Objectives. To study the clinical features of mental impairments in amyotrophic lateral sclerosis (ALS). Materials and methods. A total of 27 patients with lesions to central and peripheral motoneurons (nine men and 18 women) were investigated. Patients’ mean age was 59.8 ± 11.9 years. Mean duration of illness at the moment of investigation was 3.2 ± 2.4 years. Clinical-psychopathological and clinical follow-up methods were used. Quantitative assessment of the severity of cognitive impairments were obtained using the Edinburgh Cognitive and Behavioral ALS Screen (ECAS) and depressive impairments on the Montgomery– Asberg Depression Rating Scale (MADRS). Results. Cognitive impairments were found in 88.9% of subjects. Mnestic impairments were predominantly hypo- and dysmnesia. Thinking speed in these patients was slow, and torpidity was combined with circumstantiality and a tendency to excess detail. Attention on the background of general instability was characterized by a decrease in volume, an increase in distractibility, and difficulty concentrating. At the late stages of illness, there was an overall decline in the level of thinking and intellect, with marked perseveration and an inability to make appropriate self-assessment. More than half the patients showed symptoms of dynamic aphasia, as well as impairments to gnosis and praxis. Most (92.3%) patients showed affective impairments. Increases in the severity of symptomatology of the main neurological disease were linked with associated depressive symptomatology coming to the fore (62.9%). Particular attention should be paid to clinical cases of ALS associated with psychotic disorders in the form of acute schizophrenia-like psychotic states. Conclusions. The data obtained here provide evidence of affective, cognitive, and behavioral impairments, along with a specific neuroimaging picture, the totality of which constituted a manifestation of the behavioral variant of frontotemporal dementia. This supports the current view that ALS and frontotemporal dementia have much in common, which allows these diseases to be regarded as different phenotypic manifestations of single pathological process.
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References
A. Kurt, F. Nijboer, T. Matuz, and A. Kübler, “Depression and anxiety in individuals with amyotrophic lateral sclerosis,” CNS Drugs, 21, No. 4, 279–291 (2007), https://doi.org/10.1007/springerreference_73533.
M. H. Maurer, “Amyotrophic lateral sclerosis: an introduction to treatment and trials,” in: Amyotrophic Lateral Sclerosis, M. H. Maurer (ed.), InTechOpen, London (2012), pp. 4–8, https://doi.org/10.5772/34020.
G. N. Levitskii, A. S. Levitskii, and V. M. Gilod, “Mental disorders in patients with amyotrophic lateral sclerosis and their family members,” Zh. Nevrol. Psikhiatr., 115, No. 2, 64–67 (2015), https://doi.org/10.17116/jnevro20151152164-67.
K. Abe, “Cognitive function in amyotrophic lateral sclerosis,” J. Neurol. Sci., 148, No. 1, 95–100 (1997), https://doi.org/10.1080/146608200300080453.
S. Zago, B. Poletti, C. Morelli, et al., “Amyotrophic lateral sclerosis and frontotemporal dementia (ALS-FTD),” Arch. Ital. Biol., 149, No. 1, 39–56 (2011), https://doi.org/10.4449/aib.v149i1.1263.
G. M. Ringholz, S. H. Appel, M. Bradshaw, et al., “Prevalence and patterns of cognitive impairment in sporadic ALS,” Neurology, 65, No. 4, 586–590 (2005), https://doi.org/10.1212/01.wnl.0000172911.39167.b6.
M. Landqvist Waldö, L. Gustafson, U. Passant, and E. Englund, “Psychotic symptoms in frontotemporal dementia: a diagnostic dilemma?” Int. Psychogeriatr., 27, No. 4, 531–539 (2015), https://doi.org/10.1017/S1041610214002580.
J. Phukan, M. Elamin, P. Bede, et al., “The syndrome of cognitive impairment in amyotrophic lateral sclerosis: a population-based study,” J. Neurol. Neurosurg. Psychiatry, 83, No. 1, 102–108 (2011), https://doi.org/10.1136/jnnp-2011-300188.
P. Lillo, B. Garcin, M. Hornberger, et al., “Neurobehavioral features in frontotemporal dementia with amyotrophic lateral sclerosis,” Arch. Neurol., 67, No. 7, 826–830 (2010), https://doi.org/10.1001/archneurol.2010.146.
E. Solje, H. Aaltokallio, H. Koivumaa-Honkanen, et al., “The phenotype of the C9ORF72 expansion carriers according to revised criteria for bvFTD,” PloS One, 10, No. 7, 0131817 (2015), https://doi.org/10.1371/journal.pone.0131817.
J. J. Cooper and F. Ovsiew, “The relationship between schizophrenia and frontotemporal dementia,” J. Geriatr. Psychiatry Neurol., 26, No. 3, 131–137 (2013), https://doi.org/10.1177/0891988713490992.
M. J. Strong, G. M. Grace, M. Freedman, et al., “Consensus criteria for the diagnosis of frontotemporal cognitive and behavioural syndromes in amyotrophic lateral sclerosis,” Amyotroph. Lateral Scler., 10, No. 3, 131–146 (2009), https://doi.org/10.1080/17482960802654364.
B. L. Santos, G. R. Rodrigues, V. Tumas, and J. E. Pittella, “Behavioral changes on amyotrophic lateral sclerosis (ALS, a case of ALS/ FTD TDP-43 proteinopathy),” Arquivos de Neuro-Psiquiatria, 70, No. 3, 232–233 (2012), https://doi.org/10.1590/S0004-282X2012000300018.
I. R. A. Mackenzie, R. Rademakers, and M. Neumann, “TDP-43 and FUS in amyotrophic lateral sclerosis and frontotemporal dementia,” Lancet Neurol., 9, No. 10, 995–1007 (2010), https://doi.org/10.1016/S1474-4422(10)70195-2.
S. Shinagawa, S. Nakajima, E. Plitman, et al., “Psychosis in frontotemporal dementia,” J. Alzheimers Dis., 42, No. 2, 485–499 (2014), https://doi.org/10.3233/JAD-140312.
F. Vanderzeypen, J. C. Bier, C. Genevrois, et al., “Frontal dementia or dementia praecox? A case report of a psychotic disorder with a severe decline,” L’Encephale, 29, No. 2, 172–180 (2003).
C. J. Kerssens, W. A. Krudop, N. D. Prins, et al., “Schizophrenia as a mimic of behavioral variant frontotemporal dementia,” Neurocase, 22, No. 3, 285–288 (2016), https://doi.org/10.1080/13554794.2016.1187178.
A. C. Holm, “Neurodegenerative and psychiatric overlap in frontotemporal lobar degeneration: a case of familial frontotemporal dementia presenting with catatonia,” Int. Psychogeriatr., 26, No. 2, 345–347 (2014), https://doi.org/10.1017/S1041610213001403.
B. K. Khan, J. D. Woolley, S. Chao, et al., “Schizophrenia or neurodegenerative disease prodrome? Outcome of a first psychotic episode in a 35-year-old woman,” Psychosomatics, 53, No. 3, 280–284 (2012), https://doi.org/10.1016/j.psym.2011.04.005.
R. E. Snow and S. E. Arnold, “Psychosis in neurodegenerative disease,” Sem. Clin. Neuropsychiatry, 1, No. 4, 282–293 (1996).
Y. Utumi, E. Iseki, and H. Arai, “Three patients with mood disorders showing catatonia and frontotemporal lobes atrophy,” Psychogeriatrics, 13, No. 4, 254–259 (2013), https://doi.org/10.1111/psyg.12027.
D. Galimberti, C. Fenoglio, M. Serpente, et al., “Autosomal dominant frontotemporal lobar degeneration due to the C9ORF72 hexanucleotide repeat expansion: late-onset psychotic clinical presentation,” Biol. Psychiatry, 74, No. 5, 384–391 (2013), https://doi.org/10.1016/j.biopsych.2013.01.031.
S. C. Lanata and B. L. Miller, “The behavioural variant frontotemporal dementia (bvFTD) syndrome in psychiatry,” J Neurol. Neurosurg. Psychiatry, 87, No. 5, 501–511 (2016), https://doi.org/10.1136/jnnp-2015-310697.
G. S. Grewal, S. Kanagasundram, and S. Jambunathan, “Schizophrenia or frontotemporal dementia in a young Chinese female: A purview of possible diagnoses,” Turk. Psikiyatri Derg., 22, No. 4, 266 (2011).
J. D. Woolley, B. K. Khan, N. K. Murthy, et al., “The diagnostic challenge of psychiatric symptoms in neurodegenerative disease; rates of and risk factors for prior psychiatric diagnosis in patients with early neurodegenerative disease,” J. Clin. Psychiatry, 72, No. 2, 126 (2011), https://doi.org/10.4088/JCP.10m06382oli.
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Translated from Zhurnal Nevrologii i Psikhiatrii imeni S. S. Korsakova, Vol. 122, No. 3, Iss. 1, pp. 59–64, March, 2022.
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Butylin, D.Y., Krylov, V.I., Chesnokova, O.I. et al. Clinical Features of Mental Impairments in Amyotrophic Lateral Sclerosis. Neurosci Behav Physi 52, 982–986 (2022). https://doi.org/10.1007/s11055-022-01326-z
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DOI: https://doi.org/10.1007/s11055-022-01326-z