Neurology Resident and Fellow Section

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Neurology Resident and Fellow Section A 62-year-old lady with rapidly progressive dementia Teaching NeuroImages Neurology Resident and Fellow Section © 2017 American Academy of Neurology

Vignette A 62-year-old lady, previously healthy, presented with 3 weeks of progressive short-term memory loss She had also developed dyscalculia, dysgraphia, appetite loss, and frequent episodic left arm dystonic posturing and eyebrow elevation On examination, she also had finger agnosia, dysarthria, agraphesthesia and astereognosis bilaterally, with myoclonus, ideomotor apraxia (left worse), and wide-based gait Ganesh et al. © 2017 American Academy of Neurology

A B EEG Figure 1. Electroencephalography (EEG) findings of asymmetric slowing and periodic lateralized discharges. Representative epochs of the EEG show (A) posterior alpha rhythm on the left versus theta-delta slowing over the right hemisphere and (B) periodic lateralized discharges over the right hemisphere with triphasic morphology. Ganesh et al. © 2017 American Academy of Neurology

Imaging A B C D Ganesh et al. Figure 2. Magnetic Resonance Imaging (MRI) findings of cortical and striatal diffusion restriction. Axial sections on (A) diffusion-weighted imaging, (B) apparent diffusion coefficient, (C) T2-weighted, and (D) T1-weighted MRI sequences show true diffusion restriction in the bilateral caudate and putamen and multiple gyri, more on the right (cortical ribboning), but without other changes on T2 or T1. Ganesh et al. © 2017 American Academy of Neurology

Creutzfeldt-Jakob Disease DWI and EEG findings in Creutzfeldt-Jakob Disease EEG showed slowing and periodic lateralized discharges over the right hemisphere with triphasic morphology, less often involving the left, reflecting clinical asymmetry.1 MRI Brain showed diffusion restriction in the bilateral basal ganglia with cortical ribboning more prominent on the right involving several gyri, consistent with Creutzfeldt-Jakob disease.2 CJD is an important differential diagnosis for rapidly progressive dementia, and is differentiated from other rapid dementias by its unique DWI patterns.2 References 1. Wieser HG, Schindler K, Zumsteg D. EEG in Creutzfeldt-Jakob disease. Clin Neurophysiol. 2006;117(5):935-951. 2. Vitali P, Maccagnano E, Caverzasi E, et al. Diffusion-weighted MRI hyperintensity patterns differentiate CJD from other rapid dementias. Neurology 2011;76(20):1711-1719. Ganesh et al. © 2017 American Academy of Neurology