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Giant axonal neuropathy is a rare, autosomal recessive [1] neurological disorder that causes disorganization of neurofilaments. Neurofilaments form a structural framework that helps to define the shape and size of neurons and are essential for normal nerve function.
This condition was discovered in 1991 by Hahn et al., when they described two Chinese-Canadian siblings of the opposite sex. The male had difficulties releasing his grip, childhood-onset neuromyotonia and muscle stiffness, progressive motor neuropathy, finger cramping while and after writing, involuntary twitches of the finger, thigh and forearm muscles, foot drop-associated gait problems ...
Diffuse axonal injury (DAI) is a brain injury in which scattered lesions occur over a widespread area in white matter tracts as well as grey matter. [1] [2] [3] [4 ...
CMT2 variants are typically referred to as axonal neuropathies due to the axonal degeneration observed. CMT2 variants are a result of damage to the nerve axons, rather than damage to the myelin sheath (as is the case with CMT1). Damaged axons cause slowed transmission of signals to the muscles and brain, causing symptoms including muscle ...
An illness which is lifelong but not fatal is called a chronic condition. Terminal patients have options for disease management after diagnosis. Examples include caregiving, continued treatment, palliative and hospice care, and physician-assisted suicide. Decisions regarding management are made by the patient and their family, although medical ...
The provided axonal protection delays the onset of Wallerian degeneration. Schwann cell activation should therefore be delayed, as they would not detect axonal degradation signals from ErbB2 receptors. In experiments on Wld s mutated mice, macrophage infiltration was considerably delayed by up to six to eight days. [40]
Structure of a typical neuron with Schwann cells in the peripheral nervous system. Chronic inflammatory demyelinating polyneuropathy (or polyradiculoneuropathy) is considered an autoimmune disorder destroying myelin, the protective covering of the nerves.
According to Lopate, et al., methylprednisolone is a viable treatment for chronic inflammatory demyelinative polyneuropathy (which can also be treated with intravenous immunoglobulin). The authors also indicate that prednisone has greater adverse effects in such treatment, as opposed to intermittent (high-doses) of the aforementioned medication.