Diffusion-weighted MRI hyperintensity patterns differentiate CJD from other rapid dementias

Abstract

Background: Diffusion-weighted imaging (DWI) and fluid-attenuated inversion recovery (FLAIR)

Symptomatic nonconvulsive status epilepticus erroneously suggestive of sporadic Creutzfeldt–Jakob disease

“…e l s e v i e r . c o m / l o c a t e / j n s and before the typical EEG periodic pattern appearance [11]. The involvement of cortical and pulvinar regions has been described in both sCJD and symptomatic SE [9].…”

“Dazed and diffused”: making sense of diffusion abnormalities in neurologic pathologies

“…Although electroencephalography and cerebrospinal fluid biomarkers have been used to diagnose CJD, MRI with diffusion weighting has been proven to be the most sensitive and specific test for diagnosing CJD [25]. Diffusion restriction involving the bilateral caudate, putamen, pulvinar nuclei of the thalami, and frontotemporoparietal cortex with relative sparing of the globus pallidus and occipital cortex is the earliest imaging change [25][26][27][28] (Figure 5).…”

“…Diffusion restriction involving the bilateral caudate, putamen, pulvinar nuclei of the thalami, and frontotemporoparietal cortex with relative sparing of the globus pallidus and occipital cortex is the earliest imaging change [25][26][27][28] (Figure 5). Abnormal T 2 /fluid-attenuated inversion recovery (FLAIR) hyperintensity in the same distribution can also be seen, but these changes typically occur later in the disease and can be subtle compared with the DWI changes [25][26][27]. Moreover, T 2 /FLAIR hyperintensity in the absence of restricted diffusion is suggestive of rapidly progressive dementias other than CJD [25].…”

“…Abnormal T 2 /fluid-attenuated inversion recovery (FLAIR) hyperintensity in the same distribution can also be seen, but these changes typically occur later in the disease and can be subtle compared with the DWI changes [25][26][27]. Moreover, T 2 /FLAIR hyperintensity in the absence of restricted diffusion is suggestive of rapidly progressive dementias other than CJD [25].…”

Interpretation of cerebrospinal fluid protein tests in the diagnosis of sporadic Creutzfeldt–Jakob disease: an evidence-based approach

“…10,12 Findings on MRI (i.e., high signal in caudate nucleus or putamen with diffusion-weighted imaging or fluid-attenuated inversion recovery sequences) have proven useful in expert centres, especially in combination with EEG or analysis of cerebrospinal fluid markers. [20][21][22] However, observer dependence and variability among disease subtypes can complicate the application of MRI. 21,23 The most widely used supporting investigations are immunoassays for certain protein markers that are markedly elevated in lumbar cerebrospinal fluid in most affected patients (Box 2).…”