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. 2017 Apr 24;12:77. doi: 10.1186/s13023-017-0612-6

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© The Author(s). 2017

Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.

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Fig. 3 — Examples of subependymal lesions. a-f MRI of late-onset patient p1 at the age of 15.7 years with the (pre-existing) nodular subependymal lesion in the medial roof of the right lateral ventricle (arrows in a, d, f ₃) and four incipient lesions in the roof of both lateral ventricles (arrows in a (lat.), b and f ₂, in c and f _3,5, in e and f ₁). (a : T2w; b - f : T1w). g-l MRI of late-onset patient p2 at 14.7 years with multiple, small, FLAIR-hyperintense nodularities (g, h, arrows) resulting in a “bumpy” surface of the ventricular roof on the T2w sagittal images (i right, k left lateral ventricle) and one larger nodule in the roof of the left ventricle without enhancement (arrows in j, l ₁, l ₂). NB extensive white matter changes. (g, h: FLAIR; i-k: T2w; l ₁, l _,2: T1w ± GAD). m-r Late-onset patient p6 with the most extensive, confluent subependymal lesions with multiple, small cystic areas (hypointense on FLAIR-images m, n, arrows in n), facilitated diffusion (r), punctate susceptibility artefacts (arrows in q) and punctate enhancement (compare p with o). (m, n: FLAIR; o, p: T1w ± GAD, q: T2*; r: ADC). s-z MRIs of late-onset patient p7 at the age of 61 (S-U) and 73 years (v-z). Initially there are two lesions, a small one in the left anterior horn (arrow in s) without significant change on follow-up (arrows in v, z ₂) and a larger one in the right anterior horn (s, z ₂, arrows in t, u), which increases mildly in size from approx. 10x9x8 mm to 13x9x9 mm during the 12 year period. Punctate susceptibility artefacts (arrows in w), facilitated diffusion (x), and some superficial, linear enhancement (compare y ₁ with y ₂ and z ₁ with z ₂) are present, similar to the large lesions in p6. Two additional, small nodules in septal surface of the right anterior and the roof of the left horn (arrows in z _3,4) have been detectable and without significant changes since MRIs at 65 and 66 years. MRI at 73 years depicts an additional, incipient lesion in the roof of the right lateral ventricle (arrow in z ₅). (s, v: FLAIR; t: T2w; u, y ₁, z _2–5: T1w, y ₂,, z ₁: T1w + GAD, w: SWI; x: ADC)