OCULOMOTOR ABNORMALITIES IN LOUIS-BAR'S SYNDROME

DOI Web Site PubMed 2 Citations Open Access
  • KAMEI TAMIO
    Department of Otolaryngology, Gunma University School of Medicine
  • KANEKO YUTAKA
    Department of Otolaryngology, Gunma University School of Medicine
  • ISHII HIDEO
    Department of Otolaryngology, Gunma University School of Medicine
  • MORIKAWA AKIHIRO
    Department of Pediatrics, Gunma University School of Medicine

Bibliographic Information

Other Title
  • Louis-Bar症候群における眼運動異常について
  • Louis-Bar症候群における眼運動異常について--主として小脳性眼運動異常
  • Louis Bar ショウコウグン ニ オケル ガン ウンドウ イジョウ ニ

Search this article

Description

A case of Louis-Bar's syndrome (ataxia telangiectasia) in a 9-year-old girl was presented and * study was made of the nature and origin of oculomotor abnormalities in association with the syndrome. <br>1. Nature of the oculomotor abnormalities <br> The following abnormalities were observed: 1) hypometric saccades; 2) difficulty of keeping eye position (notably non-central gaze); 3) coarse fixation jerks; 4) downward vertical nystagmus elicited on lateral gaze; 5) weak convergence; 6) suppression of optokinetic nystagmus; 7) disturbance of smooth following eye movements; 8) Roth-Bielschowsky sign; 9) flutter-like oscillations of the eye (Cogan). In addition to these, fixation nystagmus is also reported in the literature. To be noted is that the symptoms 3), 7) and 9) are entirely new additions to the ocular manifestations of this syndrome.<br> 2. Origin <br> Aside from these oculomotor abnormalities, conspicuous cerebellar symptoms were the only neurologic manifestations in the present case. Along with recent demonstration that the pathology of the central nervous system of the syndrome is characterized by diffuse atrophy occurring almost within the confines of the cerebellum or at least by the absence of significant lesions in the brain stem and cerebrum, it was suggested that the oculomotor abnormalities mentioned above are all of cerebellar origin. In this connection, special reference was made to the symptoms 5), 6), 8) and 9).

Journal

Citations (2)*help

See more

Keywords

Details 詳細情報について

Report a problem

Back to top