MRI detection of mild malformation of cortical development with oligodendroglial hyperplasia (MOGHE) on T1WI-CHESS

Abstract

Mild malformation of cortical development with oligodendroglial hyperplasia (MOGHE) is a recently proposed epileptogenic entity that is difficult to detect on MRI. We present a case of MOGHE that was successfully detected on T1WI-chemical shift-selective saturation (CHESS) MRI. The clinical presentation, MRI including T1WI-CHESS, functional images, and pathology findings of a 14-year-old Japanese girl diagnosed with MOGHE are described. T1WI-CHESS revealed an abnormal high signal along the affected lesion, whereas the findings shown by the other MR sequences were less obvious; interictal fluorodeoxyglucose-positron emission tomography indicated slightly decreased accumulation in the lesion, and subtraction ictal single photon emission computed tomography co-registered to MRI showed an increased blood flow. Together these observations suggest that T1WI-CHESS may be a useful MR sequence for detecting the lesions in patients with MOGHE.

Keywords: CHESS; MOGHE; MRI; epilepsy.

Fig. 1

(A) T1WI, (B) T2WI, (C) FLAIR, (D) DIR, (E) T1WI-CHESS, (F) interictal FDG-PET MRI fusion image, and (G) subtraction ictal SPECT co-registered to MRI. The right medial frontal lobe showed subtle cortical thickening and blurring of gray/white matter boundaries on T1WI /T2WI/FLAIR/DIR, without signal abnormality (arrows, A–D). However, T1WI-CHESS detected a subcortical linear high signal along the affected gyri (arrows, E). Interictal FDG-PET showed a slightly decreased accumulation consistent with the lesion, compared to the opposite cortex (arrow, F). Subtraction ictal SPECT co-registered to MRI indicated increased blood flow in the lesion (arrows, G). CHESS: chemical shift selective, DIR: double inversion recovery, FDG: fluorodeoxyglucose, FLAIR: fluid-attenuated inversion recovery, PET: positron emission tomography, SPECT: single photon emission computed tomography.

Fig. 2

(A) T1WI, (B) T2WI, (C) FLAIR, (D) DIR, (E) T1WI-CHESS, (F) interictal FDG-PET MRI fusion image, and (G) subtraction ictal SPECT co-registered to MRI. T2WI/FLAIR/DIR showed minimally blurred gray/white matter boundaries with a weak subcortical high signal in the right superior frontal gyrus and decreased white matter volume in the right central posterior gyrus (arrows, B–D). T1WI-CHESS detected a high signal in the right superior frontal gyrus to the anterior cingulate gyrus and in the right central posterior gyrus (arrows, E). In these lesions, no abnormalities were detected on interictal FDG-PET and subtraction ictal SPECT co-registered to MRI were detected (F, G).

Fig. 3

The histopathological specimens shown here are the parts of the lesion indicated in Fig. 2. (A) Klüver-Barrera (KB), (B) hematoxylin and eosin-stained (H&E), (C) enlarged image of the marked area in panel B, (D) H&E, (E) myelin basic protein (MBP), (F) oligodendrocyte transcription factor 2 (Olig2), (G) neuronal nuclear protein (NeuN), and (H) MIB-1 in the anterior of right superior frontal gyrus. KB staining showed partial blurring of gray/white matter boundaries (arrows, A). B: H&E staining showed a focal increase in cell density at the gray/white matter boundaries. C: High-power magnification of the H&E image showed increased oligodendroglia and neurons. E–G: Immunohistochemical staining of the same area as the gray/white matter boundaries (D); MBP staining showed patchy loss (E), and Olig2- and NeuN-positive cells showed a heterogeneous increase (F, G). The MIB-1 positivity rate was low. Scale bar = 500 µm (A), 100 µm (B, H), 50 µm (C), and 200 µm (D–G).