title: Phenomenon of Schwann cell apoptosis in a case of congenital hypomyelinating neuropathy with basal lamina onion bulb formation.
abstract: We analyzed a sural nerve biopsy of a child with congenital hypomyelinating neuropathy. A lack of normally myelinated fibres, abnormal architecture of premyelin fibres and basal lamina onion bulbs were identified. The most prominent pathological finding was the appearance of significant death of Schwann cells with apoptotic morphology. This new finding may suggest that abnormal premyelin fibres are susceptible to death and that their disappearance is responsible for empty basal lamina onion bulb formation.
authors.affiliation: Neuromuscular Unit, Medical Research Centre, Polish Academy of Sciences, 1A, Banacha Street, 02-097 Warsaw, Poland.
authors.forename: Anna
authors.initials: A
authors.lastname: Fidziańska
authors.forename: Hanna
authors.initials: H
authors.lastname: Drac
authors.forename: Janina
authors.initials: J
authors.lastname: Rafałowska
mesh_terms: D017209:Apoptosis; D002648:Child; D006801:Humans; D008297:Male; D009186:Myelin Sheath / Q000473:pathology* / Q000648:ultrastructure; D011115:Polyneuropathies / Q000151:congenital* / Q000473:pathology*; D012583:Schwann Cells / Q000473:pathology* / Q000648:ultrastructure; D013497:Sural Nerve / Q000473:pathology / Q000648:ultrastructure