Creutzfeldt-Jakob · Disease with a Case Report

Abstract

Scientific Background: Typical Creutzfeldt-Jakob Disease (CJD) is characterized by rapidly progressive dementia associated with ataxia and myoclonus and demonstrate bilaterally synchronized - periodic discharges in EEG. 14.3.3 protein levels may be found elevated in CSF. These features may be absent in atypical subgroups. Without neuropathological examination only possible or probable cases of CJD can be diagnosed. Objective: We report a case of a 70 years old woman diagnosed as definite CJD with the aid of neuropathological examination and aim to revise information about this disease. Case: She admitted with ataxia and behavioral changes. Startle reactions and myoclonic jerks were observed following the deterioration in her conscious level. While there had been non-specific slow wave activity in EEG initially, bilateral-synchronized - triphasic periodic discharges were recorded as the clinical status progressed. 74.3.3 protein level was found elevated in cerebrospinal fluid. Probable CJD was contemplated. Necropsy and neuropathological examination was performed after her death. Findings: Neuronal loss, astrogliosis, light to moderate spongiform degeneration were detected in all cortical cross-sections and cerebellum. lmmunosytochemistry with anti-PrP monoclonal antibodies KG9 and 6H4 demonstrated the presence of protease-resistant prion protein which support the definite diagnosis of CJD. Conclusion: This is a neuropathologically established case of intermediate stage CJD. Clinical and EEG features were consistent with typical sporadic group. She was considered to be tost before proceeding to terminal stage of the disease. EEG follow-up is thought to be valuable to diagnose probable CJD. Differentiating from new variant CJD clinically could be troublesome in cases without symmetrical-periodic discharges. Definite CJD can only be identified with neuropathological examination.