Skip to main content

Advertisement

SpringerLink
Log in

Spinocerebellar ataxia type 2 olfactory impairment shows a pattern similar to other major neurodegenerative diseases

  • ORIGINAL COMMUNICATION
  • Published:
Journal of Neurology Aims and scope Submit manuscript

Abstract

Olfactory function is affected in different neurodegenerative diseases. Recently, it has been found that some hereditary ataxias are also associated with significant olfactory impairment. However, the initial findings did not examine the nature of the olfactory impairment associated with these ataxias. In the present article the effect of spinocerebellar ataxia type 2 (SCA2) on olfactory function was studied in 53 SCA2 patients and 53 healthy control subjects from Holguín, Cuba. Several tests were applied to evaluate olfactory threshold, description, identification and discrimination. The results show significant impairment in SCA2 patients on all olfactory measurements, and the pattern of olfactory deficits found suggests that they have much in common with those reported for other neurodegenerative diseases such as Parkinson’s and Alzheimer’s diseases.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2

Similar content being viewed by others

References

  1. Abele M, Riet A, Hummel T, Klockgether T, Wullner U (2003) Olfactory dysfunction in cerebellar ataxia and multiple system atrophy. J Neurol 250:1453–1455

    Article  PubMed  Google Scholar 

  2. Babovic-Vuksanovic D, Snow K, Patterson MC, Michels VV (1998) Spinocerebellar ataxia type 2 (SCA 2) in an infant with extreme CAG repeat expansion. Am J Med Genet 79:383–387

    Article  PubMed  CAS  Google Scholar 

  3. Bacon AW, Bondi MW, Salmon DP, Murphy C (1998) Very early changes in olfactory functioning due to Alzheimer’s disease and the role of apolipoprotein E in olfaction. Ann N Y Acad Sci 855:723–731

    Article  PubMed  CAS  Google Scholar 

  4. Connelly T, Farmer JM, Lynch DR, Doty RL (2003) Olfactory dysfunction in degenerative ataxias. J Neurol Neurosurg Psychiatry 74:1435–1437

    Article  PubMed  CAS  Google Scholar 

  5. Devanand DP, Michaels-Marston KS, Liu X, Pelton GH, Padilla M, Marder K, Bell K, Stern Y, Mayeux R (2000) Olfactory deficits in patients with mild cognitive impairment predict Alzheimer’s disease at follow-up. Am J Psychiatry 157:1399–1405

    Article  PubMed  CAS  Google Scholar 

  6. Distel H, Ayabe-Kanamura S, Martinez-Gomez M, Schicker I, Kobayakawa T, Saito S, Hudson R (1999) Perception of everyday odors–correlation between intensity, familiarity and strength of hedonic judgement. Chem Senses 24:191–199

    Article  PubMed  CAS  Google Scholar 

  7. Doty RL (2001) Olfaction. Annu Rev Psychol 52:423–452

    Article  PubMed  CAS  Google Scholar 

  8. Doty RL, Deems DA, Stellar S (1988) Olfactory dysfunction in parkinsonism: a general deficit unrelated to neurologic signs, disease stage, or disease duration. Neurology 38:1237–1244

    PubMed  CAS  Google Scholar 

  9. Doty RL, Shaman P, Kimmelman CP, Dann MS (1984) University of Pennsylvania Smell Identification Test: a rapid quantitative olfactory function test for the clinic. Laryngoscope 94:176–178

    Article  PubMed  CAS  Google Scholar 

  10. Estrada R, Galarraga J, Orozco G, Nodarse A, Auburger G (1999) Spinocerebellar ataxia 2 (SCA2): morphometric analyses in 11 autopsies. Acta Neuropathol (Berl) 97:306–310

    Article  CAS  Google Scholar 

  11. Fernandez-Ruiz J, Diaz R, Hall-Haro C, Vergara P, Fiorentini A, Nunez L, Drucker-Colin R, Ochoa A, Yescas P, Rasmussen A, Alonso ME (2003) Olfactory dysfunction in hereditary ataxia and basal ganglia disorders. Neuroreport 14:1339–1341

    Article  PubMed  Google Scholar 

  12. Folstein MF, Folstein SE, McHugh PR (1975) “Mini-mental state”. A practical method for grading the cognitive state of patients for the clinician. J Psychiatr Res 12:189–198

    Article  PubMed  CAS  Google Scholar 

  13. Hawkes C (2003) Olfaction in neurodegenerative disorder. Mov Disord 18:364–372

    Article  PubMed  Google Scholar 

  14. Hudson R, Arriola A, Martinez- Gomez M, Distel H (2006) Effect of air pollution on olfactory function in residents of Mexico city. Chem Senses 31:79–85

    Article  PubMed  Google Scholar 

  15. Kovacs T (2004) Mechanisms of olfactory dysfunction in aging and neurodegenerative disorders. Ageing Res Rev 3:215–232

    Article  PubMed  Google Scholar 

  16. Mainland JD, Johnson BN, Khan R, Ivry RB, Sobel N (2005) Olfactory impairments in patients with unilateral cerebellar lesions are selective to inputs from the contralesional nostril. J Neurosci 25:6362–6371

    Article  PubMed  CAS  Google Scholar 

  17. Mesholam RI, Moberg PJ, Mahr RN, Doty RL (1998) Olfaction in neurodegenerative disease: a meta-analysis of olfactory functioning in Alzheimer’s and Parkinson’s diseases. Arch Neurol 55:84–90

    Article  PubMed  CAS  Google Scholar 

  18. Orozco Diaz G, Nodarse Fleites A, Cordoves Sagaz R, Auburger G (1990) Autosomal dominant cerebellar ataxia: clinical analysis of 263 patients from a homogeneous population in Holguin, Cuba. Neurology 40:1369–1375

    PubMed  CAS  Google Scholar 

  19. Ponsen MM, Stoffers D, Booij J, van Eck-Smit BL, Wolters E, Berendse HW (2004) Idiopathic hyposmia as a preclinical sign of Parkinson’s disease. Ann Neurol 56:173–181

    Article  PubMed  Google Scholar 

  20. Qureshy A, Kawashima R, Imran MB, Sugiura M, Goto R, Okada K, Inoue K, Itoh M, Schormann T, Zilles K, Fukuda H (2000) Functional mapping of human brain in olfactory processing: a PET study. J Neurophysiol 84:1656–1666

    PubMed  CAS  Google Scholar 

  21. Rub U, Del Turco D, Del Tredici K, de Vos RA, Brunt ER, Reifenberger G, Seifried C, Schultz C, Auburger G, Braak H (2003) Thalamic involvement in a spinocerebellar ataxia type 2 (SCA2) and a spinocerebellar ataxia type 3 (SCA3) patient, and its clinical relevance. Brain 126:2257–2272

    Article  PubMed  CAS  Google Scholar 

  22. Sanpei K, Takano H, Igarashi S, Sato T, Oyake M, Sasaki H, Wakisaka A, Tashiro K, Ishida Y, Ikeuchi T, Koide R, Saito M, Sato A, Tanaka T, Hanyu S, Takiyama Y, Nishizawa M, Shimizu N, Nomura Y, Segawa M, Iwabuchi K, Eguchi I, Tanaka H, Takahashi H, Tsuji S (1996) Identification of the spinocerebellar ataxia type 2 gene using a direct identification of repeat expansion and cloning technique, DIRECT. Nat Genet 14:277–284

    Article  PubMed  CAS  Google Scholar 

  23. Schols L, Gispert S, Vorgerd M, Menezes Vieira-Saecker AM, Blanke P, Auburger G, Amoiridis G, Meves S, Epplen JT, Przuntek H, Pulst SM, Riess O (1997) Spinocerebellar ataxia type 2. Genotype and phenotype in German kindreds. Arch Neurol 54:1073–1080

    PubMed  CAS  Google Scholar 

  24. Ship JA, Weiffenbach JM (1993) Age, gender, medical treatment, and medication effects on smell identification. J Gerontol 48:M26–32

    PubMed  CAS  Google Scholar 

  25. Sobel N, Prabhakaran V, Hartley CA, Desmond JE, Zhao Z, Glover GH, Gabrieli JD, Sullivan EV (1998) Odorant-induced and sniff-induced activation in the cerebellum of the human. J Neurosci 18:8990–9001

    PubMed  CAS  Google Scholar 

  26. Trouillas P, Takayanagi T, Hallett M, Currier RD, Subramony SH, Wessel K, Bryer A, Diener HC, Massaquoi S, Gomez CM, Coutinho P, Ben Hamida M, Campanella G, Filla A, Schut L, Timann D, Honnorat J, Nighoghossian N, Manyam B (1997) International Cooperative Ataxia Rating Scale for pharmacological assessment of the cerebellar syndrome. The Ataxia Neuropharmacology Committee of the World Federation of Neurology. J Neurol Sci 145:205–211

    Article  PubMed  CAS  Google Scholar 

  27. Vanderpool HY (1996) The ethics of research involving human subjects : facing the 21st century. University Pub. Group, Frederick, Md

    Google Scholar 

  28. Velazquez-Perez L, Seifried C, Santos-Falcon N, Abele M, Ziemann U, Almaguer LE, Martinez-Gongora E, Sanchez-Cruz G, Canales N, Perez-Gonzalez R, Velazquez-Manresa M, Viebahn B, von Stuckrad-Barre S, Fetter M, Klockgether T, Auburger G (2004) Saccade velocity is controlled by polyglutamine size in spinocerebellar ataxia 2. Ann Neurol 56:444–447

    Article  PubMed  Google Scholar 

  29. Wadia NH, Swami RK (1971) A new form of heredo-familial spinocerebellar degeneration with slow eye movements (nine families). Brain 94:359–374

    PubMed  CAS  Google Scholar 

Download references

Acknowledgments

This study was partially supported by Fideicomiso UNAM to RDC and an Agreement between CIRAH and Coordinación de la Investigación Científica, UNAM.

We are grateful to the patients, control subjects, and to the Cuban Ministry of Health for the cooperation given. We also thank Rafael Ojeda for his help during this study.

Author information

Authors and Affiliations

  1. Centro para la Investigación y Rehabilitación de las Ataxias Hereditarias” Carlos J. Finlay”, Holguín, Cuba

    Luis Velázquez-Pérez MD, Ruth Pérez- González MD, Nalia Canales Ochoa MD, Gilberto Sánchez Cruz MD, Luis Enrique Almaguer Mederos PhD & Edilberto Martínez Góngora MD

  2. Departamento de Fisiología, Facultad de Medicina, Universidad Nacional Autónoma de México, México

    Juan Fernandez-Ruiz PhD & Rosalinda Díaz MSc

  3. Departamento de Biología Celular y, Fisiología Instituto de Investigaciones Biomédicas, UNAM, México

    Robyn Hudson PhD

  4. Departamento de Neurociencias, Instituto de Fisiología Celular, UNAM, Apdo. Postal 70-600, 04510, México, D.F.

    René Drucker-Colin PhD

Authors
  1. Luis Velázquez-Pérez MD
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Juan Fernandez-Ruiz PhD
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Rosalinda Díaz MSc
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Ruth Pérez- González MD
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Nalia Canales Ochoa MD
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Gilberto Sánchez Cruz MD
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Luis Enrique Almaguer Mederos PhD
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. Edilberto Martínez Góngora MD
    View author publications

    You can also search for this author in PubMed Google Scholar

  9. Robyn Hudson PhD
    View author publications

    You can also search for this author in PubMed Google Scholar

  10. René Drucker-Colin PhD
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to René Drucker-Colin PhD.

Additional information

Luis Velázquez-Pérez and Juan Fernandez-Ruiz Equal contribution

Received in revised form: 24 January 2006

Rights and permissions

Reprints and permissions

About this article

Cite this article

Velázquez-Pérez, L., Fernandez-Ruiz, J., Díaz, R. et al. Spinocerebellar ataxia type 2 olfactory impairment shows a pattern similar to other major neurodegenerative diseases. J Neurol 253, 1165–1169 (2006). https://doi.org/10.1007/s00415-006-0183-2

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00415-006-0183-2

Keywords

Search

Navigation