The site of the Media Sphera Publishers contains materials intended solely for healthcare professionals.
By closing this message, you confirm that you are a certified medical professional or a student of a medical educational institution.

Login
EN
+7 (495) 482-4118
+7 (495) 482-4329
+8 800 250-18-12
  • (toll-free number for subscriptions)
    Mon-Fri from 10 a.m. to 6 p.m.

  • © 1998-2026
+7 (495) 482-43-29
EN
All journals
Journal
Year
Year
Search result: 0

Kutlubaev M.A.

Bashkir State Medical University

Pervushina E.V.

Bashkir state medical university

Areprintceva D.K.

Bashkir state medical university

Mendelevich V.D.

Kazan State Medical University

Brylev L.V.

Buyanov Moscow City Clinical Hospital

Neuropsychiatric presentations of amyotrophic lateral sclerosis

Authors:

Kutlubaev M.A., Pervushina E.V., Areprintceva D.K., Mendelevich V.D., Brylev L.V.

More about the authors

Journal: S.S. Korsakov Journal of Neurology and Psychiatry. 2022;122(5): 36‑42

DOI: 10.17116/jnevro202212205136

Read: 1693 times

Download PDF (RU)
Contact the author
Table of contents

NEUROPSYCHIATRIC PRESENTATIONS OF AMYOTROPHIC LATERAL SCLEROSIS
NEUROPSYCHIATRIC PRESENTATIONS OF AMYOTROPHIC LATERAL SCLEROSIS

To cite this article:

Kutlubaev MA, Pervushina EV, Areprintceva DK, Mendelevich VD, Brylev LV. Neuropsychiatric presentations of amyotrophic lateral sclerosis. S.S. Korsakov Journal of Neurology and Psychiatry. 2022;122(5):36‑42. (In Russ.)
https://doi.org/10.17116/jnevro202212205136

Подписка 2026 Журнал неврологии и психиатрии им. С.С. Корсакова (S.S. Korsakov Journal of Neurology and Psychiatry)
МСФ на ЯМ
Использование инфографики авторами научных работ
Close metadata
Recommended articles:
A personalized approach to the treatment of chro­nic tension headache and como­rbid mental diso­rders. S.S. Korsakov Journal of Neurology and Psychiatry. 2025;(2):51-57
Asthenia in the acute period of ischemic stroke. S.S. Korsakov Journal of Neurology and Psychiatry. 2025;(3-2):5-10
Adolescent suicide risk asse­ssment using the Suicide Risk Rapid Survey. S.S. Korsakov Journal of Neurology and Psychiatry. 2025;(3):86-93
Oculomotor diso­rders in patients with amyotrophic late­ral scle­rosis. S.S. Korsakov Journal of Neurology and Psychiatry. 2025;(4):7-12
The state of mental health of migrant workers and citi­zens of the Russian Fede­ration: A comparative analysis as illu­strated by the Moscow region. S.S. Korsakov Journal of Neurology and Psychiatry. 2025;(4):95-101
Factors of depression acco­rding to acti­graphy in the fall season. S.S. Korsakov Journal of Neurology and Psychiatry. 2025;(5-2):27-32
Primary progressive apha­sia in the neurologist practice. S.S. Korsakov Journal of Neurology and Psychiatry. 2025;(4-2):61-66
Identification of depression biomarkers: Prospects for use in clinical practice. S.S. Korsakov Journal of Neurology and Psychiatry. 2025;(5):51-55
Depression Anxiety Stress Scale (russian language version). S.S. Korsakov Journal of Neurology and Psychiatry. 2025;(5):103-107
Expe­rience with the use of amitriptyline for chro­nic pelvic pain asso­ciated with endo­metriosis. Russian Journal of Human Reproduction. 2025;(1):86-93
Abstract:

Neuropsychiatric presentations are observed in a substantial number of patients with amyotrophic lateral sclerosis (ALS). Severe behavioral disorders develop in ALS combined with frontotemporal dementia, which are considered to be a disease continuum. Psychiatric disorders in ALS with predominantly motor symptoms are less prominent and mostly presented with apathy. Psychiatric disorders in ALS by their origin could be psychogenic, reflecting the patients’ reactions on severe disease, and organic, developing as a result of degeneration of central motor neurons and disconnection between fronto-subcortical and frontotemporal loops. An important role in the development of psychiatric disorders in ALS belongs to genetic factors, in particular to hexanucleotide expansion in the C9orf72 gene. During the first months after establishing the diagnosis of ALS, there is the high risk of developing depressive disorders, which in severe cases can lead to suicide. More research is needed in this area.

Keywords:

amyotrophic lateral sclerosis
frontotemporal dementia
suicide
apathy
depression

Authors:

Kutlubaev M.A.

Bashkir State Medical University

Pervushina E.V.

Bashkir state medical university

Areprintceva D.K.

Bashkir state medical university

Mendelevich V.D.

Kazan State Medical University

Brylev L.V.

Buyanov Moscow City Clinical Hospital

Received:

09.12.2020

Accepted:

09.12.2020

Close metadata

References:

  1. Zucchi E, Ticozzi N, Mandrioli J. Psychiatric Symptoms in Amyotrophic Lateral Sclerosis: Beyond a Motor Neuron Disorder. Front Neurosci. 2019;13:175.  https://doi.org/10.3389/fnins.2019.00175
  2. Huynh W, Ahmed R, Mahoney CJ, et al. The impact of cognitive and behavioral impairment in amyotrophic lateral sclerosis. Expert Rev Neurother. 2020;20:281-293.  https://doi.org/10.1080/14737175.2020.1727740
  3. Strong MJ, Abrahams S, Goldstein LH, et al. Amyotrophic lateral sclerosis — frontotemporal spectrum disorder (ALS-FTSD): Revised diagnostic criteria. Amyotroph Lateral Scler Frontotemporal Degener. 2017;18(3-4):153-174.  https://doi.org/10.1080/21678421.2016.1267768
  4. Raaphorst J, Beeldman E, De Visser M, et al. A systematic review of behavioural changes in motor neuron disease. Amyotroph Lateral Scler. 2012;13(6):493-501.  https://doi.org/10.3109/17482968.2012.656652
  5. Burke T, Pinto-Grau M, Lonergan K, et al. A Cross-sectional population-based investigation into behavioral change in amyotrophic lateral sclerosis: subphenotypes, staging, cognitive predictors, and survival. Ann Clin Transl Neurol. 2017;4(5):305-317.  https://doi.org/10.1002/acn3.407
  6. Lillo P, Mioshi E, Zoing MC, et al. How common are behavioural changes in amyotrophic lateral sclerosis? Amyotroph Lateral Scler. 2011;12(1):45-51.  https://doi.org/10.3109/17482968.2010.520718
  7. Pender N, Pinto-Grau M, Hardiman O. Cognitive and behavioural impairment in amyotrophic lateral sclerosis. Curr Opin Neurol. 2020;33(5):649-654.  https://doi.org/10.1097/WCO.0000000000000862
  8. Flaherty-Craig C, Eslinger P, Stephens B, et al. A rapid screening battery to identify frontal dysfunction in patients with ALS. Neurology. 2006;67(11):2070-2072. https://doi.org/10.1212/01.wnl.0000247667.89251.43
  9. Beswick E, Park E, Wong C, et al. A systematic review of neuropsychiatric and cognitive assessments used in clinical trials for amyotrophic lateral sclerosis. J Neurol. 2020;34(3):45-49.  https://doi.org/10.1007/s00415-020-10203-z
  10. Turner MR, Goldacre R, Talbot K, Goldacre MJ. Psychiatric disorders prior to amyotrophic lateral sclerosis. Ann Neurol. 2016;80(6):935-938.  https://doi.org/10.1002/ana.24801
  11. Shpilyukova YA, Fedotova EY, Berdnikovich ES, et al. C9orf72-assotsiirovannaya forma lobno-visochnoi dementsii v rossiiskoi populyatsii [C9orf72-associated frontotemporal dementia in the Russian population]. Zh Nevrol Psikhiatr im. S.S. Korsakova. 2020;120(9):98-106. (In Russ.). https://doi.org/10.17116/jnevro202012009198
  12. DeJesus-Hernandez M, Mackenzie IR, Boeve BF, et al. Expanded GGGGCC hexanucleotide repeat in noncoding region of C9ORF72 causes chromosome 9p-linked FTD and ALS. Neuron. 2011;72(2):245-256.  https://doi.org/10.1016/j.neuron.2011.09.011
  13. van der Zee J, Gijselinck I, Dillen L, et al. European Early-Onset Dementia Consortium. A pan-European study of the C9orf72 repeat associated with FTLD: geographic prevalence, genomic instability, and intermediate repeats. Hum Mutat. 2013;34(2):363-373.  https://doi.org/10.1002/humu.22244
  14. Ng ASL, Tan EK. Intermediate C9orf72 alleles in neurological disorders: does size really matter? J Med Genet. 2017;54(9):591-597.  https://doi.org/10.1136/jmedgenet-2017-104752
  15. Abramycheva NY, Lysogorskaia EV, Stepanova MS, et al. C9ORF72 hexanucleotide repeat expansion in ALS patients from the Central European Russia population. Neurobiol Aging. 2015;36(10):2908.e5-9.  https://doi.org/10.1016/j.neurobiolaging.2015.07.004
  16. Devenney EM, Ahmed RM, Halliday G, et al. Psychiatric disorders in C9orf72 kindreds: Study of 1,414 family members. Neurology. 2018;91(16):1498-1507. https://doi.org/10.1212/WNL.0000000000006344
  17. Lindquist SG, Duno M, Batbayli M, et al. Corticobasal and ataxia syndromes widen the spectrum of C9ORF72 hexanucleotide expansion disease. Clin Genet. 2013;83(3):279-283.  https://doi.org/10.1111/j.1399-0004.2012.01903.x
  18. Xi Z, Yunusova Y, van Blitterswijk M, et al. Identical twins with the C9orf72 repeat expansion are discordant for ALS. Neurology. 2014;83(16):1476-1478. https://doi.org/10.1212/WNL.0000000000000886
  19. Murphy NA, Arthur KC, Tienari PJ, et al. Age-related penetrance of the C9orf72 repeat expansion. Sci Rep. 2017;7(1):2116. https://doi.org/10.1038/s41598-017-02364-1
  20. Papma JM, Jiskoot LC, Panman JL, et al. Cognition and gray and white matter characteristics of presymptomatic C9orf72 repeat expansion. Neurology. 2017;89(12):1256-1264. https://doi.org/10.1212/WNL.0000000000004393
  21. Floeter MK, Bageac D, Danielian LE, et al. Longitudinal imaging in C9orf72 mutation carriers: Relationship to phenotype. Neuroimage Clin. 2016;12:1035-1043. https://doi.org/10.1016/j.nicl.2016.10.014
  22. Lee SE, Sias AC, Mandelli ML, et al. Network degeneration and dysfunction in presymptomatic C9ORF72 expansion carriers. Neuroimage Clin. 2016;14:286-297.  https://doi.org/10.1016/j.nicl.2016.12.006
  23. Benatar M, Wuu J, Andersen PM, et al. Neurofilament light: A candidate biomarker of presymptomatic amyotrophic lateral sclerosis and phenoconversion. Ann Neurol. 2018;84(1):130-139.  https://doi.org/10.1002/ana.25276
  24. Gendron TF, Chew J, Stankowski JN, et al. Poly(GP) proteins are a useful pharmacodynamic marker for C9ORF72-associated amyotrophic lateral sclerosis. Sci Transl Med. 2017;9(383):eaai7866. https://doi.org/10.1126/scitranslmed.aai7866
  25. Byrne S, Heverin M, Elamin M, et al. Aggregation of neurologic and neuropsychiatric disease in amyotrophic lateral sclerosis kindreds: a population-based case-control cohort study of familial and sporadic amyotrophic lateral sclerosis. Ann Neurol. 2013;74(5):699-708.  https://doi.org/10.1002/ana.23969
  26. Hall D, Finger EC. Psychotic symptoms in frontotemporal dementia. Curr Neurol Neurosci Rep. 2015;15(7):46.  https://doi.org/10.1007/s11910-015-0567-8
  27. Braak H, Ludolph A, Thal DR, et al. Amyotrophic lateral sclerosis: dash-like accumulation of phosphorylated TDP-43 in somatodendritic and axonal compartments of somatomotor neurons of the lower brainstem and spinal cord. Acta Neuropathol. 2010;120(1):67-74.  https://doi.org/10.1007/s00401-010-0683-0
  28. Mann DMA, Snowden JS. Frontotemporal lobar degeneration: Pathogenesis, pathology and pathways to phenotype. Brain Pathol. 2017;27(6):723-736.  https://doi.org/10.1111/bpa.12486
  29. Burrell JR, Kiernan MC, Vucic S, et al. Motor neuron dysfunction in frontotemporal dementia. Brain. 2011;134(Pt 9):2582-2594. https://doi.org/10.1093/brain/awr195
  30. Brettschneider J, Del Tredici K, Irwin DJ, et al. Sequential distribution of pTDP-43 pathology in behavioral variant frontotemporal dementia (bvFTD). Acta Neuropathol. 2014;127(3):423-439.  https://doi.org/10.1007/s00401-013-1238-y
  31. Henkel JS, Beers DR, Wen S, et al. Regulatory T-lymphocytes mediate amyotrophic lateral sclerosis progression and survival. EMBO Mol Med. 2013;5(1):64-79.  https://doi.org/10.1002/emmm.201201544
  32. Radewicz K, Garey LJ, Gentleman SM, et al. Increase in HLA-DR immunoreactive microglia in frontal and temporal cortex of chronic schizophrenics. J Neuropathol Exp Neurol. 2000;59(2):137-150.  https://doi.org/10.1093/jnen/59.2.137
  33. Devenney EM, Landin-Romero R, Irish M, et al. The neural correlates and clinical characteristics of psychosis in the frontotemporal dementia continuum and the C9orf72 expansion. Neuroimage Clin. 2016;13:439-445.  https://doi.org/10.1016/j.nicl.2016.11.028
  34. Zhou J, Seeley WW. Network dysfunction in Alzheimer’s disease and frontotemporal dementia: implications for psychiatry. Biol Psychiatry. 2014;75(7):565-573.  https://doi.org/10.1016/j.biopsych.2014.01.020
  35. Consonni M, Cappa SF, Dalla Bella E, et al. Cortical correlates of behavioural change in amyotrophic lateral sclerosis. J Neurol Neurosurg Psychiatry. 2019;90(4):380-386.  https://doi.org/10.1136/jnnp-2018-318619
  36. Chiò A, Moglia C, Canosa A, et al. Cognitive impairment across ALS clinical stages in a population-based cohort. Neurology. 2019;93(10):984-994.  https://doi.org/10.1212/WNL.0000000000008063
  37. Schulthess I, Gorges M, Müller HP, et al. Functional connectivity changes resemble patterns of pTDP-43 pathology in amyotrophic lateral sclerosis. Sci Rep. 2016;6:38391. https://doi.org/10.1038/srep38391
  38. Lulé D, Pauli S, Altintas E, et al. Emotional adjustment in amyotrophic lateral sclerosis (ALS). J Neurol. 2012;259(2):334-341.  https://doi.org/10.1007/s00415-011-6191-x
  39. Woolley SC, Jonathan S Katz. Cognitive and behavioral impairment in amyotrophic lateral sclerosis. Phys Med Rehabil Clin N Am. 2008;19(3):607-617, xi.  https://doi.org/10.1016/j.pmr.2008.04.002
  40. Goode DJ, Manning AA. Specific imbalance of right and left sided motor neuron excitability in schizophrenia. J Neurol Neurosurg Psychiatry. 1988;51(5):626-629.  https://doi.org/10.1136/jnnp.51.5.626
  41. Crayton JW, Meltzer HY, Goode DJ. Motoneuron excitability in psychiatric patients. Biol Psychiatry. 1977;12(4):545-561. 
  42. Turner MR, Goldacre R, Talbot K, et al. Psychiatric disorders prior to amyotrophic lateral sclerosis. Ann Neurol. 2016;80(6):935-938.  https://doi.org/10.1002/ana.24801
  43. Snowden JS, Rollinson S, Thompson JC, et al. Distinct clinical and pathological characteristics of frontotemporal dementia associated with C9ORF72 mutations. Brain. 2012;135(Pt 3):693-708.  https://doi.org/10.1093/brain/awr355
  44. Rains LS, Fallica G, O’Daly O, et al. Exploring psychotic symptoms: a comparison of motor related neuronal activation during and after acute psychosis. BMC Psychiatry. 2012;12:102.  https://doi.org/10.1186/1471-244X-12-102
  45. Roos E, Mariosa D, Ingre C, et al. Depression in amyotrophic lateral sclerosis. Neurology. 2016;86(24):2271-2277. https://doi.org/10.1212/WNL.0000000000002671
  46. Radakovic R, Stephenson L, Newton J, et al. Multidimensional apathy and executive dysfunction in amyotrophic lateral sclerosis. Cortex. 2017;94:142-151.  https://doi.org/10.1016/j.cortex.2017.06.023
  47. Caga J, Turner MR, Hsieh S, et al. Apathy is associated with poor prognosis in amyotrophic lateral sclerosis. Eur J Neurol. 2016;23(5):891-897.  https://doi.org/10.1111/ene.12959
  48. Radakovic R, Stephenson L, Newton J, et al. Multidimensional apathy and executive dysfunction in amyotrophic lateral sclerosis. Cortex. 2017;94:142-151.  https://doi.org/10.1016/j.cortex.2017.06.023
  49. Finegan E, Chipika RH, Li Hi Shing S, et al. Pathological Crying and Laughing in Motor Neuron Disease: Pathobiology, Screening, Intervention. Front Neurol. 2019;10:260.  https://doi.org/10.3389/fneur.2019.00260
  50. O’Brien M, Burke T, Heverin M, et al. Clustering of Neuropsychiatric Disease in First-Degree and Second-Degree Relatives of Patients With Amyotrophic Lateral Sclerosis. JAMA Neurol. 2017;74(12):1425-1430. https://doi.org/10.1001/jamaneurol.2017.2699
  51. Cerami C, Dodich A, Canessa N, et al. Emotional empathy in amyotrophic lateral sclerosis: a behavioural and voxel-based morphometry study. Amyotroph Lateral Scler Frontotemporal Degener. 2014;15(1-2):21-29.  https://doi.org/10.3109/21678421.2013.785568
  52. van der Hulst EJ, Bak TH, Abrahams S. Impaired affective and cognitive theory of mind and behavioural change in amyotrophic lateral sclerosis. J Neurol Neurosurg Psychiatry. 2015;86(11):1208-1215. https://doi.org/10.1136/jnnp-2014-309290
  53. Hoogenhout M, Malcolm-Smith S. Theory of mind predicts severity level in autism. Autism. 2017;21(2):242-252.  https://doi.org/10.1177/1362361316636758
  54. Kutlubaev MA. Suicidal behavior in neurological disorders; frequency, predisposing factors, approaches to prevention. Nevrologicheskii Zhurnal. 2016;3:124-130. (In Russ.).
  55. Tsai CP, Chang BH, Lee CT. Underlying cause and place of death among patients with amyotrophic lateral sclerosis in Taiwan: a population-based study, 2003—2008. J Epidemiol. 2013;23(6):424-428.  https://doi.org/10.2188/jea.je20130045
  56. Palmieri A, Sorarù G, Albertini E, Semenza C, Vottero-Ris F, D’Ascenzo C, Querin G, Zennaro A, Pegoraro E, Angelini C. Psychopathological features and suicidal ideation in amyotrophic lateral sclerosis patients. Neurol Sci. 2010;31(6):735-740.  https://doi.org/10.1007/s10072-010-0332-3
  57. Gourie-Devi M, Gupta R, Sharma V, et al. An insight into death wish among patients with amyotrophic lateral sclerosis in India using «Wish-to-Die Questionnaire». Neurol India. 2017;65(1):46-51. 
  58. Rabkin J, Goetz R, Murphy JM, et al. ALS COSMOS Study Group. Cognitive impairment, behavioral impairment, depression, and wish to die in an ALS cohort. Neurology. 2016;87(13):1320-1328. https://doi.org/10.1212/WNL.0000000000003035
  59. Albert SM, Rabkin JG, Del Bene ML, et al. Wish to die in end-stage ALS. Neurology. 2005;65(1):68-74.  https://doi.org/10.1212/01.wnl.0000168161.54833.bb
  60. Chernenkaya VY, Gorbachev KV, Gorbachev DV, et al. Édinburgskaia shkala otsenki stepeni narusheniia kognitivnykh funktsiĭ i povedeniia u patsientov s bokovym amiotroficheskim sklerozom (ECAS) — russkoiazychnaia versiia [The Edinburgh Cognitive and Behavioral ALS Screen (ECAS): a Russian version.]. Zh Nevrol Psikhiatr im. S.S. Korsakova. 2018;118(12):36-39. (In Russ.). https://doi.org/10.17116/jnevro201811812136
Contact the author
Email
Message
The publishing house "Media Sphere" does not provide treatment services, does not give recommendations on contacting medical institutions and specific specialists, on the prescription of medicines and dietary supplements.

Email Confirmation

An email was sent to test@gmail.com with a confirmation link. Follow the link from the letter to complete the registration on the site.

Email Confirmation

Submit Application

You can become an author of one of our magazines, send a request and we will consider it in during the day.

Name
Phone
E-mail
Message
Login
Registration
E-mail
Password
Forgot Password?

Log in to the site using your account in one of the services

Password recovery
E-mail
Login
Register

We use cооkies to improve the performance of the site. By staying on our site, you agree to the terms of use of cооkies. To view our Privacy and Cookie Policy, please. click here.