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Revista de la Sociedad Española del Dolor
Print version ISSN 1134-8046
Abstract
VICENTE-FATELA, L. and ACEDO, Mª S.. Pain reactivity disorders. Rev. Soc. Esp. Dolor [online]. 2004, vol.11, n.1, pp.31-37. ISSN 1134-8046.
The protective role of pain is well-known. There are some diseases in which pain perception is absent, causing multiple problems to the patients. In pain reactivity disorders can be divided into congenital and acquired disorders. Congenital disorders cover two well-differentiated clinical conditions: congenital pain insensitivity or congenital analgesia and congenital pain indifference. In the first case, pain stimuli are not adequately transmitted to the central nervous system due to a deficiency in the sensitive routes, whereas in the second the sensitive routes are intact, but the patient cannot perceive the pain stimulus as unpleas-ant. Congenital pain insensitivity is currently included in the group of the so-called sensitive-autonomous hereditary neuropathies, characterized by pain sensitivity disorders involving small myelinic and non-myelinic nerve fibres, which are the vehicles of most pain sensitivity, as well as the autonomous fibres. Five types of hereditary sensitive-autonomous neuropathies can be differentiated and are described in the text. All of them are associated to pain perception disorders, particularly the hereditary sensitive-autonomous neuropathy type IV and the congenital pain insensitivity with anhidrosis. Clinically, the loss of pain sensitivity in these neuropathies can cause damage at different levels. The most frequent associated disorders are: multiple fractures, Charcot joints, lower limb dysmetria, osteomyelitis, septic arthritis, luxations, self-amputations, self-mutilations and progressive scoliosis. Multidisciplinary and specialized therapy is required, as well as parental collaboration, in order to minimize complications of this potentially severe disease. In the absence of an etiological treatment, the symptomatic treatment becomes very important. Risk processes, fractures, bites, rubs, infections and mutilations shall be prevented as far as possible. Damage of brain areas involved in pain stimulus process-ing can cause deficits in one or several components of pain perception, resulting in clinical conditions similar to congenital pain insensitivity. Within the acquired disorders, painful asymbolia, analgotimia and painful hemiagnosia are described.
Keywords : Congenital pain insensitivity; Congenital analgesia; Pain asymbolia; Hereditary sensitive-autonomous neuropathy.