MR diagnosis of Creutzfeldt-Jakob disease

Significance of high signal intensity of the basal ganglia

D. P. Barboriak, J. M. Provenzale, Orest Boyko

Research output: Contribution to journalArticle

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Abstract

OBJECTIVE. Creutzfeldt-Jakob disease is a rare dementing illness that usually affects older adults. Currently, neuroradiologic examinations play a minor role in the diagnosis of Creutzfeldt-Jakob disease. Several single case reports have noted a distinctive finding of hyperintense signal abnormalities in the basal ganglia on T2-weighted MR images of patients with Creutzfeldt- Jakob disease. In order to assess the diagnostic utility of this finding, we studied the imaging features of four patients with Creutzfeldt-Jakob disease in whom this MR finding was present. MATERIALS AND METHODS. Two neuroradiologists retrospectively reviewed the MR images of four patients who had pathologically proved Creutzfeldt-Jakob disease and signal abnormalities in the basal ganglia on T2-weighted MR images. The patients' clinical findings were also analyzed. RESULTS. The four patients had MR examinations between 6 months and 1 year after the onset of symptoms. In all four cases, the hyperintense signal abnormalities in the basal ganglia on T2-weighted images were diffuse and bilaterally symmetric. The T1-weighted images were normal. A CT scan was obtained on a single patient and was normal. CONCLUSION. Although a lack of signal abnormality in the basal ganglia on MR imaging cannot be used to rule out a diagnosis of Creutzfeldt-Jakob disease, our experience and review of published reports suggest that in the proper clinical setting, bilaterally symmetric, diffuse hyperintense abnormalities in the basal ganglia on T2-weighted images may be a specific sign of Creutzfeldt-Jakob disease.

Original languageEnglish (US)
Pages (from-to)137-140
Number of pages4
JournalAmerican Journal of Roentgenology
Volume162
Issue number1
StatePublished - 1994
Externally publishedYes

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Creutzfeldt-Jakob Syndrome
Basal Ganglia

ASJC Scopus subject areas

  • Radiology Nuclear Medicine and imaging
  • Radiological and Ultrasound Technology

Cite this

MR diagnosis of Creutzfeldt-Jakob disease : Significance of high signal intensity of the basal ganglia. / Barboriak, D. P.; Provenzale, J. M.; Boyko, Orest.

In: American Journal of Roentgenology, Vol. 162, No. 1, 1994, p. 137-140.

Research output: Contribution to journalArticle

Barboriak, D. P. ; Provenzale, J. M. ; Boyko, Orest. / MR diagnosis of Creutzfeldt-Jakob disease : Significance of high signal intensity of the basal ganglia. In: American Journal of Roentgenology. 1994 ; Vol. 162, No. 1. pp. 137-140.
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abstract = "OBJECTIVE. Creutzfeldt-Jakob disease is a rare dementing illness that usually affects older adults. Currently, neuroradiologic examinations play a minor role in the diagnosis of Creutzfeldt-Jakob disease. Several single case reports have noted a distinctive finding of hyperintense signal abnormalities in the basal ganglia on T2-weighted MR images of patients with Creutzfeldt- Jakob disease. In order to assess the diagnostic utility of this finding, we studied the imaging features of four patients with Creutzfeldt-Jakob disease in whom this MR finding was present. MATERIALS AND METHODS. Two neuroradiologists retrospectively reviewed the MR images of four patients who had pathologically proved Creutzfeldt-Jakob disease and signal abnormalities in the basal ganglia on T2-weighted MR images. The patients' clinical findings were also analyzed. RESULTS. The four patients had MR examinations between 6 months and 1 year after the onset of symptoms. In all four cases, the hyperintense signal abnormalities in the basal ganglia on T2-weighted images were diffuse and bilaterally symmetric. The T1-weighted images were normal. A CT scan was obtained on a single patient and was normal. CONCLUSION. Although a lack of signal abnormality in the basal ganglia on MR imaging cannot be used to rule out a diagnosis of Creutzfeldt-Jakob disease, our experience and review of published reports suggest that in the proper clinical setting, bilaterally symmetric, diffuse hyperintense abnormalities in the basal ganglia on T2-weighted images may be a specific sign of Creutzfeldt-Jakob disease.",
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