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Congenital insensitivity to pain with anhidrosis (CIPA) is a rare autosomal recessive disorder of the nervous system which prevents the feeling of pain or temperature and prevents a person from sweating. Cognitive disorders are commonly coincidental.
1960: Ervin and Sternbach describe 6 members from a 2-generation family with dominantly-inherited congenital insensitivity to pain. [ 4 ] 1974: Comings and Amromin describe 3 members from a 2-generation family which consisted of a mother, her son and her daughter with the symptoms characteristic of Marsili syndrome, there was a possibility that ...
Congenital insensitivity to pain (CIP), also known as congenital analgesia, is one or more extraordinarily rare conditions in which a person cannot feel (and has never felt) physical pain. [1] The conditions described here are separate from the HSAN group of disorders, which have more specific signs and cause.
The diagnostic criteria for acquired idiopathic generalized anhidrosis is as follows: [6] Despite the widespread distribution of idiopathic anhidrosis or hypohidrosis lesions in a non-segmental spinal pattern, no additional neurological or autonomic symptoms are noted. [6] At least 25% of the body is affected by anhidrotic or hypohidrotic regions.
An extreme case of hypohidrosis in which there is a complete absence of sweating and the skin is dry is termed anhidrosis. [3] The condition is also known as adiaphoresis , [ 4 ] ischidrosis , [ 5 ] oligidria , [ 6 ] oligohidrosis [ 7 ] and sweating deficiency .
The signs and symptoms occur on the same side (ipsilateral) as it is a lesion of the sympathetic trunk. It is characterized by miosis (a constricted pupil ), partial ptosis (a weak, droopy eyelid), apparent anhidrosis (decreased sweating), with apparent enophthalmos (inset eyeball ).
Anhidrosis is not a temporary condition, and as Hamblin concludes, "a temporary inability to sweat would defy medical precedent. A long-standing detachment from the consequences of one’s actions ...
The signs and symptoms of hereditary sensory neuropathy type 1 typically appear during a person's teens or twenties. While the features of this disorder tend to worsen over time, affected individuals have a normal life expectancy if signs and symptoms are properly treated. Type 1 is the most common form among the 5 types of HSAN.