Answer of September 2007

Clinical History:

A 64-years-old lady who previously enjoyed good past health, has been suffering from progressive gait disturbance and cerebellar dysfunction for 5 months. She was afebrile all along. Physical examination revealed dystonic neck and upper limbs with myoclonus. EEG showed intermittent sharp wave/triphasic waves with slow background.

Diagnosis:

Creutzfeldt-Jakob disease

Discussion:

MR brain shows abnormal gyriform pattern of restricted diffusion at the grey matter of bilateral parietal, temporal, occipital and posterior frontal lobes, in a rather symmetrical manner. T2W hyperintense signal is present at the heads of the caudate nuclei and thalami. All the lesions show no contrast enhancement. Associated mild degree of cortical atrophy is found at bilateral parietal lobes. The cerebellum and brainstem are normal in appearance (not shown). Together with the characteristic waveform detected on EEG and clinical presentation, the overall features are compatible with Creutzfeldt-Jakob disease.