Hallervorden-Spatz Syndrome (HSS) is now known to be caused by a number of different conditions associated with iron accumulation in the brain with neurodegeneration. It was described by Hallervorden and Spatz in 1922.


Inheritance: autosomal recessive


Clinical features:

(1) occurrence in children and adolescents

(2) extrapyramidal findings (dystonic postures, muscular rigidity, choreoathetosis, tremor, involuntary movements)

(3) disorders of the corticospinal tract

(4) dementia


The disorder follows a relentlessly progressive course.


Findings on neuropathology or MRI studies:

(1) bilateral, symmetrical lesions of the globi pallidi

(2) bilateral, symmetrical lesions of the pars reticulata of the substantia nigra


Brain iron can be seen as hypointense areas on T2-weighted images using spin-echo (SE) sequence in a high field strength MRI (1.5T).


Histological examination shows:

(1) demyelination

(2) focal axonal swelling

(3) spheroids

(4) iron-containing pigment


To read more or access our algorithms and calculators, please log in or register.