Journal of Neurology

, Volume 242, Issue 8, pp 517–521 | Cite as

Fixation instability and oculomotor abnormalities in Friedreich's ataxia

  • Sybille Spicker
  • Jörg B. Schulz
  • Dirk Petersen
  • Michael Fetter
  • Thomas Klockgether
  • Johannes Dichgans
Original Communication


Eye movements were studied in 13 patients with Friedreich's ataxia and correlated with MRI findings to investigate whether oculomotor abnormalities can be traced to cerebellar disturbances in this disease. One of the most prominent eye signs was fixation instability (square-wave jerks, SWJ.). Besides SWJ the patients showed various combinations of cerebellar, vestibular and brain-stem oculomotor signs. Our patients did not comprise a homogeneous group with regard to their oculomotor findings. There was no correlation between the severity of any of the so-called cerebellar oculomotor disturbances and the number of SWJ. We tried to correlate the extent of oculomotor disturbances with floccular atrophy and atrophy of the dorsal vermis on MRI in seven of the patients. None of the oculomotor features (including SWJ) correlated with flocculus or dorsal vermis size. Furthermore, floccular and vermal measurements on MRI were normal. Accordingly, we think it unlikely that the oculomotor disturbances, including SWJ, are attributable to cerebellar pathology per se.

Key words

Friedreich's ataxia Fixation instability Oculomotor abnormalities Magnetic resonance imaging 


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  1. 1.
    Balch RW, Konrad HR, Honrubia V (1975) Vestibulo-ocular function in patients with cerebellar atrophy. Neurology 25:160–168PubMedGoogle Scholar
  2. 2.
    Dale RT, Kirby AW, Jampel RS (1978) Square wave jerks in Friedreich's ataxia. Am J Ophthalmol 85:400–406Google Scholar
  3. 3.
    Dichgans J, Jung R (1975) Oculomotor abnormalities due to cerebellar lesions. In: Lennerstrand G, Bach-y-Rita P (eds) Basic mechanisms of ocular motility and their clinical implications. Pergamon, Oxford, pp 281–298Google Scholar
  4. 4.
    Ell J, Prasher D, Rudge P (1984) Neuro-otological abnormalities in Friedreich's ataxia. J Neurol Neurosurg Psychiatry 47:26–32Google Scholar
  5. 5.
    Feldon SE, Langston JW (1977) Square-wave jerks: a disorder of microsaccades? Neurology 27:278–281PubMedGoogle Scholar
  6. 6.
    Fetter M, Klockgether T, Schulz JB, Faiss E, Koenig E, Dichgans J (1994) Oculomotor abnormalities and MRI findings in idiopathic cerebellar ataxia. J Neurol 241:234–241PubMedGoogle Scholar
  7. 7.
    Friedreich N (1863) Ober degenerative Atrophie der spinalen Hinterstrdnge. Arch Pathol Anat Physiol Klin Med 26:391–419,433–459;27:1–26Google Scholar
  8. 8.
    Furman JM, Perlman S, Baloh RW (1983) Eye movements in Friedreich's ataxia. Arch Neurol 40:343–346PubMedGoogle Scholar
  9. 9.
    Geoffroy G, Barbeau A, Breton G, Lemieux B, Aube M, Leger C, Bouchard JP (1976) Clinical description and roentgenologic evaluation of patients with Friedreich's ataxia. Can J Neurol Sci 3:279–286PubMedGoogle Scholar
  10. 10.
    Harding AE (1981) Friedreich's ataxia: a clinical and genetic study of 90 families with an analysis of early diagnostic criteria and intrafamilial clustering of clinical features. Brain 104:589–620Google Scholar
  11. 11.
    Hotson JR (1982) Cerebellar control of fixation eye movements. Neurology 32:31–36PubMedGoogle Scholar
  12. 12.
    Kase M, Nagata R, Arikado T (1985) Macrosaccadic oscillation, saccadic dysmetria and motor error in spinocerebellar degeneration. Jpn J Ophthalmol 29:369–377PubMedGoogle Scholar
  13. 13.
    Kirkham TH, Guitton D, Katsarkas A, Kline LB, Andermann E (1979) Oculomotor abnormalities in Friedreich's ataxia. Can J Neurol Sci 6:167–172PubMedGoogle Scholar
  14. 14.
    Jongkees LBW, Philipszoon AJ (1964) Electronystagmography, chapter III: caloric test. Acta Otolaryngol (Stockh) [Suppl] 189:45–54Google Scholar
  15. 15.
    Leigh RJ, Zee DS (1993) The neurology of eye movements, 2nd edn. Davis, PhiladelphiaGoogle Scholar
  16. 16.
    Monday LA, Lemieux B, St-Vincent H, Barbeau A (1978) Clinical and electronystagmographic findings in Friedreich's ataxia. Can J Neurol Sci 5:71–73PubMedGoogle Scholar
  17. 17.
    Monday L, Lespérance J, Lemieux B, St-Vincent H (1984) Follow-up study of electronystagmographic findings in Friedreich's ataxia patients and evalua tion of their relatives. Can J Neurol Sci 11:570–573PubMedGoogle Scholar
  18. 18.
    Mott FW (1907) Case of Friedreich's disease, with autopsy and systematic microscopical examination of the nervous system. Arch Neurol Pathol Lab Lond County Asylums 3:180–200Google Scholar
  19. 19.
    Oppenheimer DR (1979) Brain lesions in Friedreich's ataxia. Can J Neurol Sci 6:173–176PubMedGoogle Scholar
  20. 20.
    Schroth G, Naegele T, Klose U, Mann K, Petersen D (1988) Reversible brain shrinkage in abstinent alcoholics, measured by MRI. Neuroradiology 30:385–389PubMedGoogle Scholar
  21. 21.
    Wessel K, Schroth G, Diener HC, Müller-Forell W, Dichgans J (1989) Significance of MRI-confirmed atrophy of the cranial spinal cord in Friedreich's ataxia. Eur Arch Psychiatry Neurol Sci 238:225–230PubMedGoogle Scholar
  22. 22.
    Wiillner U, Klockgether T, Petersen D, Naegele T, Dichgans J (1993) Magnetic resonance imaging (MRI) in hereditary and idiopathic ataxia. Neurology 43:318–325PubMedGoogle Scholar

Copyright information

© Springer-Verlag 1995

Authors and Affiliations

  • Sybille Spicker
    • 1
  • Jörg B. Schulz
    • 1
  • Dirk Petersen
    • 2
  • Michael Fetter
    • 1
  • Thomas Klockgether
    • 1
  • Johannes Dichgans
    • 1
  1. 1.Department of NeurologyUniversity of TübingenTübingenGermany
  2. 2.Department of NeuroradiologyUniversity of TübingenTübingenGermany

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