Guillain-Barre syndrome demyelination

Peripheral neuropathy is degeneration of peripheral nerves. Because motor and sensory axons tun in the same nerves, usually both motor and sensory functions are affected in this disease. Neuropathies may be either acute (e.g., Charcot-Marie-Tooth disease) or chronic (e.g., Guillain-Barre syndrome) and are categorized as demyelinating or axonal. 

Guillain-Barre syndrome is a transient neurologic disorder involving inflammatory demyelination of the peripheral nerves. The syndrome is characterized by progressive symmetric weakness of the legs and arms with loss of reflexes. Occasionally sensory abnormalities and paralysis of respiratory muscles will occur.16... 

Acute inflammatoiy demyelinating polyneuropathy is a common cause of reversible paralysis. Acute inflammatory demyelinating polyneuropathy (AIDP), the classic form of the Guillain-Barre syndrome, often begins a week or two after recovery from cytomegalovirus, Epstein-Barr virus or Mycoplasma infection. Patients present with rapidly advancing symmetrical weakness, loss of deep tendon reflexes, often with distal numbness, and limb or back pain. Cerebrospinal fluid (CSF) protein concentration is elevated, but in most cases there is little or no increase in number of inflammatory cells in the CSF. This albumino-cytologic dissociation contrasts with the elevation of both... 

Magira, E. E., Papaioakim, M., Nachamkin, I. et al. Differential distribution of HLA-DQP/DRP epitopes in the two forms of Guillain-Barre syndrome, acute motor axonal neuropathy and acute inflammatory demyelinating... 

Acute autoimmune demyelinating polyneuropathies such as Guillain-Barre syndrome and chronic polyneuropathies may involve some glycolipid antigens, as described later. 

Guillain-Barre Syndrome, Chronic Inflammatory Demyelinating Polyradiculoneuropathy, and Axonal Degeneration and Regeneration... 

Ho TW, WiUison HJ, Nachamkin I, Li CY, Veitch J, Ung H, Wang GR, Liu RC, Comblath DR, Asbury AK, Griffin JW, McKhann GM (1999) Anti-GDla antibody is associated with axonal but not demyelinating forms of Guillain-Barre syndrome. Ann Neurol 45 168-173. 

Hughes R, Atkinson P, Coates P, Hall S, Leibowitz S (1992) Sural nerve biopsies in Guillain-Barre syndrome Axonal degeneration and macrophage-associated demyelination and absence of cytomegalovirus genome. Muscle Nerve 15 568-575. 

Saida T, Saida K, Lisak RP, Brown MJ, Silberberg DH, Asbury AK (1982) In vivo demyelinating activity of sera from patients with Guillain-Barre syndrome. Ann Neurol 11 69-75. 

Takigawa T, Yasuda H, Terada M, Haneda M, Kashiwagi A Saito T, Saida T, Kitasato H, Kikkawa R (2000) The sera from GMl gan-glioside antibody positive patients with Guillain-Barre syndrome or chronic inflammatory demyelinating polyneuropathy blocks Na+ currents in rat single myehnated nerve fibers. Intern Med 39 123-127. 

Taylor WA, Hughes RAC (1989) T lymphocyte activation antigens in Guillain-Barre syndrome and chronic idiopathic demyelinating polyradiculoneuropathy. J Neuroimmunol 24 33-39. 

Preparations for intravenous administration are mainly used in patients with general immune deficiency states (primary or secondary) or diseases like idiopathic thrombo-cjhopenic purpura (ITP) and autoimmune diseases (5,6). Neurological disorders (for example Guillain-Barre syndrome and chronic demyelinating polyneuropathy) have been treated with intravenous immunoglobulin (7-9). 

Isolated reports of adverse effects have included abscess formation (SEDA-11, 288) dermatomyositis, neuralgic amyotrophy, polyradiculoneuritis with paresis of the urinary bladder and bowel (SEDA-9, 283) asymmetrical polyneuropathy, demyelinating polyneuropathy, and Guillain-Barre syndrome (SEDA-14, 281) and subcutaneous nodules. Polyvinylpyrrolidone thesaurismosis, revealed by inflammatory manifestations after tetanus booster injection, has also been reported (SEDA-10,288). 

FIGURE 20.61 Locations of T lymphocytes among sural nerve biopsy specimens from 13 cases of chronic inflammatory demyelinat-ing polyneuropathy (CIDP) and 22 cases of Guillain-Barre syndrome (CBS). Percentages positive are as reported in Schmidt B, Toyka KV, Keifer R, et al. Inflammatory infiltrates in sural nerve biopsies in Guillain-Barre syndrome and chronic inflammatory demyelinating neuropathy. Muscle Nerve. 1996 19 474-487. 

Schmidt B, Toyka KV, Kiefer R, et al. Inflammatory infiltrates in strral nerve biopsies in Guillain-Barre syndrome and chronic inflammatory demyelinating neuropathy. Muscle Nerve. 1996 19 474-487. 

Peripheral neuropathies, autoimmune. Acute or chronic inflammatory neuropathies leading to demyelination and axonal damage of nerves and nerve roots associated with high-titred autoantibodies against gangliosides (e.g. Guillain-Barre syndrome, Miller-Fisher syndrome, acute sensory ataxic neuropathy). 

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