title: Decreased regional cerebral blood flow in the bilateral thalami and medulla oblongata determined by an easy Z-score (eZIS) analysis of (99m)Tc-ECD-SPECT images in a case of MM2-thalamic-type sporadic Creutzfeldt-Jakob disease.
abstract: We report a case of autopsy-verified MM2-thalamic-type sporadic Creutzfeldt-Jakob disease (sCJD) in a 46-year-old patient with a 16-month history of abnormal behavior, progressive dementia, insomnia, and speech disturbances without family history. Neurological examination revealed progressive dementia, frontal signs, insomnia, speech disturbance, gait disturbance and bilaterally exaggerated tendon reflexes. Both brain MRI and cerebrospinal fluid examinations, including 14-3-3 protein, yielded normal results. An easy Z-score (eZIS) analysis for (99m)Tc-ethyl cysteinate dimer-single photon emission computed tomography ((99m)Tc-ECD-SPECT) revealed decreased regional cerebral blood flow in the bilateral thalami and medulla oblongata. PRNP gene analysis revealed methionine homozygosity at codon 129 without mutation. Neuropathological examinations revealed severe neuronal loss, gliosis, and hypertrophic astrocytosis in the medial thalamus and inferior olivary nucleus. A slight depletion of Purkinje cells was observed. PrP immunostaining showed no obvious PrP deposits in the basal ganglia, thalamus, cerebellum, or brainstem; however, mild synaptic-type PrP deposits with some smaller plaque-like structures were only partially observed in the localized region of the frontal lobe with the spongiform change. Western blot analyses of protease-resistant PrP showed a type 2 pattern. In conclusion, eZIS analysis of (99m)Tc-ECD-SPECT images is useful for detecting both thalamic and medullary lesions. This is the first case of medullary lesions detected in a live patient with MM2-thalamic-type sCJD using SPECT.
authors.affiliation: Department of Neurology and Geriatrics, Gifu University Graduate School of Medicine, Japan. Electronic address: hayashiy@gifu-u.ac.jp.
authors.forename: Yuichi
authors.initials: Y
authors.lastname: Hayashi
authors.affiliation: Department of Neuropathology, Institute for Medical Science of Aging, Aichi Medical University, Japan.
authors.forename: Yasushi
authors.initials: Y
authors.lastname: Iwasaki
authors.affiliation: Department of Neurology and Geriatrics, Gifu University Graduate School of Medicine, Japan.
authors.forename: Nobuaki
authors.initials: N
authors.lastname: Yoshikura
authors.affiliation: Department of Radiology, Gifu University Graduate School of Medicine, Japan.
authors.forename: Takahiko
authors.initials: T
authors.lastname: Asano
authors.affiliation: Department of Neurology, Juntendo University School of Medicine, Japan.
authors.forename: Taku
authors.initials: T
authors.lastname: Hatano
authors.affiliation: Department of Neuropathology, Institute for Medical Science of Aging, Aichi Medical University, Japan.
authors.forename: Shinsui
authors.initials: S
authors.lastname: Tatsumi
authors.affiliation: Department of Molecular Microbiology and Immunology, Nagasaki University Graduate School of Biomedical Science, Japan.
authors.forename: Katsuya
authors.initials: K
authors.lastname: Satoh
authors.affiliation: Department of Neurology and Geriatrics, Gifu University Graduate School of Medicine, Japan.
authors.forename: Akio
authors.initials: A
authors.lastname: Kimura
authors.affiliation: Division of CJD Science and Technology, Department of Prion Research, Center for Translational and Advanced Animal Research on Human Diseases, Tohoku University School of Medicine, Japan.
authors.forename: Tetsuyuki
authors.initials: T
authors.lastname: Kitamoto
authors.affiliation: Department of Neuropathology, Institute for Medical Science of Aging, Aichi Medical University, Japan.
authors.forename: Mari
authors.initials: M
authors.lastname: Yoshida
authors.affiliation: Department of Neurology and Geriatrics, Gifu University Graduate School of Medicine, Japan.
authors.forename: Takashi
authors.initials: T
authors.lastname: Inuzuka
mesh_terms: D002560:Cerebrovascular Circulation / Q000502:physiology; D007562:Creutzfeldt-Jakob Syndrome / Q000000981:diagnostic imaging* / Q000503:physiopathology; D003545:Cysteine / Q000031:analogs & derivatives*; D006801:Humans; D008297:Male; D008526:Medulla Oblongata / Q000000981:diagnostic imaging*; D008875:Middle Aged; D015609:Organotechnetium Compounds; D019275:Radiopharmaceuticals; D013788:Thalamus / Q000000981:diagnostic imaging*; D015899:Tomography, Emission-Computed, Single-Photon / Q000379:methods*
chemical_list: D015609:Organotechnetium Compounds; D019275:Radiopharmaceuticals; C059659:technetium Tc 99m bicisate; D003545:Cysteine
keywords: ECD-SPECT; Easy Z-score analysis; MM2-thalamic-type; Medulla oblongata lesion; Sporadic Creutzfeldt–Jakob disease