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Roth-Bielschowsky syndrome

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This description was submitted by Zoran Bojanic, Serbia:

Internuclear ophthalmoplegia with medial rectus muscle paralysis for versions, intact convergence, and vestibular nystagmus of abducted eye. The syndrome is characterized by a failure of adduction during conjugate lateral gaze. The eye on the side of the lesion does not move inwards, while the contralateral eye remains abducted during conjugate lateral movements. Nystagmus may be present in the abducting eye. Caloric stimulation causing abduction of the homolateral eye rules out an abducens palsy. Convergence may be lost in anteriorly placed lesions and it remains intact in posterior lesions.

The syndrome is caused by a lesion in the medial longitudinal fasciculus which connects the nuclei of the third, fourth and sixth cranial nerves. Bilateral lesions are almost always due to multiple sclerosis. Unilateral lesions are due to vascular occlusion.

Sauvineau's syndrome has also been entered as a separate entity. See under Charles Sauvineau, French ophthalmologist, 1862-1924.

Bibliography

  • C. Sauvineau:
    Un nouveau type de paralysie associé des mouvements horizonteaux des yeux.
    Bulletin de la Société française d'Ophtalmologie, 1895, 13: 524-534.
  • W. K. Roth:
    Demonstration von Kranken mit Ophthalmoplegie.
    Neurologisches Centralblatt, Leipzig, 1901, 20: 922. Pseudobulbärparalyse. Neurologisches Centralblatt, Leipzig, 1909.
  • A. Bielschowsky:
    Die Innervation der Musculi recti interni als Seitenwänder.
    Berlin; Zusammenkunft Dtsch Ophthalmol Gesellschaft, 1902, 30: 164-171.
  • A. Bielschowsky:
    Das klinische Bild der assoziierten Blicklähmung und seine Bedeutung für die topische Diagnostik. Münchener medizinische Wochenschrift, 1903, 50: 1666-1670.
  • A. Lutz:
    Über die Bahnen der Blickwendung und deren Dissoziierung. (Nebst eines Falles von Ophthalmoplegia internuclearis anterior in verbindung mit Dissoziierung der Bogengänge).
    Klinische Monatsblätter für Augenheilkunde, Stuttgart, 1923, 70: 213-235.
  • A. Lutz:
    Über einseitige Ophthalmolplegia internuclearis anterior. (Beschreibung eines neuen Falls in Verbindung mit heterolateraler Herabsetzung des automatischen Blinzreflexes und Verlust der Reaktionsfähigkeit der homolateralen vertikalen Bogengang.
    Albrecht von Graefes Archiv für Ophthalmologie, 1924, 115: 692-717.
  • D. G. Cogan:
    Unilateral internuclear ophthalmoplegia: report of eight clinical cases with one postmortem study.
    Archiv für Ophthalmologie, Berlin, 1950, 44: 783-796.
  • W. J. Orlowski, P. Slomski, S. Wojtowicz:
    Bielschowsky-Lutz-Cogan syndrome.
    American Journal of Ophthalmology, Chicago, 1965, 59: 416-430.
  • H. W. Topilow:
    Posterior internuclear ophthalmoplegia of Lutz.
    Annals of Ophthalmology, Chicago, 1981, 13: 221-226.

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