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1.
Pathology ; 2024 Apr 09.
Article En | MEDLINE | ID: mdl-38710610

The protozoan parasite Dientamoeba fragilis is a frequently isolated stool organism and postulated cause of gastrointestinal symptoms. Peripheral blood eosinophilia has been described. This is the first study amongst the Australasian adult population to assess the relationship between organism detection and eosinophilia. A case-control study took place over 7 years at a single Sydney laboratory site, evaluating patients with D. fragilis identified on stool using real-time PCR with a recent full blood count, to control groups with Giardia spp. and sequential negatives with neither organism. A nested study compared those with microscopic evidence of D. fragilis as a marker of disease burden, to molecular diagnosis alone. Sixty-four D. fragilis, 30 Giardia spp., and 94 sequential controls were enrolled. Only 60.1% of samples were preserved in sodium acetate-acetic acid formalin (SAF) fixative, indication mostly not documented. The major co-organism detected amongst all participants was Blastocystis sp., particularly in the D. fragilis cohort (37.2%). The most common pathogen amongst sequential controls was Campylobacter spp. (7.4%). Patients with D. fragilis were more likely (12.5%) to have a clinically significant eosinophilia (>0.5×109/L) compared to those with Giardia spp. (3.3%) or sequential controls (4.3%) (p=0.03). A significant difference was also noted in the overall median eosinophil count of those with D. fragilis versus all controls (0.2 vs 0.1×109/L, p=0.01); however, this was within the reference interval (where up to >0.5×109/L is accepted in healthy individuals within a typical population). No eosinophil difference was found between those with molecular versus additional microscopic detection of D. fragilis (0.1 vs 0.1×109/L). These results support an association between the identification of clinically significant peripheral blood eosinophilia and D. fragilis presence, which may impact the diagnostic approach to the patient with unexplained eosinophilia. Further prospective trials may help assess any significance further and the implication of co-carriage with other enteric organisms. The importance of clinical indication and need for appropriate fixative media in diagnostic parasitology are also highlighted.

2.
Cureus ; 16(3): e56357, 2024 Mar.
Article En | MEDLINE | ID: mdl-38633948

Pemphigus vulgaris is a chronic autoimmune disease of the skin caused by the production of autoantibodies targeting desmogleins 1 and 3 usually presenting in individuals with an average age of onset of approximately 40 years. A 35-year-old obese, diabetic woman presented with fluid-filled lesions over her body for three months along with erosions and painful ulcers in her mouth and genital area for two months. Based on clinical and histopathological studies, the patient was diagnosed as a case of pemphigus vulgaris. She was started on conventional treatment with oral corticosteroids followed by pulse therapy and mycophenolate mofetil. Rituximab infusion was scheduled but could not be administered due to elevated D-dimer values. The patient underwent screening for deep vein thrombosis (DVT) and received subcutaneous enoxaparin and oral rivaroxaban. She developed severe sepsis for which she was treated with systemic antibiotics. She subsequently developed acute renal failure and underwent hemodialysis. The patient's clinical condition further deteriorated, which necessitated therapeutic plasma exchange (TPE). Collagen, colloidal silver, and silicone foam dressings were done to hasten wound healing. Two distinct approaches were employed to eliminate the pseudomembrane on the wounds. One portion was treated with hydrogen peroxide (H2O2), while the other was with hyaluronidase. The hyaluronidase treatment resulted in considerable improvement of the lesions. Intravenous immunoglobulin (IVIG) infusion was scheduled. However, the treatment could not be administered as the patient succumbed to death due to pulmonary thromboembolism (PTE) secondary to DVT.

3.
Cureus ; 16(3): e57233, 2024 Mar.
Article En | MEDLINE | ID: mdl-38686276

Cyclophosphamide, an alkylating agent, has rarely been observed to cause a bluish discoloration of nails, an occurrence that is typically underreported. We describe the case of a middle-aged male undergoing dexamethasone-cyclophosphamide pulse therapy for pemphigus foliaceus, who exhibited bluish-gray discoloration of the nails. It is crucial to differentiate this presentation from other conditions such as nail apparatus melanoma (NAM), which may manifest in a slightly different manner. We also report the onychoscopic findings observed in this case.

5.
BMJ Case Rep ; 17(4)2024 Apr 24.
Article En | MEDLINE | ID: mdl-38663898

Mycetoma is a chronic granulomatous infectious disease with a triad of subcutaneous swelling, discharging sinuses and the presence of granules. The infection may occur following minor trauma or penetrating thorn injury. We report a case of a man in his 40s with a history of thorn prick 9 years ago, followed by the formation of painless discharging sinuses on the right foot for the past 2 years. Clinical, local epidemiological, histopathological examination and Gram stain confirmed the diagnosis of actinomycetoma. Prior to initiating the Welsh regimen, a pretreatment assessment of the patient's auditory function was conducted through pure tone audiometry, indicating the existence of pre-existing high-frequency bilateral sensorineural hearing loss. The patient was treated with linezolid as an alternative to amikacin, at a dosage of 600 mg two times per day, leading to complete resolution within 3 weeks. This underscores linezolid's efficacy as a safe and cost-effective alternative for actinomycetoma, without causing ototoxic side effects.


Hearing Loss, Sensorineural , Linezolid , Mycetoma , Humans , Linezolid/therapeutic use , Linezolid/adverse effects , Linezolid/administration & dosage , Male , Hearing Loss, Sensorineural/drug therapy , Hearing Loss, Sensorineural/diagnosis , Mycetoma/drug therapy , Mycetoma/diagnosis , Adult , Anti-Bacterial Agents/therapeutic use , Anti-Bacterial Agents/adverse effects , Anti-Bacterial Agents/administration & dosage , Treatment Outcome
6.
Med Teach ; : 1-6, 2024 Feb 21.
Article En | MEDLINE | ID: mdl-38382447

PURPOSE: Undergraduate medical education has had a call to action to acknowledge racist practices that are impacting learners throughout their training. In 2020, our school performed a detailed curricular review and provided recommendations to address racism in the curriculum. Many schools have now undergone a similar curricular review process, but little is known about whether suggested antiracist curricular changes impact faculty teaching behavior or the overall curriculum. MATERIAL AND METHODS: In 2021, as part of the medical school's annual educational quality improvement process, course directors were required to answer a question about the changes they made to address racism in their courses based on recommendations provided the year prior from an antiracism curricular review. The documented changes were analyzed for themes and then organized by course and curricular year. These changes were compared with the suggested recommendations to analyze the number and types of changes implemented after one year. To evaluate student perceptions of change the general comments from academic years 2019-2021 were reviewed. RESULTS: After 1 year, approximately, 74% of our school's 328 anti-racism curricular review recommendations were implemented in courses. Over 80% were implemented in curricular year 1. The greatest number of recommendations implemented were related to the theme of critiquing the strength of evidence in race-based medical practices. The least amount change was made around the theme of challenging the biologic notion of race. CONCLUSIONS: An antiracism curricular review followed by an embedded continuous quality improvement process can be an effective approach to address racism in medical school curricula. Addressing racism in medical education requires medical schools to regularly identify curricular gaps, faculty needs and monitor their progress.

7.
Acad Med ; 99(5): 550-557, 2024 May 01.
Article En | MEDLINE | ID: mdl-38277443

PURPOSE: To gather and leverage the voices of students to drive creation of required, integrated palliative care curricula within undergraduate medical education in Massachusetts, which is lacking in a majority of U.S. medical schools. METHOD: The study was conducted by the Massachusetts Medical Schools' Collaborative, a working group committed to ensuring all medical students in Massachusetts receive foundational training in serious illness communication (SIC) and palliative care. Eight focus groups (2 per participating medical school) were conducted during January-May 2021 and included a total of 50 students from Boston University Chobanian & Avedisian School of Medicine, Harvard Medical School, Tufts University School of Medicine, and the UMass Chan Medical School. Data collected from focus groups were discussed and coded. Themes were identified using the immersion/crystallization qualitative data analysis approach. RESULTS: Six key themes emerged. Students viewed SIC as essential to high-quality medical practice regardless of specialty, and believed training in SIC skills and palliative care should be required in medical school curricula. Students preferred to learn and practice these skills using frameworks, particularly in real-world situations. Students recognized the expertise of palliative care specialists and described them as a scarce, often misunderstood resource in health care. Students reported it was mostly "luck" if they were included in family meetings and observed good role models. Finally, students desired practice in debriefing after difficult and emotional situations. CONCLUSIONS: This study confirms long-standing themes on students' experiences with SIC and palliative care topics, including feeling inadequately prepared to care for seriously ill patients as future physicians. Our study collected students' perspectives as actionable data to develop recommendations for curricular change. Collaborative faculty also created recommendations based on the focus group data for immediate and ongoing SIC and palliative care curricular change in Massachusetts, which can apply to medical schools nationwide.


Communication , Curriculum , Education, Medical, Undergraduate , Focus Groups , Palliative Care , Students, Medical , Humans , Massachusetts , Education, Medical, Undergraduate/methods , Students, Medical/psychology , Male , Female , Qualitative Research , Adult , Critical Illness/therapy , Critical Illness/psychology
8.
J Palliat Med ; 27(1): 39-46, 2024 Jan.
Article En | MEDLINE | ID: mdl-37976143

Background: Practicing physicians require serious illness communication (SIC) skills to ensure high-quality, humanistic care for patients and families as they face life-changing medical decisions. However, a majority of U.S. medical schools do not require formal training in SIC and fail to provide students deliberate practice before graduation. The Massachusetts Medical Schools' Collaborative was created to ensure that students receive foundational SIC training in undergraduate medical education. This Collaborative developed a curriculum-mapping tool to assess SIC at four medical schools. Objective: We aimed to understand existing educational activities across four medical schools and identify opportunities to build longitudinal, developmentally based curricular threads in SIC. Design: From July 2019 to April 2021, faculty, staff, and medical students assessed current educational activities related to five core competencies in SIC, adapted for students from national competencies for palliative medicine fellows, using a curriculum mapping tool. Measurements: The group selected 23 keywords and collected metrics to describe the timing, instruction and assessment for each school's educational activities. Results: On average, there were only 40 hours of required curricula in SIC over four years. Over 80% of relevant SIC hours occurred as elective experiences, mostly during the postclerkship phase, with limited capacity in these elective experiences. Only one school had SIC educational activities during the clerkship phase when students are developing clinical competencies. Assessment methods focused on student participation, and no school-assessed clinical performance in the clerkship or postclerkship phase. Conclusions: Medical schools are failing to consistently train and ensure basic competency in effective, compassionate SIC. Curriculum mapping allows schools to evaluate their current state on a particular topic such as SIC, ensure proper assessment, and evaluate curricular changes over time. Through the deliberate inclusion of SIC competencies in longitudinal curriculum design, we can fill this training gap and create best practices in undergraduate medical education.


Education, Medical, Undergraduate , Humans , Education, Medical, Undergraduate/methods , Schools, Medical , Curriculum , Massachusetts , Communication
11.
Transplant Direct ; 9(1): e1430, 2023 Jan.
Article En | MEDLINE | ID: mdl-36582672

Fungal infections are a recognized complication of immunosuppression in solid organ transplant recipients. Phaeohyphomycoses are fungal infections caused by a diverse group of dematiaceous fungi. Methods: We share the learning points from 2 Australian cases of phaeohyphomycosis secondary to Phaeacreomonium species (spp). A literature review was performed using Medline, Embase, and Google Scholar to identify this condition among kidney transplant recipients. Results: With the 2 cases reported in this article, a total of 17 cases were identified in the literature. Phaeacremonium spp is ubiquitous in humid and temperate flora, including Australia. Minor trauma is likely the source of inoculation in most cases and diagnosis is often delayed. Presently, no guidelines for management exist given the rarity of this condition. Most known cases have been treated with surgical debulking combined with long-course antifungal therapy. Conclusion: This paper describes 2 Australian cases of phaeohyphomycosis in kidney transplant recipients. A high index of suspicion, especially in the immunosuppressed, is essential for timely diagnosis in kidney transplant recipients. There are several diagnostic and therapeutic challenges that remain with this condition.

12.
Sci Total Environ ; 861: 160609, 2023 Feb 25.
Article En | MEDLINE | ID: mdl-36470384

While mounting evidence suggests that wildland fire smoke (WFS) inhalation may increase the burden of cardiopulmonary disease, the occupational risk of repeated exposure during wildland firefighting remains unknown. To address this concern, we evaluated the cardiopulmonary function in mice following a cumulative exposure to lab-scale WFS equivalent to a mid-length wildland firefighter (WLFF) career. Dosimetry analysis indicated that 80 exposure hours at a particulate concentration of 22 mg/m3 yield in mice the same cumulative deposited mass per unit of lung surface area as 3600 h of wildland firefighting. To satisfy this condition, male Apoe-/- mice were whole-body exposed to either air or smoldering Douglas fir smoke (DFS) for 2 h/day, 5 days/week, over 8 consecutive weeks. Particulate size in DFS fell within the respirable range for both mice and humans, with a count median diameter of 110 ± 20 nm. Expiratory breath hold in mice exposed to DFS significantly reduced their minute volume (DFS: 27 ± 4; Air: 122 ± 8 mL/min). By the end of the exposure time frame, mice in the DFS group exhibited a thicker (DFS: 109 ± 3; Air: 98 ± 3 µm) and less distensible (DFS: 23 ± 1; Air: 28 ± 1 MPa-1) aorta with reduced diastolic blood augmentation capacity (DFS: 53 ± 2; Air: 63 ± 2 kPa). Cardiac magnetic resonance imaging further revealed larger end-systolic volume (DFS: 14.6 ± 1.1; Air: 9.9 ± 0.9 µL) and reduced ejection-fraction (DFS: 64.7 ± 1.0; Air: 75.3 ± 0.9 %) in mice exposed to DFS. Consistent with increased airway epithelium thickness (DFS: 10.4 ± 0.8; Air: 7.6 ± 0.3 µm), airway Newtonian resistance was larger following DFS exposure (DFS: 0.23 ± 0.03; Air: 0.20 ± 0.03 cmH2O-s/mL). Furthermore, parenchyma mean linear intercept (DFS: 36.3 ± 0.8; Air: 33.3 ± 0.8 µm) and tissue thickness (DFS: 10.1 ± 0.5; Air: 7.4 ± 0.7 µm) were larger in DFS mice. Collectively, mice exposed to DFS manifested early signs of cardiopulmonary dysfunction aligned with self-reported events in mid-career WLFFs.


Pseudotsuga , Animals , Male , Mice , Aorta , Dust , Inhalation Exposure/analysis , Lung , Smoke/adverse effects , Stroke Volume
14.
Acad Med ; 97(11): 1623-1627, 2022 11 01.
Article En | MEDLINE | ID: mdl-35857397

PROBLEM: Data from the Association of American Medical Colleges (AAMC) Medical School Graduation Questionnaire (GQ) show persistent high rates of medical student mistreatment, and multiple barriers to student reporting of mistreatment exist. The authors examined whether learning environment sessions (LESs) allow students opportunities to identify and describe patterns of mistreatment missed by other avenues of reporting. APPROACH: Peer-facilitated LESs were instituted in 2018-2019 at Boston University School of Medicine. The LESs were scheduled once during every third-year core clerkship block. Third- and fourth-year students trained as peer-facilitators led discussions of topics relevant to the student clinical experience using a standardized facilitator guide. Minutes, including details of reported events, were completed during the session and visible to all students participating. These minutes were sent to clerkship leadership and the medical education office for action once student grades were submitted. OUTCOMES: Summative content analysis was conducted on 44 LES minutes from sessions held in January-November 2019. Reported incidents were categorized into broad categories of negative treatment (NT), negative learning environment (NLE), and positive learning environment (PLE). Sixty-three instances of NT were identified. Of these, 37 fit within the scope of the AAMC GQ mistreatment categories. The remaining 26 instances of NT were classified into 7 novel categories of medical student mistreatment. Instances of NLE were most discussed by students and categorized into 5 subthemes. Examples of PLE were categorized into 4 subthemes, which encompassed 11 descriptors of core qualities of an ideal preceptor or educational environment. NEXT STEPS: LESs have aided in identifying and describing new patterns of mistreatment. They fulfill a unique role by allowing students to identify, analyze, and report mistreatment in a facilitated and protected space. Formal evaluations of institutional improvement in the learning environment, reduction in medical student mistreatment, and subsequent improvement in AAMC GQ data are needed.


Clinical Clerkship , Education, Medical, Undergraduate , Medicine , Students, Medical , Humans , Learning , Surveys and Questionnaires
15.
Acad Med ; 97(12): 1799-1803, 2022 12 01.
Article En | MEDLINE | ID: mdl-35703204

PURPOSE: Physician assistants (PAs) and medical degree students (MDs) often lack training in addressing the social determinants of health (SDOH). Social work students (SWs), meanwhile, have extensive SDOH training; however, few medical professionals have opportunities to engage in interprofessional training with SWs. This study examined the feasibility, acceptability, and students' perceptions of an interprofessional virtual reality (VR) simulated learning environment (SLE) for teaching health professions students about the SDOH. METHOD: In January 2020, 15 students at Boston University School of Medicine attended web-based video conferences focused on SDOH, health equity, and team-based care. Subsequently, student dyads participated in a case-based learning activity using an immersive VR SLE to develop teamwork skills. Evaluation included a postsurvey and a focus group examining their experiences in the course to gauge feasibility and acceptability. Thematic analysis of open-ended survey responses from the postsurvey and focus group data was conducted. RESULTS: A total of 8 VR simulations were run. Findings indicated both MD and PA students learned patient engagement strategies from SW students, who enhanced their health care leadership capacity. Participants found the means of instruction acceptable, valued the hands-on VR interprofessional training, and expressed interest in learning more about the scope of one another's roles and the community resources available to patients. CONCLUSIONS: VR SLE is a feasible and acceptable means of instruction. It allowed students to connect across programmatic and geographic boundaries in a collaborative working environment mimicking the team approach to care they will use in their professional life. This experience illustrated for students the strengths a multidisciplinary team has to offer.


Lupus Erythematosus, Systemic , Students, Health Occupations , Virtual Reality , Humans , Interprofessional Relations , Social Determinants of Health
16.
Int J Infect Dis ; 121: 138-140, 2022 Aug.
Article En | MEDLINE | ID: mdl-35562042

Coxiella burnetti is the causative organism of the zoonotic infection Q fever, of which endocarditis is one of the most common manifestations of the chronic form. Polymicrobial endocarditis with Q fever is extremely rare and is yet to be described among an Australasian cohort. SUMMARY: We present the case of a 32-year-old gardener with culture-negative chronic Q fever prosthetic valve endocarditis concomitant with another bacterial pathogen, leading to aortic root abscess formation, requiring a Bentall procedure, extracorporeal membrane oxygenation, and prolonged antimicrobial therapy, with a fatal outcome. Unique to our case, Q fever was identified early, and the second pathogen was only detected on 16S ribosomal RNA (rRNA) polymerase chain reaction of explanted valvular tissue. Given the high risk for morbidity, we recommend that screening for Q fever in endemic areas among patients with infective endocarditis from other etiologies be considered. In addition, this case highlights the role for Q fever vaccination of the at-risk population with underlying valvulopathy. Furthermore, clinicians should be aware of polymicrobial infective endocarditis and suspicious in case of patients with atypical clinical features.


Coxiella burnetii , Endocarditis, Bacterial , Endocarditis , Heart Valve Prosthesis , Q Fever , Adult , Coxiella burnetii/genetics , Endocarditis/diagnosis , Endocarditis/drug therapy , Endocarditis, Bacterial/diagnosis , Endocarditis, Bacterial/drug therapy , Endocarditis, Bacterial/microbiology , Heart Valve Prosthesis/adverse effects , Heart Valve Prosthesis/microbiology , Humans , Polymerase Chain Reaction , Q Fever/complications , Q Fever/diagnosis , Q Fever/drug therapy , RNA, Ribosomal, 16S/genetics
17.
Acad Med ; 97(6): 804-811, 2022 06 01.
Article En | MEDLINE | ID: mdl-34817407

In the context of current U.S. racial justice movements, analysis of racism in medicine within medical education is a critical task for all institutions. To educate the next generation of physicians about racism in medicine and out of concern that the curriculum required critical assessment and change, a group of students and faculty at Boston University School of Medicine (BUSM) initiated a longitudinal curricular analysis through a vertical integration group, commissioned by the Medical Education Committee, from May 2019 to June 2020. The curriculum analysis and the major outcomes and guiding principles that emerged from it are described as a path forward, toward a more inclusive curriculum. The major elements of this analysis included a comprehensive internal curricular assessment and an external assessment of peer institutions that led to the development of key curricular recommendations and overarching equity and specific racially focused equity competencies. The curricular recommendations fall into the following domains: (1) challenging the persistence of biological/genetic notions of race, (2) embedding structural practices in medical education to dismantle racism in medicine, and (3) promoting institutional climate change. Initial steps to implement these recommendations are described. The authors believe that the historic and present reality of racism in America and in medicine has impacted medical education specifically, and more broadly, the practice of medicine, trainee experience, and patient outcomes. The key findings of the BUSM analysis are transferable to other medical education institutions, and the described review process can support peer institutions as they engage in the imperative work of institutional reflection and addressing the salient ideas and practices that uphold racism in medicine.


Education, Medical , Racism , Boston , Curriculum , Humans , Racism/prevention & control , Social Justice
18.
Int J Wildland Fire ; 302021 Oct 05.
Article En | MEDLINE | ID: mdl-34776721

Emission measurements are available in the literature for a wide variety of field burns and laboratory experiments, although previous studies do not always isolate the effect of individual features such as fuel moisture content (FMC). This study explores the effect of FMC on gaseous and particulate emissions from flaming and smouldering combustion of four different wildland fuels found across the United States. A custom linear tube-heater apparatus was built to steadily produce emissions in different combustion modes over a wide range of FMC. Results showed that when compared with flaming combustion, smouldering combustion showed increased emissions of CO, particulate matter and unburned hydrocarbons, corroborating trends in the literature. CO and particulate matter emissions in the flaming mode were also significantly correlated with FMC, which had little influence on emissions for smouldering mode combustion, when taking into account the dry mass of fuel burned. These variations occurred for some vegetative fuel species but not others, indicating that the type of fuel plays an important role. This may be due to the chemical makeup of moist and recently live fuels, which is discussed and compared with previous measurements in the literature.

19.
MedEdPORTAL ; 17: 11185, 2021.
Article En | MEDLINE | ID: mdl-34632053

Introduction: Data from the Association of American Medical Colleges' Medical School Graduation Questionnaire show persistent trends of medical student mistreatment nationwide. To reduce the barriers and increase actionable reporting of mistreatment, we integrated peer-facilitated learning environment sessions led by a group of trained third- and fourth-year medical students in all core clinical clerkships. Methods: During the 2018-2019 academic year, third-year medical students were recruited, oriented, and trained to act as facilitators of sessions on mistreatment. The sessions occurred once every clerkship block, using a standardized session introduction and guide. After a 6-month pilot, new medical students were recruited and worked as scribe/facilitator pairs, receiving an additional 1.5-hour training midyear, which was evaluated with a postworkshop survey. Results: Thirty-eight students implemented 43 peer-facilitated sessions and completed deidentified minutes of each session, which were shared with clerkship directors and the Medical Education Office for review. Survey data from midyear facilitator training indicated that facilitators highly agreed peer-led sessions were an important avenue for students to process experiences of mistreatment (3.9 out of 4), understood barriers to reporting (3.8 out of 4) and definitions of mistreatment (3.6 out of 4), and felt confident to facilitate these sessions (3.6 out of 4). Discussion: Peer-facilitated sessions offer a method to learn more about student experiences with mistreatment in real time and create a new avenue for communication between faculty and students. Assembling a stable core team of third- and fourth-year students trained in facilitation skills ensures the sustainability and relevance of the program.


Clinical Clerkship , Education, Medical, Undergraduate , Students, Medical , Curriculum , Humans , Peer Group
20.
J Clin Neurosci ; 86: 301-309, 2021 Apr.
Article En | MEDLINE | ID: mdl-33436304

The Heidenhain variant of Creutzfeld-Jakob disease (HvCJD) is a relentlessly progressive and fatal neurodegenerative disorder characterised by prominent visual features early in its clinical course. However, seizures are uncommonly reported in HvCJD. The case history of a patient admitted to our institution with HvCJD and seizures is described followed by a systematic review of the association between HvCJD and seizures. A systematic search of the databases Medline, PubMed, and PsycInfo was conducted, from inception to November 2019, using keywords relating to 'Creutzfeldt-Jakob disease' and 'Heidenhain variant', to ascertain the frequency of seizures in HvCJD, as well as, seizure semiology and electrographic features. The Preferred Items Reporting for Systematic Reviews and Meta-Analyses (PRISMA) guidelines were followed in the construction of this systematic review. All studies, including case reports of patients who met the diagnostic criteria for HvCJD where details pertaining to clinical presentation, imaging, biochemical and EEG findings were available were included. There were 46 articles reporting on a total of 73 patients. Seizures occurred in only four out of 73 cases (5.5%). The semiology of these seizures were focal motor seizures with or without secondary generalisation and occipital lobe seizures. Imaging and electrographic findings were most commonly abnormal in the posterior cerebral cortices (in particular the occipital and occipito-parietal regions). This systematic review suggests that seizures are uncommon in HvCJD despite the frequency of imaging and electrographic abnormalities in the posterior cerebral regions. A key limitation of this systematic review is the variability of publications in terms of incomplete reporting of clinical data, in particular potential under-reporting of seizures, as well as follow up, which may have contributed to the lower frequency of seizures reported in patients with HvCJD.


Creutzfeldt-Jakob Syndrome/diagnostic imaging , Creutzfeldt-Jakob Syndrome/genetics , Genetic Variation/genetics , Seizures/diagnostic imaging , Seizures/genetics , Creutzfeldt-Jakob Syndrome/physiopathology , Electroencephalography/methods , Female , Humans , Magnetic Resonance Imaging/methods , Middle Aged , Occipital Lobe/diagnostic imaging , Occipital Lobe/physiopathology , Parietal Lobe/diagnostic imaging , Parietal Lobe/physiopathology , Seizures/physiopathology
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