Neuronal heterotopia with capillary penetration of neurons and cortical dysplasia in a patient with complex partial seizures

Case report

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✓ Unusual pathological findings were encountered in a temporal lobectomy specimen from a 9-year-old boy with intractable complex partial seizures. Magnetic resonance imaging revealed an enlarged left temporal lobe, with diffuse high signal intensity over the cortex and poor gray-white differentiation on T2-weighted imaging; single-photon emission computerized tomography showed decreased blood flow. Active epileptiform discharges were identified in the left temporal lobe with focal slow waves and generalized epileptiform paroxysms. Pathologically, the cortex revealed changes of focal cortical dysplasia with extensive disorganization of neuronal morphology, layering, and orientation as well as focal polymicrogyria. The cortical-white matter junction was indistinct with extensive neuronal heterotopias in the white matter. Large pale balloon cells akin to those seen in tuberous sclerosis were found scattered within the cortex and white matter. The most striking finding was that of a heterotopic nodule in the white matter, which revealed abnormal neurons with penetration of cell bodies by capillaries. Ultrastructurally, there were no degenerative changes in these neurons, and this unusual phenomenon is attributed to a developmental disturbance affecting neuronal, glial, and vascular elements.

Article Information

Address reprint requests to: Venita Jay, M.D., Department of Pathology, The Hospital for Sick Children, 555 University Avenue, Toronto M5G 1X8, Ontario, Canada.

© AANS, except where prohibited by US copyright law.

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Figures

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    Left: Low-power photomicrograph showing a heterotopic nodule (N) in the white matter (W). Luxol fast blue/H & E, × 22. Right: High-power photomicrograph showing large abnormal neurons within this nodule with penetration of cytoplasm by capillaries (arrow). Luxol fast blue/H & E, × 140.

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    Photomicrograph showing accumulation of neurofilamentous material in the neurons, demonstrated by the Bielschowsky stain and immunostaining for neurofilament (inset, straight arrow). The unstained clefts represent capillaries penetrating the cytoplasm (curved arrow) × 140.

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    Electron micrographs of the nodule. Left: Section showing a neuron with penetration of the soma by a capillar, (arrow). The neuronal cytoplasm contains abundant Nissl substance, most marked in the perinuclear region, as well as microtubules, neurofilaments, and mitochondria. N = Nissl; R = red blood cell inside the capillary lumen, × 1785. Right: Section showing an intraneuronal capillary with intact basement membrane (arrow) surrounded by neuronal cytoplasm containing neurofilaments (NF) and mitochondria (M). × 2250.

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