Cognitive functioning in patients with Chiari malformation type I and relationship with neurological manifestations

Cover Page


Cite item

Full Text

Open Access Open Access
Restricted Access Access granted
Restricted Access Subscription or Fee Access

Abstract

BACKGROUND. The symptom complex of Chiari malformation type I (CMI) is traditionally characterized by the set of cerebrospinal fluid disorders, coordination disorders, brainstem and myelopathic manifestations. Cognitive impairments, as manifestations of CMI, are little-known area. Study of the characteristics of cognitive status in patients with CMI and analysis of relationship between cognitive phenotype and severity of neurological manifestations can become basis for the formation of a more detailed understanding of this pathology.

AIM. The aim was to characterize complex of the neuro-neuropsychological spectrum symptoms and their relationship in patients with CMI.

MATERIALS AND METHODS. The main group included 105 adult patients with CMI — 74 (70.5%) men and 31 (29.5%) women aged 25.61±6.89 years. The control group included 50 patients without signs of CMI, other organic brain pathology and cognitive disorders of known etiology — 31 (62%) men and 19 (38%) women aged 26.36±5.00 years. The neuroimaging verification of CMI was performed by magnetic resonance imaging of the brain. Clinical assessment consisted of the collecting complaints, anamnestic information and detailed neurological examination. Comprehensive neuropsychological testing was performed by using MMSE, MoCA and TMT (A and B).

RESULTS. Dissociation was noted between prevalence of the typical subjective symptoms and frequency of the objective neurological manifestations in patients with CMI. Among the most frequent subjective symptoms in CMI were headaches and neck pain, asthenia, disorders of the coordination sphere, as well as subjective signs of cognitive dysfunction. Objective neurological deficits were mainly represented by cerebellar disorders. Signs of cognitive dysfunction in the form of memory and attention disorders described by patients with CMI in 20% cases were confirmed by the results of complex neuropsychological testing in comparison with a group of healthy respondents. The relationship of the cognitive deficits with presence and severity of the objective neurological, mainly cerebellar, manifestations in patients with CMI was revealed.

CONCLUSIONS. The relationship of cognitive dysfunction with the presence and severity of objective neurological, mainly cerebellar, disorders indicate the complexity of neurological and neuropsychological manifestations in CMI. These data suggest an important role of the disturbed anatomy of the cerebellum and its connections with the cerebral cortex in the pathophysiology of a wide range of major clinical manifestations of CMI.

Full Text

Restricted Access

About the authors

Radmila G. Kokurkina

Kazan State Medical University

Author for correspondence.
Email: rada_nell@mail.ru
ORCID iD: 0000-0002-3182-8009
SPIN-code: 4859-3668

Postgraduate Student, Assistant, Depart. of Neurology and Rehabilitation

Russian Federation, Kazan

Elena G. Mendelevich

Kazan State Medical University

Email: emendel@mail.ru
ORCID iD: 0000-0002-6829-7942
SPIN-code: 5970-6926

M.D., D. Sci. (Med.), Prof., Depart. of Neurology and Rehabilitation

Russian Federation, Kazan

References

  1. Krupina NE. The development of ideas about Chiari malformation (literature review). Bulletin of the Ural State Medical University. 2016;1–2:87–92. (In Russ.)
  2. Holly LT, Batzdorf U. Chiari malformation and syringomyelia. J Neurosurg Spine. 2019;31(5):619–628. doi: 10.3171/2019.7.SPINE181139.
  3. Bogdanov EI. Dislocations of the cerebellar tonsils in the large occipital foramen and the spectrum of Chiari malformations type 1. Journal of Neurology and Psychiatry named after SS Korsakov. 2022;122(4):7–15. doi: 10.17116/jnevro20221220417.
  4. Smith BW, Strahle J, Bapuraj JR et al. Distribution of cerebellar tonsil position: Implications for understanding Chiari malformation. J Neurosurg. 2013;119(3):812–819. doi: 10.3171/2013.5.JNS121825.
  5. McClugage SG, Oakes WJ. The Chiari I malformation. J Neurosurg Pediatr. 2019;24(3):217–226. doi: 10.3171/2019.5.PEDS18382.
  6. Surzhenko IL, Mendelevich EG. Symptoms of isolated Chiari malformation 1 and combined with syringomyelia. Kazan Medical Journal. 2009;90(1):23–26. (In Russ.)
  7. Dunin DN, Mendelevich EG. Coordination function disorders and their evaluation in patients with Chiari malformation type 1. Neurology, Neuropsychiatry, Psychosomatics. 2012;4(4):44–47. (In Russ.)
  8. Mueller DM, Oro’ JJ. Prospective analysis of presenting symptoms among 265 patients with radiographic evidence of Chiari malformation type I with or without syringomyelia. J Am Acad Nurse Pract. 2004;16(3):134–138. doi: 10.1111/j.1745-7599.2004.tb00384.x.
  9. Fischbein R, Saling JR, Marty P et al. Patient-reported Chiari malformation type I symptoms and diagnostic experiences: A report from the national Conquer Chiari Patient Registry database. Neurol Sci. 2015;36(9):1617–1624. doi: 10.1007/s10072-015-2219-9.
  10. García M, Lázaro E, Amayra I et al. Analysis of visuospatial abilities in Chiari malformation type I. Cerebellum. 2020;19(1):6–15. doi: 10.1007/s12311-019-01056-y.
  11. Besteiro González JL, Torres Campa-Santamarina JM. Anomalies in the cognitive-executive functions in patients with Chiari malformation type I. Psicothema. 2018;30(3):316–321. doi: 10.7334/psicothema2017.401.
  12. Almotairi FS, Hellström P, Skoglund T et al. Chiari I malformation — neuropsychological functions and quality of life. Acta Neurochir (Wien). 2020;162(7):1575–1582. doi: 10.1007/s00701-019-03897-2.
  13. Kumar M, Rathore RK, Srivastava A et al. Correlation of diffusion tensor imaging metrics with neurocognitive function in Chiari I malformation. World Neurosurg. 2011;76(1–2):189–194. doi: 10.1016/j.wneu.2011.02.022.
  14. Houston ML, Houston JR, Sakaie K et al. Functional connectivity abnormalities in type I Chiari: Associations with cognition and pain. Brain Commun. 2021;3(3):137. doi: 10.1093/braincomms/fcab137.
  15. Klein R, Hopewell CA, Oien M. Chiari malformation type I: A neuropsychological case study. Mil Med. 2014;179(6):712–718. doi: 10.7205/MILMED-D-13-00227.
  16. Lacy M, Parikh S, Costello R et al. Neurocognitive functioning in unoperated adults with Chiari malformation type I. World Neurosurg. 2019;126:641–645. doi: 10.1016/j.wneu.2019.02.105.
  17. Lacy M, Ellefson SE, DeDios-Stern S, Frim DM. Parent-reported executive dysfunction in children and adolescents with Chiari malformation type 1. Pediatr Neurosurg. 2016;51(5):236–243. doi: 10.1159/000445899.
  18. Bègue I, Brakowski J, Seifritz E et al. Cerebellar and cortico-striatal-midbrain contributions to reward-cognition processes and apathy within the psychosis continuum. Schizophr Res. 2022;246:85–94. doi: 10.1016/j.schres.2022.06.010.
  19. Naeije G, Rai M, Allaerts N et al. Cerebellar cognitive disorder parallels cerebellar motor symptoms in Friedreich ataxia. Ann Clin Transl Neurol. 2020;7(6):1050–1054. doi: 10.1002/acn3.51079.
  20. Maas RPPWM, Killaars S, van de Warrenburg BPC, Schutter DJLG. The cerebellar cognitive affective syndrome scale reveals early neuropsychological deficits in SCA3 patients. J Neurol. 2021;268(9):3456–3466. doi: 10.1007/s00415-021-10516-7.

Supplementary files

Supplementary Files
Action
1. JATS XML
Download
2. Fig. 1. The characteristics and incidence of subjective symptoms in patients with Chiari malformation type 1.

Download (205KB)
Indexing metadata
3. Fig. 2. The characteristics and incidence of objective neurological symptoms in patients with Chiari malformation type 1.

Download (134KB)
Indexing metadata

Copyright (c) 2023 Eco-Vector


СМИ зарегистрировано Федеральной службой по надзору в сфере связи, информационных технологий и массовых коммуникаций (Роскомнадзор).
Регистрационный номер и дата принятия решения о регистрации СМИ: серия ПИ № ФС 77 - 75562 от 12 апреля 2019 года.


This website uses cookies

You consent to our cookies if you continue to use our website.

About Cookies