Psychological symptoms and Quality of Life in adults with Chiari malformation type I: an Assessment by the Italian version of Chiari Symptom Profile

Authors

  • Loredana Benedetto Department of Clinical and Experimental Medicine, University of Messina https://orcid.org/0000-0001-6957-2224
  • John J. Oró <span class="markfjolhjwfy"><span>Colorado Chiari Institute. Aurora, Colorado (USA)</span></span>
  • Diane M. Mueller Colorado Chiari Institute. Aurora, Colorado (USA)
  • Deborah Panarello Department of Clinical and Experimental Medicine, University of Messina
  • Erica Lo Turco Department of Biomedical and Dental Sciences and Morphofunctional Imaging, Unit of Neurosurgery, University of Messina
  • Gerardo Caruso Department of Biomedical and Dental Sciences and Morphofunctional Imaging, Unit of Neurosurgery, University of Messina
  • Antonino Francesco Germanò Department of Biomedical and Dental Sciences and Morphofunctional Imaging, Unit of Neurosurgery, University of Messina
  • Massimo Ingrassia Department of Clinical and Experimental Medicine, University of Messina https://orcid.org/0000-0003-0407-4480

DOI:

https://doi.org/10.13129/2282-1619/mjcp-3558

Keywords:

Chiari malformation, Quality of Life, Assessment, Anxiety, Depression, Psychological intervention.

Abstract

Chiari malformation type I (CM-I) is a rare condition with physical and neurological manifestation changing across people. Being a chronic and debilitating disease, a comprehensive multidisciplinary approach is needed for evaluating patient’s experienced Quality of Life (QoL) and psychological correlates of CM.

Objectives: The aim of this study was to develop the Italian adaptation of Chiari Symptom Profile (CSP), a questionnaire assessing the core symptoms of Chiari malformation and their impact on people’s lives. Secondly, the occurrence of anxiety/depression symptoms and associations with patient-reported QoL were explored.

Methods: 172 adults with diagnosed CM-I (N=79 with neurosurgery) completed an online questionnaire measuring general QoL (WHOQOL-brief), disease-related QoL (CSP), and symptoms of anxiety/depression (HADS). Participant’s demographic and clinical data were also collected.

Results: The Italian version of CSP showed excellent reliability both in total (Cronbach alpha = .97) and factorial scores (alphas from .87 to .95) assessing four domains of Chiari-related QoL (functional, physical, social, and psychological). For construct validity, significant correlations (p< .001) resulted among severity of CM symptoms, social and daily limitations assessed by CSP and general QoL. Participants’ age at neurosurgery and condition (with/without neurosurgery treatment) did not significantly impact QoL scores, but perceived QoL worsened with increasing age. Among participants, 32% reported clinical anxiety and 14% depression symptoms (with higher incidence of depression in non-operated participants). Participants with clinical anxiety/depression reported a worse QoL in all domains of CSP (functional, physical, social, and psychological).

Conclusion: Findings suggest the need to include in CM treatment a continuous psychological support, identifying the patients most at risk who, in time, they may experience greater psychological suffering.

Author Biography

  • Massimo Ingrassia, Department of Clinical and Experimental Medicine, University of Messina
    Massimo Ingrassia, PsyD, is Associate professor of Developmental and Educational Psychology at the Department of Clinical and Experimental Medicine, University of Messina, Italy. He teaches developmental and health psychology in psychology graduate and postgraduate courses. His research interests are in the areas of risk behaviors in adolescence and emerging adulthood, digital technology and internet problematic use, and learning disabilities. Dr. Ingrassia was a scientific advisor in projects to implement health services for families in pediatric palliative care.

References

Aghakhani, N., Parker, F., David, P., Morar, S., Lacroix, C., Benoudiba, F., & Tadie, M. (2009). Long-term follow-up of Chiari-related syringomyelia in adults: analysis of 157 surgically treated cases. Neurosurgery, 64(2), 308–315. https://doi.org/10.1227/01.neu.0000336768.95044.80

Allen, P.A., Houston, J.R., Pollock, J.W., Buzzelli, C., Li, X., Harrington, A.K., Martin, B.A., Loth, F., Lien, M.-C., Maleki, J., & Luciano, M.G. (2014). Task-Specific and General Cognitive Effects in Chiari Malformation Type I. PLoS ONE, 9(4), e94844. Doi: https://doi.org/10.1371/journal.pone.0094844.g001

Almotairi, F.S., Hellström, P., Skoglund, T., & Tisell, M. (2020). Chiari I malformation - neuropsychological functions and quality of life. Acta Neurochirurgica, 162, 1575–1582. https://doi.org/10.1007/s00701-019-03897-2

Bailey, K.M., Carleton, R.N., Vlaeyen, J.W., & Asmundson, G.J. (2019). Treatments addressing pain-related fear and anxiety in patients with chronic musculoskeletal pain: a preliminary review. Cognitive Behavior Therapy, 39(1), 46–63. Doi: https://doi.org/10.1080/16506070902980711

Bakim, B., Yavuz, B.G., 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. International Journal of Psychiatry in Clinical Practice, 17, 259–263. https://doi.org/10.3109/13651501.2013.778295

Bezuidenhout, A.F., Khatami, D., Heilman, C.B., Kasper, E.M., Patz, S., Madan, N., Zhao, Y., & Bhadelia, R.A. (2018). Relationship between Cough-Associated Changes in CSF Flow and Disease Severity in Chiari I Malformation: An Exploratory Study Using Real-Time MRI. AJNR American Journal of Neuroradiology, 239(7), 1267–1272. https://doi.org/10.3174/ajnr.A5670

Burke, A.L., Mathias, J.L., & Denson, L.A. (2015). Psychological functioning of people living with chronic pain: a meta-analytic review. British Journal of Clinical Psychology, 54(3), 345–360.https://doi.org/10.1111/bjc.12078

Caffo, M., Cardali, S.M., Caruso, G., Fazzari, E., Abbritti, R.V., Barresi, V., & Germanò, A. (2019). Minimally invasive posterior fossa decompression with duraplasty in Chiari malformation type I with and without syringomyelia. Surgical Neurology International, 10(88).

Available from: https://surgicalneurologyint.com/surgicalint-articles/minimally-invasive-posterior-fossa-decompression-with-duraplasty-in-chiari-malformation-type-i-with-and-without-syringomyelia/

Cohodarevic, T., Mailis-Gagnon, A., & Montanera, W. (2000). Syringomyelia: Pain, sensory abnormalities, and neuroimaging. The Journal of Pain, 1(1), 54–66. https://doi.org/10.1016/S1526-5900(00)90088-9

Costantini, M., Musso, M., Viterbori, P., Bonci, F., Del Mastro, L., Garrone, O., Venturini, M., & Morasso, G. (1999). Detecting psychological distress in cancer patients: validity of the Italian version of the Hospital Anxiety and Depression Scale. Supportive Care in Cancer, 7, 121–127.https://pubmed.ncbi.nlm.nih.gov/10335929/

de Girolamo, G., Rucci, P., Scocco, P., Becchi, A., Coppa, F., D'Addario, A., Darú, E., De Leo, D., Galassi, L., Mangelli, L., Marson, C., Neri, G, & Soldani, L. (2000). La valutazione della qualità della vita: validazione del WHOQOL-breve [Quality of Life assessment: Validation of the Italian version of the WHOQOL-brief]. Epidemiologia e Psichiatria Sociale, 9(1), 45–54. https://doi.org/10.1017/S1121189X00007740

Durham, S., Sun, P., & Schut, L. (1998). Malformación de Chiari e hidrosiringomielia [Chiari malformations and hydrosyringomyelia]. Revista de Neurología, 27, 231–237. Doi: https://doi.org/10.33588/rn.27156.97386

Feghali, J., Chen, Y., Xie, Y., Chen, C., & Huang, J. (2020). The impact of depression on surgical outcome in Chiari malformation type I: an assessment based on the Chicago Chiari Outcome Scale. Journal of Neurosurgery Spine, 24, 1–8. https://doi.org/10.3171/2020.2.spine2069

Ferré Masó, A., Poca, M.A., de la Calzada, M.D., Solana, E., Romero Tomás, O., & Sahuquillo, J. (2014). Sleep disturbance: A forgotten syndrome in patients with Chiari I Malformation. Neurología, 29(5): 294–304. https://doi.org/10.1016/j.nrl.2011.01.008

Fischbein, R., Saling, J.R., Marty, P., Kropp, D., Meeker, J., Amerine, J., & Chyatte, M.R. (2015). Patient-reported Chiari malformation type I symptoms and diagnostic experiences: a report from the national Conquer Chiari Patient Registry database. Neurological Sciences, 36, 1617–1624. https://doi.org/10.1007/s10072-015-2219-9

García M., Allen, P.A., Li, X., Houston, J.R., Loth, F., Labuda, R., & Delahanty, D.L. (2019). An examination of pain, disability, and the psychological correlates of Chiari Malformation pre- and post-surgical correction. Disability and Health Journal, 12(4), 649–656. https://doi.org/10.1016/j.dhjo.2019.05.004

García, M., Eppelheimer, M.S., Houston, J.R., Houston, M.L., Nwotchouang, B.S.T., Kaut, K.P., Labuda, R., Bapuraj, R.J., Maleki, J., Klinge, P.M., Vorster, S., Luciano, M.G., Loth, F., & Allen, P.A. (2022). Adult Age Differences in Self‑Reported Pain and Anterior CSF Space in Chiari Malformation. The Cerebellum, 21, 194–207. https://doi.org/10.1007/s12311-021-01289-w

García, M., Lázaro, E., López-Paz, J.F., Martínez, O., Pérez, M., Berrocoso, S., Al-Rashaida, M., & Amayra, I. (2018). Cognitive functioning in Chiari malformation type I without posterior fossa surgery. The Cerebellum, 17, 564–574. https://doi.org/10.1007/s12311-018-0940-7

Gatchel, R.J., Neblett, R., Kishino, N., & Ray, C.T. (2016). Fear-Avoidance Beliefs and Chronic Pain. The Journal of Orthopaedic & Sports Physical Therapy, 46(2), 38–43. https://doi.org/10.2519/jospt.2016.0601

Geerdink, N., van der Vliet, T., Rotteveel, J.J., Feuth, T., Roeleveld, N., & Mullaart, R.A. (2012). Essential features of Chiari II malformation in MR imaging: an interobserver reliability study—part 1. Child’s Nervous System, 28, 977–985. https://doi.org/10.1007/s00381-012-1761-5

Greenberg, J.K., Milner, E., Yarbrough, C.K., Lipsey, K., Piccirillo, J.F., Smyth, M.D., Park, T.S., & Limbrick, D.D., Jr. (2015). Outcome methods used in clinical studies of Chiari malformation Type I: a systematic review. Journal of Neurosurgery, 122, 262–272. https://doi.org/10.3171/2014.9.JNS14406

Haddad, F.A., Qaisi, I., Joudeh, N., Dajani, H., Jumah, F., Elmashala, A., Adeeb, N., Chern, J.J., & Tubbs, R.S. (2018). The newer classifications of the Chiari malformations with clarifications: an anatomical review. Clinical Anatomy, 31, 314–322. https://doi.org/10.1002/ca.23051

Hadley, D.M. (2002). The Chiari malformations. Journal of Neurology, Neurosurgery & Psychiatry, 72(Suppl II), ii38–ii40. https://doi.org/10.1136%2Fjnnp.72.suppl_2.ii38

Houston, J.R., Hughes, M.L., Lien, M.C., Martin, B.A., Loth, F., Luciano, M.G., Vorster, S., & Allen, P.A. (2018). An Electrophysiological Study of Cognitive and Emotion Processing in Type I Chiari Malformation. The Cerebellum, 17, 404–418. https://doi.org/10.1007/s12311-018-0923-8

Hofkes, S.K., Iskandar, B.J., Turski, P.A., Gentry, L.R., McCue, J.B., & Haughton, V.M. (2007). Differentiation between symptomatic Chiari I malformation and asymptomatic tonsillar ectopia by using cerebrospinal fluid flow imaging: initial estimate of imaging accuracy. Radiology, 245, 532–540. https://doi.org/10.1148/radiol.2452061096

Holmes, S.C., Gonzalez, A., Allen, P.A., & Johnson, D.M. (2019). Utilizing group acceptance and commitment therapy (ACT) to address chronic pain, coping, and functioning for patients with Chiari malformation: A case example. Professional Psychology: Research and Practice, 50(5), 296–306. https://psycnet.apa.org/doi/10.1037/pro0000247

Langridge, B., Phillips, E., & Choi, D. (2017). Chiari Malformation Type 1: A Systematic Review of Natural History and Conservative Management. World Neurosurgery, 104, 213–219. https://doi.org/10.1016/j.wneu.2017.04.082

Lazáro, E., García, M., Amayra, I., López-Paz, J.-F., Martínez, O., Pérez, M., Berrocoso, S., Al Rashaida, M., Fernández, P., Rodríguez, A., Jometón, A., & Ruíz, B. (2018). Anxiety and depression in Chiari malformation. Journal of Integrative Neuroscience, 17(4), 343–348. https://doi.org/10.31083/j.jin.2018.04.0414

Lethem, J., Slade, P.D., Troup, J.D., & Bentley, G. (1983). Outline of a Fear-Avoidance Model of exaggerated pain perception. Behavior Research and Therapy, 21, 401–408. Doi: https://doi.org/10.1016/0005-7967(83)90009-8

Lin, X.-J., Lin, I.-M., & Fan, S.-Y. (2013). Methodological issues in measuring health-related quality of life. Tzu Chi Medical Journal, 25(1), 8–12. https://doi.org/10.1016/j.tcmj.2012.09.002

Martínez-Sabater, A., Ballestar-Tarín, M.L., Vázquez-Seoane, M., Marí-Avargues, L., Saus-Ortega, C., & Casal-Angulo, M. (2018). Quality of Life in Individuals Affected by Arnold Chiari Malformation: Comparison and Validation of a Measurement Instrument. Endocrine, Metabolic & Immune Disorders - Drug Targets, 18(4), 388–396. https://doi.org/10.2174/1871530317666171123205628

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. Disability and Health Journal, 8(4), 521–526. Doi: https://doi.org/10.1016/j.dhjo.2015.01.003

Mestres, O., Poca, M.A., Solana, E., Radoi, A., Quintana, M., Force, E., & Sahuquillo, J. (2012). Evaluación de la calidad de vida en los pacientes con una malformación de Chiari tipo I. Estudio piloto en una cohorte de 67 pacientes [Evaluation of the quality of life of patients with a Chiari type I malformation. A pilot study in a cohort of 67 patients]. Revista de Neurologia, 55(3), 148–156. https://doi.org/10.33588/rn.5503.2012196

Mueller D.M., & Oró J.J. (2004). Prospective analysis of presenting symptoms among 265 patients with radiographic evidence of Chiari malformation type I with or without syringomyelia. Journal of the American Academy of Nurse Practitioner, 16(3), 134–138. https://doi.org/10.1111/j.1745-7599.2004.tb00384.x

Mueller, D.M., & Oró, J.J. (2005). Chiari I Malformation with or without Syringomyelia and Pregnancy: Case Studies and Review of the Literature. American Journal of Perinatology, 22, 67–70. https://doi.org/10.1055/s-2005-837271

Mueller, D.M., & Oró, J.J. (2013). The Chiari Symptom Profile: development and validation of a Chiari-/syringomyelia-specific questionnaire. Journal of Neuroscience Nursing, 45(4), 205–210. https://doi.org/10.1097/jnn.0b013e3182986573

Parker, S.L., Godil, S.S., Zuckerman, S.L., Mendenhall, S.K., Wells, J.A., Shau, D.N., & McGirt, M.J. (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. https://doi.org/10.1227/neu.0000000000000032

Patrick, D.L., & Deyo, R.A. (1989). Generic and disease-specific measures in assessing health status and quality of life. Medical Care, 27(3 Suppl), S217–S232. https://doi.org/10.1097/00005650-198903001-00018

Sheikh, S., Dahiya, S., Ansari, A.H., & Kumar, M.M. (2019). The association of quality of life between anxiety and depression in patients with chronic rheumatic heart disease. Mediterranean Journal of Clinical Psychology, 7(2), 1–12. https://doi.org/10.6092/2282-1619/2019.7.2164

WHOQOL Group (1998). The World Health Organization Quality of Life Assessment (WHOQOL): development and general psychometric properties. Social Science & Medicine, 46,1569–1585. https://doi.org/10.1016/S0277-9536(98)00009-4

Zigmond, A.S., & Snaith, R.P. (1983). The hospital anxiety and depression scale. Acta Psychiatrica Scandinavica, 67(6), 361–70. Doi: https://doi.org/10.1111/j.1600-0447.1983.tb09716.x

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Published

28-12-2022

Issue

Vol. 10 No. 3 (2022)

Section

Clinical Psychology

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