Unilateral cortical hyperintensity in diffusion-weighted MRI; New criteria for early sporadic Creutzfeldt-Jakob disease

Sporadic Creutzfeldt-Jakob disease (sCJD) is a rapidly progressive fatal prion disease. The proposed diagnostic criteria1,2 are not sufficiently helpful for diagnosis in early stages of the disorder. A 69-year-old female was brought to our hospital with a history of 3 weeks left side hemiparesis and the progressive loss of speech and attention. She was awake and mute without any purposeful behavior. Left side hemiplegia, hyperreflexia and Babinski sign was also detected. Brain magnetic resonance imaging (MRI) revealed asymmetric diffuse gyriform hyperintensity in right cortical area and fine signal changes in right caudate and putamen in diffusionweighted imaging (DWI) sequence without any significant involvement in left side and no signal abnormality in other sequences (Figure 1). Laboratory tests, including blood count, glucose, renal, hepatic and thyroid function tests, electrolytes, sedimentation rate, B12 and folic acid levels were normal. Human T-lymphotropic virus 1 (HTLV1) and 2, human immunodeficiency virus (HIV) and paraneoplastic antibodies, anti-thyroid peroxidase, anti-thyroglobulin and venereal disease research laboratory tests were negative. A repeated MRI 7 days later revealed fine hyperintense signals in left inferior frontal, angular and postcentral gyri on DWI sequences, although the signal changes were still clearly asymmetric, and no abnormality was present in other sequences. At fifteenth day of admission, she experienced myoclonic jerks and 2 days later, the typical pattern of periodic sharp wave complexes was appeared in electroencephalography. Pathological study of right frontal cortical biopsy disclosed neuronal loss and spongiform changes (Figure 2).