Case reports of two biopsy-proven patients with Creutzfeldt-Jakob disease in Singapore.
BACKGROUND
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.


METHODS
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.


RESULTS
Treatment remains palliative. Deterioration in their clinical condition was relentless, progressing to a totally dependent state within 10 to 12 months.


CONCLUSIONS
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.
See
S J
SJ
Department of Neurology (NNI-SGH), Singapore General Hospital, Singapore.
Pan
A
A
Seah
A
A
Teo
J
J
Chan
L L
LL
Wong
M C
MC
D001707:Biopsy, Needle; D007562:Creutzfeldt-Jakob Syndrome / Q000175:diagnosis / Q000473:pathology* / Q000628:therapy; D018450:Disease Progression; D004569:Electroencephalography; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D010166:Palliative Care / Q000379:methods; D012307:Risk Factors; D012720:Severity of Illness Index; D012846:Singapore