Advertisement

Journal of Neurology

, Volume 258, Issue 10, pp 1877–1879 | Cite as

Pendular nystagmus in progressive ataxia and palatal tremor

  • R. BassaniEmail author
  • C. Mariotti
  • L. Nanetti
  • M. Grisoli
  • M. Savoiardo
  • D. Pareyson
  • E. Salsano
Letter to the Editors

Dear Sirs,

Pendular nystagmus (PN) is an involuntary ocular oscillation that can be found in congenital or acquired visual or central nervous system diseases [1, 2]. We describe the case of a patient with PN associated with progressive cerebellar ataxia and palatal tremor (PAPT). The PN has been registered and analyzed with video-oculography.

A 55-year-old man complained of a 2-year history of progressive difficulty with running and walking accompanied by unsteadiness. Over this time, he described transient oscillopsia. In retrospect, he reported a long history of difficulty in biking and subtle visual disturbances and tinnitus (but no ear click). There was no relevant family and past medical history. Examination revealed wide-based and unsteady gait, impossible tandem walk and marked dystasia at the single leg stance test (Video 1). The other cerebellar tests were normal (Video 2). Tendon reflexes were brisk at four limbs. The nystagmus was almost invisible at eye (Video 3, part 1),...

Keywords

Spinocerebellar Ataxia Inferior Olive Superior Cerebellar Peduncle Friedreich Ataxia Progressive Ataxia 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

Notes

Acknowledgments

The authors thank Dr. Erik Ulmer for his technical advice and the patient for his willing collaboration and sympathy.

Conflict of interest

None reported.

Supplementary material

Video 1 The video demonstrates the presence of marked disequilibrium both in the static tests as the Single Leg Stance and in the dynamic tests as in Tandem Walking. (MPG 13090 kb)

Video 2 The video presents the principal cerebellar coordination tests to the superior and inferior limbs. The coordination tests are basically unimpaired in this patient. (MPG 4130 kb)

Video 3 The ocular oscillations are barely recognizable at clinical examination (part 1), while the nystagmus is clearly demonstrated through the infrared video-oculography. The nystagmus develops preferentially in the vertical plane. A secondary rotational counter-clockwise component can be identified (part 2). The palatal tremor is symmetrical, although it is occasionally lateralized to the left side (part 3). (MPG 3510 kb)

References

  1. 1.
    Averbuch-Heller L, Zivotofsky AZ, Das VE et al (1995) Investigations of the pathogenesis of acquired pendular nystagmus. Brain 118(2):369–378PubMedCrossRefGoogle Scholar
  2. 2.
    Gresty MA, Ell JJ, Findley LJ (1982) Acquired pendular nystagmus: its characteristics, localising value and pathophysiology. J Neurol Neurosurg Psychiatry 45:431–439PubMedCrossRefGoogle Scholar
  3. 3.
    Samuel M, Torun N, Tuite PJ, Sharpe JA, Lang AE (2004) Progressive ataxia and palatal tremor (PAPT): clinical and MRI assessment with review of palatal tremors. Brain 127(6):1252–1268Google Scholar
  4. 4.
    Kim JS, Moon SY, Choi KD, Kim JH, Sharpe JA (2007) Patterns of ocular oscillation in oculopalatal tremor: imaging correlations. Neurology 68:1128–1135PubMedCrossRefGoogle Scholar
  5. 5.
    Pareyson D, Fancellu R, Mariotti C, Romano S, Salmaggi A, Carella F, Girotti F, Gattellaro G, Carriero MR, Farina L, Ceccherini I, Savoiardo M (2008) Adult-onset Alexander disease: a series of eleven unrelated cases with review of the literature Brain 131(9):2321–2331Google Scholar
  6. 6.
    Schwankhaus JD, Parisi JE, Gulledge WR, Chin L, Currier RD (1995) Hereditary adult-onset Alexander disease with palatal myoclonus, spastic paraparesis and cerebellar ataxia. Neurology 45:2266–2271PubMedGoogle Scholar
  7. 7.
    Knight MA, Gardner RJ, Bahlo M, Matsuura T, Dixon JA, Forrest SM, Storey E (2004) Dominantly inherited ataxia and dysphonia with dentate calcification: spinocerebellar ataxia type 20. Brain 127(5):1172–1181Google Scholar
  8. 8.
    Farmer J, Hoyt CS (1984) Monocular nystagmus in infancy and early childhood. Am J Ophtalmol 98:504–509CrossRefGoogle Scholar
  9. 9.
    Aschoff JC, Conrad B, Kornhuber HH (1974) Acquired pendular nystagmus with oscillopsia in multiple sclerosis: a sign of cerebellar nuclei disease. J Neurol Neurosurg Psychiatry 37:570PubMedCrossRefGoogle Scholar
  10. 10.
    Kori A, Robin N, Jacobs J, Erchul D, Zaidat O, Remler B, Averbuch-Heller L, Dell’Osso L, Leigh R, Zinn A (1998) Pendular nystagmus in patients with peroxisomal assembly disorder. Arch Neurol 55:554–558PubMedCrossRefGoogle Scholar
  11. 11.
    Moon SY, Park SH, Hwang JM, Kim JS (2003) Oculopalatal tremor after pontine hemorrhage. Neurology 61:1621PubMedGoogle Scholar
  12. 12.
    Leigh RJ, Zee DS (2006) The neurology of eye movements. Oxford University Press, OxfordGoogle Scholar
  13. 13.
    Lopez LI, Bronstein AM, Gresty MA et al (1996) Clinical and MRI correlates in 27 patients with acquired pendular nystagmus. Brain 119(2):465–472PubMedCrossRefGoogle Scholar
  14. 14.
    Ruigrok TJ, de Zeeuw CI, Voogd J (1990) Hypertrophy of inferior olivary neurons: a degenerative regenerative or plasticity phenomenon. Eur J Morphol 28:224–239PubMedGoogle Scholar
  15. 15.
    Shaikh AG, Hong S, Liao K, Tian J, Solomon D, Zee DS, Optican LM (2010) Oculopalatal tremor explained by a model of inferior olivary hypertrophy and cerebellar plasticity. Brain 133(3):923–940PubMedCrossRefGoogle Scholar

Copyright information

© Springer-Verlag 2011

Authors and Affiliations

  • R. Bassani
    • 1
    • 2
    Email author
  • C. Mariotti
    • 3
  • L. Nanetti
    • 3
    • 4
  • M. Grisoli
    • 5
  • M. Savoiardo
    • 5
  • D. Pareyson
    • 4
  • E. Salsano
    • 4
  1. 1.Department of Neurological SciencesOspedale “G. Salvini” Garbagnate MilaneseMilanItaly
  2. 2.Oto-neurology ServiceIRCCS Foundation, “C. Besta” Neurological InstituteMilanItaly
  3. 3.Unit of Neurology VIII, Clinics of Central and Peripheral Degenerative NeuropathiesIRCCS Foundation, “C. Besta” Neurological InstituteMilanItaly
  4. 4.Unit of Genetics of Neurodegenerative and Metabolic DiseasesIRCCS Foundation, “C. Besta” Neurological InstituteMilanItaly
  5. 5.Neuroradiology DepartmentIRCCS Foundation, “C. Besta” Neurological InstituteMilanItaly

Personalised recommendations