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Personality changes

MedGen UID:
66817
Concept ID:
C0240735
Sign or Symptom
Synonyms: Personality change
SNOMED CT: Personality change (102943000); Change in personality (192073007)
 
HPO: HP:0000751

Definition

A noticeable change in a person's behavior and thinking. Causes include depression, drug or alcohol abuse, brain injuries, brain tumors, and Alzheimer's disease. [from NCI]

Conditions with this feature

Gerstmann-Straussler-Scheinker syndrome
MedGen UID:
4886
Concept ID:
C0017495
Disease or Syndrome
Genetic prion diseases generally manifest with cognitive difficulties, ataxia, and myoclonus (abrupt jerking movements of muscle groups and/or entire limbs). The order of appearance and/or predominance of these features and other associated neurologic and psychiatric findings vary. Familial Creutzfeldt-Jakob disease (fCJD), Gerstmann-Sträussler-Scheinker (GSS) syndrome, and fatal familial insomnia (FFI) represent the core phenotypes of genetic prion disease. Note: A fourth clinical phenotype, known as Huntington disease like-1 (HDL-1) has been proposed, but this is based on a single report, and the underlying pathologic features would categorize it as GSS. Although it is clear that these four subtypes display overlapping clinical and pathologic features, recognition of these phenotypes can be useful when providing affected individuals and their families with information about the expected clinical course. The age at onset ranges from the third to ninth decade of life. The course ranges from a few months to several years (typically 5-7 years; in rare instances, >10 years).
Wilson disease
MedGen UID:
42426
Concept ID:
C0019202
Disease or Syndrome
Wilson disease is a disorder of copper metabolism that can present with hepatic, neurologic, or psychiatric disturbances, or a combination of these, in individuals ranging from age three years to older than 50 years; symptoms vary among and within families. Liver disease includes recurrent jaundice, simple acute self-limited hepatitis-like illness, autoimmune-type hepatitis, fulminant hepatic failure, or chronic liver disease. Neurologic presentations include movement disorders (tremors, poor coordination, loss of fine-motor control, chorea, choreoathetosis) or rigid dystonia (mask-like facies, rigidity, gait disturbance, pseudobulbar involvement). Psychiatric disturbance includes depression, neurotic behaviors, disorganization of personality, and, occasionally, intellectual deterioration. Kayser-Fleischer rings, frequently present, result from copper deposition in Descemet's membrane of the cornea and reflect a high degree of copper storage in the body.
Huntington chorea
MedGen UID:
5654
Concept ID:
C0020179
Disease or Syndrome
Huntington disease (HD) is a progressive disorder of motor, cognitive, and psychiatric disturbances. The mean age of onset is 35 to 44 years and the median survival time is 15 to 18 years after onset.
Jakob-Creutzfeldt disease
MedGen UID:
7179
Concept ID:
C0022336
Disease or Syndrome
Genetic prion diseases generally manifest with cognitive difficulties, ataxia, and myoclonus (abrupt jerking movements of muscle groups and/or entire limbs). The order of appearance and/or predominance of these features and other associated neurologic and psychiatric findings vary. Familial Creutzfeldt-Jakob disease (fCJD), Gerstmann-Sträussler-Scheinker (GSS) syndrome, and fatal familial insomnia (FFI) represent the core phenotypes of genetic prion disease. Note: A fourth clinical phenotype, known as Huntington disease like-1 (HDL-1) has been proposed, but this is based on a single report, and the underlying pathologic features would categorize it as GSS. Although it is clear that these four subtypes display overlapping clinical and pathologic features, recognition of these phenotypes can be useful when providing affected individuals and their families with information about the expected clinical course. The age at onset ranges from the third to ninth decade of life. The course ranges from a few months to several years (typically 5-7 years; in rare instances, >10 years).
Pick 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.
Photomyoclonus, diabetes mellitus, deafness, nephropathy and cerebral dysfunction
MedGen UID:
315660
Concept ID:
C1809475
Disease or Syndrome
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.
Dermoid cysts, familial frontonasal
MedGen UID:
371575
Concept ID:
C1833473
Disease or Syndrome
Alzheimer disease, type 3
MedGen UID:
334304
Concept ID:
C1843013
Disease or Syndrome
Alzheimer disease (AD) is characterized by dementia that typically begins with subtle and poorly recognized failure of memory and slowly becomes more severe and, eventually, incapacitating. Other common findings include confusion, poor judgment, language disturbance, agitation, withdrawal, and hallucinations. Occasionally, seizures, Parkinsonian features, increased muscle tone, myoclonus, incontinence, and mutism occur. Death usually results from general inanition, malnutrition, and pneumonia. The typical clinical duration of the disease is eight to ten years, with a range from one to 25 years. Approximately 25% of all AD is familial (i.e., =2 persons in a family have AD) of which approximately 95% is late onset (age >60-65 years) and 5% is early onset (age <65 years).
Cataract, congenital, with mental impairment and dentate gyrus atrophy
MedGen UID:
334365
Concept ID:
C1843257
Disease or Syndrome
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 subcategorized 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.
Spongiform encephalopathy with neuropsychiatric features
MedGen UID:
339812
Concept ID:
C1847650
Disease or Syndrome
Polycystic lipomembranous osteodysplasia with sclerosing leukoencephalopathy
MedGen UID:
387795
Concept ID:
C1857316
Disease or Syndrome
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.
Leukoencephalopathy with vanishing white matter
MedGen UID:
347037
Concept ID:
C1858991
Disease or Syndrome
Childhood ataxia with central nervous system hypomyelination/vanishing white matter disease (CACH/VWM) is characterized by ataxia, spasticity, and variable optic atrophy. The phenotypic range includes a prenatal/congenital form, a subacute infantile form (onset age <1 year), an early childhood onset form (onset age 1-5 years), a late childhood /juvenile onset form (onset age 5-15 years), and an adult onset form. The prenatal/congenital form is characterized by severe encephalopathy. In the later onset forms initial motor and intellectual development is normal or mildly delayed followed by neurologic deterioration with a chronic progressive or subacute course. Chronic progressive decline can be exacerbated by rapid deterioration during febrile illnesses or following head trauma or major surgical procedures, or by acute psychological stresses such as extreme fright.
Huntington disease-like 1
MedGen UID:
355137
Concept ID:
C1864112
Disease or Syndrome
Genetic prion diseases generally manifest with cognitive difficulties, ataxia, and myoclonus (abrupt jerking movements of muscle groups and/or entire limbs). The order of appearance and/or predominance of these features and other associated neurologic and psychiatric findings vary. Familial Creutzfeldt-Jakob disease (fCJD), Gerstmann-Sträussler-Scheinker (GSS) syndrome, and fatal familial insomnia (FFI) represent the core phenotypes of genetic prion disease. Note: A fourth clinical phenotype, known as Huntington disease like-1 (HDL-1) has been proposed, but this is based on a single report, and the underlying pathologic features would categorize it as GSS. Although it is clear that these four subtypes display overlapping clinical and pathologic features, recognition of these phenotypes can be useful when providing affected individuals and their families with information about the expected clinical course. The age at onset ranges from the third to ninth decade of life. The course ranges from a few months to several years (typically 5-7 years; in rare instances, >10 years).
Alzheimer disease, type 10
MedGen UID:
351228
Concept ID:
C1864828
Disease or Syndrome
Leukodystrophy, adult-onset, autosomal dominant
MedGen UID:
356995
Concept ID:
C1868512
Disease or Syndrome
Autosomal dominant leukodystrophy with autonomic disease (ADLD) is a slowly progressive disorder of central nervous system white matter characterized by onset of autonomic dysfunction in the fourth to fifth decade, followed in months to years by pyramidal and cerebellar involvement. Autonomic dysfunction can include bladder dysfunction, constipation, postural hypotension, feeding difficulties, erectile dysfunction, and (less often) impaired sweating. Pyramidal signs are often more prominent in the lower extremities (i.e., spastic weakness, hypertonia, clonus, brisk deep tendon reflexes, and bilateral Babinski signs). Cerebellar signs typically appear at the same time as the pyramidal signs and can include gait ataxia, dysdiadochokinesia, intention tremor, dysmetria, and nystagmus. Although cognitive function is usually preserved or only mildly impaired early in the disease course, dementia and psychiatric manifestations can occur as late manifestations. Affected individuals may survive for decades after onset.
Parkinson disease 14
MedGen UID:
414488
Concept ID:
C2751842
Disease or Syndrome
Parkinsonism refers to all clinical states characterized by tremor, muscle rigidity, slowed movement (bradykinesia) and often postural instability. Parkinson disease is the primary and most common form of parkinsonism. Psychiatric manifestations, which include depression and visual hallucinations, are common but not uniformly present. Dementia eventually occurs in at least 20% of cases. The most common sporadic form of Parkinson disease manifests around age 60; however, young-onset and even juvenile presentations are seen.
Parkinson disease, late-onset
MedGen UID:
463618
Concept ID:
C3160718
Disease or Syndrome
Parkinsonism refers to all clinical states characterized by tremor, muscle rigidity, slowed movement (bradykinesia) and often postural instability. Parkinson disease is the primary and most common form of parkinsonism. Psychiatric manifestations, which include depression and visual hallucinations, are common but not uniformly present. Dementia eventually occurs in at least 20% of cases. The most common sporadic form of Parkinson disease manifests around age 60; however, young-onset and even juvenile presentations are seen.
Frontotemporal dementia and/or amyotrophic lateral sclerosis 4
MedGen UID:
902979
Concept ID:
C4225325
Disease or Syndrome
Frontotemporal dementia and/or amyotrophic lateral sclerosis-4 is an autosomal dominant neurodegenerative disorder characterized by adult or late adult onset of cognitive impairment, behavioral abnormalities, and speech apraxia and/or upper and lower motor neuron signs. The phenotype is highly variable (summary by Freischmidt et al., 2015). For a discussion of genetic heterogeneity of FTDALS, see FTDALS1 (105550).
Frontotemporal dementia and/or amyotrophic lateral sclerosis 3
MedGen UID:
897127
Concept ID:
C4225326
Disease or Syndrome
Frontotemporal dementia and/or amyotrophic lateral sclerosis-3 is an autosomal dominant neurodegenerative disorder characterized by adult or late adult onset of cognitive impairment, behavioral abnormalities, and speech apraxia and/or upper and lower motor neuron signs. Some patients may also develop Paget disease of bone. The phenotype is highly variable, even within families (summary by Rea et al., 2014). For a discussion of genetic heterogeneity of FTDALS, see FTDALS1 (105550).

Recent clinical studies

Etiology

Tautvydaitė D, Antonietti JP, Henry H, von Gunten A, Popp J
J Psychiatr Res 2017 Jul;90:12-20. Epub 2017 Feb 4 doi: 10.1016/j.jpsychires.2016.12.024. PMID: 28213293
Leonhardt A, Schmukle SC, Exner C
J Psychosom Res 2016 Mar;82:17-23. Epub 2016 Jan 15 doi: 10.1016/j.jpsychores.2016.01.005. PMID: 26944394
Jenkins LM, Drummond KJ, Andrewes DG
J Clin Neurosci 2016 Jul;29:128-32. Epub 2016 Feb 18 doi: 10.1016/j.jocn.2015.12.007. PMID: 26898575
Diaz AP, Schwarzbold ML, Thais ME, Cavallazzi GG, Schmoeller R, Nunes JC, Hohl A, Guarnieri R, Linhares MN, Walz R
Braz J Psychiatr 2014 Sep;36(3):213-9. Epub 2014 Apr 25 PMID: 24770655
Torrente F, Pose M, Gleichgerrcht E, Torralva T, López P, Cetkovich-Bakmas M, Manes F
Alzheimer Dis Assoc Disord 2014 Jul-Sep;28(3):261-8. doi: 10.1097/WAD.0000000000000030. PMID: 24614269

Diagnosis

López Chiriboga AS, Konno T, van Gerpen JA
JAMA Neurol 2017 Feb 1;74(2):245-246. doi: 10.1001/jamaneurol.2016.2782. PMID: 27992633
Diaz AP, Schwarzbold ML, Thais ME, Cavallazzi GG, Schmoeller R, Nunes JC, Hohl A, Guarnieri R, Linhares MN, Walz R
Braz J Psychiatr 2014 Sep;36(3):213-9. Epub 2014 Apr 25 PMID: 24770655
Torrente F, Pose M, Gleichgerrcht E, Torralva T, López P, Cetkovich-Bakmas M, Manes F
Alzheimer Dis Assoc Disord 2014 Jul-Sep;28(3):261-8. doi: 10.1097/WAD.0000000000000030. PMID: 24614269
Zhao HK, Chang CW, Geng N, Gao L, Wang J, Wang X, Wang YR, Wang XL, Gao GD
Acta Pharmacol Sin 2012 May;33(5):588-93. Epub 2012 Apr 16 doi: 10.1038/aps.2012.10. PMID: 22504903Free PMC Article
Pocnet C, Rossier J, Antonietti JP, von Gunten A
Can J Psychiatry 2011 Jul;56(7):408-17. doi: 10.1177/070674371105600704. PMID: 21835104

Therapy

Pačić-Turk L, Šulentić T, Havelka Meštrović A, Paladino J, Mrak G
Acta Clin Croat 2016 Dec;55(4):565-578. doi: 10.20471/acc.2016.55.04.06. PMID: 29117647
Dembinska-Krajewska D, Rybakowski JK
Pharmacol Rep 2015 Dec;67(6):1204-7. Epub 2015 May 28 doi: 10.1016/j.pharep.2015.05.006. PMID: 26481543
Diaz AP, Schwarzbold ML, Thais ME, Cavallazzi GG, Schmoeller R, Nunes JC, Hohl A, Guarnieri R, Linhares MN, Walz R
Braz J Psychiatr 2014 Sep;36(3):213-9. Epub 2014 Apr 25 PMID: 24770655
Serata D, Rapinesi C, Del Casale A, Simonetti A, Mazzarini L, Ambrosi E, Kotzalidis GD, Fensore C, Girardi P, Tatarelli R
Int J Neurosci 2011 Mar;121(3):165-9. Epub 2010 Dec 2 doi: 10.3109/00207454.2010.537412. PMID: 21126108
Presecki P, Mihanović M, Silić A, Vuina AL, Caratan S
Psychiatr Danub 2010 Jun;22(2):360-2. PMID: 20562783

Prognosis

Tautvydaitė D, Antonietti JP, Henry H, von Gunten A, Popp J
J Psychiatr Res 2017 Jul;90:12-20. Epub 2017 Feb 4 doi: 10.1016/j.jpsychires.2016.12.024. PMID: 28213293
Pačić-Turk L, Šulentić T, Havelka Meštrović A, Paladino J, Mrak G
Acta Clin Croat 2016 Dec;55(4):565-578. doi: 10.20471/acc.2016.55.04.06. PMID: 29117647
Miller BL, Dickerson BC, Lucente DE, Larvie M, Frosch MP
N Engl J Med 2015 Mar 19;372(12):1151-62. doi: 10.1056/NEJMcpc1409839. PMID: 25785973Free PMC Article
Diaz AP, Schwarzbold ML, Thais ME, Cavallazzi GG, Schmoeller R, Nunes JC, Hohl A, Guarnieri R, Linhares MN, Walz R
Braz J Psychiatr 2014 Sep;36(3):213-9. Epub 2014 Apr 25 PMID: 24770655
Lykou E, Rankin KP, Chatziantoniou L, Boulas C, Papatriantafyllou O, Tsaousis I, Neuhaus J, Karageorgiou C, Miller BL, Papatriantafyllou JD
Alzheimer Dis Assoc Disord 2013 Jul-Sep;27(3):258-64. doi: 10.1097/WAD.0b013e31826e5504. PMID: 23060360Free PMC Article

Clinical prediction guides

Tautvydaitė D, Antonietti JP, Henry H, von Gunten A, Popp J
J Psychiatr Res 2017 Jul;90:12-20. Epub 2017 Feb 4 doi: 10.1016/j.jpsychires.2016.12.024. PMID: 28213293
Leonhardt A, Schmukle SC, Exner C
J Psychosom Res 2016 Mar;82:17-23. Epub 2016 Jan 15 doi: 10.1016/j.jpsychores.2016.01.005. PMID: 26944394
Jenkins LM, Drummond KJ, Andrewes DG
J Clin Neurosci 2016 Jul;29:128-32. Epub 2016 Feb 18 doi: 10.1016/j.jocn.2015.12.007. PMID: 26898575
Diaz AP, Schwarzbold ML, Thais ME, Cavallazzi GG, Schmoeller R, Nunes JC, Hohl A, Guarnieri R, Linhares MN, Walz R
Braz J Psychiatr 2014 Sep;36(3):213-9. Epub 2014 Apr 25 PMID: 24770655
Torrente F, Pose M, Gleichgerrcht E, Torralva T, López P, Cetkovich-Bakmas M, Manes F
Alzheimer Dis Assoc Disord 2014 Jul-Sep;28(3):261-8. doi: 10.1097/WAD.0000000000000030. PMID: 24614269

Recent systematic reviews

Kirkland SW, Cross E, Campbell S, Villa-Roel C, Rowe BH
Cochrane Database Syst Rev 2018 Jun 2;6:CD012629. doi: 10.1002/14651858.CD012629.pub2. PMID: 29859017
Gold JA, Sher Y, Maldonado JR
Psychosomatics 2016 Sep-Oct;57(5):445-64. Epub 2016 May 16 doi: 10.1016/j.psym.2016.05.005. PMID: 27494984
Millenaar JK, Bakker C, Koopmans RT, Verhey FR, Kurz A, de Vugt ME
Int J Geriatr Psychiatry 2016 Dec;31(12):1261-1276. Epub 2016 Jun 6 doi: 10.1002/gps.4502. PMID: 27271788
Shawcross DL, Dunk AA, Jalan R, Kircheis G, de Knegt RJ, Laleman W, Ramage JK, Wedemeyer H, Morgan IE; New Insights Steering Committee.
Eur J Gastroenterol Hepatol 2016 Feb;28(2):146-52. doi: 10.1097/MEG.0000000000000529. PMID: 26600154Free PMC Article
Robins Wahlin TB, Byrne GJ
Int J Geriatr Psychiatry 2011 Oct;26(10):1019-29. Epub 2010 Nov 9 doi: 10.1002/gps.2655. PMID: 21905097

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