An ominous radiographic feature: cortical ribbon sign

SIMI 2015 A 59-year-old woman presented with a 2-month history of headache, difficulty in finding words, and slow speech. Those symptoms were associated with intermittent bilateral jerky movements of the upper extremities. The patient had noted a memory decline over the prior 2 months. Her past medical history was significant for hypothyroidism, and her medications included levothyroxine. Her father was diagnosed with Alzheimer’s disease at the age of 85. Physical examination did not reveal myoclonus, motor, or sensory deficits, hyper- or hyporeflexia, cerebellar signs, or ataxic gait. The patient had intermittent dysphonia, but her speech comprehension was intact. Results of laboratory studies, including a complete blood count, comprehensive metabolic profile, thyroid function tests, anti-thyroglobulin level, vitamin B12 level, homocysteine level, erythrocyte sedimentation rate, antinuclear antibody, anti-double stranded antibody, anti-endomysial antibody, rapid plasma reagin test, Lyme antibody test, HIV-1/2 immunoassay, serum cryptococcal antigen, paraneoplastic panel, heavy metal screening, and drug toxicological tests, were normal. The electroencephalography (EEG) study was normal. Magnetic resonance imaging (MRI) brain with and without intravenous gadolinium, including diffusion-weighted imaging (DWI), fluid-attenuated inversion recovery (FLAIR), and apparent diffusion coefficient (ADC) sequences, was performed. Cerebral MRI DWI demonstrated a ribbon-like signal hyperintensity of cerebral cortical gyri (cortical ribboning) of the right frontal, parietal, and occipital regions, known as the cortical ribbon sign