Diffusion Tensor Imaging of Guillain-Mollaret Triangle in Patients with Hypertrophic Olivary Degeneration

Abstract

A B S T R A C TThe aim of the study is to analyze diffusion tensor imaging (DTI) characteristics of the Guillain-Mollaret triangle (GMT) in patients with hypertrophic olivary degeneration (HOD) and to investigate their correlation with previously reported histopathology. DTI was performed in 10 patients diagnosed with HOD. Fractional anisotropy, apparent diffusion coefficient, axial diffusivity, and radial diffusivity were measured in the inferior olivary nucleus (IO), the central tegmental tract, the red and the dentate nuclei, and the superior cerebellar peduncle of HOD patients and compared to age, sex, and side-matched 10 neurologically normal population. The prominent finding on DTI in affected IO was an increase in radial diffusivity compatible with demyelination. While conventional magnetic resonance imaging did not show any sign of involvement in the other components of GMT, DTI demonstrated signal changes in all anatomical components of the GMT. Main DTI findings in GMT of patients with HOD were an increase in radial diffusivity representing demyelination and an increase in axial diffusivity that is reflective of neuronal hypertrophy. DTI parameters can reflect the spatiotemporal evolution of transneuronal degeneration associated with HOD in a manner consistent with the known pathologic stages of HOD.

Recessive loss of function of the neuronal ubiquitin hydrolase UCHL1 leads to early-onset progressive neurodegeneration

“…1 B-D; Table S2) (9,10). The MRI scans were also remarkable for obvious cerebellar and mild cerebral atrophy that was more severe in the older siblings ( Fig.…”

“…All MRI examinations were performed in a 3T scanner (Trio, Siemens) using 8-channels head coil. The DTI datasets were obtained and analyzed as described before (9). Briefly, high resolution cranial MRIs were performed using the following sequences: sagittal TSE T2, axial SE T1, TSE T2, FLAIR and diffusion, coronal TSE T2, high resolution 3D TurboFlair T2 and reconstructions, sagittal 3D TurboFlash T1 and reconstructions, sagittal 3D SPACE T2 and reconstructions, coronal optic nerve focused TSE -STIR, SE T1, DTI-FT, axial GRE T2.…”

Bilateral hypertrophic olivary nucleus degeneration on magnetic resonance imaging in children with Leigh and Leigh-like syndrome

“…Diffusion tensor imaging (DTI) studies in patients with inferior olivary nucleus hypertrophy have demonstrated signal changes in all anatomical components of the Guillain-Mollaret triangle even when conventional MRI did not show any signs of involvement in the other components of the Guillain-Mollaret triangle. 39,40 It was also shown that the DTI parameters can reflect the spatiotemporal evolution of transneuronal degeneration associated with hypertrophic olivary degeneration (HOD) in a manner consistent with the known pathological stages of HOD. 26 In addition, susceptibility-weighted imaging has been utilized to study the red nucleus degeneration and atrophy in children with HOD after posterior fossa tumour resection.…”

Hypertrophic olivary degeneration in a child following midbrain tumor resection: longitudinal diffusion tensor imaging studies

“…5,13,15 Recent cross-sectional studies in children and adults with HOD showed that DTI can demonstrate and quantify the disruption of the GMT's components. 4,12,17 However, HOD is a dynamic process, and progressive changes have been shown by neuropathological examination and conventional MRI.…”

“…5,13,15 Recent cross-sectional studies in children and adults with HOD showed that DTI can demonstrate and quantify the disruption of the GMT's components. 4,12,17 However, HOD is a dynamic process, and progressive changes have been shown by neuropathological examination and conventional MRI.6,7 The purpose of this study is to investigate longitudinal DTI changes of the GMT's components in a child with HOD after neurosurgery for a midbrain tumor.Hypertrophic olivary degeneration in a child following midbrain tumor resection: longitudinal diffusion tensor imaging studies Hypertrophic olivary degeneration (HOD) is a dynamic process caused by disruptive lesions affecting components of the Guillain-Mollaret triangle (GMT). The authors applied diffusion tensor imaging (DTI) to investigate longitudinal changes of the GMT components in a child with HOD after neurosurgery for a midbrain tumor.…”