The usefulness of combined brain perfusion single-photon emission computed tomography, Dopamine-transporter single-photon emission computed tomography, and 123 I-metaiodobenzylguanidine myocardial scintigraphy for the diagnosis of dementia with Lewy bodies.

BACKGROUND: Current diagnostic criteria recommend neuroimaging as a diagnostic support tool for the clinical diagnosis of dementia with Lewy bodies (DLB). Because DLB causes characteristic impairments and disabilities, such as neuroleptic hypersensitivity, which may significantly increase morbidity and mortality, its prompt and correct diagnosis is very important. The aim of this study was to evaluate the extent to which diagnostic accuracy can be increased by using different combinations of brain perfusion single-photon emission computed tomography (bp-SPECT), 123 I-metaiodobenzylguanidine myocardial scintigraphy (MIBG scintigraphy), and DAT-SPECT. Taking finances and patient burden into consideration, we compared the tests to determine priority. METHODS: Thirty-four patients with probable DLB (75.0 ± 8.3 years old; 14 men, 20 women) underwent bp-SPECT, MIBG scintigraphy, and DAT-SPECT. RESULTS: Our comparison of three functional imaging techniques indicated that MIBG scintigraphy (79%) and Dopamine-transporter (DAT) SPECT (79%) had better sensitivity for characteristic abnormalities in DLB than bp-SPECT (53%). The combination of the three modalities could increase sensitivity for diagnosis of DLB to 100%. Additionally, the ratio of patients with rapid eye movement sleep behaviour disorder was significantly higher in the positive finding group on MIBG scintigraphy than in the negative finding group. CONCLUSIONS: In terms of stand-alone diagnostic means, priority should be placed on MIBG scintigraphy or DAT-SPECT for the diagnosis of DLB. However, our results suggest that the combination of bp-SPECT, MIBG scintigraphy, and DAT-SPECT increased the accuracy of the clinical diagnosis of DLB.

Reproducibility and Variability of Quantitative Cerebral Blood Flow Measured by Multi-delay 3D Arterial Spin Labeling According to Sex and Menstrual Cycle.

Purpose : To determine the reproducibility of corrected quantitative cerebral blood flow (qCBF) through measurement of transit flow time using multi-delay three-dimensional pseudo-continuous arterial spin labeling (pCASL) in healthy men and women and to evaluate the differences in qCBF between not only men and women, but also the follicular and luteal phases of the women's menstrual cycle. Methods : The participants were 16 healthy volunteers (8 men and 8 women ; mean age, 25.3 years). Two MRI were conducted for all participants ; female participants were conducted in the follicular and luteal phases. The reproducibility of qCBF values was evaluated by the intraclass correlation coefficient (ICC) and differences between the two groups were estimated by voxel-based morphometry (VBM) analysis. Results : The qCBF values were lower in men than in women, and those in females were significantly different between the follicular and luteal phases (P < 0.05). In VBM analysis, the qCBF values of the lower frontal lobes were significantly higher in women than in men (P < 0.05). The qCBF values of the frontal pole were significantly higher in the follicular phase than in the luteal phase (P < 0.01). Conclusion : Multi-delay pCASL can reveal physiological and sex differences in cerebral perfusion. J. Med. Invest. 67 : 321-327, August, 2020.

Spinocerebellar Ataxia Type 31 with Blepharospasm.

A 58-year-old man consulted our hospital due to a 2-year history of dysarthria and a 1-month history of blepharospasm. In addition to the ataxic dysarthria and blepharospasm, a neurological examination demonstrated slight ataxia of the trunk and lower limbs. Brain MRI demonstrated atrophy of the upper portion of the cerebellar vermis. Gene analysis established a diagnosis of spinocerebellar ataxia type 31 (SCA31). Single photon emission computed tomography (SPECT) with the three-dimensional stereotaxic ROI template (3DSRT) software program demonstrated hyperperfusion in the lenticular nucleus and thalamus. Although the association between SCA31 and blepharospasm in our patient remains unclear, we considered that this combination might be more than coincidental.