Congenital insensitivity to pain with anhidrosis (CIPA): the spectrum of radiological findings

Schulman, H.; Tsodikow, Vadim; Einhorn, Menachem; Levy, Yaron Y.; Shorer, Zamir; Hertzanu, Yancu

doi:10.1007/s002470100506

Cited by 45 publications

(33 citation statements)

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“…A combined defect in sensory and autonomic neurons derived from the neural crest A reduced evoked potential (Chatrian, Farrell, Canfield, & Lettich, 1975;Shorey & Lobo, 1990) Lack of pain experience following electrical shock; (Manfredi et al, 1981) Self-mutilation and fractures (Itoh, Nakajima, Yagishita, Nakano, & Kawada, 1986;Chatrian et al, 1975;Matsuo, Kurokawa, Goya, & Ohta, 1981;Sweet, 1981;Derwin, Glover, & Wojtys, 1994;Nolano et al, 2000;Schulman et al, 2001) Lack of flare response to histamine injection (Manfredi et al, 1981;Nolano et al, 2000) Lack of temperature regulation (Itoh et al, 1986;Matsuo et al, 1981;Vital et al, 1998;Sztriha, Lestringant, Hertecant, Frossard, & Masouye, 2001) Overexpression of endogenous opioids (Dehen, Willer, Boureau, & Cambier, 1977;Dehen, Willer, Prier, Boureau, & Cambier, 1978) Reduced number of primary afferent nociceptors (Larner, Moss, Rossi, & Anderson, 1994) Loss of neurons in sympathetic ganglia (Dyck et al, 1983;Derwin et al, 1994;Shorer, Moses, Hershkovitz, Pinsk, & Levy, 2001;Sztriha et al, 2001) Loss of trkA function (receptor for nerve growth factor) as the result of mutations of the trkA receptor gene (Indo et al, 1996;Mardy et al, 1999;Yotsumoto et al, 1999;Shatzky et al, 2000;Toscano et al, 2000;Greco, Villa, Fusetti, Orlandi, & Pierotti, 2000;Miura et al, 2000a;Miura et al, 2000b;…”

Section: Congenital Insensitivity To Painmentioning

confidence: 99%

The biopsychosocial approach to chronic pain: Scientific advances and future directions.

Gatchel¹,

Peng²,

Peters³

et al. 2007

Psychological Bulletin

2,528

1,903

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The prevalence and cost of chronic pain is a major physical and mental health care problem in the United States today. As a result, there has been a recent explosion of research on chronic pain, with significant advances in better understanding its etiology, assessment and treatment. The purpose of the present article is to provide a review of the most noteworthy developments in the field. The biopsychosocial model is now widely accepted as the most heuristic approach to chronic pain. With this model in mind, a review of the basic neuroscience processes of pain (the bio part of biopsychosocial), as well as the psychosocial factors is presented. This spans research on how psychological and social factors can interact with brain processes to influence health and illness, to the development of new technologies, such as brain imaging, that provide new insights into brain-pain mechanisms.KEY WORDS: biopsychosocial; chronic pain; neuroscience of pain; pain and cognition; pain and emotion Biopsychosocial Approach to Chronic Pain 3The Biopsychosocial Approach to Chronic Pain: Scientific Advances and Future Directions During the past decade, there has been an explosion of research on chronic pain, with significant advances in understanding its etiology, assessment and treatment (Gatchel, 2004; Turk & Monarch, 2002). This research has important health care implications Epidemiological research has shown that chronic pain (loosely defined as prolonged and persistant pain of at least 3-months duration) and chronic recurrent pain (recurrent episodes of pain interspersed with pain free periods extending over months or years) affects 10% -20% of adults in the general population (Blyth et al., 2001; Gureje, Von Korff, Simon & Gater, 1998; Vehaak, Kerssens, Dekker, Sorbi & Bensing, 1998). For example, in a large-scale epidemiological study, Von Korff, Crane, Lane et al. (2005) estimated a 19% prevalence for chronic spinal pain (neck and back) in the U.S. in the previous year, and a 29% lifetime rate. The American Academy of Pain Management (2003) asserted that approximately 57% of all adult Americans reported experiencing recurrent or chronic pain in the past year. About 62% of those individuals reporting being in pain for more than 1 year, and 40% noted that they were constantly in pain. Indeed, as Gatchel (2004) has summarized that pain is a pervasive medical problem: it affects over 50 million Americans and costs more than $70 billion annually in health care costs and lost productivity; it accounts for more than 80% of all physician visits. Moreover, chronic pain is often associated with major comorbid psychiatric disorders and emotional suffering.As the above factors attest, the prevalence and cost of chronic pain is a major physical and mental health care problem in the United States. Moreover, individuals 50 years of age and older are twice as likely to have been diagnosed with chronic pain (Gatchel, 2004;. Currently, there are approximately 35 million Americans aged 65 years or older, accounting for 12.4% of t...

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Section: Congenital Insensitivity To Painmentioning

confidence: 99%

The biopsychosocial approach to chronic pain: Scientific advances and future directions.

Gatchel¹,

Peng²,

Peters³

et al. 2007

Psychological Bulletin

2,528

1,903

View full text Add to dashboard Cite

show abstract

“…TrkA is required for development of the nociceptive C and Aô fibres and sympathetic neurons (Crowley et al, 1994). As a result, these patients are unable to detect painful stimuli and suffer from self-inflicted wounds, self-mutilation, painless burns, and joint injuries (Schulman et al, 2001) Many ofthese individuals die due to internaI organ dysfunction discovered at a terminal stage because of lack of the ability of the patient to feel pain caused by the dysfunction.…”

Section: Résumémentioning

confidence: 99%

Sympathetic sprouting and changes in nociceptive sensory innervation in the glabrous skin of the rat hind paw following partial peripheral nerve injury

Yen

Bennett

Ribeiro‐da‐Silva

2006

J of Comparative Neurology

103

102

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Previous studies have suggested that sympathetic sprouting in the periphery may contribute to the development and persistence of sympathetically maintained pain in animal models of neuropathic pain. In the present study, we examined changes in the cutaneous innervation in rats with a chronic constriction injury to the sciatic nerve. At several periods postinjury, hind paw skin was harvested and processed by using a monoclonal antibody against dopamine‐β‐hydroxylase to detect sympathetic fibers and a polyclonal antibody against calcitonin gene‐related peptide to identify peptidergic sensory fibers. We observed migration and branching of sympathetic fibers into the upper dermis of the hind paw skin, where they were normally absent. This migration was first detected at 2 weeks, peaked at 4–6 weeks, and lasted for at least 20 weeks postlesion. At 8 weeks postlesion, there was a dramatic increase in the density of peptidergic fibers in the upper dermis. Quantification revealed that densities of peptidergic fibers 8 weeks postlesion were significantly above levels in sham animals. The ectopic sympathetic fibers did not innervate blood vessels but formed a novel association and wrapped around sprouted peptidergic nociceptive fibers. Our data show a long‐term sympathetic and sensory innervation change in the rat hind paw skin after the chronic constriction injury. This novel fiber arrangement after nerve lesion may play an important role in the development and persistence of sympathetically maintained neuropathic pain after partial nerve lesions. J. Comp. Neurol. 495:679–690, 2006. © 2006 Wiley‐Liss, Inc.

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“…This can cause significant bone hypertrophy in young patients. Patients with CIPA may develop recurrent, non-healing fractures with significant periosteal reactions and excessive callus formation, and 50% of patients have osteomyelitis and destruction of underlying bone [16].…”

Section: Discussionmentioning

confidence: 99%

Congenital insensitivity to pain syndrome accompanied by neglected orthopedic traumas and complications

et al. 2017

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Pain is a protective mechanism. Congenital insensitivity to pain syndrome is a very rare disease in which there is no ability to feel physical pain. It has been reported that it occurs with an incidence of 1 in 125 million newborn. Patients with congenital insensitivity to pain may have various orthopedic complications such as recurrent fractures, osteomyelitis and neuropathic joints. The most frequently affected body parts are lower extremities. Besides, curvature of spine can be seen. Injuries in epiphyseal points may cause incompatibility of extremity. Charcot joints may develop, which can lead to neuropathic arthropathy as a result of insensitivity to pain. Here, we present a patient with traumatic fracture-dislocation on left hip that neglected the treatment, had a bilateral femur fracture and then had septic arthritis of knee.

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Congenital insensitivity to pain with anhidrosis (CIPA): the spectrum of radiological findings

Abstract: CIPA is a severe autosomal recessive condition that leads to self-mutilation early in life and to fractures, osteomyelitis and limb amputation in older children. Mental retardation is common. Death from hyperpyrexia occurs in almost 20 % of patients in the first 3 years of life.

Cited by 45 publications

References 0 publications

The biopsychosocial approach to chronic pain: Scientific advances and future directions.

The biopsychosocial approach to chronic pain: Scientific advances and future directions.

Sympathetic sprouting and changes in nociceptive sensory innervation in the glabrous skin of the rat hind paw following partial peripheral nerve injury

Congenital insensitivity to pain syndrome accompanied by neglected orthopedic traumas and complications

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