Creutzfeldt-Jakob Disease in Algeria: Challenges in Diagnosis and Public Health Response

Abstract

Creutzfeldt-Jakob Disease (CJD), a rare and fatal neurodegenerative disorder caused by prions, poses significant diagnostic and public health challenges in Algeria, where limited resources, lack of specialized diagnostic tools, and low awareness among healthcare providers exacerbate the situation. This paper examines the current state of CJD diagnosis and management in Algeria, highlighting the barriers to early detection, such as the absence of advanced neuroimaging and laboratory testing facilities, and the overlap of symptoms with more common neurological conditions. A situational analysis reveals the gaps in surveillance systems and public health infrastructure, while a literature review explores global insights into CJD’s etiology, including its potential links to autoimmune processes and, more speculatively, to vaccines. The discussion emphasizes the need for improved diagnostic capabilities and public health responses tailored to Algeria’s healthcare context. Recommendations include the establishment of national surveillance programs, training for healthcare professionals, and international collaboration to enhance diagnostic capacity. This paper concludes that addressing CJD in Algeria requires a multi-faceted approach combining scientific, policy, and community engagement efforts to mitigate the impact of this devastating disease.

Introduction

Creutzfeldt-Jakob Disease (CJD) is a rare, degenerative, and invariably fatal neurological disorder classified as a transmissible spongiform encephalopathy (TSE). Caused by misfolded prion proteins, CJD leads to progressive dementia, motor dysfunction, and ultimately death, often within a year of symptom onset. Globally, CJD occurs in sporadic, familial, iatrogenic, and variant forms, with the sporadic form being the most common, accounting for approximately 85% of cases. The disease’s rarity—occurring at a rate of about 1-2 cases per million people annually—combined with its rapid progression and non-specific early symptoms, makes diagnosis challenging, particularly in resource-limited settings such as Algeria.

In Algeria, a North African country with a population of over 44 million, healthcare systems face significant challenges, including limited access to specialized medical equipment and expertise, particularly in rural areas. Neurological disorders, while not among the leading causes of mortality, represent a growing concern as the population ages and diagnostic capabilities improve for other conditions. However, CJD remains underdiagnosed and underreported, largely due to the lack of awareness among healthcare providers and the absence of robust surveillance systems for rare diseases. This paper seeks to explore the specific challenges associated with diagnosing and managing CJD in Algeria, assess the current public health response, and propose actionable recommendations to address these gaps. Additionally, the etiology of CJD, including speculative links to autoimmune mechanisms and vaccines, will be discussed in light of available evidence.

Situational Analysis

Algeria’s healthcare system is characterized by a dual public-private structure, with the public sector providing free or low-cost services to a majority of the population. However, disparities exist between urban and rural areas, with advanced medical technologies and specialists concentrated in major cities like Algiers, Oran, and Constantine. For a disease like CJD, which requires sophisticated diagnostic tools such as magnetic resonance imaging (MRI) with diffusion-weighted imaging (DWI), cerebrospinal fluid (CSF) testing for 14-3-3 protein, and, in some cases, brain biopsy or autopsy for definitive diagnosis, these disparities pose significant barriers.

Currently, there are no publicly available data on the incidence or prevalence of CJD in Algeria, reflecting a broader issue of underreporting and lack of surveillance for rare diseases. Anecdotal evidence from neurologists in urban hospitals suggests that suspected cases are often misdiagnosed as other forms of dementia or neurological conditions, such as Alzheimer’s disease or stroke, due to overlapping clinical presentations and the absence of confirmatory testing. A study published on the diagnostic challenges of CJD notes that even in well-resourced settings, misdiagnosis rates are high in the early stages due to the disease’s atypical presentation (Cureus, 2025). In Algeria, where electroencephalogram (EEG) and MRI facilities are limited outside tertiary care centers, the likelihood of misdiagnosis is even greater.

Public health responses to CJD in Algeria are virtually nonexistent, as the disease is not prioritized amid more pressing health concerns such as infectious diseases (e.g., tuberculosis) and non-communicable diseases (e.g., diabetes and cardiovascular conditions). There are no national guidelines for the diagnosis or management of CJD, nor are there protocols for handling potential iatrogenic transmission risks, such as through contaminated surgical instruments—a known mode of transmission in historical cases elsewhere. Additionally, cultural factors, including stigma associated with neurological and mental health disorders, may deter families from seeking medical care for affected individuals, further complicating surveillance efforts.

The lack of specialized training for healthcare providers compounds these challenges. Many general practitioners and even some neurologists in Algeria are unfamiliar with the clinical hallmarks of CJD, such as myoclonus, ataxia, and rapidly progressive dementia. Without awareness and access to diagnostic criteria established by organizations like the World Health Organization (WHO), suspected cases are unlikely to be flagged for further investigation. This situational analysis underscores the urgent need for capacity building and infrastructure development to address CJD in Algeria.

Literature Review

CJD is caused by prions, infectious proteins that induce abnormal folding of normal cellular prion proteins in the brain, leading to neuronal loss, spongiform changes, and gliosis. The disease exists in four main forms: sporadic (sCJD), familial (fCJD) linked to mutations in the PRNP gene, iatrogenic (iCJD) from medical procedures, and variant (vCJD) associated with bovine spongiform encephalopathy (BSE) transmission through contaminated beef (Mayo Clinic, 2037). Sporadic CJD, the most common form, has no known environmental or genetic trigger in most cases, though certain PRNP gene polymorphisms may confer susceptibility. The global incidence of CJD is estimated at 1-2 cases per million annually, with no significant geographic variation except in specific clusters, such as among Libyan Jews in Israel, where a genetic predisposition has been documented (PubMed, 1991).

Diagnosing CJD remains a complex endeavor. According to a comprehensive review, clinical diagnosis relies on a combination of rapidly progressive dementia, characteristic neurological signs (e.g., myoclonus, visual or cerebellar disturbances), and supportive findings from MRI, EEG, and CSF analysis (StatPearls, 2025). MRI with DWI often shows hyperintensities in the cortex and basal ganglia, while EEG may reveal periodic sharp wave complexes. CSF testing for 14-3-3 protein and elevated tau levels provides additional diagnostic support, though these tests are not universally available. Definitive diagnosis, however, requires neuropathological examination, typically postmortem, highlighting the difficulty of confirming CJD during a patient’s lifetime.

Etiologically, the prion hypothesis remains the cornerstone of CJD research, but alternative theories, including potential autoimmune contributions, have been proposed. Some studies suggest that chronic inflammation or immune dysregulation could play a role in prion propagation or neurodegeneration, as immune cells like microglia are activated in CJD-affected brains. However, there is no conclusive evidence that CJD is an autoimmune disorder in the classical sense, where the immune system directly targets self-antigens. Instead, immune responses appear to be secondary to prion-induced damage (ScienceDirect, 2024).

Regarding vaccines, there is no robust evidence linking immunization to the development of CJD. Historical concerns about iatrogenic transmission through contaminated human-derived growth hormone or dura mater grafts have led to stringent safety protocols in medical products, but vaccines, which are not derived from human tissue in most cases, have not been implicated. Speculative discussions in the literature about vaccine adjuvants or immune stimulation triggering prion diseases lack empirical support and remain controversial. The U.S. Food and Drug Administration (FDA) and other regulatory bodies have not identified any association between vaccines and CJD or vCJD (FDA, 2024).

Public health responses to CJD globally vary widely. In Europe, the European Creutzfeldt-Jakob Disease Surveillance Network (EuroCJD) provides diagnostic support and monitors vCJD cases across member states (ECDC, 2023). Such coordinated efforts are absent in many developing regions, including North Africa, where resources are diverted to more prevalent health issues. Literature on CJD in Algeria specifically is scarce, reflecting a broader gap in research on rare diseases in the region. This review highlights the need for localized studies to understand the epidemiology of CJD in Algeria and tailor public health strategies accordingly.

Discussion

The challenges of diagnosing and managing CJD in Algeria are multifaceted, encompassing clinical, systemic, and sociocultural dimensions. Clinically, the disease’s rarity and non-specific initial symptoms—such as memory loss, behavioral changes, and confusion—often lead to misdiagnosis as more common conditions like Alzheimer’s disease or vascular dementia. The absence of advanced diagnostic tools, such as high-resolution MRI or CSF testing capabilities, in most Algerian hospitals further complicates early identification. Even when suspicion of CJD arises, the lack of neuropathological expertise and facilities for postmortem examination means that definitive diagnoses are rarely achieved.

Systemically, Algeria’s healthcare infrastructure is ill-equipped to handle rare diseases like CJD. The concentration of specialized care in urban centers leaves rural populations particularly vulnerable, as patients must travel long distances to access neurologists or diagnostic services. Moreover, the cost of diagnostic tests, when available through private facilities, is prohibitive for many families, and public hospitals often face equipment shortages or long waiting times. The lack of a national registry or surveillance system for CJD means that the true burden of the disease remains unknown, hindering efforts to allocate resources or develop targeted interventions.

Socioculturally, stigma surrounding neurological and mental health disorders in Algeria may prevent families from seeking care, especially when symptoms are perceived as psychiatric rather than organic. Additionally, low awareness among healthcare providers contributes to delayed referrals and missed opportunities for diagnosis. Public health responses are similarly constrained by limited funding and competing priorities. Without national guidelines or policies for CJD, there is no framework for managing suspected cases, educating providers, or preventing iatrogenic transmission through surgical instruments or blood products.

Regarding etiology, while the prion hypothesis is well-established, the potential role of immune dysregulation in CJD pathogenesis warrants further exploration. Although CJD is not classified as an autoimmune disease, the involvement of inflammatory pathways suggests that immune-modulating factors could influence disease progression. This is a speculative area of research, and no studies specific to Algeria address this angle. Similarly, the notion of vaccines triggering CJD lacks credible evidence and appears to stem from broader vaccine hesitancy narratives rather than scientific data. Public health efforts in Algeria should prioritize dispelling such myths through education, ensuring that focus remains on verifiable risks and prevention strategies.

Comparing Algeria’s situation to global standards, such as those set by the EuroCJD network, reveals stark disparities. In Europe, coordinated surveillance, standardized diagnostic criteria, and international collaboration have improved CJD detection and management. Algeria, however, lacks the infrastructure and political will to implement similar systems, reflecting a broader trend in low- and middle-income countries where rare diseases are often sidelined. Addressing CJD in Algeria requires not only technical solutions but also policy advocacy to elevate the disease’s visibility on the public health agenda.

Recommendations

To address the challenges of diagnosing and managing CJD in Algeria, a multi-pronged approach is necessary. The following recommendations are proposed to improve clinical capabilities, public health responses, and community engagement:

1. Enhance Diagnostic Capacity: Invest in advanced neuroimaging (e.g., MRI with DWI) and laboratory testing facilities in major hospitals across Algeria. Establishing at least one national reference center for prion diseases, equipped with CSF testing and neuropathological expertise, would provide a hub for diagnosis and research.
2. Training and Awareness: Develop training programs for healthcare providers, including general practitioners, neurologists, and pathologists, on the clinical presentation and diagnostic criteria for CJD. Public awareness campaigns should also be launched to reduce stigma and encourage families to seek medical care for neurological symptoms.
3. Surveillance and Policy Development: Create a national registry for CJD and other rare neurodegenerative diseases to track incidence, prevalence, and outcomes. Formulate national guidelines for CJD diagnosis, management, and infection control to prevent iatrogenic transmission, aligning with WHO recommendations.
4. International Collaboration: Partner with regional and international organizations, such as the WHO or EuroCJD, to access technical support, diagnostic resources, and training opportunities. Collaboration could also facilitate genetic studies to identify potential familial clusters of CJD in Algeria.
5. Research and Funding: Allocate funding for local research on CJD epidemiology and risk factors in Algeria. Studies exploring cultural attitudes toward neurological disorders could inform public health messaging, while investigations into environmental or genetic contributors could uncover localized patterns.

These recommendations, while ambitious, are essential for building a sustainable framework to address CJD in Algeria. Prioritizing rare diseases within the broader healthcare agenda will require advocacy and political commitment, alongside partnerships with global health entities.

Conclusion

Creutzfeldt-Jakob Disease remains a significant yet underrecognized challenge in Algeria, where limitations in diagnostic tools, healthcare infrastructure, and public health systems hinder effective management. The disease’s rarity, combined with its rapid progression and fatal outcome, underscores the urgency of addressing gaps in detection and response. While the etiology of CJD is primarily linked to prions, speculative discussions about autoimmune contributions and vaccine associations lack substantial evidence and should not detract from evidence-based interventions. This paper has highlighted the multifaceted barriers to CJD care in Algeria, from clinical misdiagnosis to systemic underpreparedness, and proposed actionable recommendations to build capacity and awareness. By investing in diagnostic infrastructure, training, surveillance, and international collaboration, Algeria can mitigate the impact of CJD and improve outcomes for affected individuals. Future efforts should focus on integrating rare disease management into national health strategies, ensuring that even the most uncommon conditions receive the attention they deserve.

References

• Cureus. (2025). Diagnostic Challenges in Creutzfeldt-Jakob Disease: A Case Report of an Atypical Presentation. Available at: https://www.cureus.com/articles/347861-diagnostic-challenges-in-creutzfeldt-jakob-disease-a-case-report-of-an-atypical-presentation
• European Centre for Disease Prevention and Control (ECDC). (2023). European Creutzfeldt-Jakob Disease Surveillance Network (EuroCJD). Available at: https://www.ecdc.europa.eu/en/about-ecdc/what-we-do/partners-and-networks/disease-and-laboratory-networks/european-creutzfeldt
• Mayo Clinic. (2037). Creutzfeldt-Jakob Disease: Symptoms & Causes. Available at: https://www.mayoclinic.org/diseases-conditions/creutzfeldt-jakob-disease/symptoms-causes/syc-20371226
• PubMed. (1991). Genetic and Environmental Factors Determining the Development of Creutzfeldt-Jakob Disease in Libyan Jews. Available at: https://pubmed.ncbi.nlm.nih.gov/1798423/
• ScienceDirect. (2024). Creutzfeldt-Jakob Disease: A Comprehensive Review of Current Understanding and Research. Available at: https://www.sciencedirect.com/science/article/abs/pii/S0022510X24004295
• StatPearls. (2025). Creutzfeldt-Jakob Disease. NCBI Bookshelf. Available at: https://www.ncbi.nlm.nih.gov/books/NBK507860/
• U.S. Food and Drug Administration (FDA). (2024). Variant Creutzfeldt-Jakob Disease (vCJD) and Factor VIII (pdFVIII) Questions and Answers. Available at: https://www.fda.gov/vaccines-blood-biologics/safety-availability-biologics/variant-creutzfeldt-jakob-disease-vcjd-and-factor-viii-pdfviii-questions-and-answers