r-n-jРоссийский неврологический журналRussian neurological journal2658-79472686-7192МИА10.30629/2658-7947-2025-30-1-40-45r-n-j-664Research ArticleИССЛЕДОВАНИЯ И КЛИНИЧЕСКИЕ НАБЛЮДЕНИЯCLINICAL RESEARCHES AND CASE REPORTSСочетание бокового амиотрофического склероза с симптомами прогрессирующего надъядерного параличаComorbidity of amyotrophic lateral sclerosis with the symptoms of progressive supranuclear palsyhttps://orcid.org/0000-0002-9352-5783ПервушинаЕ. В.PervushinaE. V.

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ekaterina-pervushina@yandex.ruhttps://orcid.org/0000-0003-1001-2024КутлубаевМ. А.KutlubaevМ. А.

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mansur.kutlubaev@yahoo.comhttps://orcid.org/0000-0001-9949-9582КачемаеваО. В.KachemaevaO. V.

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olga.kachemaeva@gmail.comhttps://orcid.org/0000-0002-8199-0741ЗакироваЭ. Н.ZakirovaE. N.

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lvira_z2003@mail.ruБашкирский государственный медицинский университетРоссияBashkir State Medical UniversityRussian FederationРеспубликанская клиническая больница им. Г.Г. КуватоваРоссияRepublican Clinical Hospital named after G.G. KuvatovRussian Federation2025100520253014045Copyright © Первушина Е.В., Кутлубаев М.А., Качемаева О.В., Закирова Э.Н., 20252025Первушина Е.В., Кутлубаев М.А., Качемаева О.В., Закирова Э.Н.Pervushina E.V., Kutlubaev М.А., Kachemaeva O.V., Zakirova E.N.This work is licensed under a Creative Commons Attribution 4.0 License.https://www.r-n-j.com/jour/article/view/664

Клинически значимые глазодвигательные нарушения нехарактерны для бокового амиотрофического склероза (БАС). Представлен случай одновременного развития классических симптомов БАС и прогрессирующего надъядерного паралича в виде пареза вертикального взора, деменции и экстрапирамидных нарушений. Проанализированы потенциальные механизмы развития глазодвигательных нарушений при БАС.

Clinically significant oculomotor disorders are not typical for amyotrophic lateral sclerosis (ALS). A case of simultaneous development of classic symptoms of ALS and progressive supranuclear palsy in the form of vertical gaze palsy, dementia and extrapyramidal features is presented. Potential mechanisms of the development of oculomotor disorders in ALS are discussed.

боковой амиотрофический склерозболезнь двигательного нейронапрогрессирующий надъядерный параличглазодвигательные нарушенияamyotrophic lateral sclerosismotor neuron diseaseprogressive supranuclear palsyoculomotor disordersРабота выполнена за счет средств Программы стратегического академического лидерства Башкирского государственного медицинского университета (ПРИОРИТЕТ-2030).This work was supported by the Bashkir State Medical University Strategic Academic Leadership Program (PRIORITY-2030).ReferencesYounger DS, Brown RH Jr. Amyotrophic lateral sclerosis. Handb Clin Neurol. 2023;196:203–229. https://doi.org/10.1016/B978-0-323-98817-9.00031-4Younger DS, Brown RH Jr. Amyotrophic lateral sclerosis. Handb Clin Neurol. 2023;196:203–229. https://doi.org/10.1016/B978-0-323-98817-9.00031-4Mizutani T, Aki M, Shiozawa R, Unakami M, Nozawa T, Yajima K, Tanabe H, Hara M. Development of ophthalmoplegia in amyotrophic lateral sclerosis during long-term use of respirators. J Neurol Sci. 1990;99(2-3):311–9. https://doi.org/10.1016/0022-510x(90)90165-jMizutani T, Aki M, Shiozawa R, Unakami M, Nozawa T, Yajima K, Tanabe H, Hara M. Development of ophthalmoplegia in amyotrophic lateral sclerosis during long-term use of respirators. J Neurol Sci. 1990;99(2-3):311–9. https://doi.org/10.1016/0022-510x(90)90165-jAust E, Graupner ST, Günther R, Linse K, Joos M, Grosskreutz J, Prudlo J, Pannasch S, Hermann A. Impairment of oculomotor functions in patients with early to advanced amyotrophic lateral sclerosis. J Neurol. 2024;271(1):325–339. https://doi.org/10.1007/s00415-023-11957-yAust E, Graupner ST, Günther R, Linse K, Joos M, Grosskreutz J, Prudlo J, Pannasch S, Hermann A. Impairment of oculomotor functions in patients with early to advanced amyotrophic lateral sclerosis. J Neurol. 2024;271(1):325–339. https://doi.org/10.1007/s00415-023-11957-yKutlubaev MA, Areprintseva DK, Radakovic R, Pervushina EV. Psychometric properties of the Russian version of the Edinburgh cognitive and behavioral amyotrophic lateral sclerosis screen. Amyotroph Lateral Scler Frontotemporal Degener. 2024;25(7-8):785–787. https://doi.org/10.1080/21678421.2024.2328579Kutlubaev MA, Areprintseva DK, Radakovic R, Pervushina EV. Psychometric properties of the Russian version of the Edinburgh cognitive and behavioral amyotrophic lateral sclerosis screen. Amyotroph Lateral Scler Frontotemporal Degener. 2024;25(7-8):785–787. https://doi.org/10.1080/21678421.2024.2328579Zejlon C, Sennfält S, Finnsson J, Connolly B, Petersson S, Granberg T, Ingre C. Motor band sign is specific for amyotrophic lateral sclerosis and corresponds to motor symptoms. Ann Clin Transl Neurol. 2024;11(5):1280–1289. https://doi.org/10.1002/acn3.52066Zejlon C, Sennfält S, Finnsson J, Connolly B, Petersson S, Granberg T, Ingre C. Motor band sign is specifi c for amyotrophic lateral sclerosis and corresponds to motor symptoms. Ann Clin Transl Neurol. 2024;11(5):1280–1289. https://doi.org/10.1002/acn3.52066Turner MR. UK MND Clinical Studies Group. Diagnosing ALS: the Gold Coast criteria and the role of EMG. Pract Neurol. 2022;22(3):176–178. https://doi.org/10.1136/practneurol-2021-003256.Turner MR. UK MND Clinical Studies Group. Diagnosing ALS: the Gold Coast criteria and the role of EMG. Pract Neurol. 2022;22(3):176–178. https://doi.org/10.1136/practneurol-2021-003256.Longinetti E, Fang F. Epidemiology of amyotrophic lateral sclerosis: an update of recent literature. Curr Opin Neurol. 2019;32(5):771–776. https://doi.org/10.1097/WCO.0000000000000730Longinetti E, Fang F. Epidemiology of amyotrophic lateral sclerosis: an update of recent literature. Curr Opin Neurol. 2019;32(5):771–776. https://doi.org/10.1097/WCO.0000000000000730Федорова Н.В., Бриль Е.В., Кулуа Т.К., Михайлова А.Д. Прогрессирующий надъядерный паралич. Журнал неврологии и психиатрии им. С.С. Корсакова. 2021;121(5):111–119.Fedorova NV, Bril EV, Kulua TK, Mikhaylova AD. Progressive supranuclear palsy. S.S. Korsakov Journal of Neurology and Psychiatry. 2021;121(5):111–119. (In Russ.). https://doi.org/10.17116/jnevro2021121051111Donaghy C, Thurtell MJ, Pioro EP, Gibson JM, Leigh RJ. Eye movements in amyotrophic lateral sclerosis and its mimics: a review with illustrative cases. J Neurol Neurosurg Psychiatry. 2011;82(1):110–6. https://doi.org/10.1136/jnnp.2010.212407Donaghy C, Thurtell MJ, Pioro EP, Gibson JM, Leigh RJ. Eye movements in amyotrophic lateral sclerosis and its mimics: a review with illustrative cases. J Neurol Neurosurg Psychiatry. 2011;82(1):110–6. https://doi.org/10.1136/jnnp.2010.212407Fujita K, Matsubara T, Miyamoto R, Sumikura H, Takeuchi T, Maruyama Saladini K, Kawarai T, Nodera H, Udaka F, Kume K, Morino H, Kawakami H, Hasegawa M, Kaji R, Murayama S, Izumi Y. Co-morbidity of progressive supranuclear palsy and amyotrophic lateral sclerosis: a clinical-pathological case report. BMC Neurol. 2019;19(1):168. https://doi.org/10.1186/s12883-019-1402-7Fujita K, Matsubara T, Miyamoto R, Sumikura H, Takeuchi T, Maruyama Saladini K, Kawarai T, Nodera H, Udaka F, Kume K, Morino H, Kawakami H, Hasegawa M, Kaji R, Murayama S, Izumi Y. Co-morbidity of progressive supranuclear palsy and amyotrophic lateral sclerosis: a clinical-pathological case report. BMC Neurol. 2019;19(1):168. https://doi.org/10.1186/s12883-019-1402-7McCluskey LF, Elman LB, Martinez-Lage M, Van Deerlin V, Yuan W, Clay D, Siderowf A, Trojanowski JQ. Amyotrophic lateral sclerosis-plus syndrome with TAR DNA-binding protein-43 pathology. Arch Neurol. 2009;66(1):121–4. https://doi.org/10.1001/archneur.66.1.12McCluskey LF, Elman LB, Martinez-Lage M, Van Deerlin V, Yuan W, Clay D, Siderowf A, Trojanowski JQ. Amyotrophic lateral sclerosis-plus syndrome with TAR DNA-binding protein-43 pathology. Arch Neurol. 2009;66(1):121–4. https://doi.org/10.1001/archneur.66.1.12Zhang X, Gao J, Chi C, Zhao Z, Chan P, Ma J. An atypical ALS with PSP-like symptoms caused by ANXA11 p.D40G mutation: A case report and literature review. Front Neurol. 2023;14:1086264. https://doi.org/10.3389/fneur.2023.1086264Zhang X, Gao J, Chi C, Zhao Z, Chan P, Ma J. An atypical ALS with PSP-like symptoms caused by ANXA11 p.D40G mutation: A case report and literature review. Front Neurol. 2023;14:1086264. https://doi.org/10.3389/fneur.2023.1086264Tokushige, S.-i., Terao, Y., Matsuda, S.-i., Inomata-Terada, S., Shimizu, T., Tanaka, N., Hamada, M., Yugeta, A., Hanajima, R., Mori, H., Tsuji, S. and Ugawa, Y. (2016), Motor neuron disease with saccadic abnormalities similar to progressive supranuclear palsy. Neurol Clin Neurosci, 4:146–152. https://doi.org/10.1111/ncn3.12062Tokushige, S.-i., Terao, Y., Matsuda, S.-i., Inomata-Terada, S., Shimizu, T., Tanaka, N., Hamada, M., Yugeta, A., Hanajima, R., Mori, H., Tsuji, S. and Ugawa, Y. (2016), Motor neuron disease with saccadic abnormalities similar to progressive supranuclear palsy. Neurol Clin Neurosci, 4:146–152. https://doi.org/10.1111/ncn3.12062Maugeri G, D’Amico AG, Morello G, Reglodi D, Cavallaro S, D’Agata V. Differential Vulnerability of Oculomotor Versus Hypoglossal Nucleus During ALS: Involvement of PACAP. Front Neurosci. 2020;14:805. https://doi.org/10.3389/fnins.2020.00805Maugeri G, D’Amico AG, Morello G, Reglodi D, Cavallaro S, D’Agata V. Differential Vulnerability of Oculomotor Versus Hypoglossal Nucleus During ALS: Involvement of PACAP. Front Neurosci. 2020;14:805. https://doi.org/10.3389/fnins.2020.00805Sharma R, Hicks S, Berna CM, Kennard C, Talbot K, Turner MR. Oculomotor dysfunction in amyotrophic lateral sclerosis: a comprehensive review. Arch Neurol. 2011;68(7):857–61. https://doi.org/10.1001/archneurol.2011.130Sharma R, Hicks S, Berna CM, Kennard C, Talbot K, Turner MR. Oculomotor dysfunction in amyotrophic lateral sclerosis: a comprehensive review. Arch Neurol. 2011;68(7):857–61. https:// doi.org/10.1001/archneurol.2011.130Youn CE, Lu C, Cauchi J, MacGowan D, Morgenstern R, Scelsa S. Oculomotor Dysfunction in Motor Neuron Disease. J Neuromuscul Dis. 2023;10(3):405–410. https://doi.org/10.3233/JND-221579Youn CE, Lu C, Cauchi J, MacGowan D, Morgenstern R, Scelsa S. Oculomotor Dysfunction in Motor Neuron Disease. J Neuromuscul Dis. 2023;10(3):405–410. https://doi.org/10.3233/JND-221579

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