How is pantothenate kinase-associated neurodegeneration (PKAN) (Hallervorden-Spatz disease) differentiated from dyskinesia (TD)?

Updated: Oct 17, 2018
  • Author: James Robert Brasic, MD, MPH; Chief Editor: Selim R Benbadis, MD  more...
  • Print
Answer

Answer

Pantothenate kinase-associated neurodegeneration (PKAN) (Hallervorden-Spatz disease) occurs in patients aged 10-15 years, a different age group than that of persons with TD. PKAN is an extremely rare, progressive neurogenetic disorder with autosomal recessive inheritance associated with dementia and death (approximately 20 y after onset). It is characterized by rigidity, dystonia, choreoathetosis, spasticity, foot deformity, and intellectual deterioration. It is associated with excessive iron deposition in the basal ganglia that can be observed on MRI.


Did this answer your question?
Additional feedback? (Optional)
Thank you for your feedback!