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2016 ; 7
(ä): 138
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Creutzfeldt-Jakob Disease: Analysis of Four Cases
#MMPMID27621721
Al Balushi A
; Meeks MW
; Hayat G
; Kafaie J
Front Neurol
2016[]; 7
(ä): 138
PMID27621721
show ga
BACKGROUND: Creutzfeldt-Jakob disease (CJD) is a rare, rapidly progressive
neurodegenerative disease that almost always results in death in under a year
from onset of symptoms. Here, we report four cases of CJD with different clinical
presentations diagnosed at our institution over a 2-year period. CASES: The first
patient is an 82-year-old woman who presented with depression, cognitive decline,
and word-finding difficulty over 4?weeks. The patient deteriorated neurologically
to akinetic mutism and death within 6?weeks of presentation. The second patient
is a 54-year-old woman with liver cirrhosis who presented with confusion, ataxia,
and multiple falls over 4?weeks. She was treated initially for hepatic
encephalopathy but continued to progress to mutism, startle myoclonus, and
obtundation. Death occurred within 4?weeks of presentation. The third patient is
a 58-year-old woman who presented with an 8-week history of confusion, urinary
incontinence, Parkinsonism, ataxia, and myoclonus. Death occurred within 2?months
from presentation. The fourth patient is a 67-year-old man who presented with a
6-week history of headache, blurred vision, ataxia, and personality change and
progressed to confusion, myoclonus, akinetic mutism, and obtundation. Death
occurred within 3?weeks from presentation. CONCLUSION: These four cases highlight
the varied possible clinical presentations of CJD and demonstrate the importance
of considering CJD in patients with atypical presentations of rapidly progressive
cognitive decline. To diagnose CJD, brain biopsy remains the gold standard.
However, the presence of CSF protein 14-3-3, typical MRI findings and suggestive
EEG abnormalities, all support the diagnosis.
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