Introduction: Creutzfeldt-Jakob disease (CJD) is the most common transmissible human subacute spongiform encephalopathy. There is limited literature on CJD in Southeast Asia. We describe the clinical course and diagnostic evaluation of 2 Singapore patients with biopsy-proven CJD.Clinical Picture: Two patients presented with non-specific symptoms such as withdrawal, forgetfulness, asthenia, giddiness and insomnia. Both patients had spontaneous myoclonic jerks and impairment of multiple neurologic systems (visual, pyramidal, cerebellar and neurocognitive systems). Magnetic resonance imaging and electroencephalography provided helpful supportive evidence. Diagnosis of CJD was established on brain biopsy. Histological features included spongioform degeneration, neuronal cell loss and astrocytosis. Treatment/Outcome: Treatment remains palliative. Deterioration in their clinical condition was relentless, progressing to a totally dependent state within 10 to 12 months. Conclusion: The early features of CJD can be varied and non-specific. It is important for physicians from different specialties to be cognisant of the clinical manifestations of CJD and the appearance of supportive and definitive investigations.
Creutzfeldt-Jakob disease (CJD) is the commonest human transmissible subacute spongiform encephalopathy. It has a worldwide distribution with an estimated annual incidence of 0.5 to 1.0 per million population. However, the incidence of CJD in the Singaporean population is not known. With a population of 4 million, an estimated 2 to 4 patients newly diagnosed with CJD per year are expected.
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