[Bilateral hyperintensity of the pulvinar in sporadic Creutzfeldt-Jakob disease].
BACKGROUND
Definitive diagnosis of Creutzfeldt-Jakob disease (CJD) is made by pathological study. The premortem differentiation of variant forms is important in public health. Bilateral hyperintensity of the thalamic pulvinar nucleus in T2, FLAIR and DW sequences ("pulvinar sign") in Magnetic Resonance Imaging (MRI) is related to the variant Creutzfeldt-Jakob disease (vCJD) and is a diagnostic criterion of probable vCJD. A case of sporadic CJD with bilateral hyperintensity of pulvinar nucleus is described.


METHODS
A case of a 64 year old male with progressive gait and cognitive functions impairment, disorientation, hallucinations, delirious, language alterations and dysphagia of 4-5 months progress is presented. Clinical examination revealed unintelligible speech, almost anartric, tetraparesis with leg predominance, bilateral Babinski sign, arms dysmetria and dysdiadochokinesia, with action myoclonias.


RESULTS
progressive deterioration, akinetic mutism and death in 6-7 months following the onset of symptoms. The 14-3-3 protein was positive in cerebrospinal fluid. Electroencephalogram had no periodic sharp-wave complexes. MRI: bilateral and symmetrical lesions in putamen, caudate and pulvinar nucleus, hyperintenses in T2, FLAIR and DW; bilateral hyperintensity of frontoparietal and insular cortex; the bilateral high signal in caudates and putamen is greater than the hyperintensity in pulvinar. No mutation was found in the analysis of the prionic protein (PRNP) gene. Codon 129 polymorphism: methionine/valine heterozygosis (129 M/V). The neuropathological study confirmed the diagnosis of sporadic CJD.


CONCLUSIONS
Bilateral pulvinar hyperintensity can be present in sporadic CJD. A true pulvinar sign (consistent with vCJD) must be considered only if the high signal in the pulvinar is greater than the hyperintensity in the caudate and putamen nucleus.
de la Torre Laviana
F J
FJ
Hospital de Jerez, Jerez de la Frontera, Cádiz.
D007562:Creutzfeldt-Jakob Syndrome / Q000175:diagnosis* / Q000473:pathology* / Q000503:physiopathology; D038524:Diffusion Magnetic Resonance Imaging; D006801:Humans; D008297:Male; D008875:Middle Aged; D011110:Polymorphism, Genetic; D011328:Prions / Q000235:genetics; D020649:Pulvinar / Q000473:pathology*
D011328:Prions