(s):  
Mirza M. Baig ◽  
Martin Phillips

Creutzfeldt-Jakob disease (CJD) is a rapidly progressive and ultimately fatal disorder of the central nervous system. It occurs worldwide with an incidence of 0.5-1 new case per million population per year. No specific treatment is available and management is limited to supportive care. Autopsy or biopsy provides a definitive diagnosis. Because of the transmissible nature of the disease and hesitancy of patients/family members to give consent for biopsy, numerous challenges in confirming the clinical diagnosis are faced by healthcare professionals. We report a case of 66-year-old male who was hospitalized due to hip fracture following a fall. Acute mental status changes followed the surgical fixation of hip fracture which triggered neurologic work up. This finally revealed suspicion and confirmation of CJD. Patient had progressive cognitive decline with akinetic mutism during further hospital stay and was later discharged home with hospice. Shorter thereafter he died at home. This case demonstrates the importance of keeping an open mind towards possibility of CJD when faced with esoteric neurologic presentations. Also this case provides insight into challenges in quarantine and sterilization of surgical instruments when these patients go through major surgeries.

Delirium is a common serious complication in dementia that is associated with poor prognosis and a high burden on caregivers and healthcare professionals. Appropriate care is therefore important at an early stage for patients with delirium superimposed on dementia To gain insight into the care of six patients with delirium superimposed on dementia, 19 semi-structured interviews were conducted focused on the experiences of caregivers and professionals. The interviews revealed four themes that appeared to play a role: 1. experiences with and views on behavioral problems of these patients, 2. recognition and diagnosis of delirium in dementia, 3. views on good care and 4. organizational aspects. Knowledge gaps about delirium in dementia, as well as ethical considerations, play an important role in organizing timely and adequate care for patients with delirium superimposed on dementia.


Author(s):  
Shazma Khan ◽  
Sara Khan

<b><i>Introduction:</i></b> Sporadic Creutzfeldt-Jakob disease (sCJD) is a transmissible disorder of the central nervous system caused by the transformation of normal prion protein into an abnormal misfolded form. The process begins spontaneously and runs a vicious cycle to cause spongiform encephalopathy, rapidly resulting in death. Amply described in the western literature, CJD is scarcely reported in Asia due to certain limitations including missed diagnosis, under-reporting, and rarity of the disease. Brain MRI, electroencephalogram, cerebrospinal fluid testing, and biopsy of the infected brain tissue support the diagnosis in cases of clinical suspicion. However, the diagnosis can still be made with limited available resources in developing countries. <b><i>Method:</i></b> A review of CJD cases evaluated in the neurology department of a tertiary care hospital in Pakistan was done from 2002 to 2018. <b><i>Results:</i></b> Eleven cases labeled as sCJD are identified based on the European MRI-CJD consortium criteria. This is the first study on CJD from Pakistan, which includes both the typical and atypical presentations. <b><i>Conclusion:</i></b> Even with limited testing available, the diagnosis of CJD can be made with confidence in the developing countries, provided the suspicion is kept high in cases of rapid onset dementia and acute behavioral changes.


2016 ◽  
Vol 29 (6) ◽  
pp. 436-439 ◽  
Author(s):  
Pierre-Luc Gamache ◽  
Maude-Marie Gagnon ◽  
Martin Savard ◽  
François Émond

This article reports the case of a 68-year-old patient with anti-HU antibodies paraneoplastic encephalitis. The clinical manifestations were atypical and the paraclinical work-up, notably the magnetic resonance imaging (MRI) showing bilateral posterior thalamic hyperintensities (pulvinar sign), misleadingly pointed towards a variant Creutzfeld–Jakob disease. After presenting the case, the differential diagnosis of the pulvinar sign is discussed along with other important diagnostic considerations.


2009 ◽  
Vol 20 (06) ◽  
pp. 348-352 ◽  
Author(s):  
Sarah A. Sydlowski ◽  
Michael J. Cevette ◽  
Jon Shallop ◽  
David M. Barrs

Background: Considered a rare disorder, superficial siderosis of the central nervous system (SSCN) has become more frequently diagnosed in recent years. As it is characterized by progressive sensorineural hearing loss, patients' needs may surpass the capability of hearing aid technology. Despite the retrocochlear nature of the disorder, patients have undergone cochlear implantation (CI) with varying success. Purpose: To summarize the issues surrounding cochlear implant candidates with SSCN as well as highlight trends in performance postimplantation. Research Design: Retrospective case reports of seven cochlear implant candidates detail the symptoms, typical audiologic presentation, and array of clinical issues for patients with this progressive and potentially fatal disease. Results: Despite the retrocochlear component of a hearing loss caused by SSCN, cochlear implantation may be a viable option. Conclusions: It is essential that the CI audiologist not only be aware of the disorder but also be well versed in the resulting implications for the cochlear implant process. A more thorough case history, an expanded candidacy test battery, and knowledge of the typical presentation of SSCN are critical. The diagnosis of SSCN will impact expectations for success with the cochlear implant, and counseling should be adjusted accordingly.


2018 ◽  
Vol 17 (04) ◽  
pp. 403-408
Author(s):  
Megumi Uchida ◽  
Tatsuya Morita ◽  
Yoshinori Ito ◽  
Kazuko Koga ◽  
Tatsuo Akechi

AbstractObjectiveThis study explores the views of healthcare professionals regarding care and treatment goals in irreversible terminal delirium and their effect on patients and caregivers.MethodWe conducted a qualitative interview study of healthcare professionals (palliative care physician, oncologist, psycho-oncologist, and clinical psychologist) engaged in the treatment of terminally ill cancer patients. We assessed the views of healthcare workers regarding treatment goals in terminal delirium and their effect on patients and their families.ResultOf the 21 eligible healthcare professionals, 20 agreed to participate in this study. Three of the professionals had experience with treating terminal delirium as family caregivers. We identified five important aspects of treatment goals in terminal delirium based on the views of healthcare professionals: (1) adequate management of symptoms/distress, (2) ability to communicate, (3) continuity of self, (4) provision of care and support to families, and (5) considering a balance (between symptom alleviation and maintaining communication; between symptom alleviation and family preparations for the death of patients; balance between specific treatment goals for delirium and general treatment goals).Significance of resultsAccording to the views of healthcare workers questioned in this study, goals of care and treatment in terminal delirium are multidimensional and extend beyond simply controlling patient symptoms.


2020 ◽  
Author(s):  
Johanna H Meijer ◽  
Joric Oude Vrielink

AbstractGiven the current shortage of respirator masks and the resulting lack of personal protective equipment for use by clinical staff, we examined bottom-up solutions that would allow hospitals to fabricate respirator masks that: (i) meet requirements in terms of filtering capacities, (ii) are easy to produce rapidly and locally, and (iii) can be constructed using materials commonly available in hospitals worldwide. We found that Halyard H300 material used for wrapping of surgical instruments and routinely available in hospitals, met these criteria. Specifically, three layers of material achieved a filter efficiency of 94%, 99%, and 100% for 0.3 μm, 0.5 μm, and 3.0 μm particles, respectively; importantly, these values are close to the efficiency provided by FFP2 and N95 masks. After re-sterilization up to 5 times, the filter’s efficiency remains sufficiently high for use as an FFP1 respirator mask. Finally, using only one layer of the material satisfies the criteria for use as a ‘surgical mask’. This material can therefore be used to help protect hospital staff and other healthcare professionals who require access to suitable masks but lack commercially available solutions.


2016 ◽  
Vol 7 (40) ◽  
pp. 940 ◽  
Author(s):  
SergioV Esparza-Gutiérrez ◽  
Adrián Santana-Ramírez ◽  
Pedro Avila-Rodríguez ◽  
JEugenio Jiménez-Gómez ◽  
Ezequiel Vélez-Gómez ◽  
...  

2021 ◽  
Vol 2021 ◽  
pp. 1-18
Author(s):  
Bikash Pradhan ◽  
Saugat Bhattacharyya ◽  
Kunal Pal

The last decade has witnessed extensive research in the field of healthcare services and their technological upgradation. To be more specific, the Internet of Things (IoT) has shown potential application in connecting various medical devices, sensors, and healthcare professionals to provide quality medical services in a remote location. This has improved patient safety, reduced healthcare costs, enhanced the accessibility of healthcare services, and increased operational efficiency in the healthcare industry. The current study gives an up-to-date summary of the potential healthcare applications of IoT- (HIoT-) based technologies. Herein, the advancement of the application of the HIoT has been reported from the perspective of enabling technologies, healthcare services, and applications in solving various healthcare issues. Moreover, potential challenges and issues in the HIoT system are also discussed. In sum, the current study provides a comprehensive source of information regarding the different fields of application of HIoT intending to help future researchers, who have the interest to work and make advancements in the field to gain insight into the topic.


2018 ◽  
Vol 32 (3) ◽  
pp. 258-261
Author(s):  
Dian He ◽  
Gang Cai ◽  
Yan Li ◽  
Qi Liu ◽  
Kang Xiao ◽  
...  

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