PGBSPeter Ginn Birding Safaris (George, South Africa)
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Fetal and neonatally detected metabolic disorders of genetic basis with polyglucosan bodies (PGBs) are extremely rare and are nonreported in relation to CNS involvement.
Notably, this fetal brain presented, in addition to the PGBs, numerous vacuolated neuronal cell bodies.
The unexpected detection in our case of "aberrantly discordant" CNS parenchymal inclusions (whose staining and histomorphological characteristics were typical of PGBs) was particularly unique and actually puzzling.
Our observation on CNS PGBs thus highlights the importance of additional meticulous explorations by expert fetal neuropathologists in all cases of Sly disease to further assess the extent and prevalence of PGs in this condition: If such association is substantiated in more cases, Sly disease can then be added to the small group of disorders included under the umbrella term "polyglucosan disorders" [5].
Our present report might thus expand the spectrum of this group so-called "polyglucosan disorders" [5] that would thus span all age-groups and provides further evidence for the heterogeneous origin of this small group of disorders with PGBs.
Note also the presence of PGBs (arrows in (b)) in the neuropil.
PGB accumulation is best known in the exceptionally rare infantile metabolic disorders of glycogenosis (almost exclusively type IV [1] and possibly type VII [5], in which CNS involvement, though, still remains to be clearly shown).
Each time student papers are returned or a test grade is shared, I put a plastic sheet on the overhead showing my copy of what their PGB should look like.
Remember, workers know what the manager expects--but they also know what is really valued is what the manager inspects--so inspect the PGB often.
The PGB is also very handy when a parent wants to support me but has no idea what assignments the student has missed.