รายงานกรณีศึกษาผู้ป่วยProbable Sporadic Creutzfeldt-Jakob Diseaseที่พบในโรงพยาบาลหัวหิน

Probable Sporadic Creutzfeldt-Jakob Disease: A First Case Report in Hua-Hin Hospital, Thailand. How to Approach and Early Diagnosis?

Abstract

Sporadic Creutzfeldt-Jakob disease (sCJD) is a rare and fatal spongiform encephalopathy which characterized by rapidly progressive dementia and myoclonus. The incidence of Creutzfeldt-Jakob disease is very rare about 1-2 case per million population around the world. The rarity of this disease and varied initial symptoms make the challenge of early diagnosis. So, I would like to present a case initially admitted to the hospital for blur vision, visual hallucination and gait difficulty. Four weeks later, he developed myoclonic jerks, akinetic mutism and altered mental status. Cerebrospinal fluid (CSF) analysis for infection and immune mediated encephalitis were negative. Brain magnetic resonance imaging (MRI) showed hyperintensities in the bilateral caudate, globus pallidus, bilateral parieto-occipital regions in diffusion-weighted imaging (DWI). Then the patient was sent to performed electroencephalogram (EEG) and showed generalized periodic lateralizing epileptiform discharges. The probable diagnosis of sCJD was reached based on the clinical features, characteristic findings in MRI and EEG. The literature was also reviewed for early diagnosis and how to approach of sCJD. This report emphasizes that clinicians should be aware of sCJD presenting as a visual problem and cognitive decline. Early diagnosis is crucial for evaluated prognosis and awareness about preventing transmission of health care providers who contacted the patients.
Keyword: Sporadic Creutzfeldt-Jakob disease (sCJD), rapidly progressive dementia