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Infantile bilateral striatal necrosis

Clinical and morphological report of a case and a review of the literature

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Summary

A case of infantile bilateral striatal necrosis (IBSN) is reported, the first one found in Scandinavia. Extensive clinical and laboratory investigations did not reveal any evidence of a neurometabolic disease. By computed tomography (CT) degeneration of putamen of either side was detected. The neuropathologic findings included extensive bilateral destruction of the striatum and pallidum and degeneration of the substantia nigra and tegmental nuclei. In the damaged regions, accumulations of cells containing sudanophilic lipids were found and Alzheimer type II glial cells, which were also seen in the cortex. On the basis of the clinical picture and the destruction of nerve cells in particular areas of the brain, in the present case and previously published cases of IBSN, the possible role of glutamatc and other excitotoxic transmitters in the pathogenesis of the disease is discussed.

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Correspondence to M. Röyttä.

Additional information

Supported by a grant from “Expressens fond för perinatalforskning”

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Röyttä, M., Olsson, I., Sourander, P. et al. Infantile bilateral striatal necrosis. Acta Neuropathol 55, 97–103 (1981). https://doi.org/10.1007/BF00699234

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Key words

  • Infantile striatal necrosis
  • Computed tomography (CT)
  • Morphology
  • Excitotoxic factors