Serial Magnetic Resonance Imaging in Creutzfeldt-Jakob Disease: a Case Report and Literature Review

Abstract

Creutzfeldt-Jakob disease (CJD) is a rare, degenerative, invariably fatal brain disorder. CJD usually appears in later life and runs a rapid course. Typically, the onset of symptoms occurs about age 60 and about 90% of individuals die within one year. We report a case of 67-year-old male presented with progressive aphasia, confusion, dysphagia and inability to carry out activities of daily life (ADLs) over a period of three to four weeks. The patient had past medical history of chronic atrial fibrillation and hypertension. Prior to admission, the patient was treated for ischemic stroke of left basal ganglia but continued to have worsening encephalopathy. The spinal tap revealed a 14-3-3 protein level of thirteen times the upper limit of normal; electroencephalogram (EEG) showed a diffuse slowing of the background and periodic sharp waves with greater involvement of the left hemisphere. Diffusion-weighted imaging (DWI) magnetic resonance imaging (MRI) at the time of admission showed extensive signal abnormality in the basal ganglia bilaterally and in the cerebral cortex bilaterally, particularly over the left cerebral hemisphere. The persistence of the MRI findings over several weeks was concerning for spongiform encephalopathy. The probable diagnosis of Creutzfeldt-Jakob disease was made based on these imaging findings taken together with the patient’s clinical signs and symptoms of a rapidly progressive encephalopathy. The patient was able to have some quality time with his family as the diagnosis was made earlier than perhaps otherwise and expired peacefully after comfort care measures were chosen. Serial MRI may serve as a clue to the early diagnosis of CJD and potentially provide a better quality of life for the patients.

A Creutzfeldt‐Jakob disease case misdiagnosed with acute cerebral infarction and review of the literature

“…The hyperintensity was more evident on DWI than FLAIR (Fluid‐attenuated inversion recovery), and the restricted diffusion was noted on ADC 3,4 . One study showed that a serial MRI scan within the disease course is necessary for reaching the diagnosis of CJD 5 . Cortical DWI hyperintensity with normal ADC mapping has been reported in autoimmune encephalitis(AE), which is a key neuroimaging clue to distinct AE from sCJD 6 .…”

“…3,4 One study showed that a serial MRI scan within the disease course is necessary for reaching the diagnosis of CJD. 5 Cortical DWI hyperintensity with normal ADC mapping has been reported in autoimmune encephalitis(AE), which is a key neuroimaging clue to distinct AE from sCJD. 6 A PET-CT scan is useful for improving diagnostic accuracy by showing a hypometabolic pattern in CJD patients.…”

Creutzfeldt-Jakob Disease Presenting with Dementia and Mimic a Stroke During One Year: Case Report and Review of Literatures

“…CJD is diagnosed by afflicted cornea, larger and progressive putamen and whole atrophy and ventricular dilatation in its delayed phase. Performing MRI frequently cannot be used as a definitive diagnostic tool for CJD; however, studies show that in addition to diagnosis, it helps in monitoring the disease [ 10 ]. MRI protocol includes sagittal FLAIR with a 3mm cross section, T2- weighted with 3mm cross section, T1- weighted, and DWI, ADC map.…”

“…EEG is a definitive tool for CJD diagnosis. The periodic pattern of sharp two or three-phase wave complexes is seen in 67 to 95% of cases of the sporadic form [ 10 ]. Our patients met these CJD criteria; their MRI and EEG results confirmed our findings.…”

Creutzfeldt-Jakob Disease Presenting with Dementia and Mimic a Stroke During One Year: Case Report and Review of Literatures

“…The clinical criteria for CJD mentioned by the center for preventing and controlling diseases [12] CJD is diagnosed by afflicted cornea, larger and progressive putamen and whole atrophy and ventricular dilatation in its delayed phase. Performing MRI frequently cannot be used as a definitive diagnostic tool for CJD; however, studies show that in addition to diagnosis, it helps in monitoring the disease [10]. MRI protocol includes sagittal FLAIR with a 3mm cross section, T2-weighted with 3mm cross section, T1-weighted, and DWI, ADC map.…”