Sporadic Creutzfeldt-Jakob Disease: Diagnosing Typical and Atypical Presentations under Limited Circumstances

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.

Author(s):  
Sandhya S. ◽  
Mohanraj P.

Background: Myocardial Infarction is one of the most common causes of mortality and morbidity among the elderly patients. Also, it is known for wide range of clinical presentations other than chest pain. This article enlightens the clinical features, risk factors, complications, prognosis and outcome of Acute myocardial infarction in elderly patients. The objective of this study was to assess the risk factors, various symptoms, complications, prognosis and outcome of elderly patients with Acute myocardial Infarction (AMI).  Methods: This is a prospective study done over a period of two years in a tertiary care hospital in South India. 80 elderly patients who were diagnosed as AMI were included in the study.Results: Among the eighty patients the majority of the patients belonged to the age group 60-69 years. Twenty percent of the patients presented without chest pain. The atypical presentations included dyspnoea, giddiness, vomiting, sweating and epigastric pain. Mortality rate was 20%.Conclusions: This study showed that even though chest pain was the most common presentation in elderly AMI patients, they were also found to have atypical presentations like shortness of breath, giddiness, vomiting, sweating and epigastric pain. This signifies the need of examining physicians to meticulously identify acute myocardial infarction in elderly though they may not present typically.  


Blood ◽  
2004 ◽  
Vol 104 (11) ◽  
pp. 5294-5294
Author(s):  
Dharma R. Choudhary ◽  
Rajat Kumar ◽  
R. Saxena ◽  
Manoranjan Mahapatra ◽  
Atul Kotwal ◽  
...  

Abstract Background: There is very little published epidemiological data from developing countries regarding ITP and no large series from India. The aim of the study was to analyze the presenting features, response to different therapeutic options and suggest cost effective therapy. Method: The records of hematology department of All India Institute Of Medical Science were analyzed from January 1992 to June 2004. This is a premium tertiary care hospital in India. Diagnosis of ITP was made according to the standard criteria. Response criteria: complete response was defined as a platelet count increase to 100x109 /l or more, for at least 2 months: partial response was defined as doubling of platelet counts from initial levels and &gt; 50x 109/l for at least 2 months; no response included none of the above. Statistical methods: Database was created in MS Access and SPSS ver 11 was used for statistical analysis. Descriptive statistics were calculated and appropriate tests of significance like Chi Square, repeated measure linear model were carried out. Results: During the study period, 1230 patients of ITP were seen in hematology department, with a median age of 19.6 years (range 0.9–80). Females were 51.1% and males 48.9%. Median follow-up was of 9 months (range 0–178). Presenting features were: skin bleed − 91.1%; mucosal bleed − 57.5%; hematuria − 7.2%; gastrointestinal bleed − 12.5% and intracraniall bleed − 2.8%. Per-vaginal bleeding − 31.2 % of females. History of preceding viral fever was seen in 13.1% and palpable spleen in 2.5%. The mean platelet counts at presentation were 34+ 18.3x109/l. There were 595 (48.4%) patients of acute ITP and 635 (51.6%) patients of chronic ITP. Childhood ITP (age ≤ 12 yr) was seen in 46.5% and adult ITP in 53.5%. Response to therapy: Prednisolone was given to 99.6% patients with response of 57.3 %; Intravenous gamma globulin was given to 8.9% with response in 63.6%. Splenectomy was performed in 5% of acute ITP and 15.1% of chronic ITP (p = 0.00). The overall number of splenectomies was 126, with a response rate of 83.3%. Of these 126, acute ITP constituted 23.8% while chronic ITP formed 76.2% of cases. There was no statistically significant difference in response rate in these two groups (p =0.575). Danazole was given to 66 patients with response in 44%. Various other modalities of treatment were given to 24 patients (Anti D-14; Dapsone-2; Cyclosporin-2; Azathioprine-5; and Vincristine with Cyclophosphamide -1 patient), with a response in 25% of patients. The overall response rate with all treatment modalities was 68%: in childhood ITP − 65% and in adult ITP − 70.5%. Childhood ITP did not respond as expected, possibly due to referral bias of more refractory cases being referred to the center. The values of platelets showed a continuous increase during follow up and this increase was statistically significant (P=0.000 for all, Repeated measure model). Conclusion: Pattern of ITP in India is similar to that seen in other centers. In this study Prednisolone was given as first line agent to almost all patients with response in 57.3%; Splenectomy were done in 10.2% of prednisolone refractory or dependent patients with a response in 83.3%. These should form the primary modalities of therapy in developing countries. Significant numbers of patients were refractory to above-mentioned modalities and thus there is a requirement for other cost-effective therapies.


Author(s):  
Bilal Ur Rehman ◽  
Javid Ahmad ◽  
Rauf Ur Rashid Kaul ◽  
Mohammad Kaleem ul Haque

Background: Pregnancy is a major psychological, as well as physiological event; women may find themselves unable to cope with additional demands of pregnancy. Mental illness during pregnancy-whether anxiety, depression or more severe psychiatric disorders-can have a significant negative impact on a mother and her baby. Poor psychological health has been associated with low birth weight, premature birth, perinatal and infant death, postnatal depression, as well as long term behavioural and psychological impacts on the child. Depressive disorders are a common source of disability among women. Mental health problems during pregnancy and postpartum periods are one of the alarming health issue among women. Community-based epidemiological data on antenatal depression from developing countries is scarce. This study was conducted to assess the mental health status of pregnant women attending antenatal clinic of tertiary care hospital, SKIMS, Srinagar (J and K).Methods: A cross sectional study was conducted over a period of six months from 1stSeptember 2016 to 28th February 2017 among randomly selected pregnant women attending antenatal clinic of tertiary care hospital, SKIMS, Srinagar. A total of 200 pregnant women formed the study subjects. Data was collected by interviewing the pregnant women using pre-designed, pre-tested, semi-structured questionnaire. Data was analysed using Statistical Programme for the social science (SPSS) version 19.0.Results: Amongst the study population, prevalence of depressive disorder was 26%. The depression was significantly increasing with advancing pregnancy and advancing age. Socio-economic status and depression was associated statistically significant (p=0.024). Women with bad relationship with in laws had significantly more depression compared to those who had good relationship with in laws (P=0.0037). The association between parity and depressive disorder was statistically insignificant(P=0,7144).Conclusions: When we care for mother we care for two live and live without psychological consideration is completely materialistic. A depressive symptom occurs commonly during 2nd and 3rd trimester of pregnancy, drawing attention to a need to screen for depression during antenatal care. Maternal health policies, a priority in developing countries, must integrate maternal depression as a disorder of public health importance. Intervention should target women in the early antenatal period.


Author(s):  
Radha Kumari Paladugu ◽  
Srinivas Jagath Pentakota ◽  
Sushma N.

Background: Anaemia in pregnancy is now considered as a major public health problem, caused by iron deficiency. WHO has estimated that prevalence of anaemia in developed and developing countries in pregnant women is 14% in developed countries and 51 percent in developing countries and 65-75% in India. Various programmes regarding anaemia prophylaxis and maternal health have been implemented but still the prevalence of anaemia related complications among pregnant women continues to be high. Hence the present study was conducted to study the prevalence and pregnancy outcome as a result of anaemia in a tertiary care center in Visakhapatnam. Methods: A cross sectional descriptive study was carried out from June 2016 to August 2016 in the Department of Obstetrics and Gynecology among postnatal women in King George hospital, Visakhapatnam. A semi-structured questionnaire was prepared and information regarding age, socioeconomic status, parity, gestational age interval between pregnancies and outcome and complication of pregnancy were collected. Results: Mean age of study population is 22.4 years. Out of 100 women involved in the study 65 are from urban areas 29 from rural areas and 6 from tribal areas. Majority of the study population 66% were having HB% between 8-11% i.e. mild anaemia. When Outcome of pregnancy was seen in relation to anaemia majority of the women had live births (90%) followed by 7% IUDs and 2% still births. Conclusions: Majority of the women are in the age group of 20-24 years. Socioeconomic status is directly related to anaemic status of the women. Majority of the women had complication of puerperal sepsis. 


Author(s):  
Fizra Balkhi ◽  
Aamna Nasir ◽  
Ayesha Asghar ◽  
Farhan Patel ◽  
Marium Zafar ◽  
...  

Background: Health care worker’s hands are mostly responsible for transmission of healthcare associated infections (HCAI). In such instances, alcohol based hand rubs have been endorsed by WHO as the gold standard for hand hygiene for visibly clean hands; whereas, for visibly dirty hands soap and water is recommended. In developing countries (like Pakistan), affected people frequently exceed 25%. However, limited infrastructure in these countries hinders the performance of hand hygiene, but the use of alcohol-based hand rubs in such adverse situations is a practical solution to these constraints.Methods: In January 2021, we initiated a 12 week cross-sectional study in a public sector tertiary care hospital of Karachi, Pakistan. All participants were given a self-designed questionnaire with a consent form thereby giving us permission to use the data. Data was analyzed using statistical package for the social sciences (SPSS version 23).Results: 84.3% of the participants had received prior instructions on hand hygiene; however 71.7% respondents did not have access to alcohol based hand rubs. When questioned if they followed WHO’s five moments of hand hygiene, only 50.5% use alcohol based hand rubs before patient contact; 88.6% used it after patient contact; 83.2% after body fluid exposure risk; 77% before an aseptic task and 78.6% after contact with patient’s surroundings.Conclusions: There is a dire need for concerned authorities to enforce proper hand hygiene in public hospitals of developing countries in order to curb health care associated infections and prolonged hospital stays.


2021 ◽  
Vol 7 (2) ◽  
pp. 102-106
Author(s):  
Ruma Parvin ◽  
Sharmin Afroze ◽  
AHM Khairul Bashar ◽  
Sheikh Farjana Sonia ◽  
Nobo Krishna Ghosh

Coronavirus disease 2019 (COVID-19), caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), has spread across the world. We describe two cases of neonate, presenting with features like multisystem inflammatory syndrome (MIS-C), who presented to our neonatal unit of Dr. M R Khan Shishu Hospital, Bangladesh on August, 2020.Case 1 was a 7-day-old male infant presented at 14 hours of life with fever, poor feeding, per rectal bleeding and shock. Detailed examination revealed Down’s fancies and pallor. Case 2 was 15-day old neonate presenting with fever, rash and convulsion and his laboratory values were remarkable for high levels of C-reactive protein, D-dimer, ferritin and his Cerebrospinal fluid (CSF) findings were consistent with aseptic meningitis. Both babies were tested positive for severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). First case was very critical and referred to tertiary care hospital after stabilization. 2nd case recovered completely and was discharged to home in 15 days. Though till date COVID 19 in neonate is not very well-known to us, it is essential to disseminate information among the medical community regarding severe and atypical presentations of COVID-19 in neonate as prior knowledge can help to quickly identify and treat these patients as they present in the neonatal department. Bangladesh Journal of Infectious Diseases 2020;7(2):102-106


Author(s):  
Yasmin T ◽  

Early diagnosis of cancer gives an opportunity for early treatment which is very important. Unfortunately, late presentation and delayed diagnosis of childhood cancers remain a problem in developing countries including Bangladesh. Few studies on delayed diagnosis of childhood cancers have been conducted in Bangladesh.


2020 ◽  
Vol 4 (1) ◽  
pp. 1-5
Author(s):  
Osnat Itzhaki Ben Zadok ◽  
Katia Orvin ◽  
Edna Inbar ◽  
Eldad Rechavia

Abstract Background Creutzfeldt–Jakob disease (CJD), the most common prion disease in humans, is primarily known for its adverse neurological impact and inevitable mortality. Data regarding myocardial involvement in CJD are scarce. Case summary A 54-year-old female patient, presented with progressive effort dyspnoea, was diagnosed with unexplained non-ischaemic cardiomyopathy. An extensive cardiac work-up including cardiac magnetic resonance imaging (MRI) did not reveal any underlying aetiology. Simultaneously, the patient developed involuntary limb movements and progressive cognitive decline. Thalamic high-signal abnormalities on diffusion-weighted images were apparent on brain MRI. Based on these findings, she was subsequently referred to a neurology department, where she suddenly died the day after her admission. Brain autopsy demonstrated spongiform encephalopathy. A genetic analysis performed to her son revealed a mutation in the PRNP gene; all of these were consistent with CJD. Discussion This case describes the clinical association of CJD and cardiomyopathy and the diagnosis prion-induced cardiomyopathy by exclusion. It is not inconceivable that the coexistence of these two clinical entities may be related to genetic expression and contemporaneously deposition of infectious prions in myocardial muscle and brain tissue. Awareness of this possible association could be of important public-safety concern, and merits further collaborative cardiac-neurological work-up to elucidate this phenotype among patients with unexplained cardiomyopathy with neurological symptoms that resemble CJD.


2006 ◽  
Vol 27 (12) ◽  
pp. 1352-1357 ◽  
Author(s):  
Natalie Keeler ◽  
Lawrence B. Schonberger ◽  
Ermias D. Belay ◽  
Lynne Sehulster ◽  
George Turabelidze ◽  
...  

Objective. To investigate a case of Creutzfeldt-Jakob disease (CJD) possibly acquired from contaminated neurosurgical instruments. Design. Retrospective review of medical records, hospital databases, service log books, and state vital statistics. Setting. A tertiary care hospital (hospital A) in Missouri. Patients. The case patient was a 38-year-old African American woman with a 9-month history of progressive memory loss, visual disturbances, and dementia. She underwent neurosurgery in November 1996. CJD was confirmed in April 2004 by immunodiagnostic testing of brain biopsy samples. All patients who underwent neurosurgery at the same hospital within 6 months before or after the case patient's procedure were identified and investigated for preoperative or postoperative evidence of CJD. Results. We reviewed data on 268 neurosurgical procedures, 84 pathology log entries, and 60 death certificates for neurosurgical patients at hospital A and identified 2 suspected cases of CJD. Clinical features and definitive prion testing of stored brain biopsy samples excluded a diagnosis of CJD. Standard operating room procedures were in place, but specific protocols for handling instruments potentially contaminated with prions were not used. Conclusions. Neurosurgical instruments were not implicated as the source exposure for CJD in the case patient. The 2 patients with suspected CJD were identified from different data sources, suggesting good internal consistency in data collection. The key elements of this investigation are suggested for use in future investigations into potential cases of iatrogenic CJD.


2011 ◽  
Vol 25 (7) ◽  
pp. 368-372 ◽  
Author(s):  
Kamran Manek ◽  
Victoria Williams ◽  
Sandra Callery ◽  
Nick Daneman

BACKGROUND: The incidence and severity ofClostridium difficileinfections are increasing, and there is a need to optimize the prevention of complicated disease.OBJECTIVE: To identify modifiable processes of care associated with an altered risk ofC difficilecomplications.METHODS: A retrospective cohort study (with prospective case ascertainment) of allC difficileinfections during 2007/2008 at a tertiary care hospital was conducted.RESULTS: Severe complications were frequent (occurring in 97 of 365 [27%]C difficileepisodes), with rapid onset (median three days postdiagnosis). On multivariable analysis, nonmodifiable predictors of complications included repeat infection (OR 2.67), confusion (OR 2.01), hypotension (OR 0.97 per increased mmHg) and elevated white blood cell count (OR 1.04 per 109cells/L). Protection from complications was associated with initial use of vancomycin (OR 0.24); harm was associated with ongoing use of exacerbating antibiotics (OR 3.02).CONCLUSION:C difficileinfections often occur early in the disease course and are associated with high complication rates. Clinical factors that predicted a higher risk of complications included confusion, hypotension and leukocytosis. The most effective ways to improve outcomes for patients withC difficilecolitis are consideration of vancomycin as first-line treatment for moderate to severe cases, and the avoidance of unnecessary antibiotics.


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