Fig 1. A, Axial diffusion-weighted image shows symmetric striatal and thalamic atrophy and widespread cortical high signal intensity. Apparent diffusion coefficient maps (not shown) revealed corresponding decreased cortical signal intensity, confirming restricted water diffusion. B, Axial fluid-attenuated inversion recovery image demonstrates pontine "hot cross bun" sign, atrophic and hyperintense middle cerebellar peduncles, global cerebellar atrophy, and cortical temporal lobe high signal intensity.

We present the second variant Creutzfeldt-Jacob patient in the same district of northwest Portugal as was previously reported. A 14-year-old previously healthy girl had unexplained pain in the left leg, as well as psychiatric disturbances. This was shortly followed by progressive cognitive impairment, ataxia and generalised choreoatethosis. Neuropsychological assessment revealed severe frontal and medial temporal dysfunction, the posterior cortices being spared. An electroencephalogram was normal. CSF 14.3.3 protein was slightly positive. Magnetic resonance imaging showed the "hockey stick sign" and hyperintensities in the periaquedutal grey matter and in the right parietal cortex, the last with restriction to water molecule movement. SPECT revealed perfusion defects in the left frontotemporal and right parietal regions. PRNP gene sequencing showed no mutations, the patient being homozygous to methionine in codon 129. Five months after onset, immunocytochemical and immunoblotting analysis confirmed deposition of prion protein and a PrP4t electrophoretic pattern. The patient never travelled outside Portugal or received blood transfusions. She had surgical herniorrhaphy in 1998 (when catgut was used) and 2003. This is the second case in Portugal in a 2-year period and 20 km apart from each other, with no known common exposure apart from ingestion of cow meat. We discuss these case peculiarities and underline its epidemiological significance.

Primary bilateral thalamic gliomas are exceedingly rare and usually present with behavioral and cognitive abnormalities, sparing motor and sensory functions.1 Brain imaging reveals bilateral homogeneous enlargement of thalamic nuclei, which appear hyperintense on T2-weighted imaging.