Polyglucosan bodies in medullary catecholaminergic neurones in SUDEP.

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