Optic atrophy as the first symptom in Hallervorden-Spatz syndrome

Pier Antonio Battistella, Edoardo Midena, Agnese Suppiej, Carla Carollo

Research output: Contribution to journalArticlepeer-review


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.

Original languageEnglish
Pages (from-to)135-138
Number of pages4
JournalChild's Nervous System
Issue number3
Publication statusPublished - Mar 1998


  • Basal ganglia
  • Hallervorden-Spatz syndrome
  • Iron deposits
  • Magnetic resonance imaging
  • Optic atrophy

ASJC Scopus subject areas

  • Clinical Neurology
  • Pediatrics, Perinatology, and Child Health


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