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Hallervorden-Spatz syndrome, PANK2, and the tiger’s eyes

https://doi.org/10.1136/adc.88.6.487

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    Hallervorden and Spatz reported a rapidly progressive neurodegenerative disease of early onset in a German journal of neurology and psychiatry in 1922. The main clinical features are dystonia, dysarthria and rigidity, rapid progression, and early death. Until recently the diagnosis depended on clinical features and CT or MRI abnormalities in the globus pallidus. Pathologically, there is iron accumulation in the basal ganglia with destruction of the pallidum and substantia nigra. The classic disease is of early onset (infancy or early childhood) and rapidly progressive. Atypical forms present later and progress more slowly.

    In 2001 researchers in California and Oregon linked Hallervorden-Spatz syndrome …

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    • ↵* In an annotation (Michael Shevell. Ibid: 3–4) a call is made for the name Hallervorden-Spatz syndrome to be dropped. Julius Hallervorden was implicated in a programme of mass murder of disabled people in Nazi Germany and knowingly used the brains of victims as a basis for his reports; his name should not be perpetuated by the use of the eponym. The conditions should be called pantothenate kinase-associated degeneration or neurodegeneration with brain iron accumulation according to whether or not a PANK2 mutation is present.