Case Of the Week (COW) 30 August 2015
CJD
Findings:
Cortical ribbon sign Pulvinar sign Hockey stick sign DWI – restriction noted in caudate nucleus and medial thalami DTI –decreased FA values.
Discussion:
Creutzfeldt-Jakob disease and other transmissible spongiform encephalopathies (also known as prion diseases) are characterized by progressive dementia, other neurologic abnormalities, and, eventually, death. Types variant (vCJD) sporadic (sCJD) Familial (fCJD) Iatrogenic MRI findings may be bilateral or unilateral and symmetric or asymmetric, and include: T2 hyperintensity basal ganglia (putamen and caudate) thalamus (hockey stick sign and pulvinar sign ) cortex: most common early manifestation white matter persistent restricted diffusion on DWI (considered the most sensitive sign) Review of sequential studies also typically demonstrates rapidly progressive cerebral atrophy. Fluid-attenuated inversion recovery (FLAIR) images tend to reveal cortical abnormalities and may help to detect lesions that T2-weighted images fail to depict MR spectroscopic imaging revealed a decrease in N-acetylaspartate Brain perfusion imaging with 99mTc-ECD SPECT revealed decreased regional cerebral blood flow. Brain biopsy yields definitive diagnosis. No treatment. Prevention is the only option.
Contributed By:
Prof. S Babu Peter, Dr. S. Arunprasad
Barnard Institute of Radiology
CJD
Findings:
Cortical ribbon sign Pulvinar sign Hockey stick sign DWI – restriction noted in caudate nucleus and medial thalami DTI –decreased FA values.
Discussion:
Creutzfeldt-Jakob disease and other transmissible spongiform encephalopathies (also known as prion diseases) are characterized by progressive dementia, other neurologic abnormalities, and, eventually, death. Types variant (vCJD) sporadic (sCJD) Familial (fCJD) Iatrogenic MRI findings may be bilateral or unilateral and symmetric or asymmetric, and include: T2 hyperintensity basal ganglia (putamen and caudate) thalamus (hockey stick sign and pulvinar sign ) cortex: most common early manifestation white matter persistent restricted diffusion on DWI (considered the most sensitive sign) Review of sequential studies also typically demonstrates rapidly progressive cerebral atrophy. Fluid-attenuated inversion recovery (FLAIR) images tend to reveal cortical abnormalities and may help to detect lesions that T2-weighted images fail to depict MR spectroscopic imaging revealed a decrease in N-acetylaspartate Brain perfusion imaging with 99mTc-ECD SPECT revealed decreased regional cerebral blood flow. Brain biopsy yields definitive diagnosis. No treatment. Prevention is the only option.
Contributed By:
Prof. S Babu Peter, Dr. S. Arunprasad
Barnard Institute of Radiology