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Creutzfeldt-Jakob disease in Oman: report of two cases
E M Scrimgeour1, P R Chand, K Kenny
1Department of Medicine, Sultan Qaboos University, Al-Khod (Muscat), Oman.
Insights
This report details the first diagnosed cases of sporadic Creutzfeldt-Jakob disease (CJD) in Oman. Diagnostic challenges due to cultural practices were overcome using cerebrospinal fluid analysis.
Area of Science:
- Neurology
- Neuroscience
- Prion Diseases
Background:
- Sporadic Creutzfeldt-Jakob disease (sCJD) is a rare, fatal neurodegenerative disorder.
- Early diagnosis is crucial for patient management and understanding disease progression.
Observation:
- Two Omani Arab men, aged 50 and 75, presented with rapidly progressive dementia and myoclonic jerks.
- Clinical symptoms evolved over 3-6 months.
- Electroencephalography revealed characteristic periodic triphasic sharp waves.
Findings:
- Cerebrospinal fluid electrophoresis in one patient identified distinctive double protein spots, confirming sCJD.
- Autopsy was not feasible due to cultural practices in the Middle East.
Implications:
- This marks the first documented cases of sCJD in Oman.
- Highlights the utility of CSF analysis in diagnosing sCJD when brain biopsy/autopsy is not possible.
- Contributes to the global understanding of prion disease distribution and diagnosis.
Abstract:
Sporadic Creutzfeldt-Jakob disease (CJD) was diagnosed in two Oman Arab men, aged 50 and 75 years respectively, both with a history of rapidly developing dementia and myoclonic jerks. Illness developed over a period of 3 months in the first case and over six months in the second. Electroencephalography in both subjects showed periodic triphasic sharp waves characteristic of CJD. In neither case was it possible to obtain a brain biopsy or perform autopsy (autopsy is contrary to Islamic practice in the Middle East), however, electrophoresis of cerebrospinal fluid from the second patient revealed the distinctive double protein spots characteristic of CJD. This is the first report of CJD from Oman.