PMC5217606_01
Spectral data obtained at intermediate echo-time by single-voxel 1H magnetic resonance spectroscopy of the cerebellar vermis. The amplitude of the resonances is measured on the Y-axis using an arbitrary scale. A low N-acetyl aspartate (NAA) to creatine (Cr) area ratio was found at first assessment with NAA/Cr 0.95, normal values >1.08 (A). MRS findings at 30 months show deterioration of NAA/Cr with a drop from 0.95 to 0.83 (B)
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Fig1
However, the magnetic resonance spectroscopy (MRS) of the cerebellar vermis revealed a low N-acetyl aspartate (NAA) to Creatine (Cr) area ratio (NAA/Cr 0.95, normal values  > 1.0) (Fig. 1A).
a Axial MRI T2 image showing atrophy of the cerebellar hemispheres (grade 1). b Sagittal MRI image, showing vermian grade 2 atrophy
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Fig2
The MRI of the brain showed is mild to moderate atrophy of the vermis (grade 2) and mild atrophy (grade 1) of the hemispheres (Fig. 2), when the MRS showed further deterioration of the NAA/Cr ratios of the cerebellum (Fig. 1B). 
a Electron micrograph (original magnification x 2,600) of a mass of randomly arranged straight amyloid fibrils around a capillary (at the top of the image), and a skeletal muscle myocyte (at the bottom). b Electron micrograph (original magnification x 20,000) showing randomly arranged straight amyloid fibrils
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Fig3
Histologically, no inflammation, necrosis, or inclusions were seen (Fig. 3).