Hallervorden-Spatz syndrome
Hallervorden-Spatz syndrome is an autosomal recessive disorder causing involuntary spasticity and progressive dementia.
It is also known as pantothenate kinase-associated neurodegeneration (PKAN) which is a subset of neurodegeneration with brain iron accumulation (NBIA).
Radiographic features
MRI
T2 : T2-weighted MRI demonstrates hyperdense changes in anteriomedial part of globus pallidus and hypodense lateral part of globus pallidus and pars reticulata of substantia nigra. There is associated T2 hypointensity, which can manifest as hyperintensity if there is associated inflammation and demyelination.
Cortical and caudate atrophy may be seen. The "eye of the tiger" sign refers to a central T2 hyperintense spot (line) within the hypointense globus pallidus 3.
Etymology
- Hugo Spatz (1888-1969), anatomist; Munich, Germany
- Julius Hallervorden (1882-1965), neuropathologist; Germany
Both Hugo Spatz and Julius Hallervorden were involved in the nazi euthanasia program and, as such, the alternative name is preferred by some authors.
Brief biographical entries on Spatz [1] and Hallervorden [2] make interesting reading.
