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Personality disorder

MedGen UID:
506663
Concept ID:
CN167801
Finding
 
HPO: HP:0012075

Definition

An abnormality of mental functioning affecting the personality and behavioural tendencies of an individual and characterized by a rigid and unhealthy pattern of thinking and behavior. The definition of a personal disorder implies that the abnormality is not the result of damage or insult to the brain or from another psychiatric disorder. [from HPO]

Conditions with this feature

Homocystinuria due to CBS deficiency
MedGen UID:
461694
Concept ID:
C3150344
Disease or Syndrome
Homocystinuria caused by cystathionine ß-synthase (CBS) deficiency is characterized by developmental delay/intellectual disability, ectopia lentis and/or severe myopia, skeletal abnormalities (excessive height and length of the limbs), and thromboembolism. Expressivity is variable for all of the clinical signs. Two phenotypic variants are recognized, B6-responsive homocystinuria and B6-non-responsive homocystinuria. B6-responsive homocystinuria is typically, but not always, milder than the non-responsive variant. In the majority of untreated affected individuals, ectopia lentis occurs by age eight years. Individuals are often tall and slender with an asthenic (‘marfanoid’) habitus and are prone to osteoporosis. Thromboembolism is the major cause of early death and morbidity. IQ in individuals with untreated homocystinuria ranges widely, from 10 to 138. In B6-responsive individuals the mean IQ is 79 versus 57 for those who are B6 non-responsive. Other features that may occur include: seizures, psychiatric problems, extrapyramidal signs (e.g., dystonia), hypopigmentation, malar flush, livedo reticularis, and pancreatitis.
Epilepsy, nocturnal frontal lobe, 5
MedGen UID:
767220
Concept ID:
C3554306
Disease or Syndrome
Nocturnal frontal lobe epilepsy-5 is an autosomal dominant focal epilepsy syndrome characterized by childhood onset of clusters of motor seizures during sleep. Some patients may develop behavioral or psychiatric manifestations and/or intellectual disability. The phenotype is more severe than observed in other genetic forms of ENFL (summary by Heron et al., 2012). For a general description and a discussion of genetic heterogeneity of ENFL, see ENFL1 (600513).

Recent clinical studies

Etiology

Atmaca M, Karakoc T, Mermi O, Kilic F, Gurok MG, Yildirim H
Int J Psychiatry Med 2015;49(4):237-44. Epub 2015 Jun 9 doi: 10.1177/0091217415589286. [Epub ahead of print] PMID: 26060257
Paton C, Crawford MJ, Bhatti SF, Patel MX, Barnes TR
J Clin Psychiatry 2015 Apr;76(4):e512-8. doi: 10.4088/JCP.14m09228. PMID: 25919844
Fulford D, Eisner LR, Johnson SL
Psychiatry Res 2015 Jun 30;227(2-3):347-52. Epub 2015 Feb 7 doi: 10.1016/j.psychres.2015.02.001. [Epub ahead of print] PMID: 25892256
Ryder AG, Sunohara M, Kirmayer LJ
Curr Opin Psychiatry 2015 Jan;28(1):40-5. doi: 10.1097/YCO.0000000000000120. PMID: 25415498
Hoertel N, Peyre H, Wall MM, Limosin F, Blanco C
J Psychiatr Res 2014 Dec;59:213-9. Epub 2014 Sep 11 doi: 10.1016/j.jpsychires.2014.08.019. [Epub ahead of print] PMID: 25258339

Diagnosis

Paton C, Crawford MJ, Bhatti SF, Patel MX, Barnes TR
J Clin Psychiatry 2015 Apr;76(4):e512-8. doi: 10.4088/JCP.14m09228. PMID: 25919844
Fulford D, Eisner LR, Johnson SL
Psychiatry Res 2015 Jun 30;227(2-3):347-52. Epub 2015 Feb 7 doi: 10.1016/j.psychres.2015.02.001. [Epub ahead of print] PMID: 25892256
Atmaca M, Karakoc T, Mermi O, Gurkan Gurok M, Yildirim H
Int J Psychiatry Med 2015;48(4):317-24. doi: 10.2190/PM.48.4.f. PMID: 25817526
Ryder AG, Sunohara M, Kirmayer LJ
Curr Opin Psychiatry 2015 Jan;28(1):40-5. doi: 10.1097/YCO.0000000000000120. PMID: 25415498
Dubovsky AN, Kiefer MM
Med Clin North Am 2014 Sep;98(5):1049-64. doi: 10.1016/j.mcna.2014.06.005. PMID: 25134872

Therapy

Paton C, Crawford MJ, Bhatti SF, Patel MX, Barnes TR
J Clin Psychiatry 2015 Apr;76(4):e512-8. doi: 10.4088/JCP.14m09228. PMID: 25919844
Bateman AW, Gunderson J, Mulder R
Lancet 2015 Feb 21;385(9969):735-43. Epub 2015 Feb 20 doi: 10.1016/S0140-6736(14)61394-5. [Epub ahead of print] PMID: 25706219
Dubovsky AN, Kiefer MM
Med Clin North Am 2014 Sep;98(5):1049-64. doi: 10.1016/j.mcna.2014.06.005. PMID: 25134872
Black DW, Zanarini MC, Romine A, Shaw M, Allen J, Schulz SC
Am J Psychiatry 2014 Nov 1;171(11):1174-82. doi: 10.1176/appi.ajp.2014.13101348. PMID: 24968985
Laurenssen EM, Westra D, Kikkert MJ, Noom MJ, Eeren HV, van Broekhuyzen AJ, Peen J, Luyten P, Busschbach JJ, Dekker JJ
BMC Psychiatry 2014 May 22;14:149. doi: 10.1186/1471-244X-14-149. [Epub ahead of print] PMID: 24886402Free PMC Article

Prognosis

Schienle A, Leutgeb V, Wabnegger A
Psychiatry Res 2015 Jun 30;232(3):203-7. Epub 2015 Apr 17 doi: 10.1016/j.pscychresns.2015.04.002. [Epub ahead of print] PMID: 25937342
Tyrer P, Reed GM, Crawford MJ
Lancet 2015 Feb 21;385(9969):717-26. Epub 2015 Feb 20 doi: 10.1016/S0140-6736(14)61995-4. [Epub ahead of print] PMID: 25706217
Loas G, Baelde O, Verrier A
Psychiatry Res 2015 Feb 28;225(3):484-8. Epub 2014 Dec 9 doi: 10.1016/j.psychres.2014.11.062. [Epub ahead of print] PMID: 25529260
Sharp C, Kalpakci A, Mellick W, Venta A, Temple JR
Eur Child Adolesc Psychiatry 2015 Mar;24(3):283-90. Epub 2014 Jun 24 doi: 10.1007/s00787-014-0574-3. [Epub ahead of print] PMID: 24958159
Yang M, Mamy J, Zhou L, Liao YH, Wang Q, Seewoobudul V, Xiao SY, Hao W
Addict Behav 2014 Mar;39(3):573-9. Epub 2013 Nov 10 doi: 10.1016/j.addbeh.2013.11.003. [Epub ahead of print] PMID: 24342175

Clinical prediction guides

Schienle A, Leutgeb V, Wabnegger A
Psychiatry Res 2015 Jun 30;232(3):203-7. Epub 2015 Apr 17 doi: 10.1016/j.pscychresns.2015.04.002. [Epub ahead of print] PMID: 25937342
Loas G, Baelde O, Verrier A
Psychiatry Res 2015 Feb 28;225(3):484-8. Epub 2014 Dec 9 doi: 10.1016/j.psychres.2014.11.062. [Epub ahead of print] PMID: 25529260
Deckers JW, Lobbestael J, van Wingen GA, Kessels RP, Arntz A, Egger JI
Psychoneuroendocrinology 2015 Feb;52:119-29. Epub 2014 Nov 11 doi: 10.1016/j.psyneuen.2014.11.003. [Epub ahead of print] PMID: 25459898
Thaler L, Gauvin L, Joober R, Groleau P, de Guzman R, Ambalavanan A, Israel M, Wilson S, Steiger H
Prog Neuropsychopharmacol Biol Psychiatry 2014 Oct 3;54:43-9. Epub 2014 May 5 doi: 10.1016/j.pnpbp.2014.04.010. [Epub ahead of print] PMID: 24801751
Yang M, Mamy J, Zhou L, Liao YH, Wang Q, Seewoobudul V, Xiao SY, Hao W
Addict Behav 2014 Mar;39(3):573-9. Epub 2013 Nov 10 doi: 10.1016/j.addbeh.2013.11.003. [Epub ahead of print] PMID: 24342175

Recent systematic reviews

Bech M, Elklit A, Simonsen E
Personal Ment Health 2015 May;9(2):162-71. doi: 10.1002/pmh.1294. PMID: 25940516
van Zutphen L, Siep N, Jacob GA, Goebel R, Arntz A
Neurosci Biobehav Rev 2015 Apr;51:64-76. Epub 2015 Jan 20 doi: 10.1016/j.neubiorev.2015.01.001. [Epub ahead of print] PMID: 25616185
Hoertel N, López S, Wang S, González-Pinto A, Limosin F, Blanco C
Personal Disord 2015 Jan;6(1):81-7. doi: 10.1037/per0000091. PMID: 25580674Free PMC Article
Frías Á, Palma C
Psychopathology 2015;48(1):1-10. Epub 2014 Sep 9 doi: 10.1159/000363145. [Epub ahead of print] PMID: 25227722
Kienast T, Stoffers J, Bermpohl F, Lieb K
Dtsch Arztebl Int 2014 Apr 18;111(16):280-6. doi: 10.3238/arztebl.2014.0280. PMID: 24791755Free PMC Article

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