case_images: PMC10682068_01
case_images: Brain MRI, perfusion imaging, and macroscopic appearance of the brain in the present case. At initial visit, (A) T2-weighted imaging on brain MRI and (B) three-dimensional stereotactic surface projection (3D-SSP) analyses on 123I-IMP SPECT at the level of (i) the brainstem and cerebellum and (ii) striatum reveal no evident abnormalities. Four years after initial visit, (C) T2-weighted imaging reveals marked cerebellar atrophy, hot cross bun sign, and high intensity of middle cerebellar peduncles. (D) 3D-SSP analyses on 123I-IMP SPECT reveal (i) decreased blood flow in the bilateral cerebellum and (ii) mild decreased blood flow in the right striatum. The sectional views of the cerebellum (E), midbrain (F), and cerebrum (G) are shown. (E) Modest atrophy, notably in the white matter (arrowheads), of the cerebellum is shown. (F) Severe depigmentation of the substantia nigra is evident (black arrows). (G) The putamen shows slightly brownish color and mild atrophy (red arrows). MRI, magnetic resonance imaging; 123I-iodoamphetamine single-photon emission computed tomography, 123I-IMP SPECT; L(t), left side; R(t), right side.
case_images: fneur-14-1293732-g001.jpg
case_images: PMC10682068_01_fneur-14-1293732-g001.jpg
case_images: fig1
case_images: Brain magnetic resonance imaging (MRI) and 123I-IMP SPECT imaging revealed no evident abnormalities (Figures 1A,B). 123I-MIBG myocardial scintigraphy was normal in both the early and delayed phases. T2-weighted brain MRI revealed marked cerebellar atrophy, hot cross bun sign, and hyperintensities of the middle cerebellar peduncle (Figure 1C), with no abnormalities in the striatum (Figure 2A). 123I-IMP SPECT revealed marked bilateral cerebellar and striatal hypoperfusion (Figure 1D). The brain weighed 1,350 g, with the cerebellum showing modest atrophy, particularly in the white matter (Figure 1E). Furthermore, depigmentation of the SN (Figure 1F) and a slightly brownish putamen with mild atrophy were observed (Figure 1G).
case_images: Transaxial T2-weighted magnetic resonance imaging of cases 1 (current case) (A), 2 (B), and 3 (C) at the level of the putamen. Severe atrophy is evident in cases 2 and 3 (B,C), whereas no atrophy in the putamen is shown in the current case (A), even in the advanced phase of the disease. Magnified images of the putamen are shown in the bottom column. Dashed lines indicate the border rims of the putamen. The "years" represents the periods from onset.
case_images: fneur-14-1293732-g002.jpg
case_images: PMC10682068_01_fneur-14-1293732-g002.jpg
case_images: fig2
case_images: T2-weighted brain MRI revealed marked cerebellar atrophy, hot cross bun sign, and hyperintensities of the middle cerebellar peduncle (Figure 1C), with no abnormalities in the striatum (Figure 2A). 123I-IMP SPECT revealed marked bilateral cerebellar and striatal hypoperfusion (Figure 1D).
case_images: Microphotographs of putamen in three patients with MSA-P. Severe gliosis and neuronal loss are consistent in cases 2 and 3 (a-ii, iii) but not in the present case (a-i). Expression of dopamine receptors D1/D2 (DRD1/DRD2) (as postsynaptic markers) are severely reduced in cases 2 and 3 (c-ii, iii, d-ii, iii), but it is well-preserved in the present case (c-i, d-i). Immunoreactivity for dopamine transporter, as a presynaptic marker of nigrostriatal system, is almost reduced in all patients (b-i-iii). Fair amount of alpha-synuclein (alphaSyn)-positive GCIs are present in all patients (e-i-iii). (f) Line charts represent packing density of remaining neurons in the putamen (solid line) and substantia nigra (dotted line) in patients with MSA-P. Bar charts represent % area of immunoreactivity for DAT (light blue), DRD1 (orange), DRD2 (red), and alphaSyn (gray columns). Scale bars, 50 mum. Hematoxylin-eosin (H-E) (a-i-iii), DAT (b-i-iii), DRD1 (c-i-iii), DRD2 (d-i-iii), and alpha-synuclein (e-i-iii). MSA-P, multiple system atrophy with predominant parkinsonism; DAT, dopamine transporter; DRD1, dopamine receptor D1; DRD2, dopamine receptor D2; GCIs, glial cytoplasmic inclusions.
case_images: fneur-14-1293732-g003.jpg
case_images: PMC10682068_01_fneur-14-1293732-g003.jpg
case_images: fig3
case_images: Microscopic examination revealed alpha-synuclein-positive GCIs, a pathological hallmark of MSA, particularly in the putamen (Figures 3E-I), cerebral white matter, midbrain, pons, medulla oblongata, and cerebellar white matter.