Prion diseases are rare, fatal neurodegenerative disorders caused by misfolded proteins. Sporadic Creutzfeldt-Jakob Disease (sCJD) is the most common form and develops without any identifiable triggers. This case report documents the first probable diagnosis of sCJD in Syria, involving a 72-year-old woman who presented with depressive symptoms for several months before rapidly deteriorating, and eventually succumbing to complications of the disease. Clinical examination findings, exclusion of CJD-mimicking conditions, and specific MRI findings, supported the diagnosis in the absence of biochemical tests or postmortem confirmation. This report emphasizes the challenges of diagnosing prion diseases in resource-limited settings and calls for the development of national surveillance systems in low- and middle-income countries to aid early detection, monitor the global disease burden, and reduce the risk of outbreaks. Raising awareness of prion diseases among physicians in such contexts is crucial for improving disease recognition.
Keywords: epidemiology; neurology; prion diseases; rapidly progressive dementia.
This report describes the first likely case of a rare brain disease in Syria. The disease, called sporadic Creutzfeldt-Jakob Disease (sCJD), is caused by misfolded proteins and is always fatal. The patient was a 72-year-old woman. She first showed signs of depression for a few months. After that, her condition worsened very quickly, and she died from complications of the disease. Because specialized lab tests and an autopsy were not available, doctors diagnosed her based on her symptoms, by ruling out other diseases that look similar, and on specific brain scan (MRI) results. This case shows how hard it is to diagnose such diseases in places with limited medical resources. The report recommends that lower-income countries create national systems to track this disease. This would help find cases early, better understand how common it is worldwide, and prevent outbreaks. It is also crucial to train doctors in these areas to recognize the disease.
© 2025 Tarboosh et al.