SEVERE AXONAL DEGENERATION IN ACUTE GUILLAIN-BARRE-SYNDROME - EVIDENCE OF 2 DIFFERENT MECHANISMS

Four cases of severe acute Guillain-Barre syndrome (GBS) characterized by severe axonal degeneration are presented. All had electrically ine xcitable motor nerves as early as 4 days after onset. The disease was rapid in onset and the residual disability was severe. Two different t ypes of pathology were seen. Nerve biopsies in 3 cases showed severe a xonal degeneration without inflammation or demyelination. Autopsy in o ne of these cases showed that the dorsal and ventral roots were also s ignificantly affected. These cases illustrate the primary axonal form of GBS. Nerve biopsy in the fourth case at day 15 showed marked inflam mation and demyelination with axonal degeneration. Contralateral nerve biopsy at day 75 showed almost complete loss of axons. This case illu strates another type of axonal degeneration, that which occurs seconda ry to inflammation and demyelination.