Hallervorden-Spatz syndrome

Alternative eponyms

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A very rare disease with degeneration of the globus pallidus, red nucleus, and substantia nigra of the brain. It is characterized by progressive Parkinson-like rigidity, athetotic movements, and progressive mental and emotional retardation.

Description

A very rare disease beginning in childhood, with degeneration of the globus pallidus, red nucleus, and reticular part of the substantia nigra of the brain. It is characterized by progressive Parkinson-like rigidity, athetotic movements, and progressive mental and emotional retardation. Onset is in late childhood; death usually occurs within 10 years, but courses of up to 30 years have been described. Aetiology unknown; considered one form of iron storage disease. A heredo-familial syndrome, inherited as an autosomal recessive trait; some unrelated cases reported

The original description concerned a sibship of 12 in which 5 sisters showed clinically increasing dysarthria and progressive dementia, and at autopsy brown discoloration of the globus pallidus and substantia nigra.

Bibliography

  • J. Hallervorden, H. Spatz:
    Eigenartige Erkrankung im extrapyramidalen System mot besonderer Beteiligung des Globus pallidus und der Substantia nigra: Ein Beitrag zu den Beziehungen zwischen diesen beiden Zentren.
    Zeitschrift für die gesamte Neurologie und Psychiatrie, 1922, 79: 254-302.
  • S. Gilman, R. E. Barrett:
    Hallervorden-Spatz disease and infantile neuroaxonal dystrophy: Clinical characteristics and nosological considerations.
    Journal of Neurological Sciences, Amsterdam, 1973, 19: 189-205.

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