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Polyglucosan bodies in medullary catecholaminergic neurones in SUDEP

Patodia, S; Somani, A; Thom, M; (2021) Polyglucosan bodies in medullary catecholaminergic neurones in SUDEP. Epilepsy & Behavior Reports , Article 100430. 10.1016/j.ebr.2021.100430. (In press). Green open access

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Abstract

Polyglucosan bodies have been reported in the context of hypoxic-ischaemic perinatal brain injury, mainly in the pallidum but with rare reports in brainstem neurones. We report a case of a five year old boy with cerebral palsy, complex neurology and epilepsy with a sudden nocturnal death. At post-mortem long-standing bilateral necrosis of basal ganglia and hippocampal atrophy was identified in keeping with hypoxic-ischaemic perinatal injury. In addition numerous polyglucosan bodies, which were PAS, p62 and ubiquitin positive, were noted in brainstem neurones and dendrites, primarily involving the ventrolateral and dorsomedial medulla. Immunohistochemistry confirmed relative preservation of medullary neuronal populations in the reticular formation, including catecholaminergic (tyrosine hydroxylase, TH), serotonergic (tryptophan hydroxylase) and neurokinin1 receptor/somatostatin positive neurones. The polyglucosan bodies predominated in catecholaminergic neurones which could indicate their selective vulnerability and a functional deficiency, which during a critical peri-ictal period contributed to the sudden death in epilepsy.

Type: Article
Title: Polyglucosan bodies in medullary catecholaminergic neurones in SUDEP
Open access status: An open access version is available from UCL Discovery
DOI: 10.1016/j.ebr.2021.100430
Publisher version: https://doi.org/10.1016/j.ebr.2021.100430
Language: English
Additional information: This is an Open Access article published under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International (CC BY-NC-ND 4.0) Licence (https://creativecommons.org/licenses/by-nc-nd/4.0/).
Keywords: Polyglucosan bodies, Medulla, SUDEP
UCL classification: UCL
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Queen Square Institute of Neurology
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Queen Square Institute of Neurology > Clinical and Experimental Epilepsy
URI: https://discovery.ucl.ac.uk/id/eprint/10120492
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