Bilateral striatal necrosis, dystonia and optic atrophy in two siblings

V. Leuzzi, E. Bertini, A. M. De Negri, M. Gallucci, B. Garavaglia

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Two siblings developed a neurological disorder in the first decade characterised by generalised dystonia, hypokinesia, and subacute visual loss. CT and serial MRI examinations showed bilateral lesions of the striatum, mainly in the putamen. The classification of these patients is discussed in relation to infantile bilateral striatal necrosis (IBSN), Leigh's disease, and Leber's optic neuropathy. The literature shows a clinical and aetiopathogenetic overlap between these syndromes. In our cases parental consanguinity and the involvement of a single generation suggest a new clinical condition with autosomal recessive transmission.

Original languageEnglish
Pages (from-to)16-19
Number of pages4
JournalJournal of Neurology, Neurosurgery and Psychiatry
Issue number1
Publication statusPublished - 1992

ASJC Scopus subject areas

  • Psychiatry and Mental health
  • Neuroscience(all)
  • Neuropsychology and Physiological Psychology

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