What is axonal neuropathy?
Collapse Section. Giant axonal neuropathy is an inherited condition characterized by abnormally large and dysfunctional axons called giant axons. Axons are specialized extensions of nerve cells (neurons) that transmit nerve impulses.
What is axonal variant?
Axonal variants of Guillain-Barré syndrome (GBS) mainly include acute motor axonal neuropathy, acute motor and sensory axonal neuropathy, and pharyngeal-cervical-brachial weakness.
What is the meaning of GBS?
Guillain-Barré syndrome (GBS) is a rare neurological disorder in which the body’s immune system mistakenly attacks part of its peripheral nervous system—the network of nerves located outside of the brain and spinal cord.
What causes axonal damage?
The most common etiology of diffuse axonal injury involves high-speed motor vehicle accidents. [2] The most common mechanism involves an accelerating and decelerating motion that leads to shearing forces to the white matter tracts of the brain.
How is axonal neuropathy treated?
The standard treatment is intravenous immunoglobulin (IVIg) or plasmapheresis. SLE is a multisystem autoimmune disease which is generally treated by immunosuppressant. The association of AMSAN and SLE is rarely reported.
What causes Amsan?
Although the exact pathological mechanism is poorly understood, both AMAN and AMSAN are associated with the presence of antiganglioside antibodies (anti-GM1/GD1a/GM1b/GalNAc-GD1a) and may be caused by antibody-mediated primary axonal degeneration or antibody-mediated inhibition of voltage-gated sodium channels.
How long does it take to recover from GBS?
Guillain-Barre syndrome (GBS) may last between 14 and 30 days and you may slowly recuperate from it. Usually, recovery takes 6 to 12 months, but for some people, it could take up to 3 years.
What are the two subtypes of axonal GBS?
Axonal GBS has been classified further into two groups: acute motor axonal neuropathy (AMAN) and acute motor and sensory axonal neuropathy (AMSAN). 1 The principal clinical method for distinguishing AMAN, AMSAN, and acute inflammatory demyelinating polyneuropathy (AIDP) is electrodiagnostic, and clear criteria have been formulated.
Is there an axonal variant of GBS called Aman?
Following the first detailed description of an axonal variant of GBS ( 27 ), an axonal motor variant of GBS termed acute motor axonal neuropathy (AMAN) was report in 1993 from Northern China and hence the name Chinese paralytic illness ( 13 ).
What is the long term prognosis for axonal GBS?
A comparison of the prognosis for AMAN and AIDP patients is therefore difficult. In the multicentre study of 369 GBS patients in 11 Western countries, 29 axonal GBS accounted for 3% (10 patients); 10% of those with the axonal form and 17% with the demyelinating form could not walk or were dead 48 weeks after onset.
What are the different types of GBS syndrome?
Besides classic presentation of GBS, clinical variants are based on the types of nerve fibers involved (motor, sensory, sensory and motor, cranial or autonomic), predominant mode of fiber injury (demyelinating versus axonal), and the presence of alteration in consciousness.
