Case series of Creutzfeldt-Jakob disease in a third-level hospital in Quito

Herrán, Germaine Eleanor Torres; Herrera, Andrés Damián Ortega; Burbano, Braulio Martinez; Serrano‐Dueñas, Marcos; Yepez, Maria; Madera, R; Campaña, Luis Alfredo Masabanda; Jiménez, Guillermo David Baño; Saltos, Denny Maritza Santos; Díaz, Edgar Patricio Correa

doi:10.1186/s12883-018-1061-0

Cited by 8 publications

(7 citation statements)

References 37 publications

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“…Among the countries of Middle and South America, some such as Argentina, Chile, Mexico, Brazil, and Peru have reported their data of PrD surveillance or studies. For (49,50). It is apparent that the PrD or CJD case numbers globally are markedly undervalued, which may pose a potential biosafety risk.…”

Section: Discussionmentioning

confidence: 99%

Updated global epidemiology atlas of human prion diseases

Gao,

Tian,

Xiao

et al. 2024

Front. Public Health

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IntroductionHuman prion disease (PrD), a group of fatal and transmissible neurodegenerative diseases, consists of Creutzfeldt–Jakob disease (CJD), kuru, fatal familial insomnia (FFI), Gerstmann-Sträussler-Scheinker disease (GSS), and variably protease-sensitive prionopathy (VPSPr). The emergence of bovine spongiform encephalopathy (BSE) in cattle and variant CJD (vCJD) has greatly threatened public health, both in humans and animals. Since the 1990's, dozens of countries and territories have conducted PrD surveillance programs.MethodsIn this study, the case numbers and alternative trends of different types of PrD globally and in various countries or territories from 1993 to 2020 were collected and analyzed based on the data from the websites of the international and national PrD surveillance programs, as well as from relevant publications.ResultsThe total numbers of the reported PrD and sporadic CJD (sCJD) cases in 34 countries with accessible annual case numbers were 27,872 and 24,623, respectively. The top seven countries in PrD cases were the USA (n = 5,156), France (n = 3,276), Germany (n = 3,212), Italy (n = 2,995), China (n = 2,662), the UK (n = 2,521), Spain (n = 1,657), and Canada (n = 1,311). The annual PrD case numbers and mortalities, either globally or in the countries, showed an increased trend in the past 27 years. Genetic PrD cases accounted for 10.83% of all reported PrD cases; however, the trend varied largely among the different countries and territories. There have been 485 iatrogenic CJD (iCJD) cases and 232 vCJD cases reported worldwide.DiscussionThe majority of the countries with PrD surveillance programs were high- and upper-middle-income countries. However, most low- and lower-middle-income countries in the world did not conduct PrD surveillance or even report PrD cases, indicating that the number of human PrD cases worldwide is markedly undervalued. Active international PrD surveillance for both humans and animals is still vital to eliminate the threat of prion disease from a public health perspective.

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Section: Discussionmentioning

confidence: 99%

Updated global epidemiology atlas of human prion diseases

Gao,

Tian,

Xiao

et al. 2024

Front. Public Health

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“…It has been found that approximately one-third of sCJD cases initially present with cognitive or behavioral changes, and close to one-third have cerebellar ataxia, aphasia, visual dysfunction, and motor deficit as the initial presenting symptoms. Depression has also been found in some patients with CJD [11]. Myoclonus can sometimes be absent initially, but present in the advanced stages of sCJD [12].…”

Section: Discussionmentioning

confidence: 99%

Rapidly Progressive Dementia in an Elderly Male: Perseverance Becomes the Key to a Rare Diagnosis

Negi,

Prabhu

2023

Cureus

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Cognitive impairment in a patient with rapidly progressive dementia (RPD) develops faster than expected for a known dementia syndrome. It poses as a diagnostic challenge for the physician who must identify the diagnosis among a broad spectrum of differentials. Here, we discuss the case of a 60-year-old male who presented with a four-month history of progressive gait disturbance, incoherent talking, dysarthria, hand tremors, and new-onset bladder incontinence. Neurological examination revealed fast saccades, cerebellar dysarthria, hypertonia, and normal power in all four limbs, brisk reflexes, past pointing, intentional tremors, resting myoclonic jerks, and ataxic gait. Initial differentials of progressive paraneoplastic encephalitis, infectious encephalitis, and toxic encephalopathy were considered. However, the results of lumbar puncture and blood investigations-voltage-gated potassium channel (VGKC) antibody and Nmethyl-D-aspartate (NMDA) receptor antibody, tumor markers, viral markers being negative and ammonia and lactate levels being normal led us to think of another possibility. With such rapidly progressive dementia, myoclonic jerks, extrapyramidal signs, and cerebellar signs, a diagnosis of Creutzfeldt-Jakob disease (CJD) was taken into consideration. A cerebrospinal fluid (CSF) sample was sent for CSF protein 14-3-3 quantification by enzyme-linked immunosorbent assay (ELISA) and came out positive. During his stay in the hospital, our patient developed multiple complications, and his clinical state progressively worsened. With no signs of improvement and the known fatal nature of the disease, the goals of care were discussed with the family and we all agreed on providing palliative care. The patient passed away on day 15 of hospital admission.

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“…La ECJ se caracteriza por tener una presentación clínica heterogénea donde el síntoma cardinal es la demencia de curso rápidamente progresivo, asociado a síntomas constitucionales inespecíficos (compromiso del estado general, baja de peso, alteraciones del sueño), síntomas neuropsiquiátricos (depresión, irritabilidad, agresividad, apatía, cambios de personalidad) y síntomas neurológicos variados que revelan el depósito de la proteína priónica anormal en diferentes localizaciones del encéfalo 4,15 . Los hallazgos clínicos neurológicos que más comúnmente hacen sospechar esta enfermedad son las alteraciones de funciones cerebrales superiores, las mioclonías y la ataxia 16 , los cuales son los síndromes más frecuentemente encontrados en nuestra serie al momento de la consulta.…”

Section: Discussionunclassified

Enfermedad de Creutzfeldt-Jakob: experiencia de 5 años en un hospital terciario de Chile

Legua-Koc¹,

Castillo-Torres

León-Mantero

et al. 2021

Rev. méd. Chile

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Background: Creutzfeldt-Jakob disease (CJD) is a prion affection that typically produces a rapidly progressive dementia with different neurologic and extra-neurologic manifestations. Aim: To characterize clinical, imaging and electroencephalography findings in patients with a probable CJD. Patients and Methods: A case series study of patients admitted in the Neurology department at a public hospital, between 2014 and 2019. Demographic, clinical, imaging, and electroencephalographic data of patients with probable CJD were analyzed. Results: Seventeen patients aged 63 ± 11 years (53% women) with a probable CJD were gathered. The incidence was 4.7 cases/year per million inhabitants. Twenty four percent of patients had a family history of CJD. The median time between the onset of symptoms and the hospital admission was three months with a survival of four months. The most common clinical manifestations were an amnesic syndrome in 88%, myoclonus in 76%, frontal syndrome and ataxia in 71%. Brain MRI was abnormal in all patients. The preponderant finding was the involvement of the caudate nucleus in 82% of cases. In the EEG, 94% of patients had abnormalities. All had a theta-delta slowing as a base rhythm. The pseudo-periodic pattern was observed in the 29% and status epilepticus in 18%. Conclusions: In this group of patients we observed the heterogeneity of the clinical manifestations of the disease, the frequent imaging and electroencephalographic alterations and the short evolution time leading to death.

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Case series of Creutzfeldt-Jakob disease in a third-level hospital in Quito

Cited by 8 publications

References 37 publications

Updated global epidemiology atlas of human prion diseases

Updated global epidemiology atlas of human prion diseases

Rapidly Progressive Dementia in an Elderly Male: Perseverance Becomes the Key to a Rare Diagnosis

Enfermedad de Creutzfeldt-Jakob: experiencia de 5 años en un hospital terciario de Chile

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