Inexcitability of nerves in a fulminant case of Guillain-Barré syndrome.
A 45-year-old woman presented with a recent sensorimotor deficiency in all 4 limbs, and the next day she was totally paralyzed. A slight motor improvement began on day 27. The cerebrospinal fluid had normal cellularity, but the protein varied from 90 mg/dL on the first day to 800 mg/dL on day 15, and then 290 mg/dL on day 33. Electrophysiologic studies performed on days 15 and 23 revealed a universal peripheral nerve inexcitability. A superficial peroneal nerve biopsy was performed on day 23. Nine fascicles were examined on semi-thin sections and myelinated fiber damage varied greatly from one fascicle to another. At ultrastructural examination, certain axons were severely damaged, but the others were quite well preserved and were naked or wrapped in a myelin sheath presenting a multivesicular degeneration. A few fibers had a better-preserved myelin sheath that was sometimes dissociated by elongated processes from an invading histiocyte. Six cases of fulminant Guillain-Barré syndrome with inexcitability of nerves and ultrastructural examination of nerve fragments have been reported. Electrophysiologic study is often ambiguous and cannot determine the precise origin of such an axonal degeneration. Therefore, ultrastructural analysis of a nerve biopsy is mandatory in this setting.
Vital
C
C
Laboratoire de Neuropathologie, Université Victor Segalen, Bordeaux, France. claude.vital@neuropath.u-bordeaux2.fr
Vital
A
A
Arne
P
P
Hilbert
G
G
Gruson
D
D
Gbikpi-Benissan
G
G
Cardinaud
J P
JP
Petry
K
K
D001369:Axons / Q000648:ultrastructure; D001706:Biopsy; D003711:Demyelinating Diseases / Q000473:pathology; D005260:Female; D020275:Guillain-Barre Syndrome / Q000473:pathology / Q000503:physiopathology*; D006801:Humans; D008875:Middle Aged; D009410:Nerve Degeneration / Q000473:pathology; D010543:Peroneal Nerve / Q000473:pathology / Q000503:physiopathology*