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Congenital Insensitivity/Indifference to Pain
Background
Congenital Insensitivity to Pain: Congenital Indifference to Pain; Hereditary and Sensory Autonomic Neuropathy Types I-IV; HSAN Types I-IV
In Congenital Insensitivity to Pain, there are structural abnormalities in peripheral nerves which are the peripheral pathways carrying electrical impulses from pain sensitive nerve endings in both superficial and deep tissues.
In Congenital Indifference to Pain, the peripheral nerves are intact and the defect is apparently in the central structures such as the thalamus where painful impulses are normally interpreted. However, it is now thought that some individuals, formerly given a diagnosis of Congenital Indifference to Pain, have been shown by refined histological techniques, which look at the minute structures of bodies, to also have peripheral nerve abnormalities and are therefore examples of Congenital Insensitivity to Pain. Nevertheless, Congenital Indifference to Pain almost certainly exists as an independent condition, but is very rare.
Congenital Insensitivity to Pain (of which types I to IV are generally accepted, with some other very rare conditions) is usually classified under the more general heading of Hereditary and Sensory Autonomic Neuropathy (HSAN). The various categories are distinguished according to clinical features, including age of onset, progressive or non-progressive, presence or absence of abnormalities of the autonomic nervous system, if the system is sympathetic (augmenting actions) or parasympathetic (inhibiting actions) and also according to the nature of structural abnormalities in peripheral nerves.
Abnormalities of peripheral nerves complicate many hereditary neurological and neurometabolic diseases, but they are incidental findings, whereas absent or reduced pain sensation is the hallmark presenting feature in Congenital Insensitivity to Pain.
This classification of hereditary sensory neuropathies overlaps with classifications of hereditary sensory neuropathies in which there are varying degrees of impairment of tactile sensation, with or without impairment of pain sensation. There are also other very rare HSAN which have been identified, mostly as single families, since the original classification was proposed by Dr P J Dyck in 1984. This almost certainly reflects the number of genes which are responsible for the development and maintenance of healthy nerve function, defects in any one of which can lead to impairment of one or many sensory modalities (senses).
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