Limbic and Nigral Lewy Bodies and Alzheimer's Disease Pathology Mimicking Progressive Supranuclear Palsy in a 75-Year-Old Man with Preserved Cardiac Uptake of MIBG

Article type: Short Communication

Authors: Kasahata, Naoki^{a; b; *} | Uchihara, Toshiki^c | Orimo, Satoshi^d | Nakamura, Ayako^c | Makita, Yoshihisa^e

Affiliations: [a] Department of Neurology, Makita General Hospital, Tokyo, Japan | [b] Department of Medicine, Division of Neurology, Tokyo Metropolitan Ohtsuka Hospital, Tokyo, Japan | [c] Laboratory of Structural Neuropathology, Tokyo Metropolitan Institute of Medical Science, Tokyo, Japan | [d] Department of Neurology, Kanto Central Hospital, Tokyo, Japan | [e] Department of Pathology, Makita General Hospital, Tokyo, Japan

Correspondence: [*] Correspondence to: Naoki Kasahata, Department of Medicine, Division of Neurology, Tokyo Metropolitan Ohtsuka Hospital, 2-8-1 Minamiohtsuka, Toshima-ku, Tokyo 170-8476, Japan. Tel.: +81 3 3941 3211; Fax: +81 3 3941 9557; E-mail: n_kasa-o@ohtsuka-hospital.toshima.tokyo.jp.

Abstract: A 75-year-old man developed l-dopa non-responsive parkinsonism, supranuclear ophthalmoplegia, neck dorsiflexion, and dementia. Atrophy of the midbrain tegmentum on MRI and normal myocardial uptake of MIBG led to the clinical diagnosis of progressive supranuclear palsy (PSP). Autopsy revealed depigmentation of the substantia nigra and locus ceruleus. Alzheimer's disease pathology was advanced with PSP-like neurofibrillary tangles distribution, and Lewy bodies were abundant in limbic lobe, while scarce in lower brainstem nuclei. Tuft-shaped astrocytes were not apparent. Although decreased myocardial uptake of MIBG is a rule in patients harboring Lewy bodies, its normal uptake may be related to their absence in lower brainstem nuclei.

Keywords: Alzheimer disease, Lewy body, limbic system, MIBG, progressive supranuclear palsy (PSP)

DOI: 10.3233/JAD-2012-120445

Journal: Journal of Alzheimer's Disease, vol. 32, no. 4, pp. 889-894, 2012