Case 2 -

Focal Cortical Dysplasia, Taylor Type IIB Rebecca Folkerth Brigham and Women’s Hospital Boston, MA

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Clinical History This 14-year-old right-handed girl was seen by her primary physician for a 2-month history of progressively severe headaches, which occurred mostly in the morning. There was no history of nausea or vomiting, or of seizures. She also complained of a recent sore throat. On physical examination, her temperature was 38.4C, and she had erythematous and enlarged tonsils. Laboratory analysis disclosed an elevated peripheral white count and leukocytosis. Neurologic examination was normal. Magnetic resonance imaging revealed a T2-hyperintense, rounded, partially enhancing lesion in the right superior frontal gyrus near the vertex. The lesion was based in the cortex, extending to involve the subcortical white matter, with T2 prolongation and FLAIR hyperintensity. There was also evidence of hemosiderin and/or calcification. At surgery, the cerebral cortical surface was normal, so ultrasound was used to localize the lesion. Upon incision, a small cyst was found to be surrounded by abnormal rubbery tissue. A complete surgical resection was accomplished. Case 2 - Slide 1 Click to view with ImageScope Click to view with a Web-Based Viewer Case 2 - Figure 1 Low-power view of lesional cells: lack of organized cytoarchitecture, and presence of bizarre cells with cytologic features of neurons (vesicular cytoplasm, prominent nucleoli, Nissl substance) (arrows) as well as ballooned cells with glassy cytoplasm (arrowheads). Case 2 - Figure 2 Lesion with mineralizations: "droplet"-like calcifications, astrocyte with abundant cytoplasm (arrow), and clustered round glial nuclei ("oligodendroglial hyperplasia") (arrowheads). Case 2 - Figure 3 Lesional cell, mineralization, and Rosenthal fiber (arrow). Case 2 - Figure 4 High-power view of lesional cells: bizarre cells with cytologic features of neurons (vesicular cytoplasm, prominent nucleoli, Nissl substance) as well as ballooned cells with glassy cytoplasm. Case 2 - Figure 5 Luxol-fast blue and cresyl violet stain: Nissl substance in cytoplasm of ballooned cell, and disorganized myelinated fibers (blue threads) in background white matter. Case 2 - Figure 6 Immunostain for glial fibrillary acidic protein: variably sized astrocytes, some with large, bizarre nuclei; largely negative cytoplasm of balloon cell. Case 2 - Figure 7 Immunostain for phosphorylated neurofilaments: negative in balloon cell cytoplasm. Case 2 - Figure 8 Immunostain for NeuN neuronal antigen: malorientation of cortical neurons, with apical dendrites pointing in all directions. Differential Diagnosis Neoplasm Ganglioglioma Gangliocytoma Malformation Focal cortical dysplasia Cortical tuber of tuberous sclerosis Hemimegalencephaly Final Diagnosis and References Focal cortical dysplasia, Taylor type IIB Key words Balloon cell - Brain malformation - Epilepsy – Mesial temporal sclerosis References Andre VM, Wu N, Yamazaki I et al. Cytomegalic interneurons: A new abnormal cell type in severe pediatric cortical dysplasia. J Neuropathol Exp Neurol 2007; 66:491-504 Becker AJ, Urbach H, Scheffler B et al. Focal cortical dysplasia of Taylor's balloon cell type: Mutational analysis of the TSC1 gene indicates a pathogenic relationship to tuberous sclerosis. Ann Neurol 2002; 52:29-37 Englund C, Folkerth RD, Born D, Lacy JM, Hevner RF. Aberrant neuronal-glial differentiation in Taylor-type focal cortical dysplasia (type IIA/B). Acta Neuropathol 2005; 109:519-533 Fauser S, Schultze-Bonhage A, Honegger J et al. Focal cortical dysplasias: Surgical outcome in 67 patients in relation to histological subtypes and dual pathology. Brain 2004; 127:2406-2418 Lamparello P, Baybis M, Pollard J et al. Developmental lineage of cell types in cortical dysplasia with balloon cells. Brain 2007; 130:2276-2276. Tassi L, Colombo N, Garbelli R et al. Focal cortical dysplasia: Neuropathological subtypes, EEG, neuroimaging and surgical outcome. Brain 2002; 125:1719-1732 Taylor DC, Falconer MA, Bruton CJ, Corsellis JAN. Focal dysplasia of the cerebral cortex in epilepsy. J Neurol Neurosurg Psychiatry 1971; 34:369-387 Taylor JP, Sater R, French J, Baltuch G, Crino PB. Transcription of intermediate filament genes is enhanced in focal cortical dysplasia. Acta Neuropathol 2001; 102:141-148 Urbach H, Scheffler B, Heinrichsmeier T et al. Focal cortical dysplasia of Taylor's balloon cell type: A clinicopathological entity with characteristic neuroimaging and histopathological features, and favorable postsurgical outcome. Epilepsia 2002; 43:33-40