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Disinhibition

MedGen UID:
504576
Concept ID:
CN000690
Finding
 
HPO: HP:0000734

Definition

A lack of restraint manifested in several ways, including disregard for social conventions, impulsivity, and poor risk assessment. [from HPO]

Term Hierarchy

Conditions with this feature

Pick's disease
MedGen UID:
116020
Concept ID:
C0236642
Disease or Syndrome
The clinical manifestations of MAPT-related disorders (MAPT-related tauopathies) are most typically those of frontotemporal dementia (FTDP-17), but also include progressive supranuclear palsy (PSP), corticobasal degeneration (CBD), mild late-onset parkinsonism, and dementia with epilepsy. Clinical presentation of frontotemporal dementia (FTD) is variable: some present with slowly progressive behavioral changes, language disturbances, and/or extrapyramidal signs, whereas others present with rigidity, bradykinesia, supranuclear palsy, and saccadic eye movement disorders. Onset is usually between ages 40 and 60 years, but may be earlier or later. The disease progresses over a few years into profound dementia with mutism. PSP is characterized by progressive vertical gaze palsy in combination with a prominent loss of balance at early stages of the disease. With progression, axial rigidity, dysarthria, and dysphagia become prominent, often in combination with a frontal dysexecutive syndrome. CBD is a progressive neurodegenerative disorder which affects both the frontoparietal cortex and the basal ganglia, resulting in a mild to moderate dementia in combination with asymmetric parkinsonism, ideomotor apraxia, aphasia, and an alien-hand syndrome.
Frontotemporal dementia
MedGen UID:
83266
Concept ID:
C0338451
Disease or Syndrome
The clinical manifestations of MAPT-related disorders (MAPT-related tauopathies) are most typically those of frontotemporal dementia (FTDP-17), but also include progressive supranuclear palsy (PSP), corticobasal degeneration (CBD), mild late-onset parkinsonism, and dementia with epilepsy. Clinical presentation of frontotemporal dementia (FTD) is variable: some present with slowly progressive behavioral changes, language disturbances, and/or extrapyramidal signs, whereas others present with rigidity, bradykinesia, supranuclear palsy, and saccadic eye movement disorders. Onset is usually between ages 40 and 60 years, but may be earlier or later. The disease progresses over a few years into profound dementia with mutism. PSP is characterized by progressive vertical gaze palsy in combination with a prominent loss of balance at early stages of the disease. With progression, axial rigidity, dysarthria, and dysphagia become prominent, often in combination with a frontal dysexecutive syndrome. CBD is a progressive neurodegenerative disorder which affects both the frontoparietal cortex and the basal ganglia, resulting in a mild to moderate dementia in combination with asymmetric parkinsonism, ideomotor apraxia, aphasia, and an alien-hand syndrome.
Choreoacanthocytosis
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.
Frontotemporal Dementia, Chromosome 3-Linked
MedGen UID:
318833
Concept ID:
C1833296
Disease or Syndrome
Chromosome 3-linked frontotemporal dementia (FTD3) has been described in a single family from Denmark and in one individual with familial FTD3 from Belgium. It typically starts between ages 46 and 65 years with subtle personality changes and slowly progressive behavioral changes, dyscalculia, and language disturbances. Disinhibition or loss of initiative is the most common presenting symptom. The disease progresses over a few years into profound dementia with mutism. Several individuals have developed an asymmetric akinetic rigid syndrome with arm and gait dystonia and pyramidal signs that may be related to treatment with neuroleptic drugs. Disease duration may be as short as three years or longer than 20 years.
Fragile X tremor/ataxia syndrome
MedGen UID:
333403
Concept ID:
C1839780
Disease or Syndrome
FMR1-related disorders include fragile X syndrome, fragile X-associated tremor/ataxia syndrome (FXTAS), and FMR1-related primary ovarian insufficiency (POI). Fragile X syndrome occurs in individuals with an FMR1 full mutation or other loss-of-function mutation and is nearly always characterized by moderate intellectual disability in affected males and mild intellectual disability in affected females. Because FMR1 mutations are complex alterations involving non-classic gene-disrupting alterations (trinucleotide repeat expansion) and abnormal gene methylation, affected individuals occasionally have an atypical presentation with an IQ above 70, the traditional demarcation denoting intellectual disability (previously referred to as mental retardation). Males with an FMR1 full mutation accompanied by aberrant methylation may have a characteristic appearance (large head, long face, prominent forehead and chin, protruding ears), connective tissue findings (joint laxity), and large testes after puberty. Behavioral abnormalities, sometimes including autism spectrum disorder, are common. FXTAS occurs in males (and some females) who have an FMR1 premutation and is characterized by late-onset, progressive cerebellar ataxia and intention tremor. FMR1-related POI (age at cessation of menses <40 years) occurs in approximately 20% of females who have an FMR1 premutation.
Frontotemporal dementia, ubiquitin-positive
MedGen UID:
375285
Concept ID:
C1843792
Disease or Syndrome
The spectrum of frontotemporal dementia associated with GRN (also known as PGRN) mutation (FTD-GRN or FTD-PGRN) includes the behavioral variant (FTD-bv), primary progressive aphasia (PPA; further sub-categorized as progressive non-fluent aphasia [PNFA] and semantic dementia [SD]), and movement disorders with extrapyramidal features such as parkinsonism and corticobasal syndrome. A broad range of clinical features both within and across families is observed. The age of onset ranges from 35 to 87 years. Behavioral disturbances are the most common early feature, followed by progressive aphasia. Impairment in executive function manifests as loss of judgment and insight. In early stages, PPA often manifests as deficits in naming, word finding, or word comprehension. In late stages, affected individuals often become mute and lose their ability to communicate. Early findings of parkinsonism include rigidity, bradykinesia or akinesia (slowing or absence of movements), limb dystonia, apraxia (loss of ability to carry out learned purposeful movements), and disequilibrium. Late motor findings may include myoclonus, dysarthria, and dysphagia. Most affected individuals eventually lose the ability to walk. Disease duration is three to 12 years.
Neuroferritinopathy
MedGen UID:
381211
Concept ID:
C1853578
Disease or Syndrome
Neuroferritinopathy typically presents with progressive adult-onset chorea or dystonia affecting one or two limbs, and subtle cognitive deficits. The movement disorder involves additional limbs within five to ten years and becomes more generalized within 20 years. When present, asymmetry remains throughout the course of the disorder. The majority of individuals develop a characteristic orofacial action-specific dystonia related to speech that leads to dysarthrophonia. Frontalis overactivity and orolingual dyskinesia are common. Cognitive deficits and behavioral issues become major problems with time.
Polycystic lipomembranous osteodysplasia with sclerosing leukoencephalopathy
MedGen UID:
387795
Concept ID:
C1857316
Congenital Abnormality
Polycystic lipomembranous osteodysplasia with sclerosing leukoencephalopathy (PLOSL) is characterized by fractures (resulting from radiologically demonstrable polycystic osseous lesions), frontal lobe syndrome, and progressive presenile dementia beginning in the fourth decade. The clinical course of PLOSL can be divided into four stages: (1) The latent stage is characterized by normal early development. (2) The osseous stage (3rd decade of life) is characterized by pain and tenderness, mostly in ankles and feet, usually following strain or injury. Fractures are typically diagnosed several years later, most commonly in the bones of the extremities. (3) In the early neurologic stage (4th decade of life), a change of personality begins to develop insidiously. Affected individuals show a frontal lobe syndrome (loss of judgment, euphoria, loss of social inhibitions, disturbance of concentration, and lack of insight, libido, and motor persistence) leading to serious social problems. (4) The late neurologic stage is characterized by progressive dementia and loss of mobility. Death usually occurs before age 50 years.
Spastic paraplegia 4, autosomal dominant
MedGen UID:
401097
Concept ID:
C1866855
Disease or Syndrome
Spastic paraplegia 4 (SPG4; also known as SPAST-associated HSP) is characterized by insidiously progressive bilateral lower-limb gait spasticity. More than 50% of affected individuals have some weakness in the legs and impaired vibration sense at the ankles. About one third have sphincter disturbances. Onset is insidious, mostly in young adulthood, although symptoms may start as early as age one year and as late as age 76 years. Intrafamilial variation is considerable.
Amyotrophic lateral sclerosis type 10
MedGen UID:
383137
Concept ID:
C2677565
Disease or Syndrome
TARDBP-related amyotrophic lateral sclerosis (TARDBP-related ALS) is characterized by upper motor neuron (UMN) and lower motor neuron (LMN) disease that appears indistinguishable from ALS of other known and unknown causes based on gender ratio, age of onset, symptom distribution, and severity of disease. The male to female ratio is 1.6 to 1. Mean age of onset is 54 ± 12 years. UMN manifestations can include stiffness, spasticity, hyperreflexia, and pseudobulbar affect; LMN manifestations often include weakness accompanied by muscle atrophy, fasciculations, and cramping. Limb onset occurs in 80% and bulbar onset in 20%. Affected individuals typically succumb to respiratory failure when phrenic and thoracic motor neurons become severely involved.

Recent clinical studies

Etiology

Rooney M, Chronis-Tuscano AM, Huggins S
J Atten Disord 2015 Apr;19(4):313-27. Epub 2012 Nov 1 doi: 10.1177/1087054712459885. [Epub ahead of print] PMID: 23117860
Archer NP, Wilkinson AV, Ranjit N, Wang J, Zhao H, Swann AC, Shete S
Brain Behav 2014 Jul;4(4):521-30. Epub 2014 May 16 doi: 10.1002/brb3.236. PMID: 25161819Free PMC Article
Himes SK, LaGasse LL, Derauf C, Newman E, Smith LM, Arria AM, Della Grotta SA, Dansereau LM, Abar B, Neal CR, Lester BM, Huestis MA
Ther Drug Monit 2014 Aug;36(4):535-43. doi: 10.1097/FTD.0000000000000049. PMID: 24518561Free PMC Article
Mayer MK, Reiter PL, Zucker RA, Brewer NT
Sex Transm Dis 2013 Oct;40(10):822-8. doi: 10.1097/OLQ.0000000000000021. PMID: 24275737Free PMC Article
Lee Y, Chong MF, Liu JC, Libedinsky C, Gooley JJ, Chen S, Wu T, Tan V, Zhou M, Meaney MJ, Lee YS, Chee MW
Am J Clin Nutr 2013 May;97(5):919-25. Epub 2013 Apr 3 doi: 10.3945/ajcn.112.053801. [Epub ahead of print] PMID: 23553164

Diagnosis

Rooney M, Chronis-Tuscano AM, Huggins S
J Atten Disord 2015 Apr;19(4):313-27. Epub 2012 Nov 1 doi: 10.1177/1087054712459885. [Epub ahead of print] PMID: 23117860
Powers JP, Massimo L, McMillan CT, Yushkevich PA, Zhang H, Gee JC, Grossman M
Cogn Behav Neurol 2014 Dec;27(4):206-14. doi: 10.1097/WNN.0000000000000044. PMID: 25539040Free PMC Article
Himes SK, LaGasse LL, Derauf C, Newman E, Smith LM, Arria AM, Della Grotta SA, Dansereau LM, Abar B, Neal CR, Lester BM, Huestis MA
Ther Drug Monit 2014 Aug;36(4):535-43. doi: 10.1097/FTD.0000000000000049. PMID: 24518561Free PMC Article
Schiehser DM, Liu L, Lessig SL, Song DD, Obtera KM, Burke Iii MM, Earl SR, Vincent Filoteo J
J Int Neuropsychol Soc 2013 Mar;19(3):295-304. Epub 2013 Jan 28 doi: 10.1017/S1355617712001385. [Epub ahead of print] PMID: 23351239
Handley ED, Chassin L, Haller MM, Bountress KE, Dandreaux D, Beltran I
J Abnorm Psychol 2011 Aug;120(3):528-42. doi: 10.1037/a0024162. PMID: 21668077Free PMC Article

Therapy

Mayer MK, Reiter PL, Zucker RA, Brewer NT
Sex Transm Dis 2013 Oct;40(10):822-8. doi: 10.1097/OLQ.0000000000000021. PMID: 24275737Free PMC Article
Tripathi A, Whiteside YO, Duffus WA
South Med J 2013 Oct;106(10):558-64. doi: 10.1097/SMJ.0000000000000010. PMID: 24096949
Kembabazi A, Bajunirwe F, Hunt PW, Martin JN, Muzoora C, Haberer JE, Bangsberg DR, Siedner MJ
PLoS One 2013;8(7):e69634. Epub 2013 Jul 19 doi: 10.1371/journal.pone.0069634. PMID: 23894514Free PMC Article
Vrieze SI, McGue M, Miller MB, Hicks BM, Iacono WG
Behav Genet 2013 Mar;43(2):97-107. Epub 2013 Jan 31 doi: 10.1007/s10519-013-9584-z. [Epub ahead of print] PMID: 23362009Free PMC Article
Gallant AR, Tremblay A, Pérusse L, Després JP, Bouchard C, Drapeau V
Br J Nutr 2012 Dec 14;108(11):1976-9. Epub 2012 Feb 28 doi: 10.1017/S0007114512000505. [Epub ahead of print] PMID: 22369701

Prognosis

Samek DR, Iacono WG, Keyes MA, Epstein M, Bornovalova MA, McGue M
J Child Psychol Psychiatry 2014 Jul;55(7):784-92. PMID: 25083529Free PMC Article
Conradt E, Lagasse LL, Shankaran S, Bada H, Bauer CR, Whitaker TM, Hammond JA, Lester BM
Dev Neurosci 2014;36(3-4):306-15. Epub 2014 Jul 15 doi: 10.1159/000365004. [Epub ahead of print] PMID: 25033835
Carr KA, Lin H, Fletcher KD, Epstein LH
Obesity (Silver Spring) 2014 Jan;22(1):254-9. Epub 2013 May 29 doi: 10.1002/oby.20392. [Epub ahead of print] PMID: 23512958Free PMC Article
Murray JD, Anticevic A, Gancsos M, Ichinose M, Corlett PR, Krystal JH, Wang XJ
Cereb Cortex 2014 Apr;24(4):859-72. Epub 2012 Nov 29 doi: 10.1093/cercor/bhs370. [Epub ahead of print] PMID: 23203979Free PMC Article
Riggs NR, Tate EB, Ridenour TA, Reynolds MD, Zhai ZW, Vanyukov MM, Tarter RE
J Adolesc Health 2013 Oct;53(4):465-70. Epub 2013 Jul 20 doi: 10.1016/j.jadohealth.2013.05.017. [Epub ahead of print] PMID: 23876782Free PMC Article

Clinical prediction guides

Archer NP, Wilkinson AV, Ranjit N, Wang J, Zhao H, Swann AC, Shete S
Brain Behav 2014 Jul;4(4):521-30. Epub 2014 May 16 doi: 10.1002/brb3.236. PMID: 25161819Free PMC Article
Van Gucht D, Soetens B, Raes F, Griffith JW
Appetite 2014 May;76:137-43. Epub 2014 Feb 12 doi: 10.1016/j.appet.2014.01.078. [Epub ahead of print] PMID: 24530692
Himes SK, LaGasse LL, Derauf C, Newman E, Smith LM, Arria AM, Della Grotta SA, Dansereau LM, Abar B, Neal CR, Lester BM, Huestis MA
Ther Drug Monit 2014 Aug;36(4):535-43. doi: 10.1097/FTD.0000000000000049. PMID: 24518561Free PMC Article
Murray JD, Anticevic A, Gancsos M, Ichinose M, Corlett PR, Krystal JH, Wang XJ
Cereb Cortex 2014 Apr;24(4):859-72. Epub 2012 Nov 29 doi: 10.1093/cercor/bhs370. [Epub ahead of print] PMID: 23203979Free PMC Article
Riggs NR, Tate EB, Ridenour TA, Reynolds MD, Zhai ZW, Vanyukov MM, Tarter RE
J Adolesc Health 2013 Oct;53(4):465-70. Epub 2013 Jul 20 doi: 10.1016/j.jadohealth.2013.05.017. [Epub ahead of print] PMID: 23876782Free PMC Article

Recent systematic reviews

Stevens L, Verdejo-García A, Goudriaan AE, Roeyers H, Dom G, Vanderplasschen W
J Subst Abuse Treat 2014 Jul;47(1):58-72. Epub 2014 Feb 10 doi: 10.1016/j.jsat.2014.01.008. [Epub ahead of print] PMID: 24629886
Sharma L, Markon KE, Clark LA
Psychol Bull 2014 Mar;140(2):374-408. Epub 2013 Oct 7 doi: 10.1037/a0034418. [Epub ahead of print] PMID: 24099400
Di Pietro F, McAuley JH, Parkitny L, Lotze M, Wand BM, Moseley GL, Stanton TR
J Pain 2013 Nov;14(11):1270-88. Epub 2013 Sep 12 doi: 10.1016/j.jpain.2013.07.004. [Epub ahead of print] PMID: 24035350
Kennealy PJ, Skeem JL, Walters GD, Camp J
Psychol Assess 2010 Sep;22(3):569-80. doi: 10.1037/a0019618. PMID: 20822269
Shulman KI
J Affect Disord 1997 Dec;46(3):175-82. PMID: 9547115

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