Probable Creutzfeldt Jacob Disease (CJD), Bansang District, Prachinburi Province, August - October 2010

Abstract

Background: Bureau of Epidemiology (BoE) got a report from Prachinburi Provincial Health Office on 23 August 2010 of a suspected CJD case admitted and died at Chaophraya Abhaibhubejhr Hospital on 12 Aug 2010. BoE team, Surveillance Rapid Response Team (SRRT) of Bansang Hospital, Prachinburi Provincial Health Office and officers of Prasat Neurological Institute conducted this investigation from 26 August to 15 October 2010 to verify diagnosis
Methods: We reviewed medical record in hospitals and interviewed physicians, family members, and colleges. Active case finding was performed by interview case’s contacts and village health volunteer. A suspect case was defined as a family member of the case or colleges, lived in Bansang district, Prachinburi province, had a history of either progressive dementia or neuropsychiatric symptoms. A probable case was defined as a suspect case that had at least two following signs: pyramidal/extrapyramidal signs, akinetic mutism, chorea, hyperreflexia, myoclonus, visual or cerebellar signs. A confirmed case was defined as a probable case that had the confirmation of neuropathological spongiform change.
Results: The case was a Thai female teacher 59 years old who lived in Bansang district, Prachinburi province. Her onset was in April, 2010. During late June to August 2010, she had dizziness, vertigo, ataxia, and insomnia, followed by progressive memory loss, behavior and mood changed and loss of direction. She had no history of surgery, corneal graft, transplantation, transfusion and travel aboard, no history of cow or buffalo care, no familial history of dementia or psychiatric symptoms. Abnormal neurological sign included disorientation to time, recent memory, calculation, hyperreflexia and wide base gait. Brain MRI finding is multifocal abnormal hypersignal intensities at the cortices of bilateral cerebral hemispheres and at bilateral caudate nuclei on DWI. Diagnosis was rapid progressive dementia associated with sCJD.
Conclusions: This is a case investigation of clinically probable sporadic CJD without pathological confirmation. Thai medical neurological expert consultants or committees should be established to conclude the definite diagnosis in CJD dead case conference. Sentinel case registry for disease caused by abnormal prion protein, especially vCJD and BSE should be established soon.