John Libbey Eurotext

Clinical seizure manifestations in the absence of synaptic connections Volume 23, issue 1, February 2021

Figures

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Authors
1 Department of Neurology, The Royal Children's Hospital, Parkville, Victoria, Australia
2 The University of Melbourne, Parkville, Victoria, Australia
3 Murdoch Children's Research Institute, Parkville, Victoria, Australia
4 Department of Neurosurgery, The Royal Children's Hospital, Parkville, Victoria, Australia
5 Department of Medical Imaging, The Royal Children's Hospital, Parkville, Victoria, Australia
6 Department of Anatomical Pathology, The Royal Children's Hospital, Parkville, Victoria, Australia
* Correspondence: Emma Macdonald-Laurs Department of Neurology, Royal Children's Hospital, 50 Flemington Road, Parkville, VIC, 3052, Australia

We report a child with a history of temporal-parietal-occipital disconnection for epilepsy secondary to posterior quadrantic dysplasia who developed recurrent and prolonged bouts of distress and autonomic disturbance associated with EEG and PET evidence of status epilepticus confined to his disconnected cortex. These bouts were refractory to antiseizure medications but resolved following resection of the disconnected cortex. In the absence of synaptic connections, we hypothesise that his seizure-related symptoms were mediated either by neurochemical transmission in preserved vascular and lymphatic channels or by ephaptic transmission to trigeminal nerve fibres in overlying dura, producing symptoms akin to migraine. The case highlights potential means by which seizures may manifest clinically, without synaptic connections, and adds to the differential for symptoms post-disconnection surgery.