Schwannian crystalline-like inclusions bodies (Fardeau-Engel bodies) revisited in peripheral neuropathies.
In 1969, Fardeau and Engel described polygonal organelles containing crystalline-like structures located in the Schwann cell cytoplasm of unmyelinated fibers. Such inclusions were reported in various conditions, mainly with axonal lesions. They were also reported in a few cases of peripheral neuropathy associated with primary mitochondriopathy. Although they are surrounded by a double membrane, their mitochondrial origin is not definitely proven. Their significance remains obscure but they deserve to be better known as Fardeau-Engel bodies, so as not to be mistaken with mitochondrial crystalline inclusions, which are frequently observed in patients with ragged-red fibers in muscle.
Vital
Claude
C
Neuropathology Department, Victor Segalen University, Bordeaux, France.
Bouillot
Sandrine
S
Canron
Marie-Hélène
MH
Vital
Anne
A
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001369:Axons / Q000648:ultrastructure; D002648:Child; D003460:Crystallization; D005260:Female; D006801:Humans; D002479:Inclusion Bodies / Q000648:ultrastructure*; D008297:Male; D008875:Middle Aged; D008931:Mitochondria, Muscle / Q000648:ultrastructure; D017240:Mitochondrial Myopathies / Q000473:pathology; D009413:Nerve Fibers, Myelinated / Q000648:ultrastructure; D010523:Peripheral Nervous System Diseases / Q000473:pathology*; D010543:Peroneal Nerve / Q000648:ultrastructure*; D012583:Schwann Cells / Q000648:ultrastructure*