Clinics in diagnostic imaging (193). Sporadic Creutzfeldt-Jakob disease (sCJD)

Abstract

A 68-year-old man presented with a three-week history of rapidly progressive dementia, gait ataxia and myoclonus. Subsequent electroencephalography showed periodic sharp wave complexes, and cerebrospinal fluid assay revealed the presence of a 14-3-3 protein. A probable diagnosis of sporadic Creutzfeldt-Jakob disease was made, which was further supported by magnetic resonance (MR) imaging of the brain showing asymmetric signal abnormality in the cerebral cortices and basal ganglia. The aetiology, clinical features, diagnostic criteria, various MR imaging patterns and radiologic differential diagnosis of sporadic Creutzfeldt-Jakob disease are discussed in this article.

Keywords: Creutzfeldt-Jakob disease; MR imaging; human prion disease.

Fig. 1

(a & b) Fluid-attenuated inversion recovery (FLAIR) and (c & d) diffusion-weighted images.

Fig. 2

(a) Pre- and (b) post-gadolinium T1-weighted images.

Fig. 3

Hypoxic-ischaemic encephalopathy in a 60-year-old man who presented with acute pulmonary embolism. Post-embolectomy, he developed a poor Glasgow Coma Scale score and was placed on extracorporeal membrane oxygenation. (a–c) FLAIR images show symmetrical hyperintensity in the bilateral caudate and inferior lentiform nuclei. (d–f) DW images and (g–i) apparent diffusion coefficient (ADC) map show mildly reduced diffusion in the affected areas.

Fig. 4

Herpes simplex virus in a 66-year-old woman who presented with altered mental status, dysphagia, facial asymmetry and high fever. (a & b) FLAIR and (c & d) DW images show abnormal high signal and swelling in the left hippocampus, left mesial and inferior temporal lobe, left insular cortex and left basifrontal lobe, with no (e & f) appreciable corresponding restricted diffusion in these areas on ADC map. (g & h) Post-gadolinium T1-W images show slight leptomeningeal enhancement on the left.

Fig. 5

Posterior reversible encephalopathy syndrome in a 26-year-old woman who presented with seizures and neurodeficits. MR images of the brain show bilateral fairly symmetrical signal abnormalities in the left frontal and bilateral parietal lobes in the cortical and subcortical regions on (a & b) FLAIR and (c & d) DW images, with (e & f) no apparent restricted diffusion in the affected areas and (g & h) no abnormal susceptibility suggesting intracranial haemorrhage. Normal appearance of the basal ganglia is seen in (b).

Fig. 6

Postictal state in a 67-year-old woman with a known history of gastrointestinal stromal tumours on imatinib, who presented with seizure. (a & b) FLAIR and (c & d) DW images show increased signal intensity and swelling in the body and tail of the right hippocampus. (e & f) ADC map shows no restricted diffusion detected. The left hippocampus appears unremarkable. (g & h) Post-gadolinium T1-W images show no appreciable abnormal parenchymal or leptomeningeal enhancement.

Fig. 7

Voltage-gated potassium channel antibody disease in a 52-year-old woman with suspected encephalitis, who presented with lower limb incoordination and progressive confusion. MR images show (a & b) areas of FLAIR hyperintensity in the bilateral cortices, left hippocampus, bilateral caudate nuclei and cerebellar hemispheres, with (c–f) associated mild diffusion restriction but without (g & h) abnormal susceptibility or (i & j) enhancement.

Fig. 8

Wilson’s disease in a 15-year-old boy with dysarthria, dystonia and an abnormal liver function test. MR image of the brain shows symmetric abnormal FLAIR hyperintensity in the bilateral basal ganglia and thalami. No cortical signal abnormality is noted.

Fig. 9

Probable Wernicke’s encephalopathy in a 59-year-old non-alcoholic female patient with gait disturbance, cognitive impairment and memory loss. (a–c) FLAIR and (d–f) DW images of the brain reveal symmetric hyperintensities in the periventricular region of the third ventricle, medial thalami, tectal plate and periaqueductal region, with no (g–i) associated enhancement. The lack of a history of chronic alcohol abuse suggests that this is a non-alcoholic subtype of Wernicke’s encephalopathy.