POLR3A and leukodystrophy: Compared with classic 4H leukodystrophy, infratentorial involvement was practically inverted in patients with the striatal variant of POLR3A-related disease: While MCP was normal in the striatal variant, T2-hyperintensity of MCP has been noted in reported cases of 4H leukodystrophy [22–24] and was present in 29 of the 36 the previously reported patients with classic 4H leukodystrophy [7] re-reviewed for comparison.