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. 2020 Jan 15;21(2):121–133. doi: 10.1007/s10048-019-00602-4

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© The Author(s) 2020

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Fig. 4 — Evolution of brainstem and striatal changes in patients 1 (A, B) and 5 (C, D). A, In patient 1 at 0.5 years, the decussation of SCP mildly hyperintense (A₄; inset: normal finding in age-matched control) and the striatum is normal (A₅). B At 2 years ICP (B₁), hila of dentate nuclei and peridentate white matter (B_1,2) are newly hyperintense, and SCP is now clearly hyperintense along its mesencephalic course (B₃) and in the decussation (B₄). The striatum is homogeneously T2-hyperintense and atrophic (B₅).

C Patient 5 also has a normal striatum at 2 years (C₅). ICP (C₁), hila of dentate nuclei, peridentate white matter (C₂) and SCP (C₃), along to the red nucleus (C₄) are T2-hyperintense. At 4.8 years (D), infratentorial changes (D_1–4) are regressing whereas the striatum is newly T2-hyperintense and atrophic (D₅)