|Generate QR code
Open Access
Subscription Access
Comorbidity of amyotrophic lateral sclerosis with the symptoms of progressive supranuclear palsy
https://doi.org/10.30629/2658-7947-2025-30-1-40-45
Abstract
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.
Keywords
amyotrophic lateral sclerosis,
motor neuron disease,
progressive supranuclear palsy,
oculomotor disorders
Supporting agencies: This work was supported by the Bashkir State Medical University Strategic Academic Leadership Program (PRIORITY-2030).
About the Authors
E. V. Pervushina
Bashkir State Medical University
Russian Federation
Russian Federation
Ufa
М. А. Kutlubaev
Bashkir State Medical University
Russian Federation
Russian Federation
Ufa
O. V. Kachemaeva
Bashkir State Medical University
Russian Federation
Russian Federation
Ufa
E. N. Zakirova
Republican Clinical Hospital named after G.G. Kuvatov
Russian Federation
Russian Federation
Ufa
References
1. Younger DS, Brown RH Jr. Amyotrophic lateral sclerosis. Handb Clin Neurol. 2023;196:203–229. https://doi.org/10.1016/B978-0-323-98817-9.00031-4
2. Mizutani 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-j
3. Aust 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-y
4. Kutlubaev 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/10.1080/21678421.2024.2328579
5. Zejlon 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/acn3.52066
6. 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.
7. 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.0000000000000730
8. 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/jnevro2021121051111
9. Donaghy 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.2010.212407
10. Fujita 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/s12883-019-1402-7
11. McCluskey 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.66.1.12
12. Zhang 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.2023.1086264
13. Tokushige, 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/ncn3.12062
14. Maugeri 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.2020.00805
15. Sharma 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.2011.130
16. Youn 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
Review
For citations:
Pervushina E.V., Kutlubaev М.А., Kachemaeva O.V., Zakirova E.N. Comorbidity of amyotrophic lateral sclerosis with the symptoms of progressive supranuclear palsy. Russian neurological journal. 2025;30(1):40-45. (In Russ.) https://doi.org/10.30629/2658-7947-2025-30-1-40-45
Views:
64
Email this article 