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Huntington Hastalığı’nın Nöropsikiyatrik Yönü

Yıl 2022, Cilt: 14 Sayı: 1, 98 - 112, 31.03.2022
https://doi.org/10.18863/pgy.952664

Öz

Huntington Hastalığı; kore ve distoni, koordinasyon bozukluğu, bilişsel performansta bozulmalar ve davranışsal sorunlar gibi farklı
fenotipler ile ortaya çıkabilen, genetik olarak otozomal dominant geçiş özelliğine sahip, ilerleyici tipte bir nörodejeneratif hastalıktır. Bilişsel
ve motor belirtilerin yanı sıra nöropsikiyatrik belirtiler de hastalığın çekirdek belirtileri arasında yer almaktadır. Huntington Hastalığı’nda
nöropsikiyatrik belirtiler oldukça sık görülmekte ve hastalığın farklı dönemlerinde psikiyatrik bozuklukların görülme prevelansı %33-76 olarak
tahmin edilmektedir. Nöropsikiyatrik belirtilerin görülme sıklığı hastalığın evrelerine göre farklılık gösterse de başlangıcının motor belirtiler
başlamadan yıllar önce olabileceği de bilinmektedir. Huntington Hastalığı’nda sık görülen nöropsikiyatrik belirti ve bozukluklar depresyon,
anksiyete, intihar, irritabilite, apati, obsesif-kompulsif belirtiler, perseverasyonlar, psikoz, uyku bozuklukları ve cinsel işlev bozuklukları olarak
sayılabilir. Nöropsikiyatrik belirtiler aileler üzerindeki yükün en önemli nedenlerinden birini oluşturmakta, günlük işlevsellikteki azalma ile
bakım kurumlarına yerleştirilme ve hastaneye yatışların en önemli öngörücüsü olarak değerlendirilmektedir. Hem sıklığı hem de sonuçları
göz önüne alındığında Huntington Hastalığı’ndaki nöropsikiyatrik belirtilerin tanınması ve bu belirtilere müdahale edilmesi hastalar, hasta
yakınları ve bakımverenleri için oldukça önemlidir. Hastalıkta görülen nöropsikiyatrik belirtilerin tedavisi ile ilgili yüksek kanıt düzeyine
sahip araştırmalar olmasa da daha düşük kanıt düzeyine sahip çalışmalar, vaka bildirimleri ve uzman görüşlerine dayalı tedavi kılavuzları
son yıllarda yazında kendine yer bulmuştur. Bu alanda dikkat edilmesi gereken başka bir konu da risk altındaki bireylerin değerlendirilmesi,
genetik danışmanlık ve bu değerlendirmeler sırasında güvenli bir protokolün oluşturulmasıdır. Bu yazıda Huntington Hastalığı’nda sık görülen
nöropsikiyatrik bozukluklar, bu bozuklukların tedavisi ve risk altındaki bireyleri değerlendirmede dikkat edilmesi gereken durumlar güncel
yazın ışığında derlenmiştir.

Kaynakça

  • A’Campo LEI, Spliethoff-Kamminga NGA, Roos RAC (2012) The Patient Education Program for Huntington’s Disease (PEP-HD). J Huntingtons Dis, 1:47-56.
  • Adrissi J, Nadkarni NA, Gausche E, Bega D (2019) Electroconvulsive therapy (ECT) for refractory psychiatric symptoms in Huntington’s disease: a case series and review of the literature. J Huntingtons Dis, 8:291-300.
  • Anderson KE (2005) Huntington’s disease, and related disorders. Psychiatr Clin North Am, 28:275-290.
  • Anderson KE, van Duijn E, Craufurd D, Drazinic C, Edmondson M, Goodman N et al. (2018). Clinical management of neuropsychiatric symptoms of huntington disease: expert-based consensus guidelines on agitation, anxiety, apathy, psychosis and sleep disorders. J Huntingtons Dis, 7:355-366.
  • Anderson KE, Eberly S, Groves M, Kayson E, Marder K, Young AB et al. (2016) Risk factors for suicidal ideation in people at risk for Huntington’s disease. J Huntingtons Dis, 5:389-394.
  • Arran N, Craufurd D, Simpson J (2014) Illness perceptions, coping styles and psychological distress in adults with Huntington’s disease. Psychol Health Med, 19:169-179.
  • Ataöv G, Akbaş I, Gül O, Balaban OD, Tanrıverdi Seda (2020) Psikiyatri servisinde Huntington Hastalığı vakası. 5. Psikiyatri Zirvesi ve 12. Anksiyete Kongresi, 29-31 Ekim Antalya, Türkiye. Kongre Özet Kitabı pp: 272.
  • Bachoud-Lévi AC, Ferreira J, Massart R, Youssov K, Rosser A, Busse M et al. (2019). International guidelines for the treatment of Huntington's disease. Front Neurol, 10:710.
  • Baker CR, Domínguez D JF, Stout JC, Gabery S, Churchyard A, Chua P et al. (2016) Subjective sleep problems in Huntington’s disease: a pilot investigation of the relationship to brain structure, neurocognitive, and neuropsychiatric function. J Neurol Sci, 364:148-153.
  • Bayram E, Mercan FN, Akbostancı MC (2015) Uneventful recovery from a suicide attempt with tetrabenazine: a case report. Turk J Neurol, 21:175-176.
  • Beglinger LJ, Paulsen JS, Watson DB, Wang MC, Duff K, Langbehn DR et al. & PREDICT-HD Investigators of the Huntington Study Group (2008) Obsessive and compulsive symptoms in prediagnosed Huntington's disease. J Clin Psychiatry, 69:1758-1765.
  • Bhattacharyya KB (2016). The story of George Huntington and his disease. Ann Indian Acad Neurol, 19:25-28.
  • Blackwell AD, Paterson NS, Barker RA, Robbins TW, Sahakian BJ (2008) The effects of modafinil on mood and cognition in Huntington’s disease. Psychopharmacology, 199:29-36.
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Neuropsychiatric Aspects of Huntington’s Disease

Yıl 2022, Cilt: 14 Sayı: 1, 98 - 112, 31.03.2022
https://doi.org/10.18863/pgy.952664

Öz

Huntington’s Disease is a progressive neurodegenerative disorder inherited in an autosomal dominant fashion with distinct phenotypes
as chorea and dystonia, incoordination, cognitive disorders, and behavioural problems. In addition to cognitive and motor symptoms,
neuropsychiatric symptoms are among the core symptoms of the disease. Neuropsychiatric symptoms are quite common in Huntington’s
Disease and the prevalence of neuropsychiatric disorders in different stages of the disease is estimated to be 33-76%. Although the prevelance
of neuropsychiatric symptoms varies among the stages of the disease, it is also known that the onset of these symptoms may be years before
the onset of motor symptoms. Common neuropsychiatric symptoms and disorders in Huntington’s Disease include depression, anxiety, suicide,
irritability, apathy, obsessive-compulsive symptoms, perseverations, psychosis, sleep disorders, and sexual dysfunctions. Neuropsychiatric
symptoms constitute one of the most important factors for the burden on families, and are considered to be the most important predictors of
decrease in daily functionality and institutionalizations in care centers and hospitalizations. Considering both its frequency and outcomes, it is
very important for patients, their relatives and caregivers to recognize and intervene in neuropsychiatric symptoms of Huntington’s Disease.
Although there are no studies with a high level of evidence on the treatment of neuropsychiatric symptoms of the disease, studies with lower
levels of evidence, case reports and treatment guidelines based on expert opinions are found in the literature in recent years. Another issue to
be considered in this area is the evaluation of individuals at genetic risk, genetic counseling and setting a safe protocol during these evaluations.
In this article, neuropsychiatric disorders common in Huntington’s Disease, the management of these disorders and the conditions to be
considered in the evaluation of individuals at genetic risk are reviewed in the light of current literature.

Kaynakça

  • A’Campo LEI, Spliethoff-Kamminga NGA, Roos RAC (2012) The Patient Education Program for Huntington’s Disease (PEP-HD). J Huntingtons Dis, 1:47-56.
  • Adrissi J, Nadkarni NA, Gausche E, Bega D (2019) Electroconvulsive therapy (ECT) for refractory psychiatric symptoms in Huntington’s disease: a case series and review of the literature. J Huntingtons Dis, 8:291-300.
  • Anderson KE (2005) Huntington’s disease, and related disorders. Psychiatr Clin North Am, 28:275-290.
  • Anderson KE, van Duijn E, Craufurd D, Drazinic C, Edmondson M, Goodman N et al. (2018). Clinical management of neuropsychiatric symptoms of huntington disease: expert-based consensus guidelines on agitation, anxiety, apathy, psychosis and sleep disorders. J Huntingtons Dis, 7:355-366.
  • Anderson KE, Eberly S, Groves M, Kayson E, Marder K, Young AB et al. (2016) Risk factors for suicidal ideation in people at risk for Huntington’s disease. J Huntingtons Dis, 5:389-394.
  • Arran N, Craufurd D, Simpson J (2014) Illness perceptions, coping styles and psychological distress in adults with Huntington’s disease. Psychol Health Med, 19:169-179.
  • Ataöv G, Akbaş I, Gül O, Balaban OD, Tanrıverdi Seda (2020) Psikiyatri servisinde Huntington Hastalığı vakası. 5. Psikiyatri Zirvesi ve 12. Anksiyete Kongresi, 29-31 Ekim Antalya, Türkiye. Kongre Özet Kitabı pp: 272.
  • Bachoud-Lévi AC, Ferreira J, Massart R, Youssov K, Rosser A, Busse M et al. (2019). International guidelines for the treatment of Huntington's disease. Front Neurol, 10:710.
  • Baker CR, Domínguez D JF, Stout JC, Gabery S, Churchyard A, Chua P et al. (2016) Subjective sleep problems in Huntington’s disease: a pilot investigation of the relationship to brain structure, neurocognitive, and neuropsychiatric function. J Neurol Sci, 364:148-153.
  • Bayram E, Mercan FN, Akbostancı MC (2015) Uneventful recovery from a suicide attempt with tetrabenazine: a case report. Turk J Neurol, 21:175-176.
  • Beglinger LJ, Paulsen JS, Watson DB, Wang MC, Duff K, Langbehn DR et al. & PREDICT-HD Investigators of the Huntington Study Group (2008) Obsessive and compulsive symptoms in prediagnosed Huntington's disease. J Clin Psychiatry, 69:1758-1765.
  • Bhattacharyya KB (2016). The story of George Huntington and his disease. Ann Indian Acad Neurol, 19:25-28.
  • Blackwell AD, Paterson NS, Barker RA, Robbins TW, Sahakian BJ (2008) The effects of modafinil on mood and cognition in Huntington’s disease. Psychopharmacology, 199:29-36.
  • Boyle A, Malloy PF, Salloway S, Cahn-Weiner DA, Cohen R, Cummings JL (2003) Executive dysfunction and apathy predict functional impairment in Alzheimer disease. Am J Geriatr Psychiatry, 11:214-221.
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  • Levy ML, Cummings JL, Fairbanks LA, Masterman D, Miller BL, Craig AH et al. (1998) Apathy is not depression. J Neuropsychiatry Clin Neurosci, 10:314-319.
  • Loi SM, Walterfang M, Velakoulis D, Looi JC (2018) Huntington’s disease: Managing neuropsychiatric symptoms in Huntington’s disease. Australas Psychiatry, 26:376-380.
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  • Marshall J, White K, Weaver M, Wetherill LF, Hui S, Stout JC et al. (2007). Specific psychiatric manifestations among preclinical Huntington disease mutation carriers. Arch Neurol, 64:116-121.
  • Martinez-Horta S, Perez-Perez J, van Duijn E, Fernandez-Bobadilla R, Carceller M, Pagonabarraga J et al. (2016). Neuropsychiatric symptoms are very common in premanifest and early stage Huntington's disease. Parkinsonism Relat Disord, 25:58-64.
  • Morris M (1991) Psychiatric aspects of Huntington’s disease. In Huntington’s Disease,( Ed. PS Harper):81–126. London, UK, WB Saunders.
  • Nakano T, Ono S, Yamaguchi J, Sugimoto R, Yamaguchi N, Morimoto Y et al. (2013) Modified electroconvulsive therapy for the treatment of refractory schizophrenia-like psychosis associated with Huntington’s disease. J Neurol, 260:312-314.
  • Nance M, Paulsen JS, Rosenblatt A, Wheelock V (2011) A Physician’s Guide to the Management of Huntington’s Disease. United States, Huntington’s Disease Society of America.
  • Neutel D, Tchikviladzé M, Charles P, Leu-Semenescu S, Roze E, Durr A et al. (2015) Nocturnal agitation in Huntington disease is caused by arousal-related abnormal movements rather than by rapid eye movement sleep behavior disorder. Sleep Med, 16:754-759.
  • Nimmagadda SR, Agrawal N, Worrall-Davies A, Markova I, Rickards H (2011) Determinants of irritability in Huntington’s disease. Acta Neuropsychiatr, 23:309-314.
  • Nock MK, Borges G, Bromet EJ, Alonso J, Angermeyer M, Beautrais A et al. (2008) Cross-national prevalence and risk factors for suicidal ideation, plans and attempts. Br J Psychiatry, 192:98-105.
  • Oosterloo M, Craufurd D, Nijsten H, van Duijn E (2019) Obsessive-compulsive and perseverative behaviors in Huntington’s disease. J Huntingtons Dis, 8:1-7.
  • Paoli RA, Botturi A, Ciammola A, Silani V, Prunas C, Lucchiari C et al. (2017) Neuropsychiatric burden in Huntington’s disease. Brain Sci,7:67.
  • Paulsen JS (2009) Functional imaging in Huntington’s disease. Exp Neurol, 216:272-277.
  • Paulsen JS, Ready RE, Hamilton JM, Mega MS, Cummings JL (2001) Neuropsychiatric aspects of Huntington’s disease. J Neurol Neurosurg Psychiatry, 71:310-314.
  • Paulsen JS, Miller AC, Hayes T, Shaw E (2017) Cognitive and behavioral changes in Huntington disease before diagnosis. In: Handbook of Clinical Neurology. Elsevier, pp. 69-91.
  • Paulsen JS, Hoth KF, Nehl C, Stierman L, The Huntington Study Group (2005) Critical periods of suicide risk in Huntington's disease. Am J Psychiatry 162:725-731.
  • Ratna N, Kamble NL, Venkatesh SD, Purushottam M, Pal PK, Jain S (2020) Psychiatric morbidity and poor follow-up underlie suboptimal functional and survival outcomes in Huntington’s disease. BMC Neurol, 20:87.
  • Rawlins M (2010) Huntington’s disease out of the closet? Lancet, 376:1372-1373.
  • Ready RE, Matthews M, Leserman A, Paulsen JS (2008) Patient and caregiver quality of life in Huntington’s disease. Mov Disord, 23:721-726.
  • Reedeker W, van der Mast RC, Giltay EJ, Kooistra TA, Roos RA, van Duijn E (2012a) Psychiatric disorders in Huntington’s disease: A 2-year follow-up study. Psychosomatics, 53:220-229.
  • Reedeker N, Bouwens JA, Giltay EJ, Le Mair SE, Roos RA, van der Mast RC et al. (2012b). Irritability in Huntington’s disease. Psychiatry Res, 200:813-818.
  • Rodrigues FB, Abreu D, Damásio J, Goncalves N, Correia-Guedes L, Coelho M et al. (2017) Survival, mortality, causes and places of death in a European Huntington's disease prospective cohort. Mov Disord Clin Pract, 4:737-742.
  • Ross CA, Tabrizi SJ (2011) Huntington's disease: from molecular pathogenesis to clinical treatment. Lancet Neurol, 10:83-98.
  • Ross CA, Aylward EH, Wild EJ (2014) Huntington disease: natural history, biomarkers and prospects for therapeutics. Nat Rev Neurol, 10:204-216.
  • Rossi G, Oh JC (2020) Management of agitation in huntington's disease: a review of the literature. Cureus,12:e9748.
  • Salman S, Balaban OD, Erten E, Karamustafalioglu N (2018) İlk başvurusu psikiyatrik belirtilerle olan bir Huntington Hastalığı olgusu. 3. Psikiyatri Zirvesi ve 10. Anksiyete Kongresi, 15-18 Mart 2018 Antalya, Türkiye. Kongre Özet Kitabı sayfa: 249-250.
  • Savva E, Schnorf H, Burkhard PR (2009) Restless legs syndrome: an early manifestation of Huntington’s disease? Acta Neurol Scand, 119:274-276.
  • Scheuing L, Chiu CT, Liao HM, Linares GR, Chuang DM (2014) Preclinical and clinical investigations of mood stabilizers for Huntington's disease: what have we learned? Int J Biol Sci, 10:1024-1038.
  • Serpell L, Waller G, Fearon P, Meyer C (2009) The roles of persistence and perseveration in psychopathology. Behav Ther, 40:260-271.
  • Sexton A, West K, Gill G, Wiseman A, Taylor J, Purvis R et al. (2021) Suicide in frontotemporal dementia and Huntington disease: analysis of family-reported pedigree data and implications for genetic healthcare for asymptomatic relatives. Psychol Health, 36:1397-1402.
  • Silver A (2003) Cognitive-behavioural therapy with a Huntington’s gene positive patient. Patient Educ Couns, 49:133-138.
  • Sinanović O (2020) Psychiatric disorders in neurological diseases. In Mind and Brain: Bridging Neurology and Psychiatry, (Ed V Demarin): 65-79.Cham, Switzerland, Springer.
  • Slaughter JR, Martens M.P, Slaughter KA (2001) Depression and Huntington’s disease: Prevalence, clinical manifestations, aetiology, and treatment. CNS Spectr, 6:306-326.
  • Solberg OK, Filkuková P, Frich JC, Feragen KJB (2018) Age at death and causes of death in patients with Huntington Disease in Norway in 1986–2015. J Huntingtons Dis, 7:77-86.
  • Squitieri F, Cannella M, Porcellini A, Brusa L, Simonelli M, Ruggieri S (2001) Short-term effects of olanzapine in Huntington disease. Neuropsychiatry Neuropsychol Behav Neurol, 14:69-72.
  • Stahl CM, Feigin A (2020) Medical, surgical, and genetic treatment of Huntington Disease. Neurol Clin, 38:367-378.
  • Stober T, Wussow W, Schimrigk K (1984) Bicaudate diameter: the most specifi c and simple CT parameter in the diagnosis of Huntington’s disease. Neuroradiology, 26:25-28.
  • Strassburger K, Andrich J, Saft C (2008) Bupropion: first experience in Huntington’s Disease. J Neurol Neurosurg Psychiatry, 79:29-30.
  • Szymuś K, Bystrzyński A, Kwaśniak-Butowska M, Konkel A, Leśnicka A, Nowacka M et al. (2020). Sexual dysfunction in Huntington’s Disease-a systematic review. Neurol Neurochir Pol, 54:305-311.
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  • van der Meer L, van Duijn E, Wolterbeek R, Tibben A (2014) Offspring of a parent with genetic disease: childhood experiences and adult psychological characteristics. Health Psychol, 33:1445-1453.
  • van Duijn E, Kingma EM, van der Mast RC (2007) Psychopathology in verified Huntington's disease gene carriers. J Neuropsychiatry Clin Neurosci, 19:441-448.
  • van Duijn E, Kingma EM, Timman R, Zitman FG, Tibben A, Roos RAC et al. (2008) Cross-sectional study on prevalences of psychiatric disorders in mutation carriers of Huntington’s disease compared with mutation-negative firstdegree relatives. J Clin Psychiatry, 69:1804-1810.
  • van Duijn E, Reedeker N, Giltay EJ, Roos RAC, van der Mast RC (2010) Correlates of apathy in huntington’s disease.J Neuropsychiatry Clin Neurosci, 22:287-94.
  • van Duijn E, Craufurd D, Hubers AA, Giltay EJ, Bonelli R, Rickards H et al. for the European Huntington’s Disease Network Behavioural Phenotype Working Group (2014) Neuropsychiatric symptoms in a European Huntington’s disease cohort (REGISTRY). J Neurol Neurosurg Psychiatry, 85:1411-1418.
  • van Duijn E, Vrijmoeth EM, Giltay EJ, Bernhard F, Landwehrmeyer G, REGISTRY investigators of the European Huntington's Disease Network (2018) Suicidal ideation and suicidal behavior according to the C-SSRS in a European cohort of Huntington's disease gene expansion carriers. J Affect Disord, 228:194-204.
  • Wahlin TBR (2007). To know or not to know: a review of behaviour and suicidal ideation in preclinical Huntington's disease. Patient Educ Couns, 65:279-287.
  • Walker FO (2007) Huntington’s disease. Lancet, 369:218-228.
  • Wesson M, Boileau NR, Perlmutter JS, Paulsen JS, Barton SK, McCormack MK et al. (2018) Suicidal ideation assessment in individuals with premanifest and manifest Huntington Disease. J Huntingtons Dis, 7:239-249.
  • Wetzel HH, Gehl CR, Dellefave-Castillo L, Schiffman JF, Shannon KM, Paulsen JS (2011) Huntington Study Group: Suicidal ideation in Huntington disease: the role of comorbidity. Psychiatry Res, 188:372-376.
  • Wolf RC, Vasic N, Schönfeldt-Lecuona C, Ecker D, Landwehrmeyer GB (2008). Functional imaging of cognitive processes in Huntington's disease and its presymptomatic mutation carriers. Der Nervenarzt, 79:408-420.
Toplam 104 adet kaynakça vardır.

Ayrıntılar

Birincil Dil Türkçe
Konular Psikiyatri
Bölüm Derleme
Yazarlar

Özlem Devrim Balaban 0000-0001-7903-549X

E. Cem Atbaşoğlu 0000-0002-8211-6095

Yayımlanma Tarihi 31 Mart 2022
Kabul Tarihi 23 Ağustos 2021
Yayımlandığı Sayı Yıl 2022 Cilt: 14 Sayı: 1

Kaynak Göster

AMA Balaban ÖD, Atbaşoğlu EC. Huntington Hastalığı’nın Nöropsikiyatrik Yönü. Psikiyatride Güncel Yaklaşımlar. Mart 2022;14(1):98-112. doi:10.18863/pgy.952664

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