PULVINAR or HOCKEY STICK SIGN IN CJD

Abstract

Creutzfeldt-Jakob Disease (CJD) is a
rare transmissible spongiform encephalopathy
caused by proteinecious infectious
particle called PrP(prion).Among
subtypes CJD most common is sporadic
CJD(s CJD) and less common is variant
CJD(v CJD).MRI is indispensable in
making diagnosis and ruling out other
conditions. Bilateral symmetrical abnormal
high signal intensity in the posterior
thalamus is the pulvinar sign and in dorsomedial
thalamic nuclei pulvinar is the
hockey-stick sign. In the appropriate
clinical context the MRI identification of
pulvinarhockey stick sign is a useful noninvasive
test for the diagnosis of vCJD
(upto 90sensitivity) and can avoid tonsillar
biopsy.The pulvinar sign was originally
defined simply as hyperintensity of
the pulvinar. In young patients, the normal
basal ganglia are relatively hyperintense
compared to old and rarely in
sCJD patients can have pulvinar sign
and this could be mistaken for vCJD.
Hence the pulvinar sign is redefined as
hyperintensity of the pulvinar
relative to the signal intensity of the anterior
putamen. Hyperintensity of all deep
grey-matter nuclei, with the pulvinar less
bright than the anterior putamen and caudate
is a feature of sCJD. Here we describe
a case of sCJD with pulvinarhockey
stick sign mimicking vCJD. Our patient had
pulvinar hyperintensity less bright than putamen,
cortical ribboning and age of onset
in fifth decade which were typically suggestive
of sCJD. So far vCJD has not been reported
in our country.