Radiologic-pathologic correlation in focal cortical dysplasia and hemimegalencephaly in 18 children - 17/08/11

Doi : 10.1016/S0887-8994(01)00318-6

Chris L.F. Woo, MBBS ⁎, Sylvester H. Chuang, MD ^†, Laurence E. Becker, MD ^‡, Venita Jay, MD ^‡, Hiroshi Otsubo, MD , James T. Rutka, MD, PhD ^§, O.Carter Snead, MD
Division of Neurology, The Hospital for Sick Children, Toronto, Ontario, Canada
^† Division of Diagnostic Imaging, The Hospital for Sick Children, Toronto, Ontario, Canada
^‡ Division of Pathology, The Hospital for Sick Children, Toronto, Ontario, Canada
^§ Division of Neurosurgery, The Hospital for Sick Children, Toronto, Ontario, Canada

Communications should be addressed to: Dr. Woo c/o Dr. Becker; Division of Pathology; The Hospital for Sick Children; 555 University Avenue; Toronto, Ontario, M5G 1X8, Canada

Abstract

To describe the radiologic-pathologic correlation in children who underwent epilepsy surgery for medically intractable epilepsy with pathologically confirmed focal cortical dysplasia and hemimegalencephaly, we conducted a retrospective review on the magnetic resonance imaging and pathology of 18 children (10 boys and 8 girls). The preoperative MRIs were reviewed by one neuroradiologist who did not know the radiologic diagnosis and the pathology reports. MRI revealed focal cortical dysplasia (10), hemimegalencephaly (3), hamartomas (2), polymicrogyria (1), pial hemosiderosis (1), and no abnormality (1). Pathologic examination revealed focal cortical dysplasia (9), forme fruste of tuberous sclerosis (5), hemimegalencephaly (3), and focal cortical dysplasia with mesial temporal sclerosis (1). MRI was accurate in making the preoperative diagnosis in 16 out of 18 patients. On MRI, 12 patients had abnormal gyral formation and 12 had abnormal cortical thickness. Eleven patients manifested loss of gray-white differentiation, and 11 patients had abnormal signal on T₂-weighted image. Pathologically, 15 patients had neuronal heterotopia, 12 had misalignment or disorientation of neurons, 11 had large neurons, and 10 had abnormal cortical lamination. The presence of ectopic and large neurons and abnormal cortical lamination may be responsible for the MRI characteristics.

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