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 Volume 64, Number 4, February 22, 2005
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NEUROLOGY 2005;64:643-648
© 2005 American Academy of Neurology

Clinical diagnosis of MM2-type sporadic Creutzfeldt-Jakob disease

T. Hamaguchi, MD, T. Kitamoto, MD, PhD, T. Sato, MD, PhD, H. Mizusawa, MD, PhD, Y. Nakamura, MD, MPH, FFPHM, M. Noguchi, MD, Y. Furukawa, MD, C. Ishida, MD, PhD, I. Kuji, MD, PhD, K. Mitani, MD, S. Murayama, MD, PhD, T. Kohriyama, MD, PhD, S. Katayama, MD, PhD, M. Yamashita, MD, PhD, T. Yamamoto, MD, PhD, F. Udaka, MD, PhD, A. Kawakami, MD, PhD, Y. Ihara, MD, PhD, T. Nishinaka, MD, PhD, S. Kuroda, MD, PhD, N. Suzuki, MD, Y. Shiga, MD, PhD, H. Arai, MD, PhD, M. Maruyama, MD, PhD and M. Yamada, MD, PhD

From the Department of Neurology and Neurobiology of Aging (Drs. Hamaguchi, Noguchi, Furukawa, and Yamada), Kanazawa University Graduate School of Medical Science, Departments of Neurological Science (Dr. Kitamoto), Neurology (Drs. Suzuki and Shiga), and Geriatric and Complementary Medicine (Drs. Arai and Maruyama), Tohoku University Graduate School of Medicine, Sendai, National Center for Neurology and Psychiatry (Dr. Sato), Kohnodai Hospital, Ichikawa, Department of Neurology and Neurological Science (Dr. Mizusawa), Graduate School, Tokyo Medical and Dental University, Department of Public Health (Dr. Nakamura), Jichi Medical School, Minamikawachi, Department of Neurology (Dr. Ishida), Noto General Hospital, Nanao, Department of Nuclear Medicine (Dr. Kuji), Kanazawa University Hospital, Department of Neurology (Dr. Mitani), Tokyo Metropolitan Geriatric Medical Center, Department of Neuropathology (Dr. Murayama), Tokyo Metropolitan Institute of Gerontology, Department of Neurology (Drs. Kohriyama and Katayama), Hiroshima University Hospital, Department of Neurology (Drs. Yamashita and Yamamoto), Saiseikai Nakatsu Hospital and Medical Center, Osaka, Department of Neurology (Dr. Udaka), Sumitomo Hospital, Osaka, Department of Neurology (Dr. Kawakami), Kaetsu Hospital, Niitsu, Department of Neurology (Drs. Ihara and Nishinaka), Clinical Research Institute, National Hospital Organization Minami-Okayama Medical Center, Okayama, Department of Neuropsychiatry (Dr. Kuroda), Okayama University Graduate School of Medicine and Dentistry, and Creutzfeldt-Jakob Disease Surveillance Committee (Drs. Kitamoto, Sato, Mizusawa, Nakamura, Murayama, Kuroda, Shiga, and Yamada), Japan.

Address correspondence and reprint requests to Dr. M. Yamada, Department of Neurology and Neurobiology of Aging, Kanazawa University Graduate School of Medical Science, 13-1, Takara-machi, Kanazawa 920-8640, Japan; e-mail: m-yamada{at}med.kanazawa-u.ac.jp

Background: No method for the clinical diagnosis of MM2-type sporadic Creutzfeldt-Jakob disease (sCJD) has been established except for pathologic examination.

Objective: To identify a reliable marker for the clinical diagnosis of MM2-type sCJD.

Methods: CSF, EEG, and neuroimaging studies were performed in eight patients with MM2-type sCJD confirmed by neuropathologic, genetic, and western blot analyses.

Results: The eight cases were pathologically classified into the cortical (n = 2), thalamic (n = 5), and combined (corticothalamic) (n = 1) forms. The cortical form was characterized by late-onset, slowly progressive dementia, cortical hyperintensity signals on diffusion-weighted imaging (DWI) of brain, and elevated levels of CSF 14-3-3 protein. The thalamic form showed various neurologic manifestations including dementia, ataxia, and pyramidal and extrapyramidal signs with onset at various ages and relatively long disease duration. Characteristic EEG and MRI abnormalities were almost absent. However, all four patients examined with cerebral blood flow (CBF) study using SPECT showed reduction of the CBF in the thalamus as well as the cerebral cortex. The combined form had features of both the cortical and the thalamic forms, showing cortical hyperintensity signals on DWI and hypometabolism of the thalamus on [18F]2-fluoro-2-deoxy-d-glucose PET.

Conclusion: For the clinical diagnosis of MM2-type sporadic Creutzfeldt-Jakob disease, cortical hyperintensity signals on diffusion-weighted MRI are useful for the cortical form and thalamic hypoperfusion or hypometabolism on cerebral blood flow SPECT or [18F]2-fluoro-2-deoxy-d-glucose PET for the thalamic form.


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