Focal Cortical Dysplasia, Taylor Type IIB
Brigham and Women’s Hospital
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.
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).
Lesion with mineralizations: "droplet"-like calcifications, astrocyte with abundant cytoplasm (arrow), and clustered round glial nuclei ("oligodendroglial hyperplasia") (arrowheads).
Lesional cell, mineralization, and Rosenthal fiber (arrow).
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.
Luxol-fast blue and cresyl violet stain: Nissl substance in cytoplasm of ballooned cell, and disorganized myelinated fibers (blue threads) in background white matter.
Immunostain for glial fibrillary acidic protein: variably sized astrocytes, some with large, bizarre nuclei; largely negative cytoplasm of balloon cell.
Immunostain for phosphorylated neurofilaments: negative in balloon cell cytoplasm.
Immunostain for NeuN neuronal antigen: malorientation of cortical neurons, with apical dendrites pointing in all directions.
- Focal cortical dysplasia
- Cortical tuber of tuberous sclerosis
Final Diagnosis and References
Focal cortical dysplasia, Taylor type IIB
Balloon cell - Brain malformation - Epilepsy – Mesial temporal sclerosis
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