Curly Hair as Presenting Symptom in Giant Axonal Neuropathy
Giant Axonal Neuropathy (GAN) is a chronic progressive peripheral polyneuropathy of unknown etiology. Symptoms such as weakness of distal muscles, loss of reflexes and sensation or nystagmus appear usually at the age of 2 or 3 years. With regard to the pathomorphology of the disease, it is characterized by segmental swelling of myelinated and unmyelinated axons in peripheral nerves. Ultrastructural investigations have disclosed, that this enlargement of axons is produced by accumulation of tightly packed filaments with a diameter of approximately 100 Å (Asbury et al. 1972, Carpenter et al. 1974, Igisu et al. 1975, Prineas et al. 1976, Gambarelli et al. 1977, Peiffer et al. 1977, Jedrzejowska et al. 1977).
KeywordsSchwann Cell Sural Nerve Hair Shaft Unmyelinated Axon Osmic Acid
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