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Polyglucosan bodies in medullary catecholaminergic neurones in SUDEP

Smriti Patodia1, Alyma Somani1, Maria Thom1

  • 1Department of Clinical and Experimental Epilepsy, UCL Queen Square Institute of Neurology, London WC1N 3BG, United Kingdom.

Epilepsy & Behavior Reports
|February 19, 2021
PubMed

Insights

Polyglucosan bodies, associated with perinatal brain injury, were found in a child with epilepsy who died suddenly. These bodies predominantly affected catecholaminergic neurons in the brainstem, suggesting selective vulnerability.

Area of Science:

  • Neuropathology
  • Neuroscience
  • Epilepsy Research

Background:

  • Polyglucosan bodies are linked to hypoxic-ischaemic perinatal brain injury, typically in the basal ganglia.
  • Rare occurrences in brainstem neurons have been noted previously.

Observation:

  • A five-year-old boy with cerebral palsy, epilepsy, and sudden death was examined post-mortem.
  • Significant findings included basal ganglia necrosis, hippocampal atrophy, and numerous polyglucosan bodies in brainstem neurons.
  • These bodies were PAS, p62, and ubiquitin positive, primarily in the ventrolateral and dorsomedial medulla.

Findings:

  • Polyglucosan bodies were predominantly found in catecholaminergic (tyrosine hydroxylase, TH) neurons within the medulla.
  • Immunohistochemistry revealed relative preservation of other medullary neuronal populations, including serotonergic and neurokinin1 receptor/somatostatin positive neurons.
  • This suggests selective vulnerability of catecholaminergic neurons to polyglucosan accumulation.

Implications:

  • The accumulation of polyglucosan bodies in catecholaminergic neurons may indicate a functional deficiency.
  • This deficiency could have contributed to the sudden unexpected death in epilepsy (SUDEP), particularly during a peri-ictal period.
  • Further research into the role of polyglucosan bodies in specific neuronal populations and SUDEP is warranted.

Keywords:
MR, Median rapheMedullaPGB, polyglucosan bodiesPolyglucosan bodiesSUDEPSUDEP, Sudden unexpected death in epilepsyTH, Tyrosine hydroxylaseTPH, Tryptophan hydroxylaseVLM, Ventrolateral medulla

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