Chiari I malformation—neuropsychological functions and quality of life

  • Fawaz S. AlmotairiEmail author
  • Per Hellström
  • Thomas Skoglund
  • Åsa Lundgren Nilsson
  • Magnus Tisell
Original Article - Neurosurgery general
Part of the following topical collections:
  1. Neurosurgery general



To assess the neuropsychological (NP) functioning and quality of life (QOL) before and 3 months after surgery on adults with Chiari I malformation (CMI).

Patients and methods

All adult patients who had been diagnosed with CMI were invited to participate. Those who participated were assessed using a Hospital Anxiety and Depression scale (HAD) and NP examinations. Their QOL was assessed using the self-reported life satisfaction checklist, LiSat-11 and the five-dimensional EuroQol measurement of health outcome, EQ-5D-5L. All assessments were carried out both before and 3 months after surgery was performed. Demographic data and comorbidities were also registered.


Of the 11 patients who underwent NP assessment, the majority demonstrated cognitive functioning within the normal range. However, postoperatively, their performance in verbal learning, psychomotor speed, colour naming speed and the ability to manage interference through response selection and inhibition (aspects of executive functioning) was significantly improved. Thirteen patients completed QOL assessments. When LiSat-11 item domains were compared with those of healthy subjects, patients reported a lower level of satisfaction with their life quality both before and after surgery. However, the EQ-5D-5L measurements, i.e., the descriptive system and the visual analogue, indicated that their QOL of life was significantly improved after surgery.


There is scarcely any literature documenting effects of surgery on the QOL of CMI patients. The study we present here breaks new ground by comparing pre- and postoperative NP functions in CMI. We also examine the value of surgery for improving both NP functions and QOL in CMI.


Arnold-Chiari malformation Cognitive dysfunction Decompressive craniectomy Psychological tests Quality of life Treatment outcome 



We would like to thank all the patients who participated in this study. Special thanks are also owed to Gudrun Barrows, Clinical Coordinator at the Department of Neurosurgery, Sahlgrenska University Hospital, for her invaluable help with the administration of this study.


This research received funding from Rune och Ulla Amlövs Stiftelse för Neurologisk och Reumatologisk Forskning and Göteborg Läkaresällskap (Gothenburg Medical Society). The sponsor had no role in the design or conduct of this research.

Compliance with ethical standards

Conflict of interest

All authors certify that they have no affiliations with or involvement in any organisation or entity with any financial interest (such as honoraria; educational; participation in speakers’ bureaus; membership, employment, consultancies, stock ownership, or other equity interest; and expert testimony or patent- licensing arrangements), or non-financial interest (such as personal or professional relationships, affiliations, knowledge or beliefs) in the subject matter or materials discussed in this manuscript.

Ethical approval

The study was approved by the regional ethics board of the University of Gothenburg (Diary nr 865–14) and was carried out following the principles of the 1964 Declaration of Helsinki and its later amendments or comparable ethical standards.

Informed consent

Informed consent was obtained from all individual participants included in the study.


  1. 1.
    Allen PA, Houston JR, Pollock JW, Buzzelli C, Li X, Harrington AK, Martin BA, Loth F, Lien MC, Maleki J, Luciano MG (2014) Task-specific and general cognitive effects in Chiari malformation type I. PLoS One 9:e94844. CrossRefGoogle Scholar
  2. 2.
    Almotairi FS, Tisell M (2016) Cerebrospinal fluid disturbance in overweight women after occipitocervical decompression in Chiari malformation type I. Acta Neurochir 158:589–594; discussion 594. CrossRefGoogle Scholar
  3. 3.
    Bakim B, Goksan Yavuz B, Yilmaz A, Karamustafalioglu O, Akbiyik M, Yayla S, Yuce I, Alpak G, Tankaya O (2013) The quality of life and psychiatric morbidity in patients operated for Arnold-Chiari malformation type I. Int J Psychiatry Clin Pract 17:259–263. CrossRefGoogle Scholar
  4. 4.
    Barkovich AJ, Wippold FJ, Sherman JL, Citrin CM (1986) Significance of cerebellar tonsillar position on MR. AJNR Am J Neuroradiol 7:795–799Google Scholar
  5. 5.
    Caykoylu A, Ekinci O, Albayrak Y, Kuloglu M, Deniz O (2008) Arnold-Chiari I malformation association with generalized anxiety disorder: a case report. Prog Neuro-Psychopharmacol Biol Psychiatry 32:1613–1614. CrossRefGoogle Scholar
  6. 6.
    Devlin NJ, Shah KK, Feng Y, Mulhern B, van Hout B (2017) Valuing health-related quality of life: an EQ-5D-5L value set for England. Health Econ.
  7. 7.
    Ellenbogen RG, Armonda RA, Shaw DW, Winn HR (2000) Toward a rational treatment of Chiari I malformation and syringomyelia. Neurosurg Focus 8:E6CrossRefGoogle Scholar
  8. 8.
    Eshetu T, Meoded A, Jallo GI, Carson BS, Huisman TA, Poretti A (2014) Diffusion tensor imaging in pediatric Chiari type I malformation. Dev Med Child Neurol 56:742–748. CrossRefGoogle Scholar
  9. 9.
    Fischbein R, Saling JR, Marty P, Kropp D, Meeker J, Amerine J, Chyatte MR (2015) Patient-reported Chiari malformation type I symptoms and diagnostic experiences: a report from the national Conquer Chiari Patient Registry database. Neurol Sci 36:1617–1624. CrossRefGoogle Scholar
  10. 10.
    Grosso S, Scattolini R, Paolo G, Di Bartolo RM, Morgese G, Balestri P (2001) Association of Chiari I malformation, mental retardation, speech delay, and epilepsy: a specific disorder? Neurosurgery 49:1099–1103; discussion 1103-1094 Google Scholar
  11. 11.
    Herdman M, Gudex C, Lloyd A, Janssen M, Kind P, Parkin D, Bonsel G, Badia X (2011) Development and preliminary testing of the new five-level version of EQ-5D (EQ-5D-5L). Qual Life Res Int J Qual Life Asp Treat Care Rehab 20:1727–1736. Google Scholar
  12. 12.
    Heuer GG, Gabel B, Lemberg PS, Sutton LN (2008) Chiari I malformation presenting with hearing loss: surgical treatment and literature review. Childs Nerv Syst 24:1063–1066. CrossRefGoogle Scholar
  13. 13.
    Klein R, Hopewell CA, Oien M (2014) Chiari malformation type I: a neuropsychological case study. Mil Med 179:e712–e718. CrossRefGoogle Scholar
  14. 14.
    Koehler PJ (1991) Chiari's description of cerebellar ectopy (1891). With a summary of Cleland's and Arnold's contributions and some early observations on neural-tube defects. J Neurosurg 75:823–826. CrossRefGoogle Scholar
  15. 15.
    Krishna V, Sammartino F, Yee P, Mikulis D, Walker M, Elias G, Hodaie M (2016) Diffusion tensor imaging assessment of microstructural brainstem integrity in Chiari malformation type I. J Neurosurg 125:1112–1119. CrossRefGoogle Scholar
  16. 16.
    Kumar A, Patni AH, Charbel F (2002) The Chiari I malformation and the neurotologist. Otol Neurotol 23:727–735CrossRefGoogle Scholar
  17. 17.
    Kumar M, Rathore RK, Srivastava A, Yadav SK, Behari S, Gupta RK (2011) Correlation of diffusion tensor imaging metrics with neurocognitive function in Chiari I malformation. World Neurosurg 76:189–194. CrossRefGoogle Scholar
  18. 18.
    Lacy M, Ellefson SE, DeDios-Stern S, Frim DM (2016) Parent-reported executive dysfunction in children and adolescents with Chiari malformation type 1. Pediatr Neurosurg 51:236–243. CrossRefGoogle Scholar
  19. 19.
    Leggio MG, Tedesco AM, Chiricozzi FR, Clausi S, Orsini A, Molinari M (2008) Cognitive sequencing impairment in patients with focal or atrophic cerebellar damage. Brain 131:1332–1343. CrossRefGoogle Scholar
  20. 20.
    Lugaresi A, Zucconi M, Gerardi R, Sforza E, Contin M, Cortelli P, Cirignotta F (1987) Autonomic failure in a case of Chiari malformation type I. Funct Neurol 2:511–513Google Scholar
  21. 21.
    Meeker J, Amerine J, Kropp D, Chyatte M, Fischbein R (2015) The impact of Chiari malformation on daily activities: a report from the national conquer Chiari patient registry database. Disabil Health J 8:521–526. CrossRefGoogle Scholar
  22. 22.
    Melin R, Fugl-Meyer KS, Fugl-Meyer AR (2003) Life satisfaction in 18- to 64-year-old swedes: in relation to education, employment situation, health and physical activity. J Rehabil Med 35:84–90CrossRefGoogle Scholar
  23. 23.
    Milhorat TH, Chou MW, Trinidad EM, Kula RW, Mandell M, Wolpert C, Speer MC (1999) Chiari I malformation redefined: clinical and radiographic findings for 364 symptomatic patients. Neurosurgery 44:1005–1017CrossRefGoogle Scholar
  24. 24.
    Mueller D, Oro JJ (2005) Prospective analysis of self-perceived quality of life before and after posterior fossa decompression in 112 patients with Chiari malformation with or without syringomyelia. Neurosurg Focus 18:Ecp2CrossRefGoogle Scholar
  25. 25.
    Parker SL, Godil SS, Zuckerman SL, Mendenhall SK, Wells JA, Shau DN, McGirt MJ (2013) Comprehensive assessment of 1-year outcomes and determination of minimum clinically important difference in pain, disability, and quality of life after suboccipital decompression for Chiari malformation I in adults. Neurosurgery 73:569–581; discussion 581. CrossRefGoogle Scholar
  26. 26.
    Rekate HL, Nadkarni TD, Teaford PA, Wallace D (1999) Brainstem dysfunction in chiari malformation presenting as profound hypoglycemia: presentation of four cases, review of the literature, and conjecture as to mechanism. Neurosurgery 45:386–391CrossRefGoogle Scholar
  27. 27.
    Riva D, Usilla A, Saletti V, Esposito S, Bulgheroni S (2011) Can Chiari malformation negatively affect higher mental functioning in developmental age? Neurol Sci 32(Suppl 3):S307–S309. CrossRefGoogle Scholar
  28. 28.
    Rosenthal R, Cooper H, Hedges L (1994) Parametric measures of effect size. The handbook of research synthesis:231–244Google Scholar
  29. 29.
    Schady W, Metcalfe RA, Butler P (1987) The incidence of craniocervical bony anomalies in the adult Chiari malformation. J Neurol Sci 82:193–203CrossRefGoogle Scholar
  30. 30.
    Schmahmann JD, Sherman JC (1998) The cerebellar cognitive affective syndrome. Brain 121(Pt 4):561–579CrossRefGoogle Scholar
  31. 31.
    Stovner LJ, Sjaastad O (1995) Segmental hyperhidrosis in two siblings with Chiari type I malformation. Eur Neurol 35:149–155CrossRefGoogle Scholar
  32. 32.
    Tisell M, Wallskog J, Linde M (2009) Long-term outcome after surgery for Chiari I malformation. Acta Neurol Scand 120:295–299. CrossRefGoogle Scholar
  33. 33.
    Tubbs RS, Beckman J, Naftel RP, Chern JJ, Wellons JC 3rd, Rozzelle CJ, Blount JP, Oakes WJ (2011) Institutional experience with 500 cases of surgically treated pediatric Chiari malformation type I. J Neurosurg Pediatr 7:248–256. CrossRefGoogle Scholar
  34. 34.
    Tubbs RS, Shoja MM, Ardalan MR, Shokouhi G, Loukas M (2008) Hindbrain herniation: a review of embryological theories. Ital J Anat Embryol 113:37–46Google Scholar
  35. 35.
    Zigmond AS, Snaith RP (1983) The hospital anxiety and depression scale. Acta Psychiatr Scand 67:361–370CrossRefGoogle Scholar

Copyright information

© Springer-Verlag GmbH Austria, part of Springer Nature 2019

Authors and Affiliations

  1. 1.Institute of Neuroscience and PhysiologyThe Sahlgrenska Academy, University of GothenburgGothenburgSweden
  2. 2.Department of NeurosurgeryThe Sahlgrenska Academy, University of GothenburgGothenburgSweden
  3. 3.Division of Neurosurgery, Department of Surgery, College of MedicineKing Saud UniversityRiyadhKingdom of Saudi Arabia

Personalised recommendations