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Optic atrophy as the first symptom in Hallervorden-Spatz syndrome

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Abstract

A 16-year-old boy with the classic or postinfantile type of Hallervorden-Spatz syndrome is described. Bilateral optic atrophy with visual loss but without retinal changes was the only presenting symptom. Mild cognitive impairment, behavioural disturbances and insidious extrapyramidal involvement appeared later. MRI showed marked symmetrical hypointensity of the globi pallidi and substantia nigra. This new observation suggests that the occurrence of optic atrophy in a patient with Hallervorden-Spatz syndrome should be regarded as noncoincidental and stresses the importance of an accurate neurological work-up in all adolescents with any unusual form of progressive optic atrophy.

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Authors and Affiliations

  1. Department of Pediatrics, University of Padua, Via Giustiniani, 3, I-35128 Padua, Italy Tel.: +39-49-82 13 505 Fax: +39-49-82 13 509, IT

    P. A. Battistella & Agnese Suppiej

  2. Institute of Ophthalmology, University of Padua, Padua, Italy, IT

    Edoardo Midena

  3. Neuroradiological Service, University of Padua, Padua, Italy, IT

    Carla Carollo

Authors
  1. P. A. Battistella
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  2. Edoardo Midena
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  3. Agnese Suppiej
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  4. Carla Carollo
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Received: 27 January 1997

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Battistella, P., Midena, E., Suppiej, A. et al. Optic atrophy as the first symptom in Hallervorden-Spatz syndrome. Child's Nerv Syst 14, 135–138 (1998). https://doi.org/10.1007/s003810050196

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  • DOI: https://doi.org/10.1007/s003810050196

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