The Italian Journal of Neurological Sciences

, Volume 13, Issue 2, pp 151–156 | Cite as

The role of MRI in the diagnosis of olivopontocerebellar atrophy

  • Giuliani G. 
  • Chiaramoni L. 
  • Foschi N. 
  • Terziani S. 
Case Reports

Abstract

Under the term of olivopontocerebellar atrophy different nosological pictures are grouped, all characterized by showing clinical signs of deficiency of the structures of the pons and of the cerebellum.

The diagnosis of olivopontocerebellar atrophy has been made, until now, by clinical criteria while typical anatomopathological changes are found at the autoptic studies.

We describe three patients affected by olivopontocerebellar atrophy, of different types and at different stages of disease.

In all cases MRI showed a similar and typical picture of atrophy of the olivary eminences of the medulla oblongata resulting in straightening of the angle usually present on the ventral frontier between pons and medulla oblongata. This diagnostic tool demonstrated thus to be of primary relevance yet in the early phases of the disease.

Key Words

Olivopontocerebellar atrophy MRI 

Sommario

Sotto il termine di atrofia olivo-pontocerebellare si raggruppano quadri nosografici di verosimile diversa origine, caratterizzati tutti dalla presenza di segni clinici deficitari pontini e cerebellari. La diagnosi è stata sino ad ora solo clinica con conferma autoptica del danno pontocerebellare. Sono descritti tre casi di atrofia olivo-pontocerebellare, appartenenti, a tre distinti sottogruppi ed in fasi diverse della malattia. In tutti e tre i casi la MRI ha evidenziato un quadro di atrofia pontina e cerebellare ed una riduzione dimensionale delle olive bulbari che risultava in un allargamento dell'angolo pontobulbare normalmente rilevabile sulla faccia anteriore del tronco in proiezione laterale. L'esame si conferma di importanza diagnostica essenziale nella malattia sin dalle sue fasi iniziali.

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

References

  1. [1]
    Berciano J.:Olivopontocerebellar atrophy. A review of 117 cases. J. Neurol. Sci., 53-253-272, 1982.Google Scholar
  2. [2]
    Bradley W.B., Waluch V., Yadley, R.A., Wycoff, R.R.:Comparison of CT and MR in 400 patients with suspected disease of the brain and spinal cord. Neuroradiology, 152:695–702, 1984.Google Scholar
  3. [3]
    Bydder, G.M., Steiner, R.E., Young I.R. et al.:Clinical NMR imaging of the brain: 140 cases. A.J.N.R., 3:459–480, 1982.Google Scholar
  4. [4]
    Duvoisin R.C.:The olivopontocerebellar atrophies. In: Marsden C.D., Fahn, S., eds.: Movement disorders Butterworths, London, 7: 249–269, 1987.Google Scholar
  5. [5]
    Escourolle R., Masson M.:Atrophies cérébelleuses. In: Encyclopédie Médico-Chirurgicale, Editions Techniques, vol 3o, 17066 A 10, 1967.Google Scholar
  6. [6]
    Garcia De La Rocha M.L., Moreno Martinez J.M., Garrido Carrion A., Margalet Fernandez P., Martin Araguz A.:Present criteria for diagnosis in vivo of olivopontocerebellar atrophy. Ann. Neurol. Scand., 77:234–238, 1988.Google Scholar
  7. [7]
    Gilman S., Markel D.S., Koeppe R.A., et al.:Cerebellar and Brainstem Hypometabolism in Olivopontocerebellar Atrophy Detected with Positron Emission Tomography Ann. Neurol. 23:223–230, 1988.PubMedGoogle Scholar
  8. [8]
    Graham J.C., Oppenheimer D.R.:Orthostatic hypotension in a case of multiple system atrophy. J. Neurol. Neurosurg. Psychiatry, 32:28–34, 1969.PubMedGoogle Scholar
  9. [9]
    Greenfield J.G.:The spino-cerebellar degenerations. Oxford, Blackwell, England, 1954.Google Scholar
  10. [10]
    Harding A.E.:The hereditary ataxias and related disorders. Churchill Livingstone London, 1984.Google Scholar
  11. [11]
    Harding A.E.:Commentary: Olivopontocerebellar atrophy is not a useful concept. In: Marsden C.D., Fahn S., eds. Movement disorders, Butterworths, London, 7:269–271, 1987.Google Scholar
  12. [12]
    Kinkel P.R.:Nuclear Magnetic Resonance Imaging in Clinical Neurology, In: A.B. Baker and R.J. Joint, eds. Clinical Neurology, Vol. 1, Cap 4A, J.B. Lippincot Company, Philadelphia, 1988Google Scholar
  13. [13]
    Koehler P.R., Haughton V.M., Daniels D.L., et al.:MR Measurement of Normal and Pathologic Brainstem Diameters. A.J.N.R.; 6:425–427, 1985.Google Scholar
  14. [14]
    Koningsmark B.W., Weiner L.P.:The olivopontocerebellar atrophies: a review. Medicine, 49:227–234, 1970.PubMedGoogle Scholar
  15. [15]
    Oppenheimer D.R.:Diseases of the basal ganglia, cerebellum and motor neurons. In: Adams, J.H., Corsellis J.A.N., Duchen L.W., eds.: Greenfield's Neuropathology, Edward Arnold, London, 1984.Google Scholar
  16. [16]
    Pederesen L. andGyldensted C.:Computerized tomography in hereditary ataxia. Acta Neurol. Scand., 58:81–88, 1978.PubMedGoogle Scholar
  17. [17]
    Rosenhagen H.:Die primàre Atrophie des Bruckenfusses und der unteren Oliven. Arch. Psychiatr. Nervenkr., 116:163–172, 1943.Google Scholar
  18. [18]
    Savoiardo M., Bracchi M., Passerini A. et al.:Computed tomography of olivopontocerebellar degeneration. A.J.N.R., 4:509–512, 1983.Google Scholar
  19. [19]
    Savoiardo M., Strada L., Girotti F. et al.:Olivopontocerebellar atrophy: MR diagnosis and relationship to multisystem atrophy. Radiology, 174:693–696, 1990.PubMedGoogle Scholar
  20. [20]
    Van Bogaert L.:Les maladies-dégénéreuses systematisés In: Traité de Médecine, Tome XVI, 142, Masson, Paris, 1949.Google Scholar

Copyright information

© Masson Italia Periodici S.r.l. 1992

Authors and Affiliations

  • Giuliani G. 
    • 1
  • Chiaramoni L. 
    • 1
  • Foschi N. 
    • 1
  • Terziani S. 
    • 1
  1. 1.Clinica Neurologica dell'Università di AnconaOspedale Generale Regionale, USL 12Ancona
  2. 2.Clinica NeurologicaOspedale Generale Regionale, TorretteAncona

Personalised recommendations