r-n-jРоссийский неврологический журналRussian neurological journal2658-79472686-7192МИА10.30629/2658-7947-2021-26-2-17-24r-n-j-166Research ArticleИССЛЕДОВАНИЯ И КЛИНИЧЕСКИЕ НАБЛЮДЕНИЯCLINICAL RESEARCHES AND CASE REPORTSСиндром боковой амиотрофический склероз — альцгеймеровская деменцияSyndrome amyotrophic lateral sclerosis — Alz heimer's dementiahttps://orcid.org/0000-0002-3110-4764КоберскаяН. Н.KoberskayaN. N.

Москва

Moscow

koberskaya_n_n@mail.ruhttps://orcid.org/0000-0003-2424-3245ГришинаД. А.GrishinaD. A.

Москва

Moscow

https://orcid.org/0000-0002-8255-5645ЯхноН. Н.YakhnoN. N.

Москва

Moscow

Первый МГМУ им. И.М. Сеченова (Сеченовский Университет), Институт клинической медицины им. Н.В. Склифосовского, кафедра нервных болезней и нейрохирургииРоссияI.M. Sechenov First Moscow State Medical University (Sechenov University), N.V. Sklifosovsky Institute of Clinical Medicine, Department of Nervous Diseases and NeurosurgeryRussian Federation2021290520212621724Copyright © Коберская Н.Н., Гришина Д.А., Яхно Н.Н., 20212021Коберская Н.Н., Гришина Д.А., Яхно Н.Н.Koberskaya N.N., Grishina D.A., Yakhno N.N.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://www.r-n-j.com/jour/article/view/166

В последнее время появляется все больше доказательств наличия когнитивного дефекта различной степени выраженности в клинической картине БАС. Редкой формой заболевания является комплекс боковой амиотрофический склероз (БАС) — деменция, характериующийся сочетанием деменции (чаще лобно-височной) с симптомами БАС. Профиль когнитивного дефицита при БАС включает нарушение управляющих функций, памяти, речевые и зрительно-пространственные расстройства. Приводится литературный обзор по данной проблеме с описанием клинического наблюдения синдрома БАС–деменция (лобный вариант возможной болезни Альцгеймера). У пациентки с достоверным диагнозом БАС наблюдались быстропрогрессирующие когнитивные расстройства в виде гиппокампальных нарушений памяти, речевых, зрительно-пространственных нарушений и дефектности управляющих функций, сопровождающиеся поведенческими изменениями (апатией, снижением критики). При магнитно-резонансном исследовании головного мозга обнаружена значительная атрофия гиппокампов, коры лобных долей и левой височной доли. В литературе практически отсутствуют описания пациентов с клинической картиной сочетания БА и БАС. Обсуждаются сложности диагностики данного состояния. Обсуждается взаимосвязь этих нейродегенеративных заболеваний. Представленные данные литературы и приведенное клиническое наблюдение подтверждают целесообразность исследования когнитивных функций у больных с предполагаемым или достоверным БАС, с одной стороны, и анализ состояния центрального и периферического нейрона у пациентов с нервно-психическими расстройствами лобно-височного типа — с другой, что может быть полезным для диагностики и проведения лечебно-реабилитационных мероприятий.

Recently, there is more and more evidence of the presence of a cognitive defect of varying severity in the clinical picture of ALS. A rare form of the disease is the amyotrophic lateral sclerosis (ALS) — dementia complex, characterized by a combination of dementia (usually frontotemporal) with ALS symptoms. The profile of cognitive deficit in ALS includes impairment of executive functions, memory, speech and visual-spatial disorders. A literature review on this problem is presented with a description of the clinical observation of ALS–dementia syndrome (frontal variant of possible Alzheimer’s disease). A patient with a reliable diagnosis of ALS showed rapidly progressive cognitive impairments in the form of hippocampal memory impairments, speech, visual-spatial impairments, and defective executive functions, accompanied by behavioral changes (apathy, decreased criticism). Magnetic resonance imaging of the brain revealed significant atrophy of the hippocampus, frontal lobe cortex, and left temporal lobe. In the literature, there are practically no descriptions of patients with a clinical picture of a combination of AD and ALS. Difficulties in diagnosing this condition are discussed. The relationship between these neurodegenerative diseases is discussed. The presented literature data and the presented clinical observation confi rm the expediency of studying cognitive functions in patients with suspected or signifi cant ALS, on the one hand, and analysis of the state of the central and peripheral neurons in patients with neuropsychiatric disorders of the frontotemporal type, on the other hand, which can be useful for diagnostics and treatment and rehabilitation measures.

деменциябоковой амиотрофический склерозболезнь Альцгеймералобно-височная деменциякогнитивные нарушенияdementiaamyotrophic lateral sclerosisAlzheimer’s diseasefronto-tempral dementiacognitive impairmentReferencesLomen-Hoerth C., Anderson T. and Miller B. The overlap of amyotrophic lateral sclerosis and frontotemporal dementia. Neurology. 2002;59(7):1077–1079.Lomen-Hoerth C., Anderson T. and Miller B. The overlap of amyotrophic lateral sclerosis and frontotemporal dementia. Neurology. 2002;59(7):1077–1079.McGeer P.L., Schwab C., McGeer E.G., Haddock R.L., Steele J.C. Familial nature and continuing morbidity of the amyotrophic lateral sclerosis-parkinsonism dementia complex of Guam. Neurology. 1997;47:400–409. https://doi.org/10.1212/wnl.49.2.400McGeer P.L., Schwab C., McGeer E.G., Haddock R.L., Steele J.C. Familial nature and continuing morbidity of the amyotrophic lateral sclerosis-parkinsonism dementia complex of Guam. Neurology. 1997;47:400–409. https://doi.org/10.1212/wnl.49.2.400Rakowicz W.P., Hodges J.R. Dementia and aphasia in motor neuron disease: an under recognised association? J Neurol Neurosurg Psychiatry. 1998;65:881–889. https://doi.org/10.1136/jnnp.65.6.881Rakowicz W.P., Hodges J.R. Dementia and aphasia in motor neuron disease: an under recognised association? J Neurol Neurosurg Psychiatry. 1998;65:881–889. https://doi.org/10.1136/jnnp.65.6.881Raaphorst J., de Visser M., Linssen W.H. et al. The cognitive profi le of amyotrophic lateral sclerosis: a meta-analysis. Amyotroph Lateral Scler. 2010;11:27–37. https://doi.org/10.3109/17482960802645008Raaphorst J., de Visser M., Linssen W.H. et al. The cognitive profi le of amyotrophic lateral sclerosis: a meta-analysis. Amyotroph Lateral Scler. 2010;11:27–37. https://doi.org/10.3109/17482960802645008Lomen-Hoerth C., Murphy J., Langmore S., Kramer J.H., Olney R.K., Miller B. Are amyotrophic lateral sclerosis patients cognitively normal? Neurology. 2003;60:1094–1097. https://doi.org/10.1212/01.wnl.0000055861.95202.8dLomen-Hoerth C., Murphy J., Langmore S., Kramer J.H., Olney R.K., Miller B. Are amyotrophic lateral sclerosis patients cognitively normal? Neurology. 2003;60:1094–1097. https://doi.org/10.1212/01.wnl.0000055861.95202.8dStrong M.J., Grace G.M., Freedman M. et al. Consensus criteria for the diagnosis of frontotemporal cognitive and behavioural syndromes in amyotrophic lateral sclerosis. Amyotroph Lateral Scler. 2009;10:131–46. https://doi.org/10.1080/17482960802654364Strong M.J., Grace G.M., Freedman M. et al. Consensus criteria for the diagnosis of frontotemporal cognitive and behavioural syndromes in amyotrophic lateral sclerosis. Amyotroph Lateral Scler. 2009;10:131–46. https://doi.org/10.1080/17482960802654364Phukan J., Elamin M., Bede P., Jordan N., Gallagher L., Byrne S. et al. The syndrome of cognitive impairment in amyotrophic lateral sclerosis: a population-based study. J Neurol Neurosurg Psychiatry. 2012;83(1):102–8. https://doi.org/10.1136/jnnp-2011-300188 Epub 2011 Aug 11. PMID: 21836033.Phukan J., Elamin M., Bede P., Jordan N., Gallagher L., Byrne S. et al. The syndrome of cognitive impairment in amyotrophic lateral sclerosis: a population-based study. J Neurol Neurosurg Psychiatry. 2012;83(1):102–8. https://doi.org/10.1136/jnnp-2011-300188 Epub 2011 Aug 11. PMID: 21836033.Achi E.Y., Rudnicki S.A. ALS and Frontotemporal Dysfunction: A Review. Neurol Res Int. 2012;2012:806306. https://doi.org/10.1155/2012/806306. Epub 2012 Aug 7. PMID: 22919484; PMCID: PMC3423946.Achi E.Y., Rudnicki S.A. ALS and Frontotemporal Dysfunction: A Review. Neurol Res Int. 2012;2012:806306. https://doi.org/10.1155/2012/806306. Epub 2012 Aug 7. PMID: 22919484; PMCID: PMC3423946.Deng Z., Sheehan P., Chen S., Yue Z. Is amyotrophic lateral sclerosis/ frontotemporal dementia an autophagy disease? Mol Neurodegener. 2017;12(1):90. https://doi.org/10.1186/s13024-017-0232-6. PMID: 29282133; PMCID: PMC5746010.Deng Z., Sheehan P., Chen S., Yue Z. Is amyotrophic lateral sclerosis/ frontotemporal dementia an autophagy disease? Mol Neurodegener. 2017;12(1):90. https://doi.org/10.1186/s13024-017-0232-6. PMID: 29282133; PMCID: PMC5746010.Burrell J.R., Kiernan M.C., Vucic S., Hodges J.R. Motor neuron dysfunction in frontotemporal dementia. Brain. 2011;134 (Pt 9):2582–2594. https://doi.org/10.1093/brain/awr195Burrell J.R., Kiernan M.C., Vucic S., Hodges J.R. Motor neuron dysfunction in frontotemporal dementia. Brain. 2011;134 (Pt 9):2582–2594. https://doi.org/10.1093/brain/awr195Huynh W. et al. The impact of cognitive and behavioral impairment in amyotrophic lateral sclerosis. Expert Review of Neurotherapeutics. 2020;(20):281–293. http://dx.doi.org/10.1080/14737175.2020.1727740Huynh W. et al. The impact of cognitive and behavioral impairment in amyotrophic lateral sclerosis. Expert Review of Neurotherapeutics. 2020;(20):281–293. http://dx.doi.org/10.1080/14737175.2020.1727740Strong M.J., Abrahams S., Goldstein L.H., Woolley S., McLaughlin P., Snowden J. et al. Amyotrophic lateral sclerosisfrontotemporal spectrum disorder (ALS-FTSD): revised diagnostic criteria. Amyotroph Later Scler Frontotemporal Degener. 2017;18:1–22. https://doi.org/10.1080/21678421.2016.1267768Strong M.J., Abrahams S., Goldstein L.H., Woolley S., McLaughlin P., Snowden J. et al. Amyotrophic lateral sclerosisfrontotemporal spectrum disorder (ALS-FTSD): revised diagnostic criteria. Amyotroph Later Scler Frontotemporal Degener. 2017;18:1–22. https://doi.org/10.1080/21678421.2016.1267768Raaphorst J., van Tol M.J., de Visser M., van der Kooi A.J., Majoie C.B., van den Berg L.H. et al. Prose memory impairment in amyotrophic lateral sclerosis patients is related to hippocampus volume. Eur J Neurol. 2015;22(3):547–54. https://doi.org/10.1111/ene.12615Raaphorst J., van Tol M.J., de Visser M., van der Kooi A.J., Majoie C.B., van den Berg L.H. et al. Prose memory impairment in amyotrophic lateral sclerosis patients is related to hippocampus volume. Eur J Neurol. 2015;22(3):547–54. https://doi.org/10.1111/ene.12615Abdulla S., Machts J., Kaufmann J., Patrick K., Kollewe K., Dengler R. et al. Hippocampal degeneration in patients with amyotrophic lateral sclerosis. Neurobiol Aging. 2014;35(11):2639–2645. https://doi.org/10.1016/j.neurobiolaging.2014.05.035Abdulla S., Machts J., Kaufmann J., Patrick K., Kollewe K., Dengler R. et al. Hippocampal degeneration in patients with amyotrophic lateral sclerosis. Neurobiol Aging. 2014;35(11):2639–2645. https://doi.org/10.1016/j.neurobiolaging.2014.05.035Witgert M., Salamone A.R., Strutt A.M., Jawaid A., Massman P.J., Bradshaw M. et al. Frontal-lobe mediated behavioral dysfunction in amyotrophic lateral sclerosis. European Journal of Neurology. 2010;17(1):103–11. http://dx.doi.org/10.1111/j.1468-1331.2009.02801.xWitgert M., Salamone A.R., Strutt A.M., Jawaid A., Massman P.J., Bradshaw M. et al. Frontal-lobe mediated behavioral dysfunction in amyotrophic lateral sclerosis. European Journal of Neurology. 2010;17(1):103–11. http://dx.doi.org/10.1111/j.1468-1331.2009.02801.xWoolley S.C., Strong M.J. Frontotemporal Dysfunction and Dementia in Amyotrophic Lateral Sclerosis. Neurol Clin. 2015;33(4):787–805. https://doi.org/10.1016/j.ncl.2015.07.011. Epub 2015 Sep 8. PMID: 26515622.Woolley S.C., Strong M.J. Frontotemporal Dysfunction and Dementia in Amyotrophic Lateral Sclerosis. Neurol Clin. 2015;33(4):787–805. https://doi.org/10.1016/j.ncl.2015.07.011. Epub 2015 Sep 8. PMID: 26515622.Burrell J.R., Halliday G.M., Kril J.J., Ittner L.M., Götz J., Kiernan M.C. et al. The frontotemporal dementia-motor neuron disease continuum. Lancet. 2016;388(10047):919–31. https://doi.org/10.1016/S0140-6736(16)00737-6. Epub 2016 Mar 14. PMID: 26987909.Burrell J.R., Halliday G.M., Kril J.J., Ittner L.M., Götz J., Kiernan M.C. et al. The frontotemporal dementia-motor neuron disease continuum. Lancet. 2016;388(10047):919–31. https://doi.org/10.1016/S0140-6736(16)00737-6. Epub 2016 Mar 14. PMID: 26987909.Agosta F., Canu E., Valsasina P., Riva N., Prelle A., Comi G. et al. Divergent brain network connectivity in amyotrophic lateral sclerosis. Neurobiol Aging. 2013;34(2):419–27. https://doi.org/10.1016/j.neurobiolaging.2012.04.015. Epub 2012 May 18. PMID: 22608240.Agosta F., Canu E., Valsasina P., Riva N., Prelle A., Comi G. et al. Divergent brain network connectivity in amyotrophic lateral sclerosis. Neurobiol Aging. 2013;34(2):419–27. https://doi.org/10.1016/j.neurobiolaging.2012.04.015. Epub 2012 May 18. PMID: 22608240.Lillo P., Mioshi E., Burrell J.R., Kiernan M.C., Hodges J.R., Hornberger M. Grey and white matter changes across the amyotrophic lateral sclerosis-frontotemporal dementia continuum. PLoS One. 2012;7(8):e43993. https://doi.org/10.1371/journal.pone.0043993. Epub 2012 Aug 29. PMID: 22952843; PMCID: PMC3430626.Lillo P., Mioshi E., Burrell J.R., Kiernan M.C., Hodges J.R., Hornberger M. Grey and white matter changes across the amyotrophic lateral sclerosis-frontotemporal dementia continuum. PLoS One. 2012;7(8):e43993. https://doi.org/10.1371/journal.pone.0043993. Epub 2012 Aug 29. PMID: 22952843; PMCID: PMC3430626.Oyanagi K., Makifuchi T., Ohtoh T., Chen K.M., van der Schaaf T., Gajdusek D.C. et al. Amyotrophic lateral sclerosis of Guam: the nature of the neuropathological fi ndings. Acta Neuropathol. 1994;88(5):405–12. https://doi.org/10.1007/BF00389491. PMID: 7847068.Oyanagi K., Makifuchi T., Ohtoh T., Chen K.M., van der Schaaf T., Gajdusek D.C. et al. Amyotrophic lateral sclerosis of Guam: the nature of the neuropathological fi ndings. Acta Neuropathol. 1994;88(5):405–12. https://doi.org/10.1007/BF00389491. PMID: 7847068.Hirano A., Kurland L., Krooth R., Lessell S. Parkinsonismdementia complex, an endemic disease on the island of Guam. I. Clinical features. Brain. 1961;84:642–61. https://doi.org/10.1093/brain/84.4.642. PMID: 13907609.Hirano A., Kurland L., Krooth R., Lessell S. Parkinsonismdementia complex, an endemic disease on the island of Guam. I. Clinical features. Brain. 1961;84:642–61. https://doi.org/10.1093/brain/84.4.642. PMID: 13907609.Hudson A.J. Amyotrophic lateral sclerosis and its association with dementia, parkinsonism and other neurological disorders: a review. Brain. 1981;104(2):217–47. https://doi.org/10.1093/brain/104.2.217. PMID: 7016254.Hudson A.J. Amyotrophic lateral sclerosis and its association with dementia, parkinsonism and other neurological disorders: a review. Brain. 1981;104(2):217–47. https://doi.org/10.1093/brain/104.2.217. PMID: 7016254.Delisle M.B., Gorce P., Hirsch E., Hauw J.J., Rascol A., Bouissou H. Motor neuron disease, parkinsonism and dementia. Report of a case with diffuse Lewy body-like intracytoplasmic inclusions. Acta Neuropathol. 1987;75(1):104–8. https://doi.org/10.1007/BF00686799. PMID: 3434209.Delisle M.B., Gorce P., Hirsch E., Hauw J.J., Rascol A., Bouissou H. Motor neuron disease, parkinsonism and dementia. Report of a case with diffuse Lewy body-like intracytoplasmic inclusions. Acta Neuropathol. 1987;75(1):104–8. https://doi.org/10.1007/BF00686799. PMID: 3434209.Frecker M.F., Fraser F.C., Andermann E., Pryse-Phillips W.E. Association between Alzheimer disease and amyotrophic lateral sclerosis? Can J Neurol Sci. 1990;17:12–14. https://doi.org/10.1017/S0317167100029942Frecker M.F., Fraser F.C., Andermann E., Pryse-Phillips W.E. Association between Alzheimer disease and amyotrophic lateral sclerosis? Can J Neurol Sci. 1990;17:12–14. https://doi.org/10.1017/S0317167100029942Müller M., Vieregge P., Reusche E., Ogomori K. Amyotrophic lateral sclerosis and frontal lobe dementia in Alzheimer’s disease. Case report and review of the literature. Eur Neurol. 1993;33(4):320–4. https://doi.org/10.1159/000116962. PMID: 8348920.Müller M., Vieregge P., Reusche E., Ogomori K. Amyotrophic lateral sclerosis and frontal lobe dementia in Alzheimer’s disease. Case report and review of the literature. Eur Neurol. 1993;33(4):320–4. https://doi.org/10.1159/000116962. PMID: 8348920.Hedera P., Lerner A.J., Castellani R., Friedland R.P. Concurrence of Alzheimer’s disease, Parkinson’s disease, diff use Lewy body disease, and amyotrophic lateral sclerosis. J Neurol Sci. 1995;128:219–224. https://doi.org/10.1016/0022-510X(94)00222-AHedera P., Lerner A.J., Castellani R., Friedland R.P. Concurrence of Alzheimer’s disease, Parkinson’s disease, diff use Lewy body disease, and amyotrophic lateral sclerosis. J Neurol Sci. 1995;128:219–224. https://doi.org/10.1016/0022-510X(94)00222-ADeng H.X., Chen W., Hong S.T., Boycott K.M., Gorrie G.H., Siddique N. et al. Mutations in UBQLN2 cause dominant Xlinked juvenile and adult-onset ALS and ALS/dementia. Nature. 2011;477(7363):211–5. https://doi.org/10.1038/nature10353. PMID: 21857683; PMCID: PMC3169705.Deng H.X., Chen W., Hong S.T., Boycott K.M., Gorrie G.H., Siddique N. et al. Mutations in UBQLN2 cause dominant Xlinked juvenile and adult-onset ALS and ALS/dementia. Nature. 2011;477(7363):211–5. https://doi.org/10.1038/nature10353. PMID: 21857683; PMCID: PMC3169705.Deng H.X., Hentati A., Tainer J.A., Iqbal Z., Cayabyab A., Hung W.Y. et al. Amyotrophic lateral sclerosis and structural defects in Cu,Zn superoxide dismutase. Science. 1993;261(5124):1047–51. https://doi.org/10.1126/science.8351519. PMID: 8351519.Deng H.X., Hentati A., Tainer J.A., Iqbal Z., Cayabyab A., Hung W.Y. et al. Amyotrophic lateral sclerosis and structural defects in Cu,Zn superoxide dismutase. Science. 1993;261(5124):1047–51. https://doi.org/10.1126/science.8351519. PMID: 8351519.Kwiatkowski TJ Jr, Bosco D.A., Leclerc A.L., Tamrazian E., Vanderburg C.R., Russ C. et al. Mutations in the FUS/TLS gene on chromosome 16 cause familial amyotrophic lateral sclerosis. Science. 2009;323(5918):1205–8. https://doi.org/10.1126/science.1166066. PMID: 19251627.Kwiatkowski TJ Jr, Bosco D.A., Leclerc A.L., Tamrazian E., Vanderburg C.R., Russ C. et al. Mutations in the FUS/TLS gene on chromosome 16 cause familial amyotrophic lateral sclerosis. Science. 2009;323(5918):1205–8. https://doi.org/10.1126/science.1166066. PMID: 19251627.Sreedharan J., Blair I.P., Tripathi V.B., Hu X., Vance C., Rogelj B. et al. TDP-43 mutations in familial and sporadic amyotrophic lateral sclerosis. Science. 2008;319(5870):1668–72. https://doi.org/10.1126/science.1154584. Epub 2008 Feb 28. PMID: 18309045.Sreedharan J., Blair I.P., Tripathi V.B., Hu X., Vance C., Rogelj B. et al. TDP-43 mutations in familial and sporadic amyotrophic lateral sclerosis. Science. 2008;319(5870):1668–72. https://doi.org/10.1126/science.1154584. Epub 2008 Feb 28. PMID: 18309045.Vance C., Rogelj B., Hortobágyi T., De Vos K.J., Nishimura A.L., Sreedharan J. et al. Mutations in FUS, an RNA processing protein, cause familial amyotrophic lateral sclerosis type 6. Science. 2009;323(5918):1208–1211. https://doi.org/10.1126/science.1165942. PMID: 19251628; PMCID: PMC4516382.Vance C., Rogelj B., Hortobágyi T., De Vos K.J., Nishimura A.L., Sreedharan J. et al. Mutations in FUS, an RNA processing protein, cause familial amyotrophic lateral sclerosis type 6. Science. 2009;323(5918):1208–1211. https://doi.org/10.1126/science.1165942. PMID: 19251628; PMCID: PMC4516382.Garruto R.M. Pacifi c paradigms of environmentally-induced neurological disorders: clinical, epidemiological and molecular perspectives. Neurotoxicology. 1991;12(3):347–77. PMID: 1745428.Garruto R.M. Pacifi c paradigms of environmentally-induced neurological disorders: clinical, epidemiological and molecular perspectives. Neurotoxicology. 1991;12(3):347–77. PMID: 1745428.Wang X., Blanchard J., Grundke-Iqbal I., Wegiel J., Deng H.X., Siddique T. et al. Alzheimer disease and amyotrophic lateral sclerosis: an etiopathogenic connection. Acta Neuropathol. 2014;127(2):243–56. https://doi.org/10.1007/s00401-013-1175-9. Epub 2013 Oct 18. PMID: 24136402; PMCID: PMC3947003.Wang X., Blanchard J., Grundke-Iqbal I., Wegiel J., Deng H.X., Siddique T. et al. Alzheimer disease and amyotrophic lateral sclerosis: an etiopathogenic connection. Acta Neuropathol. 2014;127(2):243–56. https://doi.org/10.1007/s00401-013-1175-9. Epub 2013 Oct 18. PMID: 24136402; PMCID: PMC3947003.Hamilton R.L., Bowser R. Alzheimer disease pathology in amyotrophic lateral sclerosis. Acta Neuropathol. 2004;107(6):515–22. https://doi.org/10.1007/s00401-004-0843-1. Epub 2004 Mar 16. PMID: 15024584.Hamilton R.L., Bowser R. Alzheimer disease pathology in amyotrophic lateral sclerosis. Acta Neuropathol. 2004;107(6):515–22. https://doi.org/10.1007/s00401-004-0843-1. Epub 2004 Mar 16. PMID: 15024584.Moglia C., Calvo A., Grassano M., Canosa A., Manera U., D’Ovidio F.,. Piemonte and Valle d’Aosta Register for ALS (PARALS). Early weight loss in amyotrophic lateral sclerosis: outcome relevance and clinical correlates in a population-based cohort. 2019;90(6):666–673. https://doi.org/10.1136/jnnp-2018-319611. Epub 2019 Jan 10. PMID: 30630957.Moglia C., Calvo A., Grassano M., Canosa A., Manera U., D’Ovidio F.,. Piemonte and Valle d’Aosta Register for ALS (PARALS). Early weight loss in amyotrophic lateral sclerosis: outcome relevance and clinical correlates in a population-based cohort. 2019;90(6):666–673. https://doi.org/10.1136/jnnp-2018-319611. Epub 2019 Jan 10. PMID: 30630957.van Es M.A., Hardiman O., Chio A., Al-Chalabi A., Pasterkamp R.J., Veldink J.H. et al. Amyotrophic lateral sclerosis. Lancet. 2017;390(10107):2084–2098. https://doi.org/10.1016/S0140-6736(17)31287-4. Epub 2017 May 25. PMID: 28552366.van Es M.A., Hardiman O., Chio A., Al-Chalabi A., Pasterkamp R.J., Veldink J.H. et al. Amyotrophic lateral sclerosis. Lancet. 2017;390(10107):2084–2098. https://doi.org/10.1016/S0140-6736(17)31287-4. Epub 2017 May 25. PMID: 28552366.Степкина Д.А., Захаров В.В., Яхно Н.Н. Синдром первичной прогрессирующей афазии. Неврологический журнал. 2014;19(5):22.Stepkina D.A., Zakharov V.V., Yakhno N.N. Primary progressive aphasia syndrome. The Neurological Journal. 2015;19(5):22. (In Russian). https://doi.org/10.18821/1560-9545-2014-19-5-22-28Mendez M.F., Lee A.S., Joshi A., Shapira J.S. Nonamnestic presentations of early-onset Alzheimer’s disease. Am J Alzheimers Dis Other Demen. 2012;27(6):413–20. https://doi.org/10.1177/1533317512454711. Epub 2012 Aug 7. PMID: 22871906; PMCID: PMC3625669.Mendez M.F., Lee A.S., Joshi A., Shapira J.S. Nonamnestic presentations of early-onset Alzheimer’s disease. Am J Alzheimers Dis Other Demen. 2012;27(6):413–20. https://doi.org/10.1177/1533317512454711. Epub 2012 Aug 7. PMID: 22871906; PMCID: PMC3625669.Mez J., Cosentino S., Brickman A.M., Huey E.D., Manly J.J., Mayeux R. Dysexecutive versus amnestic Alzheimer disease subgroups: analysis of demographic, genetic, and vascular factors. Alzheimer Dis Assoc Disord. 2013;27(3):218–25. https://doi.org/10.1097/WAD.0b013e31826a94bd. PMID: 23954887; PMCID: PMC3748394.Mez J., Cosentino S., Brickman A.M., Huey E.D., Manly J.J., Mayeux R. Dysexecutive versus amnestic Alzheimer disease subgroups: analysis of demographic, genetic, and vascular factors. Alzheimer Dis Assoc Disord. 2013;27(3):218–25. https://doi.org/10.1097/WAD.0b013e31826a94bd. PMID: 23954887; PMCID: PMC3748394.Коберская Н.Н., Ковальчук Н.А. Болезнь Альцгеймера с ранним дебютом. Медицинский совет. 2019;1:10–16.Koberskaya N.N., Kovalchuk N.A. Alzheimer’s disease with an early debut. Meditsinskiy sovet. 2019;(1):10–16. (In Russian). https://doi.org/10.21518/2079-701X-2019-1-10-16Rabinovici G.D., Rosen H.J., Alkalay A., Kornak J., Furst A.J., Agarwal N. et al. Amyloid vs FDG-PET in the diff erential diagnosis of AD and FTLD. Neurology. 2011;77(23):2034–42. https://doi.org/10.1212/WNL.0b013e31823b9c5e. Epub 2011 Nov 30. PMID: 22131541; PMCID: PMC3236517.Rabinovici G.D., Rosen H.J., Alkalay A., Kornak J., Furst A.J., Agarwal N. et al. Amyloid vs FDG-PET in the diff erential diagnosis of AD and FTLD. Neurology. 2011;77(23):2034–42. https://doi.org/10.1212/WNL.0b013e31823b9c5e. Epub 2011 Nov 30. PMID: 22131541; PMCID: PMC3236517.Taylor K.I., Probst A., Miserez A.R., Monsch A.U., Tolnay M. Clinical course of neuropathologically confirmed frontal-variant Alzheimer’s disease. Nat Clin Pract Neurol. 2008;4:226–32. http://dx.doi.org/10.1038/ncpneuro0746.Taylor K.I., Probst A., Miserez A.R., Monsch A.U., Tolnay M. Clinical course of neuropathologically confirmed frontal-variant Alzheimer’s disease. Nat Clin Pract Neurol. 2008;4:226–32. http://dx.doi.org/10.1038/ncpneuro0746.McKhann G.M., Knopman D.S., Chertkow H., Hyman B.T., Jack C.R. Jr, Kawas C.H. et al. The diagnosis of dementia due to Alzheimer’s disease: recommendations from the National Institute on Aging-Alzheimer’s Association workgroups on diagnostic guidelines for Alzheimer’s disease. Alzheimers Dement. 2011;7(3):263–9. https://doi.org/10.1016/j.jalz.2011.03.005. Epub 2011 Apr 21. PMID: 21514250; PMCID: PMC3312024.McKhann G.M., Knopman D.S., Chertkow H., Hyman B.T., Jack C.R. Jr, Kawas C.H. et al. The diagnosis of dementia due to Alzheimer’s disease: recommendations from the National Institute on Aging-Alzheimer’s Association workgroups on diagnostic guidelines for Alzheimer’s disease. Alzheimers Dement. 2011;7(3):263–9. https://doi.org/10.1016/j.jalz.2011.03.005. Epub 2011 Apr 21. PMID: 21514250; PMCID: PMC3312024.Rascovsky K., Hodges J.R., Knopman D., Mendez M.F., Kramer J.H., Neuhaus J. et al. Sensitivity of revised diagnostic criteria for the behavioural variant of frontotemporal dementia. Brain. 2011;134(Pt9):2456–77. https://doi.org/10.1093/brain/awr179. Epub 2011 Aug 2. PMID: 21810890; PMCID: PMC3170532.Rascovsky K., Hodges J.R., Knopman D., Mendez M.F., Kramer J.H., Neuhaus J. et al. Sensitivity of revised diagnostic criteria for the behavioural variant of frontotemporal dementia. Brain. 2011;134(Pt9):2456–77. https://doi.org/10.1093/brain/awr179. Epub 2011 Aug 2. PMID: 21810890; PMCID: PMC3170532.Гришина Д.А., Яхно Н.Н., Захаров В.В. Некогнитивные нервнопсихические нарушения при синдроме первичной прогрессирующей афазии. Неврологический журнал. 2014;19(6):17.Grishina D.A., Yakhno N.N., Zakharov V.V. Non-cognitive neuropsychiatric disorders in patients with primary progressive aphasia syndrome. The Neurological Journal. 2014;19(6):17. (In Russian). https://doi.org/10.18821/1560-9545-2014-19-6-17-22Степкина Д.А., Захаров В.В., Яхно Н.Н. Лобно-височная дегенерация (обзор литературы и собственные наблюдения). Доктор.Ру. 2013;5(83):44–50.Styopkina D.A., Zakharov V.V., Yakhno N.N. Frontotemporal Lobar Degeneration: Review of Literature and Own Observations. Doctor.ru. 2013;5(83):44–50. (In Russian)

The authors declare that there are no conflicts of interest present.