case_images: PMC10246376_01
case_images: Preoperative axial magnetic resonance imaging. It shows a predominantly right hemispheric lesion, a characteristic laminar appearance is observed due to abnormal thickening of the cerebellar folia ("tiger-stripe appearance"). In T1-weighted (a) the lesion shows hypointensity, in the contrasted T1-weighted (b) no contrast-enhancement, no edema in T2-weighted (c) and fluid attenuated inversion recovery-weighted (d), no diffusion restriction, and no calcifications or hemorrhage in the susceptibility (e and f).
case_images: SNI-14-185-g001.jpg
case_images: PMC10246376_01_SNI-14-185-g001.jpg
case_images: F1
case_images: The T1 appeared a hypointense, and T2 was a hyperintense-weighted image with parallel linear striations with a characteristic "tiger-striped appearance" visible on T2-weighted imaging [Figures 1 and 2].
case_images: Magnetic resonance imaging sagittal and coronal projections. (a and b) fluid attenuated inversion recovery-weighted; infiltration is observed in the inferior, middle, and superior cerebellar peduncles, displacing the fourth ventricle and narrowing the anterior and lateral cisterns of the posterior fossa.
case_images: SNI-14-185-g002.jpg
case_images: PMC10246376_01_SNI-14-185-g002.jpg
case_images: F2
case_images: The T1 appeared a hypointense, and T2 was a hyperintense-weighted image with parallel linear striations with a characteristic "tiger-striped appearance" visible on T2-weighted imaging [Figures 1 and 2].
case_images: Photomicrographs of Lhermitte-Duclos disease showing the spectrum of changes. (a) Cerebellar cortex replaced by the presence of dysplastic cells characteristic of the disease, encompassing virtually all three histologic strata. H and E x100 (b) Cluster of dysplastic cells with a large eosinophilic cytoplasm of globoid appearance, with a large nucleus, slightly recessed to the periphery. (Black arrowhead) Amphophilic nucleolus. (black arrows) Open neuropil, with edema and vacuolation. Alternates with apoptotic appearing neurons. H and E x400 (c) KluverBarrera/H and E staining showing the direction in different directions of axons and loss of layering of neuronal somas. Note that the fibers are tortuous, serpiginous, and disorganized (blue). x400 (d) Neuronal bodies of different sizes and morphology (brown). In addition, little gliosis or satellitosis was evident. IHC NeuN x400.
case_images: SNI-14-185-g003.jpg
case_images: PMC10246376_01_SNI-14-185-g003.jpg
case_images: F3
case_images: The surgical specimen measuring 4.5 cm x 2.5 cm x 1.3 cm was sent to the neuropathology area for analysis, and 24 h later, a histopathological report was obtained describing the presence of a solid neoplasm expanding the cerebellar folia at the expense of proliferating dysplastic ganglion cells with large nuclei of granular chromatin with the presence of positive glial fibrillary acidic protein in reactive astrocytes and positive neuronal specific enolase in dysplastic neurons [Figure 3].