Table 2:

Comparison of neuroimaging features in NBIA

DisorderIron DepositionWhite Matter InvolvementOther Findings
PKANGlobus pallidus, substantia nigra (mild)NoEye-of-the-tiger sign
PLANGlobus pallidus,a substantia nigraaMildModerate cerebellar atrophy
NFT“Patchy” globus pallidus, putamen, caudate, dentate, thalamusMild, moderateCystic cavitation, mild cerebral, cerebellar atrophy
ACPGlobus pallidus, putamen, caudate, thalamus, red nucleus, dentateModerate, severeMild cerebellar atrophy
FAHNGlobus pallidus, substantia nigrabModeratePontocerebellar atrophy
KRSGlobus pallidus, putamen, caudatebSevere cerebral, cerebellar, brain stem atrophy
WSSGlobus pallidusbSevere, confluent
SENDASubstantia nigra, globus pallidusOccasionalMidbrain T1 hyperintensity
  • a Inconsistent finding.

  • b Numbers of genetically confirmed cases are still too small to determine the frequency of iron deposition.