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Delusions

MedGen UID:
504582
Concept ID:
CN000702
Finding
 
HPO: HP:0000746

Definition

A belief that is pathological and is held despite evidence to the contrary. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVDelusions

Conditions with this feature

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).
Metachromatic leukodystrophy
MedGen UID:
6071
Concept ID:
C0023522
Disease or Syndrome
Arylsulfatase A deficiency (also known as metachromatic leukodystrophy or MLD) is characterized by three clinical subtypes: late-infantile MLD (50%-60% of cases); juvenile MLD (20%-30% of cases); and adult MLD (15%-20% of cases). Age of onset within a family is usually similar. The disease course may be from three to ten or more years in the late-infantile form and up to 20 years or more in the juvenile and adult forms. Late-infantile MLD. Onset is between ages one and two years. Typical presenting findings include weakness, hypotonia, clumsiness, frequent falls, toe walking, and slurred speech. Later signs include inability to stand, difficulty with speech, deterioration of mental function, increased muscle tone, pain in the arms and legs, generalized or partial seizures, compromised vision and hearing, and peripheral neuropathy. In the final stages children have tonic spasms, decerebrate posturing, and general unawareness of their surroundings. Juvenile MLD. Onset is between age four years and sexual maturity (age 12-14 years). Initial manifestations include decline in school performance and emergence of behavioral problems, followed by clumsiness, gait problems, slurred speech, incontinence, and bizarre behaviors. Seizures may occur. Progression is similar to but slower than the late-infantile form. Adult MLD. Onset occurs after sexual maturity, sometimes not until the fourth or fifth decade. Initial signs can include problems in school or job performance, personality changes, alcohol or drug abuse, poor money management, and emotional lability; in others, neurologic symptoms (weakness and loss of coordination progressing to spasticity and incontinence) or seizures predominate initially. Peripheral neuropathy is common. Disease course is variable, with periods of stability interspersed with periods of decline, and may extend over two to three decades. The final stage is similar to that for the earlier-onset forms.
Schizophrenia
MedGen UID:
48574
Concept ID:
C0036341
Mental or Behavioral Dysfunction
Schizophrenia is a psychosis, a disorder of thought and sense of self. Although it affects emotions, it is distinguished from mood disorders in which such disturbances are primary. Similarly, there may be mild impairment of cognitive function, and it is distinguished from the dementias in which disturbed cognitive function is considered primary. There is no characteristic pathology, such as neurofibrillary tangles in Alzheimer disease (104300). Schizophrenia is a common disorder with a lifetime prevalence of approximately 1%. It is highly heritable but the genetics are complex. This may not be a single entity. Reviews In a review of schizophrenia, van Os and Kapur (2009) noted that in Japan the term schizophrenia was abandoned and the illness is now called integration-dysregulation syndrome.
Schizophrenia 1
MedGen UID:
65084
Concept ID:
C0220702
Disease or Syndrome
Cholestanol storage disease
MedGen UID:
116041
Concept ID:
C0238052
Disease or Syndrome
Cerebrotendinous xanthomatosis (CTX) is a lipid storage disease characterized by infantile-onset diarrhea, childhood-onset cataract, adolescent- to young adult-onset tendon xanthomas, and adult-onset progressive neurologic dysfunction (dementia, psychiatric disturbances, pyramidal and/or cerebellar signs, dystonia, atypical parkinsonism, peripheral neuropathy, and seizures). Chronic diarrhea from infancy may be the earliest clinical manifestation. In approximately 75% of affected individuals, cataracts are the first finding, often appearing in the first decade of life. Xanthomas appear in the second or third decade; they occur on the Achilles tendon, the extensor tendons of the elbow and hand, the patellar tendon, and the neck tendons. Xanthomas have been reported in the lung, bones, and central nervous system. Some individuals show cognitive impairment from early infancy, whereas the majority have normal or only slightly impaired intellectual function until puberty; dementia with slow deterioration in intellectual abilities occurs in the 20s in more than 50% of individuals. Neuropsychiatric symptoms such as behavioral changes, hallucinations, agitation, aggression, depression, and suicide attempts may be prominent. Pyramidal signs (i.e., spasticity) and/or cerebellar signs almost invariably become evident between ages 20 and 30 years. The biochemical abnormalities that distinguish CTX from other conditions with xanthomas include high plasma and tissue cholestanol concentration, normal-to-low plasma cholesterol concentration, decreased chenodeoxycholic acid, increased concentration of bile alcohols and their glyconjugates, and increased concentrations of cholestanol and apolipoprotein B in cerebrospinal fluid.
Schizophrenia 10
MedGen UID:
107776
Concept ID:
C0543918
Mental or Behavioral Dysfunction
Lewy body dementia
MedGen UID:
199874
Concept ID:
C0752347
Disease or Syndrome
Dementia with Lewy bodies (DLB) is a neurodegenerative disorder clinically characterized by dementia and parkinsonism, often with fluctuating cognitive function, visual hallucinations, falls, syncopal episodes, and sensitivity to neuroleptic medication. Pathologically, Lewy bodies are present in a pattern more widespread than usually observed in Parkinson disease (see PD; 168600). Alzheimer disease (AD; 104300)-associated pathology and spongiform changes may also be seen (McKeith et al., 1996; Mizutani, 2000; McKeith et al., 2005).
Huntington disease-like 2
MedGen UID:
341120
Concept ID:
C1847987
Disease or Syndrome
Huntington disease-like 2 (HDL2) typically presents in midlife with a relentless progressive triad of movement, emotional, and cognitive abnormalities progressing to death over ten to 20 years. In some individuals the presentation resembles juvenile-onset Huntington disease (HD) or the Westphal variant of HD, usually presenting in the fourth decade (ages 29 to 41 years) with diminished coordination and weight loss despite increase in food intake. Neurologic abnormalities include parkinsonism (rigidity, bradykinesia, tremor), dysarthria, and hyperreflexia. In others the presentation is more variable but, in general, corresponds to typical HD.
Spinocerebellar ataxia 12
MedGen UID:
347653
Concept ID:
C1858501
Disease or Syndrome
Spinocerebellar ataxia type 12 (SCA12) is characterized by onset of action tremor of the upper extremities in the fourth decade, slowly progressing to include ataxia and other cerebellar and cortical signs. Given the small number of individuals known to have SCA12, it is possible that other clinical manifestations have not yet been recognized.
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.
Frontotemporal dementia and/or amyotrophic lateral sclerosis
MedGen UID:
350795
Concept ID:
C1862937
Disease or Syndrome
Frontotemporal dementia (FTD) and/or amyotrophic lateral sclerosis (ALS) is an autosomal dominant neurodegenerative disorder characterized by adult onset of one or both of these features in an affected individual, with significant intrafamilial variation. The disorder is genetically and pathologically heterogeneous (summary by Vance et al., 2006). Patients with C9ORF72 repeat expansions tend to show a lower age of onset, shorter survival, bulbar symptom onset, increased incidence of neurodegenerative disease in relatives, and a propensity toward psychosis or hallucinations compared to patients with other forms of ALS and/or FTD (summary by Harms et al., 2013). Patients with C9ORF72 repeat expansions also show psychiatric disturbances that may predate the onset of dementia (Meisler et al., 2013; Gomez-Tortosa et al., 2013). For a general phenotypic description of frontotemporal dementia, also known as frontotemporal lobar degeneration (FTLD), see 600274. For a general discussion of motor neuron disease (MND), see amyotrophic lateral sclerosis-1 (ALS1; 105400). Genetic Heterogeneity of Frontotemporal Dementia and/or Amyotrophic Lateral Sclerosis See also FTDALS2 (615911), caused by mutation in the CHCHD10 gene (615903) on chromosome 22q11; FTDALS3 (616437), caused by mutation in the SQSTM1 gene (601530) on chromosome 5q35; and FTDALS4 (616439), caused by mutation in the TBK1 gene (604834) on chromosome 12q14.
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).

Recent clinical studies

Etiology

Perez DL, Pan H, Weisholtz DS, Root JC, Tuescher O, Fischer DB, Butler T, Vago DR, Isenberg N, Epstein J, Landa Y, Smith TE, Savitz AJ, Silbersweig DA, Stern E
Psychiatry Res 2015 Sep 30;233(3):352-66. Epub 2015 Jun 23 doi: 10.1016/j.pscychresns.2015.06.002. [Epub ahead of print] PMID: 26208746Free PMC Article
Boyden P, Knowles R, Corcoran R, Hamilton S, Rowse G
Cogn Neuropsychiatry 2015;20(2):109-21. Epub 2014 Nov 11 doi: 10.1080/13546805.2014.974802. [Epub ahead of print] PMID: 25384453
Whitford TJ, Kubicki M, Pelavin PE, Lucia D, Schneiderman JS, Pantelis C, McCarley RW, Shenton ME
Schizophr Res 2015 Jan;161(1):36-41. Epub 2014 Oct 11 doi: 10.1016/j.schres.2014.08.033. [Epub ahead of print] PMID: 25311780Free PMC Article
Factor SA, Scullin MK, Sollinger AB, Land JO, Wood-Siverio C, Zanders L, Freeman A, Bliwise DL, McDonald WM, Goldstein FC
J Neurol Sci 2014 Dec 15;347(1-2):316-21. Epub 2014 Oct 23 doi: 10.1016/j.jns.2014.10.033. [Epub ahead of print] PMID: 25466695
Freeman D, Lister R, Evans N
J Behav Ther Exp Psychiatry 2014 Dec;45(4):454-8. Epub 2014 Jun 21 doi: 10.1016/j.jbtep.2014.06.005. [Epub ahead of print] PMID: 25000504

Diagnosis

Kimhy D
Am J Psychother 2016;70(1):117-23. PMID: 27052609
Goto A, Miyawaki D, Kusaka H, Okada Y, Asada N, Iwakura Y, Yanagihara E, Inoue K
Osaka City Med J 2015 Dec;61(2):73-80. PMID: 26995851
Perez DL, Pan H, Weisholtz DS, Root JC, Tuescher O, Fischer DB, Butler T, Vago DR, Isenberg N, Epstein J, Landa Y, Smith TE, Savitz AJ, Silbersweig DA, Stern E
Psychiatry Res 2015 Sep 30;233(3):352-66. Epub 2015 Jun 23 doi: 10.1016/j.pscychresns.2015.06.002. [Epub ahead of print] PMID: 26208746Free PMC Article
Sanati A, Kyratsous M
J Eval Clin Pract 2015 Jun;21(3):479-85. Epub 2015 Mar 31 doi: 10.1111/jep.12347. [Epub ahead of print] PMID: 25828924
Wade DM, Brewin CR, Howell DC, White E, Mythen MG, Weinman JA
Br J Health Psychol 2015 Sep;20(3):613-31. Epub 2014 Jun 18 doi: 10.1111/bjhp.12109. [Epub ahead of print] PMID: 24944013

Therapy

Dodds PR, Dodds TJ, Jacoby TL
Conn Med 2016 Mar;80(3):159-61. PMID: 27169299
Kimhy D
Am J Psychother 2016;70(1):117-23. PMID: 27052609
Gerretsen P, Flint AJ, Whyte EM, Rothschild AJ, Meyers BS, Mulsant BH
J Clin Psychiatry 2015 Apr;76(4):427-33. doi: 10.4088/JCP.14m09003. PMID: 25919834
Freeman D, Emsley R, Dunn G, Fowler D, Bebbington P, Kuipers E, Jolley S, Waller H, Hardy A, Garety P
Schizophr Bull 2015 Jul;41(4):971-9. Epub 2014 Dec 20 doi: 10.1093/schbul/sbu173. [Epub ahead of print] PMID: 25528759Free PMC Article
Freeman D, Pugh K, Dunn G, Evans N, Sheaves B, Waite F, Cernis E, Lister R, Fowler D
Schizophr Res 2014 Dec;160(1-3):186-92. Epub 2014 Nov 11 doi: 10.1016/j.schres.2014.10.038. [Epub ahead of print] PMID: 25468186Free PMC Article

Prognosis

Gawęda Ł, Prochwicz K
Eur Psychiatry 2015 Nov;30(8):943-9. Epub 2015 Oct 13 doi: 10.1016/j.eurpsy.2015.08.003. [Epub ahead of print] PMID: 26647870
Gerretsen P, Flint AJ, Whyte EM, Rothschild AJ, Meyers BS, Mulsant BH
J Clin Psychiatry 2015 Apr;76(4):427-33. doi: 10.4088/JCP.14m09003. PMID: 25919834
Ting WK, Fischer CE, Millikin CP, Ismail Z, Chow TW, Schweizer TA
Curr Alzheimer Res 2015;12(2):165-72. PMID: 25654501
Tagawa R, Hashimoto H, Matsuda Y, Uchida K, Yoshida A, Higashiyama S, Kawabe J, Toshihiro K, Shiomi S, Mori H, Inoue K
Osaka City Med J 2014 Dec;60(2):73-80. PMID: 25803882
Mehl S, Landsberg MW, Schmidt AC, Cabanis M, Bechdolf A, Herrlich J, Loos-Jankowiak S, Kircher T, Kiszkenow S, Klingberg S, Kommescher M, Moritz S, Müller BW, Sartory G, Wiedemann G, Wittorf A, Wölwer W, Wagner M
Schizophr Bull 2014 Nov;40(6):1338-46. Epub 2014 Apr 17 doi: 10.1093/schbul/sbu040. [Epub ahead of print] PMID: 24743864Free PMC Article

Clinical prediction guides

Goto A, Miyawaki D, Kusaka H, Okada Y, Asada N, Iwakura Y, Yanagihara E, Inoue K
Osaka City Med J 2015 Dec;61(2):73-80. PMID: 26995851
Gawęda Ł, Prochwicz K
Eur Psychiatry 2015 Nov;30(8):943-9. Epub 2015 Oct 13 doi: 10.1016/j.eurpsy.2015.08.003. [Epub ahead of print] PMID: 26647870
Perez DL, Pan H, Weisholtz DS, Root JC, Tuescher O, Fischer DB, Butler T, Vago DR, Isenberg N, Epstein J, Landa Y, Smith TE, Savitz AJ, Silbersweig DA, Stern E
Psychiatry Res 2015 Sep 30;233(3):352-66. Epub 2015 Jun 23 doi: 10.1016/j.pscychresns.2015.06.002. [Epub ahead of print] PMID: 26208746Free PMC Article
Gerretsen P, Flint AJ, Whyte EM, Rothschild AJ, Meyers BS, Mulsant BH
J Clin Psychiatry 2015 Apr;76(4):427-33. doi: 10.4088/JCP.14m09003. PMID: 25919834
Whitford TJ, Kubicki M, Pelavin PE, Lucia D, Schneiderman JS, Pantelis C, McCarley RW, Shenton ME
Schizophr Res 2015 Jan;161(1):36-41. Epub 2014 Oct 11 doi: 10.1016/j.schres.2014.08.033. [Epub ahead of print] PMID: 25311780Free PMC Article

Recent systematic reviews

Reeve S, Sheaves B, Freeman D
Clin Psychol Rev 2015 Dec;42:96-115. Epub 2015 Sep 9 doi: 10.1016/j.cpr.2015.09.001. [Epub ahead of print] PMID: 26407540Free PMC Article
Cook CC
Int J Soc Psychiatry 2015 Jun;61(4):404-25. Epub 2015 Mar 12 doi: 10.1177/0020764015573089. [Epub ahead of print] PMID: 25770205Free PMC Article
Taylor P, Hutton P, Dudley R
Syst Rev 2014 May 8;3:44. doi: 10.1186/2046-4053-3-44. [Epub ahead of print] PMID: 24887076Free PMC Article
Garety PA, Freeman D
Br J Psychiatry 2013 Nov;203(5):327-33. doi: 10.1192/bjp.bp.113.126953. PMID: 24187067
Kesting ML, Lincoln TM
Compr Psychiatry 2013 Oct;54(7):766-89. Epub 2013 May 17 doi: 10.1016/j.comppsych.2013.03.002. [Epub ahead of print] PMID: 23684547

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