Background Creutzfeldt-Jacob disease or CJD is a rare neurodegenerative disorder. It is characterised by visual disturbances, myoclonus, pyramidal/extrapyramidal disturbance and rapidly progressive cognitive decline. As these symptoms are features of other more commonly presenting neurological conditions, diagnosis of CJD is difficult.
Case We describe an atypical case of an 83 year old lady who presented to a district general hospital, following a fall. She was noted to have right arm apraxia and a 3-month history of falls, but no evidence of cognitive decline. She was admitted with continuous twitching of her right arm, diagnosed as epilepsia partilais continua. MRI brain showed cortical ribboning and some apparent mild gyriform restricted diffusion seen predominantly within the left parietal and posterior frontal lobes. CSF samples showed a high protein count, and were later found to be positive for protein 14-3-3. She deteriorated rapidly and was transferred to the local neurology unit for further investigation, where she died several weeks later.
Discussion This case describes an atypical presentation of CJD with epilepsia partilais continua and without initial cognitive decline. Therefore, CJD should be considered in these cases, mostly when there are compatible findings on MRI.
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