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Creutzfeldt-Jakob disease (CJD) is a rare and rapidly progressive neurodegenerative disorder which is often fatal within a year of onset of symptoms. We describe a case of a 62-year-old woman who presented with symptoms of disorientation, particularly environmental and visual agnosia, worsening over 18 months without any cognitive deficit. It was a change in her speech and some unsteadiness in her gait which led her to seek neurology advice. An MRI revealed Gyriform restricted diffusion with corresponding low ADC signal, particularly in both parietal and occiptial lobes suspicous of spongiform encephalopathy, particularly Heidenhain variant of CJD. CSF samples were sent for RT-QUic which came back positive, confirming a diagnosis of CJD (likely sporadic). The absence of any behavioural changes or obvious memory loss and the unusually long course of symptoms for CJD made the diagnosis more challenging. Heidenhain variant more commonly presents with blurred vision, visual field restriction, metamorphopsia or cortical blindness and is often associated with a shorter disease course, further conflicting with our patient’s journey. This case highlights the importance of considering a wide differential when faced with an unusual set of symptoms, particularly if things don’t follow the common path.v.maccallum@nhs.net