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NEUROLOGY 2004;63:443-449
© 2004 American Academy of Neurology

Diffusion-weighted MRI abnormalities as an early diagnostic marker for Creutzfeldt–Jakob disease

Y. Shiga, MD PhD, K. Miyazawa, MD, S. Sato, MD PhD, R. Fukushima, MD, S. Shibuya, MD PhD, Y. Sato, MD PhD, H. Konno, MD PhD, K. Doh-ura, MD PhD, S. Mugikura, MD PhD, H. Tamura, MD PhD, S. Higano, MD PhD, S. Takahashi, MD PhD and Y. Itoyama, MD PhD

From the Department of Neurology (Drs. Shiga, Miyazawa, and Itoyama), Tohoku University School of Medicine, Department of Neurology (Dr. S. Sato), Kohnan Hospital, Second Department of Internal Medicine (Dr. Fukushima), Hiraka General Hospital, Department of Neurology (Dr. Shibuya), Miyagi National Hospital, Department of Neurology (Dr. Y. Sato), Research Institute for Brain and Blood Vessels, Akita, Department of Neurology (Dr. Konno), Nishitaga National Hospital, Department of Neuropathology (Dr. Doh-ura), Institute of Brain Diseases, Kyushu University Faculty of Medicine, and Department of Diagnostic Radiology (Drs. Mugikura, Tamura, Higano, and Takahashi), Tohoku University School of Medicine, Japan.

Address correspondence and reprint requests to Dr. Y. Shiga, Department of Neurology, Tohoku University School of Medicine, 1-1 Seiryo-machi, Aoba-ku, Sendai 980-8574, Japan; e-mail: yshiga{at}em.neurol.med.tohoku.ac.jp

Objective: To evaluate the usefulness of diffusion-weighted MRI (DWI) for the early diagnosis of Creutzfeldt–Jakob disease (CJD).

Methods: Thirty-six consecutive patients (age 56 to 82 years) were enrolled, and 26 were examined by DWI. Nine were definite based on the World Health Organization criteria, and 27 were probable. The percentages of DWI abnormalities, periodic sharp wave complexes (PSWCs) on the EEG, detection of CSF 14-3-3 protein, and increase of CSF neuron-specific enolase (>25 ng/mL) on the first examination were compared. For DWI, 32 patients (age 31 to 84 years) who showed progressive dementia or impaired consciousness served as disease controls.

Results: The percentage of DWI abnormalities was 92.3%, of PSWCs 50.0%, of 14-3-3 protein detection 84.0%, and of NSE increase 73.3%. Two of the 32 control subjects were falsely positive on DWI. The sensitivity of DWI was 92.3% (95% CI 74.8 to 99.5%) and specificity 93.8% (95% CI 79.2 to 99.2%). In 17 patients who did not show PSWCs on the first EEG, abnormal DWI findings were still clearly detected. Four patients who were negative for 14-3-3 protein also showed DWI abnormalities. DWI abnormalities were detected as early as at 3 weeks of symptom duration in four patients in whom PSWCs were not yet evident.

Conclusions: DWI can detect characteristic lesions in the majority of patients with CJD regardless of the presence of PSWCs. DWI was the most sensitive test for the early clinical diagnosis of CJD; consideration should be given to its inclusion in the clinical diagnostic criteria of CJD.


Received April 16, 2003. Accepted in final form March 18, 2004.

See also pages 410, 436, and 450

Presented in part at the 127th Annual Meeting of the American Neurologic Association, October 14, 2002, New York.




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