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Hyaline protoplasmic astrocytopathy is a rare disorder marked by an accumulation of protein material in the cytoplasm of astrocytic cells, mostly in the cortex. The finding has been described in Aicardi syndrome (agenesis of the corpus callosum, chorioretinal lacunae and infantile spasms) as well as in patients with pharmacoresistant epilepsy and in association with focal cortical dysplasia, polymicrogyria and nodular heterotopia. This report describes the first case of this entity described in a patient with tuberous sclerosis. The patient was a 3-year-old boy who presented at age 2months with medically intractable seizures. Has mother has a tuberous sclerosis 2 (TSC 2) gene abnormality and a diagnosis of tuberous sclerosis. On imaging, he was noted to have multiple lesions in the left parietal and temporal lobes consistent with focal cortical dysplasia and a subependymal nodule. He additionally had two hypopigmented lesions on the skin. He underwent resection of the left parietal lobe 32months after seizure onset. Histopathologic examination showed eosinophilic cytoplasmic inclusions within astrocytes in the cortex and superficial white matter focally accompanied by a disordered cortical architecture with dysmorphic neurons and balloon cells, consistent with focal cortical dysplasia classified as type IIb according to International League Against Epilepsy classification criteria (ILAE type IIb). At the time of most recent follow-up, 93months postoperatively, he is still experiencing seizures with overall worthwhile improvement while on seizure medication., (Copyright © 2016 Elsevier Ltd. All rights reserved.)

We report a rare case of a 22-year-old woman with biopsy-proven pigmented ganglioglioma. The patient initially underwent a right temporal lobectomy for intractable seizures at the age of 9 and remained seizure free for several years but subsequently developed complex partial seizures. Due to enhancement of a left mesial occipital lesion on preoperative MRI of the brain, the patient underwent a left subdural electrode placement and simultaneous biopsy of the left mesial occipital lesion. Biopsy results revealed a rare pigmented ganglioglioma, World Health Organization Grade I. The seizure focus was identified in the left mesial occipital lobe and the patient underwent tumor resection. An extensive literature search revealed that our patient is the fourth case of pigmented ganglioglioma described in the literature and was positive for BRAF V600E mutation by molecular studies., (Copyright © 2015 Elsevier Ltd. All rights reserved.)

Focal cortical dysplasia (FCD) is of increasing interest as a cause of focal epilepsy. We aimed to determine whether the existence of FCD influences the epileptogenicity induced by electrical kindling stimulation of the cortices. We created an FCD rat model by focal contact of a frozen metal probe on the scalp immediately after birth. To produce afterdischarges (ADs), electrical stimulation was applied to the frontal cortices once daily for 20 consecutive days from postnatal day 38 (P38). Thresholds and durations of ADs were measured. Brains were exposed and examined histologically at P58. We observed mild FCD, which consisted of disorganized cortices with extra sulci; however, there was no statistical difference in the thresholds or durations of ADs between FCD rats and control animals. These results suggest that FCD might not influence vulnerability to epileptogenicity, at least in some patients with mild FCD.