NR ALGJ
AU Summers,D.M.; Collie,D.A.; Sellar,R.J.; Zeidler,M.; Knight,R.S.G.; Will,R.G.; Ironside,J.W.
TI The pulvinar sign and diagnosis of Creutzfeldt-Jakob disease
QU Neurology 2002 Sep 24; 59(6): 962; discussion 962
KZ Neurology. 2002 Jan 8;58(1):148-9. PMID: 11781427
PT comment; letter
VT
To the Editor:
Haïk et al. present a case report of a patient with histologically confirmed sporadic Creutzfeldt-Jakob disease (sCJD) with bilateral increased signal in the pulvinar of the thalamus. [1] We felt it important to clarify recent changes in the definition of the pulvinar sign to avoid difficulties in future diagnosis of variant CJD (vCJD) using MRI.
The presence of pulvinar hyperintensity was originally described as a characteristic feature of vCJD by Sellar et al. [2] in 1997, and the high sensitivity and specificity of this sign were subsequently documented by our group. [3] Clinical criteria for a diagnosis of vCJD were formulated that incorporated these MRI findings with diagnostic changes defined as bilateral pulvinar high signal on MRI scan. [4] It has been recognized that this definition was ambiguous, as it was unclear with which structure the degree of pulvinar hyperintensity was compared.
Recently, a more specific definition of the pulvinar sign has been developed and included in the recently published World Health Organization (WHO) revised case definition of vCJD. [5] The new definition of the pulvinar sign in vCJD is bilateral symmetrical pulvinar high signal relative to the signal intensity of other deep grey matter nuclei and cortical grey matter. This definition emphasizes that, for the sign to be considered positive, the highest signal of grey matter is within the pulvinar of the thalamus in vCJD; this does not exclude some increase in signal in other grey matter structures. Recent analysis by our group of MRI scans of patients with definite vCJD has shown that the sensitivity of the pulvinar sign for a diagnosis of vCJD remains very high and in fact has increased with the wider use of fluid-attenuated inversion recovery imaging and with improvements of MRI scanner technology.
There is considerable overlap in the appearances of grey matter structures in different forms of CJD. However, to date the pulvinar sign (as defined in the WHO criteria) remains a robust, sensitive, and highly specific noninvasive diagnostic sign of vCJD that has not been reported in other forms of CJD. The imaging appearances correlate with extensive neuronal loss and gliosis within the pulvinar on neuropathologic studies, [3] changes that have not been reported in other forms of CJD.
We have encountered several cases where a false-positive diagnosis of vCJD has been suggested by radiologists and clinicians that are not experienced in the diagnosis of this form of the disease. We hope the improved definition of the pulvinar sign of vCJD will reduce the number of these cases and are happy to provide a second opinion of MRI scans in which the diagnosis is suspected clinically but interpretation of the scan is considered difficult.
MH Creutzfeldt-Jakob Syndrome/*diagnosis; Human; Pulvinar/*pathology
AD D. M. Summers, MD; D. A. Collie, MD; R. J. Sellar, MD; M. Zeidler, MD; R. Knight, MD; R. G. Will, MD; J. W. Ironside, MD; Edinburgh, Scotland, UK
SP englisch
PO USA