Congenital insensitivity to pain: Review with dental implications

Kumar, Ashish; Jaishankar, H. P.; Naik, Purnachandrarao

doi:10.4103/0970-5333.128880

Cited by 8 publications

(7 citation statements)

References 2 publications

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“…[1,3] Its other synonyms are hereditary sensory autonomic neuropathy (HSAN), congenital general pure analgesia (Dearborn, 1932), congenital universal insensitiveness to pain (Ford and Wilkins, 1938), congenital universal indifference to pain (Boyd and Nie, 1949), congenital absence of pain (Winkelmann, 1962), and Morvan syndrome. [6] Three diagnostic criteria was proposed by Thrush for defining congenital insensitivity to pain:…”

Section: Discussionmentioning

confidence: 99%

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Congenital insensitivity to pain - A rare case report

Nagaraj¹,

Nigam²,

Gogula³

et al. 2018

ijmdcr

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Congenital insensitivity to pain is defined as persistent occurrences of infections and inexplicable fever, anhidrosis (inability to sweat), and lack of response to deleterious stimuli, self-mutilating performance, mental abnormalities, and injuries to oral cavity. This syndrome can be diagnosed by clinical features, and genetic test is considered as a diagnostic tool. The diagnosis of this syndrome should be done earlier to provide greater lifespan to the patient, thus preventing ejections. In the present case, 3-year-old boy came to our department with the chief complaint of shaking upper front teeth for 1 day and his parents gave a history of congenitally absence of pain and temperature sensations since birth.

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Section: Discussionmentioning

confidence: 99%

“…Although patients are sensitive to thermal stimuli, they may be unaware of the pain associated with high temperatures, thus leading to burns of oral mucosa, especially palate, due to hot food or beverages, etc. [6]…”

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confidence: 99%

Congenital insensitivity to pain - A rare case report

Nagaraj¹,

Nigam²,

Gogula³

et al. 2018

ijmdcr

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“…Sistemik anhidroz, göz kuruluğu, terleme eksikliği, ekstremitelerde ağrısız yara ve zeka geriliği sendromun tipik bulgularındandır. Konjenital ağrı duyarsızlığı sendromu olan, 8 …”

Section: öZetunclassified

“…J Dent Fac Atatürk Uni Supplement: 11, Yıl: 2015, Sayfa : 8-12 J Dent Fac Atatürk Uni Supplement: 11, Yıl: 2015, Sayfa : [8][9][10][11][12]…”

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confidence: 99%

Konjeni̇tal Ağri Duyarsizliği Ve Anhi̇drosi̇s (Cipa): Olgu Sunumu

Kılınç¹,

Çetin²

2015

Atatürk Üniversitesi Diş Hekimliği Fakültesi Dergisi

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Congenital sensitivity to pain and anhidrosis (CIPA), appear in early childhood, is quite rare, autosomal recessive disorder characterized by insensitivity to pain is an autonomic neuropathy. In all cases there is no sensation of pain from birth. Systemic anhydrous, eye dryness, lack of sweating, lack of pain in the extremities and mental retardation are the typical signs of the syndrome. The 8-year-old male patient having congenital insensitivity to pain syndrome, was admitted to Dokuz Eylul University Faculty of Medicine Children's Dental clinics with complaints of injuries in his tongue. During the oral examination; an extreme bite wound with excessive bleeding on the right side of the language determined. Upper right 1st. and 2nd primary molars extracted in order to prevent the patient's tongue biting. In this study we aimed to present CIPA; hereditary, sensory and autonomic neuropathy (HSAN) known as type 4.

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“…[ 5 ] IV, V NTRK1, NGFB Japan/ N/A RA N/A N/A N/A Repeated fractures, joint dislocations, arthritis, osteomyelitis, avascular necrosis, Charcot arthropathy Oral self-mutilation (including auto-extraction of teeth) 2014 Kumar et al . [ 69 ] IV NTRK1 India/ N/A RA N/A N/A N/A N/A Orofacial self-mutilation, premature loss of teeth, osteomyelitis, fractures of the jaws 2013 Limeres et al . [ 26 ] IV N/A Spain/ N/A RA N/A N/A N/A N/A Oral self-mutilation (including auto-extraction of teeth) 2012 Mass [ 70 ] III IKBKAP N/A / N/A RA N/A N/A N/A Insensitivity to pain and temperature, vasomotor instability, respiratory distress, orthostatic hypotension, insensitivity to hypoxia, decreased deep tendon reflexes, alacrima Absence of fungiform papilla, dental traumata, orofacial self-mutilation, proportionally small jaws, crowding of teeth, low caries rate, hypersalivation, impaired taste sensation 2003 Nagasako et al .…”

Section: Introductionmentioning

confidence: 99%

Oral manifestations, dental management, and a rare homozygous mutation of the PRDM12 gene in a boy with hereditary sensory and autonomic neuropathy type VIII: a case report and review of the literature

et al. 2017

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BackgroundHereditary sensory and autonomic neuropathy type VIII is a rare autosomal recessive inherited disorder. Chen et al. recently identified the causative gene and characterized biallelic mutations in the PR domain-containing protein 12 gene, which plays a role in the development of pain-sensing nerve cells. Our patient’s family was included in Chen and colleagues’ study. We performed a literature review of the PubMed library (January 1985 to December 2016) on hereditary sensory and autonomic neuropathy type I to VIII genetic disorders and their orofacial manifestations. This case report is the first to describe the oral manifestations, and their treatment, of the recently discovered hereditary sensory and autonomic neuropathy type VIII in the medical and dental literature.Case presentationWe report on the oral manifestations and dental management of an 8-month-old white boy with hereditary sensory and autonomic neuropathy-VIII over a period of 16 years. Our patient was homozygous for a mutation of PR domain-containing protein 12 gene and was characterized by insensitivity to pain and thermal stimuli, self-mutilation behavior, reduced sweat and tear production, absence of corneal reflexes, and multiple skin and bone infections. Oral manifestations included premature loss of teeth, associated with dental traumata and self-mutilation, severe soft tissue injuries, dental caries and submucosal abscesses, hypomineralization of primary teeth, and mandibular osteomyelitis.ConclusionsThe lack of scientific knowledge on hereditary sensory and autonomic neuropathy due to the rarity of the disease often results in a delay in diagnosis, which is of substantial importance for the prevention of many complications and symptoms. Interdisciplinary work of specialized medical and dental teams and development of a standardized treatment protocols are essential for the management of the disease. There are many knowledge gaps concerning the management of patients with hereditary sensory and autonomic neuropathy-VIII, therefore more research on an international basis is needed.

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Congenital insensitivity to pain: Review with dental implications

Cited by 8 publications

References 2 publications

Congenital insensitivity to pain - A rare case report

Congenital insensitivity to pain - A rare case report

Konjeni̇tal Ağri Duyarsizliği Ve Anhi̇drosi̇s (Cipa): Olgu Sunumu

Oral manifestations, dental management, and a rare homozygous mutation of the PRDM12 gene in a boy with hereditary sensory and autonomic neuropathy type VIII: a case report and review of the literature

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