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Self-mutilation of tongue and lips due to involuntary movements

MedGen UID:
348566
Concept ID:
C1860219
Finding
HPO: HP:0008767

Definition

Uncontrolled movements result in tissue damage to the tongue and lips. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVSelf-mutilation of tongue and lips due to involuntary movements

Conditions with this feature

Chorea-acanthocytosis
MedGen UID:
98277
Concept ID:
C0393576
Disease or Syndrome
Chorea-acanthocytosis (ChAc) is characterized by a progressive movement disorder, cognitive and behavior changes, a myopathy that can be subclinical, and chronic hyperCKemia in serum. Although the disorder is named for acanthocytosis of the red blood cells, this feature is variable. The movement disorder is mostly limb chorea, but some individuals present with parkinsonism. Dystonia is common and affects the oral region and especially the tongue, causing dysarthria and serious dysphagia with resultant weight loss. Habitual tongue and lip biting are characteristic, as well as tongue protrusion dystonia. Progressive cognitive and behavioral changes resemble those in a frontal lobe syndrome. Seizures are observed in almost half of affected individuals and can be the initial manifestation. Myopathy results in progressive distal muscle wasting and weakness. Mean age of onset in ChAc is about 30 years, although ChAc can develop as early as the first decade or as late as the seventh decade. It runs a chronic progressive course and may lead to major disability within a few years. Life expectancy is reduced, with age of death ranging from 28 to 61 years.

Recent clinical studies

Etiology

Sahoo LK, Swain KP, Mallick AK, Mohanty G, Samanta M, Sahoo SK
J Assoc Physicians India 2017 Apr;65(4):93-94. PMID: 28527175
Arhakis A, Topouzelis N, Kotsiomiti E, Kotsanos N
Dent Traumatol 2010 Dec;26(6):496-500. doi: 10.1111/j.1600-9657.2010.00930.x. PMID: 21078074

Diagnosis

Sahoo LK, Swain KP, Mallick AK, Mohanty G, Samanta M, Sahoo SK
J Assoc Physicians India 2017 Apr;65(4):93-94. PMID: 28527175

Prognosis

Arhakis A, Topouzelis N, Kotsiomiti E, Kotsanos N
Dent Traumatol 2010 Dec;26(6):496-500. doi: 10.1111/j.1600-9657.2010.00930.x. PMID: 21078074

Clinical prediction guides

Ruiz-Sandoval JL, García-Navarro V, Chiquete E, Dobson-Stone C, Monaco AP, Alvarez-Palazuelos LE, Padilla-Martínez JJ, Barrera-Chairez E, Rodríguez-Figueroa EI, Pérez-García G
Arch Neurol 2007 Nov;64(11):1661-4. doi: 10.1001/archneur.64.11.1661. PMID: 17998451

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